1
|
Weinberg S, Amarnani A, Jolly M. Gastrointestinal and hepatic manifestations. DUBOIS' LUPUS ERYTHEMATOSUS AND RELATED SYNDROMES 2025:505-520. [DOI: 10.1016/b978-0-323-93232-5.00045-9] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/03/2025]
|
2
|
Louthrenoo W, Gumtorntip W, Thanunchai P, Amantakul A, Kasitanon N, Pojchamarnwiputh S. Clinical features, imaging findings, and outcomes of acute abdominal pain in systemic lupus erythematosus: comparing mesenteric vasculitis, non-mesenteric vasculitis, and surgical conditions. Clin Rheumatol 2024; 43:3699-3712. [PMID: 39422805 DOI: 10.1007/s10067-024-07189-8] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/12/2024] [Revised: 09/14/2024] [Accepted: 10/11/2024] [Indexed: 10/19/2024]
Abstract
OBJECTIVES To determine the spectrum, clinical features and outcomes in systemic lupus erythematosus (SLE) patients with acute abdominal pain (AAP). METHOD Medical records of SLE patients in a lupus cohort from January 1987 to June 2023 were reviewed. Patients with AAP requiring hospitalization were identified and categorized into 3 groups: lupus mesenteric vasculitis (LMV), non-LMV, and surgical AAP. Each AAP episode represented one patient. RESULTS Of 1,538 patients in the cohort, 62 (4.03%) had 93 episodes of AAP. After exclusion, 31 patients had 39 LMV episodes, and 30 had 40 non-LMV episodes (19 due to surgical AAP). Seventy-six of the 79 AAP episodes (96.20%) were in females, with a mean ± SD age and median (IQR) disease duration of 36.76 ± 13.60 years and 6 (2, 9) years, respectively. Patients in the LMV group had more fever, nausea and vomiting, and diarrhea than those in the non-LMV group. They also had more small bowel involvement, bowel wall thickening, target water enhancement signs, mesenteric vessels engorgement and mesenteric fat cloudiness, and higher SLE disease activity. These differences were more pronounced when compared to the surgical AAP group. Treatment with corticosteroids and immunosuppressive drugs gave favorable outcomes in the LMV group. Two of 40 (5.00%) non-LMV AAP patients died, of which 1 (5.26%) was in the surgical AAP group. CONCLUSION LMV was common among SLE patients admitted for AAP. LMV usually presented with fever, gastrointestinal dysmotility symptoms, diffused abdominal pain, together with evidence of active disease. Localized abdominal pain with peritoneal signs favored surgical AAP. Key Points • Lupus mesenteric vasculitis is common among SLE patients presenting with acute abdominal pain. Its presence often associates with gastrointestinal symptoms together with other clinical manifestations of SLE • The signs in abdominal computed tomography findings are not specific and could be observed in other causes of abdominal pain in SLE. Interpretation of these signs should be cautionary and accompanied by history taking and physical abdominal findings • Treatment of lupus mesenteric vasculitis with corticosteroids alone, or in combination with immunosuppressive drugs, usually results in good outcomes.
Collapse
Affiliation(s)
- Worawit Louthrenoo
- Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand.
| | - Wanitcha Gumtorntip
- Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand
| | - Piyanut Thanunchai
- Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand
| | - Amonlaya Amantakul
- Division of Diagnostic Radiology, Department of Radiology, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand
| | - Nuntana Kasitanon
- Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand
| | - Suwalee Pojchamarnwiputh
- Division of Diagnostic Radiology, Department of Radiology, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand
| |
Collapse
|
3
|
Sönmez HE, Batu ED, İşgüder R, Şahin N, Aliyev E, Aslan E, Çoban S, Güngörer V, Karadağ ŞG, Karaçayır N, Kısaoğlu H, Yekedüz Bülbül A, Garip S, Karalı Y, Ayduran S, Demir S, Kaya Akça Ü, Başaran Ö, Şahin S, Kasap B, Kilic SS, Kışla Ekinci RM, Kısaarslan AP, Kalyoncu M, Bakkaloğlu S, Yüksel S, Aktay Ayaz N, Çelikel Acar B, Sözeri B, Kasapçopur Ö, Ünsal E, Özen S. Exploring gastrointestinal manifestations in childhood onset systemic lupus erythematosus - Insights from a multicenter study. Lupus 2024; 33:1358-1364. [PMID: 39186467 DOI: 10.1177/09612033241279071] [Citation(s) in RCA: 1] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 08/28/2024]
Abstract
Objective Systemic lupus erythematosus (SLE) constitutes an autoimmune disorder with potential involvement of the gastrointestinal system (GIS). Our objective was to assess the gastrointestinal (GI) manifestations in patients diagnosed with childhood onset SLE. Methods The study cohort consisted of 123 patients with childhood onset-SLE and GIS involvement from 16 referral departments of pediatric rheumatology. All participants met the Systemic Lupus International Collaborating Clinics criteria. Results Out of 123 patients, 78 (63.4%) exhibited GIS involvement at the initial SLE diagnosis, whereas the remaining 45 (36.6%) developed GI symptoms after a median duration of 12 (3–140) months. Eighty-two (66.7%) individuals experienced symptoms related to the GI tract, whereas the remaining patients received a diagnosis of GI involvement through laboratory assessments. The predominant initial GIS involvement symptom was abdominal pain, observed in 77 (62.6%) patients, followed by elevated hepatic transaminases in 70 (56.9%), hepatomegaly in 40 (32.5%), diarrhea in 26 (21.1%), and jaundice in 11 (8.9%) patients. The GIS involvement was associated with SLE in 82 (78.6%), while it resulted from drug-related adverse events in 35 (28.5%) patients or comorbidities in 6 (0.5%) patients. Conclusion GIS involvement should be considered in all childhood onset-SLE patients, especially in the presence of suggestive symptoms or elevated hepatic transaminases. It is also crucial to consider SLE in the differential diagnosis of GIS manifestations in children. Apart from GIS involvement directly associated with SLE, adverse events of drugs should be kept in mind.
Collapse
Affiliation(s)
- Hafize Emine Sönmez
- Faculty of Medicine, Department of Pediatric Rheumatology, Kocaeli University, Kocaeli, Turkey
| | - Ezgi Deniz Batu
- Faculty of Medicine, Department of Pediatric Rheumatology, Hacettepe University, Ankara, Turkey
| | - Rana İşgüder
- Faculty of Medicine, Department of Pediatric Rheumatology, Dokuz Eylül University, Izmir, Turkey
| | - Nihal Şahin
- Faculty of Medicine, Department of Pediatric Rheumatology, Kocaeli University, Kocaeli, Turkey
| | - Emil Aliyev
- Faculty of Medicine, Department of Pediatric Rheumatology, Hacettepe University, Ankara, Turkey
| | - Esma Aslan
- Cerrahpaşa Medical Faculty, Department of Pediatric Rheumatology, Istanbul University-Cerrahpaşa, Istanbul, Turkey
| | - Sümeyra Çoban
- Department of Pediatric Rheumatology, Umraniye Training and Research Hospital, Istanbul, Turkey
| | - Vildan Güngörer
- Department of Pediatric Rheumatology, Ankara Bilkent City Hospital, Ankara, Turkey
| | - Şerife Gül Karadağ
- Faculty of Medicine, Department of Pediatric Rheumatology, Istanbul University, Istanbul, Turkey
| | - Nihal Karaçayır
- Department of Pediatric Rheumatology, Gazi University Faculty of Medicine, Ankara, Turkey
| | - Hakan Kısaoğlu
- Department of Pediatric Rheumatology, Karadeniz Technical University Faculty of Medicine, Trabzon, Turkey
| | | | - Sevinç Garip
- Department of Pediatric Gastroenterology, Adana City Training and Research Hospital, Adana, Turkey
| | - Yasin Karalı
- Department of Pediatric Rheumatology, Uludağ University, Bursa, Turkey
| | - Semra Ayduran
- Department of Pediatric Rheumatology, Pamukkale University, Denizli, Turkey
| | - Selcan Demir
- Department of Pediatric Rheumatology, Osmangazi University, Eskişehir, Turkey
| | - Ümmüşen Kaya Akça
- Department of Pediatric Rheumatology, Aydın State Hospital, Aydın, Turkey
| | - Özge Başaran
- Faculty of Medicine, Department of Pediatric Rheumatology, Hacettepe University, Ankara, Turkey
| | - Sezgin Şahin
- Cerrahpaşa Medical Faculty, Department of Pediatric Rheumatology, Istanbul University-Cerrahpaşa, Istanbul, Turkey
| | - Belde Kasap
- Department of Pediatric Rheumatology, Izmir Katip Celebi University, Izmir, Turkey
| | - Sara Sebnem Kilic
- Department of Pediatric Rheumatology, Uludağ University, Bursa, Turkey
| | - Rabia Miray Kışla Ekinci
- Department of Pediatric Gastroenterology, Adana City Training and Research Hospital, Adana, Turkey
| | | | - Mukaddes Kalyoncu
- Department of Pediatric Rheumatology, Karadeniz Technical University Faculty of Medicine, Trabzon, Turkey
| | - Sevcan Bakkaloğlu
- Department of Pediatric Rheumatology, Gazi University Faculty of Medicine, Ankara, Turkey
| | - Selçuk Yüksel
- Department of Pediatric Rheumatology, Çanakkale Onsekiz Mart University, Çanakkale, Turkey
| | - Nuray Aktay Ayaz
- Faculty of Medicine, Department of Pediatric Rheumatology, Istanbul University, Istanbul, Turkey
| | - Banu Çelikel Acar
- Department of Pediatric Rheumatology, Ankara Bilkent City Hospital, Ankara, Turkey
| | - Betül Sözeri
- Department of Pediatric Rheumatology, Umraniye Training and Research Hospital, Istanbul, Turkey
| | - Özgür Kasapçopur
- Cerrahpaşa Medical Faculty, Department of Pediatric Rheumatology, Istanbul University-Cerrahpaşa, Istanbul, Turkey
| | - Erbil Ünsal
- Faculty of Medicine, Department of Pediatric Rheumatology, Dokuz Eylül University, Izmir, Turkey
| | - Seza Özen
- Faculty of Medicine, Department of Pediatric Rheumatology, Hacettepe University, Ankara, Turkey
| |
Collapse
|
4
|
Zhou L, Cai SZ, Dong LL. Recent advances in pathogenesis, diagnosis, and therapeutic approaches for digestive system involvement in systemic lupus erythematosus. J Dig Dis 2024; 25:410-423. [PMID: 39317429 DOI: 10.1111/1751-2980.13307] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/06/2023] [Revised: 07/30/2024] [Accepted: 08/01/2024] [Indexed: 09/26/2024]
Abstract
Systemic lupus erythematosus (SLE) is a systemic autoimmune disease characterized by the presence of large amounts of autoantibodies and immune complex formation. Because of their atypical clinical symptoms, SLE patients with digestive system involvement may not be recognized or treated precisely and extensively. Clinicians should pay close attention to SLE with digestive system involvement, as these conditions can easily worsen the condition and possibly endanger the patient's life. In this review we summarized the pathogenesis, pathological characteristics, clinical manifestations, diagnosis, and therapies for digestive system involvement in SLE.
Collapse
Affiliation(s)
- Liang Zhou
- Department of Rheumatology and Immunology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei Province, China
| | - Shao Zhe Cai
- Department of Rheumatology and Immunology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei Province, China
| | - Ling Li Dong
- Department of Rheumatology and Immunology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei Province, China
| |
Collapse
|
5
|
Muñoz-Urbano M, Sangle S, D'Cruz DP. Lupus enteritis: a narrative review. Rheumatology (Oxford) 2024; 63:1494-1501. [PMID: 38216993 DOI: 10.1093/rheumatology/kead689] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/02/2023] [Revised: 10/20/2023] [Accepted: 12/02/2023] [Indexed: 01/14/2024] Open
Abstract
Lupus enteritis (LE) is a rare manifestation of systemic lupus erythematosus. The pathophysiology of LE has not been fully elucidated, although inflammatory and thrombotic processes are likely important factors. The underlying pathophysiological mechanisms may depend on which portion of the intestine is affected. Over half of the patients with LE also present with renal or haematological complications. The diagnosis of LE is based on clinical, histopathological and imaging findings; abdominal computed tomography (CT) is the gold standard in diagnosis. Abdominal CT can also identify factors that predict complications and could potentially guide pharmacological and nutritional management. Timely identification and prompt treatment initiation are paramount to avoid life and organ threatening complications. Glucocorticoids are often the first-line treatment. Additional therapy including immunosuppressive therapy is utilised on a case-by-case basis as there are no clinical trials to define the optimal therapeutic approach. Surgical intervention may be needed especially if there is bowel perforation or peritonitis. In general, the prognosis of LE is good.
Collapse
Affiliation(s)
| | - Shirish Sangle
- Louise Coote Lupus Unit, Guy's Hospital, Guy's and St Thomas' Hospitals NHS Foundation Trust, London, UK
| | - David P D'Cruz
- Louise Coote Lupus Unit, Guy's Hospital, Guy's and St Thomas' Hospitals NHS Foundation Trust, London, UK
| |
Collapse
|
6
|
Chen YL, Meng J, Li C. Intestinal perforation with systemic lupus erythematosus: A systematic review. Medicine (Baltimore) 2023; 102:e34415. [PMID: 37543816 PMCID: PMC10402941 DOI: 10.1097/md.0000000000034415] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/31/2023] [Revised: 06/27/2023] [Accepted: 06/29/2023] [Indexed: 08/07/2023] Open
Abstract
Intestinal perforation (IP) is a rare complication of systemic lupus erythematosus (SLE), and the timely diagnosis and treatment of IP are necessary to prevent death. In this study, the clinical features of IP in SLE were described in an attempt to enhance its understanding to reduce mortality. The clinical data of IP in SLE from 1984 to 2022 were retrospectively collected. A total of 18 patients were enrolled, and data on clinical symptoms, preoperative evaluation, surgical procedures, and postoperative outcomes were collected and retrospectively analyzed. The analysis included 15 females and 3 males, with a mean age of 49.2 years. Fifteen patients (83.3%) had a history of the disease for >5 years, and the SLE disease activity index score of 1 (5.6%) patient was <5 points and that of 17 (94.4%) patients was >10 points. A total of 9 (50%), 5 (27.7%), 3 (16.7%), and 1 (5.6%) patient had lesions in the rectum, colon, ileum, and both ileum and appendix, respectively. The cause of perforation in 12 (66.7%) patients was lupus mesenteric vasculitis and in 3 (16.7%) patients was chronic inflammation. Seven (38.9%) patients had other immune system diseases. All patients were treated with steroids and surgical treatment. However, 5 patients died after surgery. A disease duration of >5 years, SLE disease activity index score of >10, nonstandard use of steroids, and concomitant presence of other immune system diseases are the possible risk factors of IP in SLE. The most common site of perforation was the rectum, which was caused by lupus mesenteric vasculitis. The results suggest that the key to successfully manage such cases is early diagnosis, aggressive resuscitation, antibiotics, steroid therapy, and prompt surgical intervention.
Collapse
Affiliation(s)
- Ya Lan Chen
- Department of Gastroenterology, The Affiliated Hospital of Hebei University, Baoding, P.R. China
| | - Jie Meng
- Department of Gastroenterology, The Affiliated Hospital of Hebei University, Baoding, P.R. China
| | - Cong Li
- Department of Hepatobiliary Surgery, The Affiliated Hospital of Hebei University, Baoding, P.R. China
| |
Collapse
|
7
|
Muñoz-Urbano M, Sanchez-Bautista J, Ramírez A, Santamaría-Alza Y, Quintero-González DC, Vanegas-García AL, Vásquez G, González LA. Lupus enteritis: A 10-year experience in a single Latin American center. Lupus 2023:9612033231175782. [PMID: 37184366 DOI: 10.1177/09612033231175782] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 05/16/2023]
Abstract
OBJECTIVE The objective is to compare the clinical and laboratory characteristics of systemic lupus erythematosus (SLE) patients with and without lupus enteritis (LE) and to identify the factors associated with the occurrence of LE. METHODS We performed a retrospective, case-control study in hospitalized patients with SLE who were admitted to our tertiary hospital between January 2012 and December 2021. Sixteen LE patients (cases) were matched (1:3 ratio) for sex and birth year with 48 non-LE patients (controls). Univariable and multivariable logistic regression analyses were used to identify the variables associated with LE. RESULTS Of 2,479 SLE patients who were admitted to our hospital as inpatients, 16 (0.65%) were diagnosed as having LE. All patients, cases and controls, were of Mestizo ethnicity. SLE was diagnosed simultaneously with the first episode of LE in 10 (62.5%) patients. The median time from SLE diagnosis to the first episode of LE was 7 (IQR 0-78) months. LE patients had a shorter median disease duration [7 (0-78) vs 34 (9.5-79) months], and a significantly longer hospital stay (28.3 ± 15.8 vs 6.5 ± 7.9 days, p < 0.001) than non-LE patients. Most LE patients (93.8%) had concomitant lupus nephritis. LE patients had higher SLEDAI-2K scores than those without LE (20.5 ± 9.4 vs 9.8 ± 10.4, p < 0.001). By multivariable analysis, a higher SLEDAI-2K score (OR 1.10, 95% CI 1.02-1.18; p = 0.015) was independently associated with LE occurrence after adjusting for cutaneous involvement, lymphocyte count, serum creatinine, and serum complement C4. Recurrence was observed in two patients (12.5%), both with a bowel wall thickening > 8 mm. The two patients with large intestine-dominant LE developed intestinal pseudo-obstruction. No patient had life-threatening complications (intestinal hemorrhage, infarction, or perforation), and there were no deaths induced directly by LE itself. CONCLUSION In patients of Mestizo ethnicity, LE occurs during the early course of SLE, frequently is one of the presenting manifestations of SLE, and in most cases, it presents with concomitant lupus nephritis. Higher levels of disease activity at diagnosis were independently associated with LE occurrence and when recurrences occur, they do so in the context of severe wall thickness.
Collapse
Affiliation(s)
- Marcela Muñoz-Urbano
- Division of Rheumatology, Department of Internal Medicine, School of Medicine, Universidad de Antioquia, Hospital San Vicente Fundación, Medellín, Colombia
| | - Julián Sanchez-Bautista
- Division of Rheumatology, Department of Internal Medicine, School of Medicine, Universidad de Antioquia, Hospital San Vicente Fundación, Medellín, Colombia
| | - Andrés Ramírez
- Division of Rheumatology, Department of Internal Medicine, School of Medicine, Universidad de Antioquia, Hospital San Vicente Fundación, Medellín, Colombia
| | - Yeison Santamaría-Alza
- Division of Rheumatology, Department of Internal Medicine, School of Medicine, Universidad de Antioquia, Hospital San Vicente Fundación, Medellín, Colombia
| | - Diana C Quintero-González
- Division of Rheumatology, Department of Internal Medicine, School of Medicine, Universidad de Antioquia, Hospital San Vicente Fundación, Medellín, Colombia
| | - Adriana-Lucía Vanegas-García
- Division of Rheumatology, Department of Internal Medicine, School of Medicine, Universidad de Antioquia, Hospital San Vicente Fundación, Medellín, Colombia
| | - Gloria Vásquez
- Division of Rheumatology, Department of Internal Medicine, School of Medicine, Universidad de Antioquia, Hospital San Vicente Fundación, Medellín, Colombia
| | - Luis A González
- Division of Rheumatology, Department of Internal Medicine, School of Medicine, Universidad de Antioquia, Hospital San Vicente Fundación, Medellín, Colombia
| |
Collapse
|
8
|
Mauro A, Giani T, Di Mari C, Sandini M, Talenti A, Ansuini V, Biondi L, Di Nardo G, Bernardo L. Gastrointestinal Involvement in Children with Systemic Lupus Erythematosus. CHILDREN (BASEL, SWITZERLAND) 2023; 10:309. [PMID: 36832440 PMCID: PMC9955206 DOI: 10.3390/children10020309] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Subscribe] [Scholar Register] [Received: 10/27/2022] [Revised: 01/05/2023] [Accepted: 02/02/2023] [Indexed: 02/08/2023]
Abstract
Systemic lupus erythematosus (SLE) is a systemic autoimmune disorder. When it presents before the age of 18 years (childhood-onset systemic lupus erythematosus, cSLE), the disease course tends to be more severe with a higher rate of organ involvement and requires an early diagnosis. Gastrointestinal involvement in cSLE is rare and scarcely reported in the literature. Any organ of the gastrointestinal system may be affected, either as a direct consequence of the disease, as a subsequent complication, or as an adverse drug event. Abdominal pain is the most common GI symptom, it can be diffuse or well localized, and can underline different conditions such as hepatitis, pancreatitis, appendicitis, peritonitis, or enteritis. cSLE may have an alteration of the intestinal barrier with features of protein-losing enteropathy or, in genetically predisposed patients, may develop associated autoimmune disorders such as Coeliac Disease or Autoimmune Hepatitis. The aim of this manuscript is to provide a narrative review of gastrointestinal manifestations in cSLE focused on hepatic, pancreatic, and intestinal involvement. A comprehensive literature search based on the PubMed database was performed.
Collapse
Affiliation(s)
- Angela Mauro
- Pediatric Rheumatology Unit, Department of Childhood and Developmental Medicine, Fatebenefratelli-Sacco Hospital, Piazzale Principessa Clotilde, 20121 Milano, Italy
| | - Teresa Giani
- Department of Pediatrics, Meyer Children’s Hospital, 50139 Firenze, Italy
| | - Clelia Di Mari
- Pediatric Rheumatology Unit, Department of Childhood and Developmental Medicine, Fatebenefratelli-Sacco Hospital, Piazzale Principessa Clotilde, 20121 Milano, Italy
| | - Martina Sandini
- Pediatric Rheumatology Unit, Department of Childhood and Developmental Medicine, Fatebenefratelli-Sacco Hospital, Piazzale Principessa Clotilde, 20121 Milano, Italy
| | - Antonella Talenti
- Pediatric Rheumatology Unit, Department of Childhood and Developmental Medicine, Fatebenefratelli-Sacco Hospital, Piazzale Principessa Clotilde, 20121 Milano, Italy
| | - Valentina Ansuini
- Pediatric Rheumatology Unit, Department of Childhood and Developmental Medicine, Fatebenefratelli-Sacco Hospital, Piazzale Principessa Clotilde, 20121 Milano, Italy
| | - Luigi Biondi
- Pediatric Rheumatology Unit, Department of Childhood and Developmental Medicine, Fatebenefratelli-Sacco Hospital, Piazzale Principessa Clotilde, 20121 Milano, Italy
| | - Giovanni Di Nardo
- Faculty of Medicine and Psycology, Sapienza University of Rome—NESMOS Department, Sant’Andrea University Hospital, Via Grottarossa 1035-1039, 00189 Roma, Italy
| | - Luca Bernardo
- Pediatric Rheumatology Unit, Department of Childhood and Developmental Medicine, Fatebenefratelli-Sacco Hospital, Piazzale Principessa Clotilde, 20121 Milano, Italy
| |
Collapse
|
9
|
Alharbi S. Gastrointestinal Manifestations in Patients with Systemic Lupus Erythematosus. Open Access Rheumatol 2022; 14:243-253. [PMID: 36281321 PMCID: PMC9587305 DOI: 10.2147/oarrr.s384256] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/29/2022] [Accepted: 10/11/2022] [Indexed: 11/05/2022] Open
Abstract
Systemic lupus erythematosus (SLE) is an autoimmune disorder of unknown etiology. Women of childbearing age are affected approximately nine times more often than men. Its presentation and course are highly variable, ranging from mild to fulminant systemic disease. Any organ can be affected by SLE. Although less common than in other systems, such as the skin, joints, and kidneys, 40%–60% of SLE patients have gastrointestinal (GI) involvement. SLE can affect any part of the GI tract, from the mouth to the anus. GI manifestations can be caused by SLE, medication-related side effects, or non-SLE causes including infection. This article reviews the most common types of GI involvement associated with SLE.
Collapse
Affiliation(s)
- Samar Alharbi
- Department of Medicine, College of Medicine, Taibah University, Medina, Saudi Arabia,Correspondence: Samar Alharbi, Department of Medicine, College of Medicine, Taibah University, Medina, 42312-3779, Saudi Arabia, Tel +96 6553018777, Fax +96 648461172, Email
| |
Collapse
|
10
|
Leone P, Prete M, Malerba E, Bray A, Susca N, Ingravallo G, Racanelli V. Lupus Vasculitis: An Overview. Biomedicines 2021; 9:1626. [PMID: 34829857 PMCID: PMC8615745 DOI: 10.3390/biomedicines9111626] [Citation(s) in RCA: 36] [Impact Index Per Article: 9.0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/28/2021] [Revised: 10/29/2021] [Accepted: 11/03/2021] [Indexed: 12/21/2022] Open
Abstract
Lupus vasculitis (LV) is one of the secondary vasculitides occurring in the setting of systemic lupus erythematosus (SLE) in approximately 50% of patients. It is most commonly associated with small vessels, but medium-sized vessels can also be affected, whereas large vessel involvement is very rare. LV may involve different organ systems and present in a wide variety of clinical manifestations according to the size and site of the vessels involved. LV usually portends a poor prognosis, and a prompt diagnosis is fundamental for a good outcome. The spectrum of involvement ranges from a relatively mild disease affecting small vessels or a single organ to a multiorgan system disease with life-threatening manifestations, such as mesenteric vasculitis, pulmonary hemorrhage, or mononeuritis multiplex. Treatment depends upon the organs involved and the severity of the vasculitis process. In this review, we provide an overview of the different forms of LV, describing their clinical impact and focusing on the available treatment strategies.
Collapse
Affiliation(s)
- Patrizia Leone
- Department of Biomedical Sciences and Human Oncology, “Aldo Moro” University of Bari Medical School, 70124 Bari, Italy; (P.L.); (M.P.); (E.M.); (A.B.); (N.S.)
| | - Marcella Prete
- Department of Biomedical Sciences and Human Oncology, “Aldo Moro” University of Bari Medical School, 70124 Bari, Italy; (P.L.); (M.P.); (E.M.); (A.B.); (N.S.)
| | - Eleonora Malerba
- Department of Biomedical Sciences and Human Oncology, “Aldo Moro” University of Bari Medical School, 70124 Bari, Italy; (P.L.); (M.P.); (E.M.); (A.B.); (N.S.)
| | - Antonella Bray
- Department of Biomedical Sciences and Human Oncology, “Aldo Moro” University of Bari Medical School, 70124 Bari, Italy; (P.L.); (M.P.); (E.M.); (A.B.); (N.S.)
| | - Nicola Susca
- Department of Biomedical Sciences and Human Oncology, “Aldo Moro” University of Bari Medical School, 70124 Bari, Italy; (P.L.); (M.P.); (E.M.); (A.B.); (N.S.)
| | - Giuseppe Ingravallo
- Section of Pathology, Department of Emergency and Organ Transplantation, “Aldo Moro” University of Bari Medical School, 70124 Bari, Italy;
| | - Vito Racanelli
- Department of Biomedical Sciences and Human Oncology, “Aldo Moro” University of Bari Medical School, 70124 Bari, Italy; (P.L.); (M.P.); (E.M.); (A.B.); (N.S.)
| |
Collapse
|
11
|
Patra PK, Bhattarai D, Banday AZ, Gupta A, Peters NJ, Bal A, Saxena A. Recurrent lupus enteritis in a child-chase the calm before the storm. Lupus 2021; 30:2003-2007. [PMID: 34652230 DOI: 10.1177/09612033211045923] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/16/2022]
Abstract
Systemic lupus erythematosus (SLE) is a complex multisystemic autoimmune disease. Lupus enteritis (LE), one of the less commonly described manifestations of childhood SLE, presents with relatively nonspecific clinical and laboratory features. In addition, recurrent episodes of LE occurring in temporal proximity are rare in children. Presence of disease activity at other sites (which may not be seen universally) supports the diagnosis of LE in an appropriate setting. Because of its potential role to cause ischemic complications, early recognition and prompt treatment are necessary for a good outcome. Herein, we describe a child with recurrent LE with an interval of about 3 months between the first and the second episode. The first episode correlated with systemic disease activity and bowel thickening was noted on abdominal ultrasonography. This episode was successfully managed with intravenous methylprednisolone pulse therapy. Conversely, the second episode was not associated with significant clinical and laboratory evidence of disease activity at other sites and the initial abdominal ultrasonography was non-contributory. Diagnostic and therapeutic delays, hence, led to the development of fatal complications. We highlight that a high index of suspicion of LE and a timely aggressive treatment is imperative for optimal outcomes even in rare pediatric cases of recurrent LE that may have normal imaging findings initially and may not be associated with systemic lupus erythematosus disease activity index (SLEDAI).
Collapse
Affiliation(s)
- Pratap Kumar Patra
- Department of Pediatrics, Allergy Immunology Unit, 29751Post Graduate Institute of Medical Education and Research, Chandigarh, India
| | - Dharmagat Bhattarai
- Department of Pediatrics, Allergy Immunology Unit, 29751Post Graduate Institute of Medical Education and Research, Chandigarh, India
| | - Aaqib Zaffar Banday
- Department of Pediatrics, Allergy Immunology Unit, 29751Post Graduate Institute of Medical Education and Research, Chandigarh, India
| | - Anju Gupta
- Department of Pediatrics, Allergy Immunology Unit, 29751Post Graduate Institute of Medical Education and Research, Chandigarh, India
| | - Nitin James Peters
- Department of Pediatric Surgery, 29751Post Graduate Institute of Medical Education and Research, Chandigarh, India
| | - Amanjit Bal
- Department of Histopathology, 29751Post Graduate Institute of Medical Education and Research, Chandigarh, India
| | - Akshay Saxena
- Department of Radiodiagnosis, 29751Post Graduate Institute of Medical Education and Research, Chandigarh, India
| |
Collapse
|
12
|
Tejera Segura B, Altabás González I, Rúa-Figueroa I, Pérez Veiga N, Del Campo Pérez V, Olivé-Marqués A, Galindo M, Calvo J, Ovalles-Bonilla JG, Fernández-Nebro A, Menor-Almagro R, Tomero E, Del Val Del Amo N, Uriarte IE, Martínez-Taboada VM, Andreu JL, Boteanu A, Narváez J, Movasat A, Montilla C, Senabre Gallego JM, Hernández-Cruz B, Andrés M, Salgado E, Freire M, Machín García S, Moriano C, Expósito L, Pérez Velásquez C, Velloso-Feijoo ML, Cacheda AP, Lozano-Rivas N, Bonilla G, Arévalo M, Jiménez I, Quevedo-Vila V, Manero-Ruiz FJ, de la Peña Lefebvre G, Vázquez-Rodríguez TR, Ibañez-Ruan J, Cobo-Ibañez T, Pego-Reigosa JM. Relevance of gastrointestinal manifestations in a large Spanish cohort of patients with systemic lupus erythematosus: what do we know? Rheumatology (Oxford) 2021; 60:5329-5336. [PMID: 33950249 DOI: 10.1093/rheumatology/keab401] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/14/2021] [Revised: 04/28/2021] [Indexed: 01/10/2023] Open
Abstract
BACKGROUND Systemic lupus erythematosus (SLE) can affect any part of the gastrointestinal (GI) tract. GI symptoms are reported to occur in more than 50% of SLE patients. AIMS To describe the GI manifestations of SLE in the RELESSER (Registry of Systemic Lupus Erythematosus Patients of the Spanish Society of Rheumatology) cohort and to determine if these are associated with a more severe disease, damage accrual and a worse prognosis. METHODS We conducted a nationwide, retrospective, multicenter, cross-sectional cohort study of 3658 SLE patients who fulfill ≥ 4 ACR-97 criteria. Data on demographics, disease characteristics, activity (SLEDAI-2K or BILAG), damage (SLICC/ACR/DI) and therapies were collected. Demographic and clinical characteristics were compared between lupus patients with and without GI damage to establish whether GI damage is associated with a more severe disease. RESULTS From 3654 lupus patients, 3.7% developed GI damage. Patients in this group (group 1) were older, they had longer disease duration, and were more likely to have vasculitis, renal disease and serositis than patients without GI damage (group 2). Hospitalizations and mortality were significantly higher in group 1. Patients in group 1 had higher modified SDI. The presence of oral ulcers reduced risk of developing damage in 33% of patients. CONCLUSIONS Having GI damage is associated with a worse prognosis. Patients on high dose of glucocorticoids are at higher risk of developing GI damage which reinforces the strategy of minimizing glucocorticoids. Oral ulcers appear to decrease the risk of GI damage.
Collapse
Affiliation(s)
- Beatriz Tejera Segura
- Rheumatology Department, Hospital Universitario Insular de Gran Canaria, Islas Canarias, Spain
| | - Irene Altabás González
- Rheumatology Department, Complejo Hospitalario Universitario de Vigo, Vigo, Galicia, Spain.,Rheumatology & Immuno-Mediated Diseases Research Group (IRIDIS), Galicia Sur Health Research Institute (IIS Galicia Sur), Spain
| | - Iñigo Rúa-Figueroa
- Rheumatology Department, Hospital Universitario de Gran Canaria Dr Negrin, Las Palmas de Gran Canaria, Islas Canarias, Spain
| | - Natalia Pérez Veiga
- Rheumatology & Immuno-Mediated Diseases Research Group (IRIDIS), Galicia Sur Health Research Institute (IIS Galicia Sur), Spain
| | | | - Alejandro Olivé-Marqués
- Rheumatology Department, Germans Trías i Pujol University Hospital, Badalona, Cataluña, Spain
| | - María Galindo
- Rheumatology Department, 12 de Octubre University Hospital, Madrid, Spain
| | - Jaime Calvo
- Rheumatology Department, Hospital Araba, Araba, Spain
| | | | - Antonio Fernández-Nebro
- Instituto de Investigación Biomédica de Málaga - IBIMA, Málaga. Spain.,Rheumatology Department, Hospital Regional Universitario de Málaga, Málaga.,Departamento de Medicina, Universidad de Málaga, Málaga
| | | | - Eva Tomero
- Rheumatology Department. Hospital Universitario de la Princesa. Madrid, Spain
| | | | | | | | - Jose L Andreu
- Rheumatology Department, Puerta de Hierro-Majadahonda Hospital, Madrid, Spain
| | - Alina Boteanu
- Rheumatology Department, Hospital Gregorio Marañón, Madrid, Spain.,Rheumatology Department, Hospital Ramon y Cajal, Madrid, Spain
| | - Javier Narváez
- Rheumatology Department, Hospital de Bellvitge, Hospitalet Llobregat, Cataluña, Spain
| | - Atusa Movasat
- Rheumatology Department, Hospital Universitario Príncipe de Asturias, Madrid, Spain
| | - Carlos Montilla
- Rheumatology Department, Salamanca Clinic University Hospital, Salamanca, Spain
| | | | | | - Mariano Andrés
- Rheumatology Department, Hospital General Universitario de Alicante, Elche, Alicante, Spain
| | - Eva Salgado
- Rheumatology Department, Hospital Universitario de Orense, Galicia, Spain
| | - Mercedes Freire
- Rheumatology Department, Hospital Juan Canalejo de La Coruña, A Coruna, Galicia, Spain
| | - Sergio Machín García
- Rheumatology Department, Hospital Universitario Insular de Gran Canaria, Islas Canarias, Spain
| | - Clara Moriano
- Rheumatology Department, Hospital Universitario de León, León, Spain
| | - Lorena Expósito
- Rheumatology Department, Hospital Universitario de Canarias, La Laguna, Tenerife, Spain
| | | | - M L Velloso-Feijoo
- Rheumatology Department, Hospital Universitario de Valme, Sevilla, Spain
| | - Ana Paula Cacheda
- Rheumatology Department, Hospital Son Llatzer, Mallorca, Islas Baleares, Spain
| | - Nuria Lozano-Rivas
- Rheumatology Department, Hospital Universitario Virgen de la Arrixaca, Murcia, Spain
| | - Gema Bonilla
- Rheumatology Department, La Paz University Hospital, Madrid, Spain
| | - Marta Arévalo
- Rheumatology Department, Consorci Sanitari Parc Taulí, Sabadell, Cataluña, Spain
| | | | | | | | | | | | | | - Tatiana Cobo-Ibañez
- Rheumatology Department, Hospital Universitario Infanta Sofía, Madrid, Spain
| | - Jose María Pego-Reigosa
- Rheumatology Department, Complejo Hospitalario Universitario de Vigo, Vigo, Galicia, Spain.,Rheumatology & Immuno-Mediated Diseases Research Group (IRIDIS), Galicia Sur Health Research Institute (IIS Galicia Sur), Spain
| |
Collapse
|
13
|
Taibi A, Durand Fontanier S, Charissoux A, Mathonnet M. Man With Joint Pain and Abdominal Pain. Ann Emerg Med 2020; 76:153-190. [PMID: 32713480 DOI: 10.1016/j.annemergmed.2020.01.023] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/26/2019] [Indexed: 11/16/2022]
Affiliation(s)
- Abdelkader Taibi
- Digestive Surgery Department, Dupuytren University Hospital, Limoges, France; University Limoges, CNRS, XLIM, UMR 7252, Limoges, France
| | - Sylvaine Durand Fontanier
- Digestive Surgery Department, Dupuytren University Hospital, Limoges, France; University Limoges, CNRS, XLIM, UMR 7252, Limoges, France
| | | | - Muriel Mathonnet
- Digestive Surgery Department, Dupuytren University Hospital, Limoges, France
| |
Collapse
|
14
|
Chowichian M, Aanpreung P, Pongpaibul A, Charuvanij S. Lupus enteritis as the sole presenting feature of systemic lupus erythematosus: case report and review of the literature. Paediatr Int Child Health 2019; 39:294-298. [PMID: 30191770 DOI: 10.1080/20469047.2018.1504430] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/17/2023]
Abstract
Systemic lupus erythematosus (SLE) is a multisystem, autoimmune inflammatory disease which can affect any organ, including the gastrointestinal tract. Lupus enteritis is one of the manifestations of gastrointestinal involvement in SLE patients. However, it is exceedingly rare that lupus enteritis is the sole initial presentation of SLE. A 12-year-old Thai girl who had had recurrent abdominal pain for 2 months with no other signs of SLE on initial presentation is described. A single-balloon enteroscopy demonstrated segmental erythema of the proximal and mid-jejunum. Histopathology demonstrated active enteritis and submucosal vasculitis. On the basis of evidence of intestinal vasculitis, autoimmune profiles were performed; the results supported the possibility of SLE. She subsequently developed leucopenia, lymphopenia and an oral ulcer, leading to a robust diagnosis of SLE. Her clinical condition improved dramatically with prednisolone. Even though lupus enteritis is rare, it can be the initial presentation of SLE. In young adolescent girls with recurrent abdominal pain, the possibility of lupus enteritis should be borne in mind.
Collapse
Affiliation(s)
- Monthira Chowichian
- Division of Rheumatology, Department of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol University , Bangkok , Thailand
| | - Prapun Aanpreung
- Division of Gastroenterology, Department of Pediatrics, Mahidol University , Bangkok , Thailand
| | - Ananya Pongpaibul
- Department of Pathology, Faculty of Medicine Siriraj Hospital, Mahidol University , Bangkok , Thailand
| | - Sirirat Charuvanij
- Division of Rheumatology, Department of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol University , Bangkok , Thailand
| |
Collapse
|
15
|
Gastrointestinal Tract Vasculopathy: Clinicopathology and Description of a Possible "New Entity" With Protean Features. Am J Surg Pathol 2019; 42:866-876. [PMID: 29624512 DOI: 10.1097/pas.0000000000001060] [Citation(s) in RCA: 15] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/16/2022]
Abstract
Noninfectious gastrointestinal (GI) vasculopathic disorders are rare and are often overlooked in histopathologic examination or when forming differential diagnoses due to their rarity. However, involvement of the GI tract may lead to serious complications, including ischemia and perforation. Since awareness of the types of vasculopathy that may involve the GI tract is central to arriving at a correct diagnosis, we reviewed our institutional experience with GI tract vasculopathy in order to enhance diagnostic accuracy of these rare lesions. We report the clinical and histologic features of 16 cases (excluding 16 cases of immunoglobulin A vasculitis) diagnosed over a 20-year period. Of the 16 patients, 14 presented with symptoms related to the GI vasculopathy (including 2 presenting with a mass on endoscopic examination). The remaining 2 patients presented with incarcerated hernia and invasive adenocarcinoma. The vasculopathy was not associated with systemic disease and appeared limited to the GI tract in 8 patients. Eight had associated systemic disease, but only 6 had a prior diagnosis. The underlying diagnoses in these 6 patients included systemic lupus erythematosus (1), dermatomyositis (2), rheumatoid arthritis (1), eosinophilic granulomatosis with polyangiitis (1), and Crohn disease (1). One patient with granulomatous polyangiitis and 1 patient with systemic lupus erythematosus initially presented with GI symptoms. The 8 cases of isolated GI tract vasculopathy consisted of enterocolic lymphocytic phlebitis (4), idiopathic myointimal hyperplasia of the sigmoid colon (1), idiopathic myointimal hyperplasia of the ileum (1), granulomatous vasculitis (1), and polyarteritis nodosa-like arteritis (1). Isolated GI tract vasculopathy is rare, but appears to be almost as common as that associated with systemic disease. The chief primary vasculopathies are enterocolic lymphocytic colitis and idiopathic myointimal hyperplasia. Although the latter occurs predominantly in the left colon, rare examples occur in the small bowel and likely represent a complex, more protean disorder.
Collapse
|
16
|
A rare case of laparoscopy towards SLE with lupus mesenteric vasculitis induced ascites. BMC Surg 2019; 19:73. [PMID: 31266494 PMCID: PMC6604445 DOI: 10.1186/s12893-019-0533-5] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/04/2018] [Accepted: 06/19/2019] [Indexed: 11/20/2022] Open
Abstract
Background Diagnosis and management of acute abdomen secondary to systematic lupus erythematosus (SLE) has always been a clinical challenge. Case presentation A 21-year-old lady, with BMI 17.7, presented to our department with acute abdomen. Laparoscopy was carried out to exclude surgical emergency when conservative regimen failed. The patient revealed a history of purpuric changes and lupus test was positive for SLE. Conclusion Based on our experience, early laparoscopy to alleviate acute abdomen has shown to improve the prognosis of the patient.
Collapse
|
17
|
Liang Y, Di Re A, El Khoury T. A rare case of lupus-related gastrointestinal vasculitis presenting as rectal gangrene. J Surg Case Rep 2019; 2019:rjz128. [PMID: 31044066 PMCID: PMC6486653 DOI: 10.1093/jscr/rjz128] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/11/2019] [Accepted: 04/09/2019] [Indexed: 11/14/2022] Open
Abstract
Systemic lupus erythematosus (SLE) related gastrointestinal vasculitis is a rare condition limited to case studies within the literature however, no cases of rectal gangrene and perforation have been previously described. A 32-year-old male presented with abdominal pain, vomiting and fevers. CT demonstrated free gas and free fluid around the rectum indicative of a perforation. He proceeded to urgent laparotomy, confirming a diagnosis of rectal infarction and perforation. Uniquely, the involved segment of gangrene extended from the rectosigmoid to the anorectal junction. A Hartmanns procedure was performed. Histopathology confirmed underlying stenosis of the rectal arteries secondary to chronic vasculitis related to the affected areas. The current case is a unique presentation of SLE-related vasculitis. It highlights the need to judiciously investigate SLE patients presenting with surgical acute abdomen.
Collapse
Affiliation(s)
- Yi Liang
- Westemead Hospital, Cnr Hawkesbury and Darcy Road, Westmead NSW, Australia
| | - Angelina Di Re
- Westemead Hospital, Cnr Hawkesbury and Darcy Road, Westmead NSW, Australia
| | - Toufic El Khoury
- Westemead Hospital, Cnr Hawkesbury and Darcy Road, Westmead NSW, Australia.,University of Notre Dame Medical School, 160 Oxford Street, Darlinghurst NSW, Australia
| |
Collapse
|
18
|
Smith EMD, Lythgoe H, Hedrich CM. Vasculitis in Juvenile-Onset Systemic Lupus Erythematosus. Front Pediatr 2019; 7:149. [PMID: 31143758 PMCID: PMC6521594 DOI: 10.3389/fped.2019.00149] [Citation(s) in RCA: 16] [Impact Index Per Article: 2.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/25/2018] [Accepted: 04/01/2019] [Indexed: 01/08/2023] Open
Abstract
Juvenile-onset systemic lupus erythematosus (JSLE) is a rare, heterogeneous multisystem autoimmune disease that can affect any organ, and present with diverse clinical and serological manifestations. Vasculitis can be a feature of JSLE. It more commonly presents as cutaneous vasculitis than visceral vasculitis, which can affect the central nervous system, peripheral nervous system, lungs, gut, kidneys, heart, and large vessels. The incidence and prevalence of vasculitis in JSLE has not been well described to date. Symptoms of vasculitis can be non-specific and overlap with other features of JSLE, requiring careful consideration for the diagnosis to be achieved and promptly treated. Biopsies are often required to make a definitive diagnosis and differentiate JSLE related vasculitis from other manifestations of JSLE, vasculopathies, and JSLE related antiphospholipid syndrome. Visceral vasculitis can be life threatening, and its presence at the time of JSLE diagnosis is associated with permanent organ damage, which further highlights the importance of prompt recognition and treatment. This review will focus on the presentation, diagnosis, management and outcomes of vasculitis in JSLE, highlighting gaps in the current evidence base.
Collapse
Affiliation(s)
- Eve M D Smith
- Department of Women and Children's Health, Institute of Translational Medicine, University of Liverpool, Liverpool, United Kingdom.,Department of Paediatric Rheumatology, Alder Hey Children's NHS Foundation Trust, Liverpool, United Kingdom
| | - Hanna Lythgoe
- St Helen's and Knowsley Teaching Hospital NHS Trust, St Helens, United Kingdom
| | - Christian M Hedrich
- Department of Women and Children's Health, Institute of Translational Medicine, University of Liverpool, Liverpool, United Kingdom.,Department of Paediatric Rheumatology, Alder Hey Children's NHS Foundation Trust, Liverpool, United Kingdom
| |
Collapse
|
19
|
Maruyama A, Nagashima T, Iwamoto M, Minota S. Clinical characteristics of lupus enteritis in Japanese patients: the large intestine-dominant type has features of intestinal pseudo-obstruction. Lupus 2018; 27:1661-1669. [PMID: 30028259 DOI: 10.1177/0961203318785770] [Citation(s) in RCA: 14] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/16/2022]
Abstract
This study was performed to investigate the clinical characteristics of lupus enteritis in Japanese patients with systemic lupus erythematosus (SLE). A total of 481 patients with SLE admitted to our hospital between 2001 and 2015 were retrospectively reviewed. Diagnosis of lupus enteritis was based on the following three criteria: (1) abdominal symptoms, (2) diffuse long-segment bowel thickening and (3) a requirement for glucocorticoid therapy. Lupus enteritis was identified in 17 patients (3.5%) and there were two distinct types: small intestine-dominant and large intestine-dominant. Significant differences between the two types were noted with respect to the age, frequency of biopsy-proven lupus nephritis, frequency of rectal involvement, maximum bowel wall thickness, and requirement for steroid pulse therapy. Among patients with large intestine-dominant lupus enteritis, 60% had extra-intestinal symptoms (hydroureter, bladder wall thickening, and bile duct dilatation) that are known complications of intestinal pseudo-obstruction. Two patients with large intestine-dominant lupus enteritis developed intestinal pseudo-obstruction either before or after diagnosis of lupus enteritis. Five patients (29%) developed recurrence during a median observation period of 7.2 years (1.4-14.4 years). In conclusion, large intestine-dominant lupus enteritis resembles intestinal pseudo-obstruction and these two diseases may have a common pathogenesis.
Collapse
Affiliation(s)
- A Maruyama
- Division of Rheumatology and Clinical Immunology, Department of Medicine, Jichi Medical University, Shimotsuke, Japan
| | - T Nagashima
- Division of Rheumatology and Clinical Immunology, Department of Medicine, Jichi Medical University, Shimotsuke, Japan
| | - M Iwamoto
- Division of Rheumatology and Clinical Immunology, Department of Medicine, Jichi Medical University, Shimotsuke, Japan
| | - S Minota
- Division of Rheumatology and Clinical Immunology, Department of Medicine, Jichi Medical University, Shimotsuke, Japan
| |
Collapse
|
20
|
Iwasaki K, Morimoto M, Ota G, Koinuma K, Horie H, Sata N, Nakaya T. Partial small intestinal resection for successful surgical management of refractory protein-losing gastroenteropathy in systemic lupus erythematosus: A case report and literature review. Medicine (Baltimore) 2018; 97:e11357. [PMID: 30045258 PMCID: PMC6078739 DOI: 10.1097/md.0000000000011357] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/25/2022] Open
Abstract
RATIONALE Although systemic lupus erythematosus (SLE) can be complicated by various gastrointestinal tract diseases, it is rarely associated with lupus enteritis and protein-losing enteropathy (PLE). We report here the successful surgical treatment of lupus enteritis and therapy-resistant and refractory PLE in a patient with SLE. We also provide a review of relevant literature. PATIENT CONCERNS A 16-year-old girl presenting with polyarthritis, malar rash, and palmar erythema was indicated for steroid therapy on the basis of positive results for antinuclear, anti-Smith, and antiphospholipid antibodies, which confirmed the diagnosis of SLE. During the course of steroid therapy, the patient developed acute abdomen and hypoalbuminemia. DIAGNOSES Computed tomography and Tc-labeled human serum albumin scintigraphy revealed abnormal findings, and a diagnosis of lupus enteritis and PLE was made. Steroid treatment was continued but no significant improvement was observed, and the patient was referred and admitted to our hospital. Double-balloon enteroscopy revealed multiple ischemic stenoses and mucosal necroses in the small intestine, suggesting that PLE was associated with ischemic enteritis due to antiphospholipid syndrome. The patient received steroids, immunosuppressive drugs, and antithrombotic therapy, with no improvement in symptoms. Thus, the disease was judged to be refractory and resistant to medical therapy, and the patient was indicated for surgical treatment. INTERVENTIONS Partial small intestinal resection was performed by removing the segment of the small intestine presenting PLE lesions, and a double-end ileostomy was created. OUTCOMES Multiple stenotic lesions were confirmed in the resected segment. Histopathology evaluation revealed marked inflammatory cell infiltration in the intestinal tract wall and recanalization of the vessels, suggesting a circulatory disorder caused by vasculitis and antiphospholipid syndrome. Postoperatively, the clinical course was good. Serum albumin levels and body weight increased as nutritional status improved significantly. Secondary enteroenterostomy with ileostomy closure could be performed at 2 months after the initial surgery. LESSONS Timely surgical treatment can be successful in managing therapy-resistant and refractory PLE in patients with SLE.
Collapse
Affiliation(s)
| | | | | | | | | | | | - Takeo Nakaya
- Department of Pathology, Jichi Medical University, Tochigi, Japan
| |
Collapse
|
21
|
Gayam V, Mandal AK, Khalid M, Kaler J, Thapa S, Garlapati P, Gill A, Alex R, Shrestha B. A Rare Case of Systemic Lupus Erythematosus with Gastric Ulcer and Acute Pancreatitis: A Case Report and Literature Review. Gastroenterology Res 2018; 11:321-325. [PMID: 30116433 PMCID: PMC6089583 DOI: 10.14740/gr1048w] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/15/2018] [Accepted: 06/17/2018] [Indexed: 12/31/2022] Open
Abstract
Systemic lupus erythematosus (SLE) is a chronic inflammatory disease which can manifest in many different organ systems. Gastrointestinal (GI) involvement is common in SLE, but the symptoms are usually mild. More severe GI complications including acute pancreatitis and peptic ulcer bleeding are rare but represent a significant risk of morbidity and mortality. We present a case of a 25-year-old Hispanic female with a severe SLE flare. The initial presentation included symptoms of hematemesis and epigastric abdominal pain secondary to both gastric ulceration and acute pancreatitis, an atypical presentation of an SLE flare. The non-specific symptom of abdominal pain makes both acute pancreatitis and gastric ulcer disease a clinical challenge; however, clinicians need to have a high suspicion for these conditions co-existing at the same time due to higher mortality rates.
Collapse
Affiliation(s)
- Vijay Gayam
- Department of Medicine, Interfaith Medical Center, Brooklyn, NY, USA
| | | | - Mazin Khalid
- Department of Medicine, Interfaith Medical Center, Brooklyn, NY, USA
| | - Jaspreet Kaler
- Department of Medicine, Interfaith Medical Center, Brooklyn, NY, USA
| | - Shivani Thapa
- Department of Medicine, Interfaith Medical Center, Brooklyn, NY, USA
| | - Pavani Garlapati
- Department of Medicine, Interfaith Medical Center, Brooklyn, NY, USA
| | - Arshpal Gill
- Department of Medicine, Interfaith Medical Center, Brooklyn, NY, USA
| | - Ragin Alex
- Department of Medicine, Interfaith Medical Center, Brooklyn, NY, USA
| | - Binav Shrestha
- Department of Medicine, Interfaith Medical Center, Brooklyn, NY, USA
| |
Collapse
|
22
|
Gastrointestinal system manifestations in juvenile systemic lupus erythematosus. Clin Rheumatol 2017; 36:1521-1526. [PMID: 28204893 DOI: 10.1007/s10067-017-3571-3] [Citation(s) in RCA: 18] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/01/2016] [Revised: 01/28/2017] [Accepted: 02/03/2017] [Indexed: 12/11/2022]
|
23
|
Sharma A, Dhooria A, Aggarwal A, Rathi M, Chandran V. Connective Tissue Disorder-Associated Vasculitis. Curr Rheumatol Rep 2017; 18:31. [PMID: 27097818 DOI: 10.1007/s11926-016-0584-x] [Citation(s) in RCA: 10] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/17/2022]
Abstract
Vasculitides secondary to connective tissue diseases are classified under the category of 'vasculitis associated with systemic disease' in the revised International Chapel Hill Consensus Conference (CHCC) nomenclature. These secondary vasculitides may affect any of the small, medium or large vessels and usually portend a poor prognosis. Any organ system can be involved and the presentation would vary depending upon that involvement. Treatment depends upon the type and severity of presentation. In this review, we describe secondary vasculitis associated with rheumatoid arthritis, systemic lupus erythematosus, sarcoidosis, relapsing polychondritis, systemic sclerosis, Sjogren's syndrome and idiopathic inflammatory myositis, focusing mainly on recent advances in the past 3 years.
Collapse
Affiliation(s)
- Aman Sharma
- Department of Internal Medicine, Post Graduate Institute of Medical Education and Research, Chandigarh, India, 160012.
| | - Aadhaar Dhooria
- Department of Internal Medicine, Post Graduate Institute of Medical Education and Research, Chandigarh, India, 160012
| | - Ashish Aggarwal
- Department of Internal Medicine, Post Graduate Institute of Medical Education and Research, Chandigarh, India, 160012
| | - Manish Rathi
- Department of Nephrology, Post Graduate Institute of Medical Education and Research, Chandigarh, India
| | - Vinod Chandran
- Department of Medicine, Division of Rheumatology, & Division of Laboratory Medicine and Pathobiology, University of Toronto, Toronto, ON, Canada.,Institute of Medical Science, University of Toronto, Toronto, ON, Canada.,Centre for Prognosis Studies in the Rheumatic Diseases, Krembil Research Institute, University Health Network, Toronto, ON, Canada
| |
Collapse
|
24
|
Kwok SK, Seo SH, Ju JH, Park KS, Yoon CH, Kim WU, Min JK, Park SH, Cho CS, Kim HY. Lupus enteritis: clinical characteristics, risk factor for relapse and association with anti-endothelial cell antibody. Lupus 2016; 16:803-9. [PMID: 17895303 DOI: 10.1177/0961203307082383] [Citation(s) in RCA: 55] [Impact Index Per Article: 6.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/16/2022]
Abstract
The study was undertaken to evaluate clinical and laboratory characteristics of patients with lupus enteritis and to investigate its association with anti-endothelial cell antibodies (AECAs). Systemic lupus erythematosus (SLE) patients who were admitted to Kangnam St. Mary's Hospital with complaints of acute abdominal pain from January 1990 to July 2006 were reviewed retrospectively. The clinical features, laboratory data and prognosis of these patients were analyzed. Among the 706 SLE patients admitted during the study period, 87 were found to admit for acute abdominal pain. Among them, 41 patients were identified with lupus enteritis. The SLE disease activity index score at admission and the mean prednisolone dose administered during the last three months prior to admission were significantly higher in patients with lupus enteritis than those with other causes ( P < 0.001, P = 0.036). Serum anti-endothelial cell antibody (AECA-IgG) titer was also significantly higher in patients with lupus enteritis than those with other manifestations or healthy controls ( P = 0.040, P < 0.001). Four out of 13 recurrent patients had pre-existing anti-phospholipid syndrome (APS), whereas only one out of 28 non-recurrent patients had pre-existing APS ( P = 0.028). Most of the patients with lupus enteritis showed good response to high-dose intravenous steroids and there was no death directly associated with lupus enteritis. Lupus (2007) 16, 803—809.
Collapse
Affiliation(s)
- S-K Kwok
- Division of Rheumatology, Department of Internal Medicine, School of Medicine, The Catholic University of Korea, Seoul, South Korea
| | | | | | | | | | | | | | | | | | | |
Collapse
|
25
|
Alves SC, Fasano S, Isenberg DA. Autoimmune gastrointestinal complications in patients with systemic lupus erythematosus: case series and literature review. Lupus 2016; 25:1509-1519. [PMID: 27329649 DOI: 10.1177/0961203316655210] [Citation(s) in RCA: 33] [Impact Index Per Article: 3.7] [Reference Citation Analysis] [Abstract] [Key Words] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/10/2015] [Accepted: 05/25/2016] [Indexed: 02/06/2023]
Abstract
The association of systemic lupus erythematosus (SLE) with gastrointestinal autoimmune diseases is rare, but has been described in the literature, mostly as case reports. However, some of these diseases may be very severe, thus a correct and early diagnosis with appropriate management are fundamental. We have analysed our data from the SLE patient cohort at University College Hospital London, established in 1978, identifying those patients with an associated autoimmune gastrointestinal disease. We have also undertaken a review of the literature describing the major autoimmune gastrointestinal pathologies which may be coincident with SLE, focusing on the incidence, clinical and laboratory (particularly antibody) findings, common aetiopathogenesis and complications.
Collapse
Affiliation(s)
- S Custódio Alves
- Internal Medicine Unit, Department of Medicine, Hospital de Cascais, Cascais, Portugal
| | - S Fasano
- Rheumatology Unit, Department of Clinical and Experimental Medicine, Second University of Naples, Naples, Italy
| | - D A Isenberg
- Centre for Rheumatology, Department of Medicine, University College London, London, UK
| |
Collapse
|
26
|
Gu Y, Zhu T, Wang Y, Xu H. Systemic lupus erythematosus with intestinal perforation: A case report. Exp Ther Med 2015; 10:1234-1238. [PMID: 26622471 DOI: 10.3892/etm.2015.2639] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/09/2014] [Accepted: 07/07/2015] [Indexed: 12/13/2022] Open
Abstract
Systemic lupus erythematosus (SLE) is a systemic autoimmune inflammatory disease, which can affect almost all systems and organs. Gastrointestinal disorder is one of the most noteworthy complications of patients with SLE. However, gastrointestinal disorder with intestinal perforation is rare, but potentially life-threatening if not treated promptly. The present study reported a case of SLE with intestinal perforation, where surgical intervention was performed and a crevasse (~3 cm in diameter) was detected in the ileum, ~60 cm from the ileocecal valve. Following surgery, the patient suffered from difficult ventilator weaning, septic shock and intestinal obstruction. The patient was successfully treated and discharged from the hospital after ~4 months of treatment. Intestinal perforation in SLE patients is potentially life-threatening; early diagnosis and prompt treatment are crucial to the management of this rare complication of SLE.
Collapse
Affiliation(s)
- Yuqing Gu
- Department of General Surgery, Taicang Hospital Affiliated to Soochow University, Taicang, Jiangsu 215400, P.R. China
| | - Tao Zhu
- Department of Intensive Care Unit, Taicang Hospital Affiliated to Soochow University, Taicang, Jiangsu 215400, P.R. China
| | - Yiqing Wang
- Department of General Surgery, Taicang Hospital Affiliated to Soochow University, Taicang, Jiangsu 215400, P.R. China
| | - Hongxing Xu
- Department of General Surgery, Taicang Hospital Affiliated to Soochow University, Taicang, Jiangsu 215400, P.R. China
| |
Collapse
|
27
|
Mesenteric vasculitis in children with systemic lupus erythematosus. Clin Rheumatol 2015; 35:785-93. [DOI: 10.1007/s10067-015-2892-3] [Citation(s) in RCA: 13] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/24/2014] [Revised: 01/26/2015] [Accepted: 01/31/2015] [Indexed: 01/25/2023]
|
28
|
Joo SH, Lee JM, Park JK, Lee EY, Lee EB, Song YW. A Case of Ischemic Enteritis with Small Bowel Perforation during Management of Lupus Enteritis. JOURNAL OF RHEUMATIC DISEASES 2015. [DOI: 10.4078/jrd.2015.22.6.378] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/22/2022]
Affiliation(s)
- Sang Hyun Joo
- Division of Rheumatology, Department of Internal Medicine, Seoul National University Hospital, Seoul, Korea
| | - Jae Myung Lee
- Division of Rheumatology, Department of Internal Medicine, Seoul National University Hospital, Seoul, Korea
| | - Jin Kyun Park
- Division of Rheumatology, Department of Internal Medicine, Seoul National University Hospital, Seoul, Korea
| | - Eun Young Lee
- Division of Rheumatology, Department of Internal Medicine, Seoul National University Hospital, Seoul, Korea
| | - Eun Bong Lee
- Division of Rheumatology, Department of Internal Medicine, Seoul National University Hospital, Seoul, Korea
| | - Yeong Wook Song
- Division of Rheumatology, Department of Internal Medicine, Seoul National University Hospital, Seoul, Korea
| |
Collapse
|
29
|
Abdel Galil SM. Hydroxychloroquine-induced toxic hepatitis in a patient with systemic lupus erythematosus: a case report. Lupus 2014; 24:638-40. [PMID: 25424894 DOI: 10.1177/0961203314561667] [Citation(s) in RCA: 34] [Impact Index Per Article: 3.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/28/2014] [Accepted: 11/05/2014] [Indexed: 12/22/2022]
Abstract
Increased serum level of liver enzymes is a common finding in patients with systemic lupus erythematosus (SLE). Hepatotoxic drugs, viral hepatitis and fatty liver are thought to be the main causes of hepatic lesion in these patients. Our aim was to determine the cause of strikingly elevated liver enzymes in a case with systemic lupus presenting with acute abdomen. Liver enzyme abnormality was defined as a 10-fold or greater increase in aspartate aminotransferase and alanine aminotransferase. Acute toxic hepatitis was diagnosed, which rapidly returned to normal after cessation of the suspected causative medication, hydroxychloroquine, and subsequent administration of mycophenolate mofetil. Elevated liver enzymes are a major concern and should be well investigated in SLE patients.
Collapse
Affiliation(s)
- S M Abdel Galil
- Rheumatology & Rehabilitation Department, Faculty of Medicine, Zagazig University, Egypt Medicine Department, Faculty of Medicine, Umm Al-Qura University, Saudi Arabia
| |
Collapse
|
30
|
Kaieda S, Kobayashi T, Moroki M, Honda S, Yuge K, Kawano H, Mitsuyama K, Sata M, Ida H, Hoshino T, Fukuda T. Successful treatment of rectal ulcers in a patient with systemic lupus erythematosus using corticosteroids and tacrolimus. Mod Rheumatol 2014; 24:357-60. [PMID: 24593214 DOI: 10.3109/14397595.2013.852846] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/13/2022]
Abstract
Systemic lupus erythematosus (SLE) is frequently accompanied by gastrointestinal symptoms. Although all parts of the gastrointestinal tract may be affected, colonic involvement is quite rare. Colonic ulceration, particularly in the rectum, is associated with a high mortality rate in patients with SLE, despite immunosuppressive therapy. While a standard regimen for treating rectal ulcers as a complication of SLE has not been established, combination therapy with steroids and immunosuppressive agents is necessary because of the associated high mortality rate. In this report, we describe a patient with SLE whose condition was complicated with ulcerative lesions in the rectum and sigmoid colon; the lesions were successfully treated with a combination of corticosteroids and tacrolimus therapy. Tacrolimus could be a useful additional or alternative modality for treating rectal involvement in SLE.
Collapse
Affiliation(s)
- Shinjiro Kaieda
- Division of Respirology, Neurology and Rheumatology, Department of Medicine, Kurume University School of Medicine , Kurume , Japan
| | | | | | | | | | | | | | | | | | | | | |
Collapse
|
31
|
López CAG, Laredo-Sánchez F, Malagón-Rangel J, Flores-Padilla MG, Nellen-Hummel H. Intestinal pseudo-obstruction in patients with systemic lupus erythematosus: A real diagnostic challenge. World J Gastroenterol 2014; 20:11443-11450. [PMID: 25170234 PMCID: PMC4145788 DOI: 10.3748/wjg.v20.i32.11443] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/05/2014] [Revised: 03/11/2014] [Accepted: 04/16/2014] [Indexed: 02/06/2023] Open
Abstract
Intestinal pseudo-obstruction secondary to systemic lupus erythematosus (SLE) is a rare syndrome described in recent decades. There are slightly over 30 published cases in the English language literature, primarily associated with renal and hematological disease activity. Its presentation and evolution are a diagnostic challenge for the clinician. We present four cases of intestinal pseudo-obstruction due to lupus in young Mexican females. One patient had a previous diagnosis of SLE and all presented with a urinary tract infection of varying degrees of severity during their evolution. We consider that recognition of the disease is of vital importance because it allows for establishing appropriate management, leading to a better prognosis and avoiding unnecessary surgery and complications.
Collapse
|
32
|
|
33
|
Wakui H, Togashi M, Omokawa A, Okuyama S, Masai R, Komatsuda A, Ogasawara H, Sawada KI. Simultaneous herpes simplex virus esophagitis and lupus enteritis in a patient with systemic lupus erythematosus. Mod Rheumatol 2014. [DOI: 10.3109/s10165-009-0237-2] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/13/2022]
|
34
|
Moon SJ, Kwok SK, Park KS, Kim WU, Park SH, Kim HY. Simultaneous presentation of hemophagocytic syndrome and mesenteric vasculitis in a patient with systemic lupus erythematosus. Mod Rheumatol 2014. [DOI: 10.3109/s10165-010-0401-8] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/22/2022]
Affiliation(s)
- Su-Jin Moon
- Division of Rheumatology, Department of Internal Medicine, School of Medicine, The Catholic University of Korea, Seoul St. Mary’s Hospital, 505 Banpo-Dong, Seocho-Gu, Seoul 137-040,, South Korea
| | - Seung-Ki Kwok
- Division of Rheumatology, Department of Internal Medicine, School of Medicine, The Catholic University of Korea, Seoul St. Mary’s Hospital, 505 Banpo-Dong, Seocho-Gu, Seoul 137-040,, South Korea
| | - Kyung-Su Park
- Division of Rheumatology, Department of Internal Medicine, School of Medicine, The Catholic University of Korea, Seoul St. Mary’s Hospital, 505 Banpo-Dong, Seocho-Gu, Seoul 137-040,, South Korea
| | - Wan-Uk Kim
- Division of Rheumatology, Department of Internal Medicine, School of Medicine, The Catholic University of Korea, St. Vincent’s Hospital, 93 Chi-Dong, Suwon 442-723, South Korea
| | - Sung-Hwan Park
- Division of Rheumatology, Department of Internal Medicine, School of Medicine, The Catholic University of Korea, Seoul St. Mary’s Hospital, 505 Banpo-Dong, Seocho-Gu, Seoul 137-040,, South Korea
| | - Ho-Youn Kim
- Division of Rheumatology, Department of Internal Medicine, School of Medicine, The Catholic University of Korea, Seoul St. Mary’s Hospital, 505 Banpo-Dong, Seocho-Gu, Seoul 137-040,, South Korea
| |
Collapse
|
35
|
Lupus mesenteric vasculitis: clinical features and associated factors for the recurrence and prognosis of disease. Semin Arthritis Rheum 2013; 43:759-66. [PMID: 24332116 DOI: 10.1016/j.semarthrit.2013.11.005] [Citation(s) in RCA: 41] [Impact Index Per Article: 3.4] [Reference Citation Analysis] [Abstract] [Key Words] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/22/2013] [Revised: 11/06/2013] [Accepted: 11/07/2013] [Indexed: 01/07/2023]
Abstract
OBJECTIVE To evaluate the clinical characteristics of lupus mesenteric vasculitis (LMV) and identify the potential factors and appropriate treatments that are associated with disease relapse and prognosis in LMV. METHODS A retrospective cohort study was performed among patients admitted to the First Affiliated Hospital of Sun Yet-sen University between 2002 and 2011. Demographic information, clinical symptoms, laboratory findings, imaging characteristics like abdominal CT scan, ultrasonography, medications including corticosteroid, cyclophosphamide, and other immunosuppressive agents, and outcomes were documented. The endpoints of the study were defined as occurrence of severe complications that needed surgical intervention, disease recurrence, or death. RESULTS Out of 3823 systemic lupus erythematosus (SLE) patients, 97 were diagnosed with mesenteric vasculitis with the overall prevalence of 2.5%. Among these 97 LMV patients, 13 died because of serious complications (13/97, 13.4%) and 2 presented intestinal perforation during the induction therapy stage. The logistic regression multivariate analysis indicated that leukopenia [peripheral WBC, odds ratio (OR) = 0.640, 95% confidence interval (CI): 0.456-0.896, P = 0.009], hypoalbuminemia (serum albumin, OR = 0.891, 95% CI: 0.798-0.994, P = 0.039) and elevated serum amylase (OR = 7.719, 95% CI: 1.795-33.185, P = 0.006) were positively associated with the occurrence of serious complications, while intravenous cyclophosphamide (CYC) therapy inhibited the occurrence of serious complications (OR = 0.220, 95% CI: 0.053-0.903, P = 0.036). A total of 79 patients who achieved remission were followed-up for 2-96 months and 18 cases experienced disease relapse (18/79, 22.8%). The statistical analysis adjusted by Cox proportional hazards models indicated that high-dose CYC therapy (≥ 1.0 g/m(2)/month) was a protective factor for disease relapse and led to better outcomes [hazard ratio (HR) = 0.209, 95% CI: 0.049-0.887, P = 0.034], while the severe thickness of the bowel wall (>8mm) was a risk factor (HR = 7.308, 95% CI: 1.740-30.696, P = 0.007). LMV and lupus cystitis occurred concurrently in 22 (22/97, 22.7%) patients, and the symptoms of urinary tract resolved after treatment with corticosteroid and immunosupressants. CONCLUSION LMV is one of the serious complications of SLE with high mortality. The current study demonstrated that leukopenia, hypoalbuminemia, and elevated serum amylase were associated with severe adverse events, while CYC therapy led to better outcomes during remission-induction stage. Severe thickness of the bowel was a risk factor while high-dose CYC therapy was a protective factor for disease relapse in intensification therapy stage. It is necessary to evaluate the urinary tract involvement once LMV is diagnosed due to the frequent coexistence of these 2 diseases.
Collapse
|
36
|
Yuan S, Lian F, Chen D, Li H, Qiu Q, Zhan Z, Ye Y, Xu H, Liang L, Yang X. Clinical Features and Associated Factors of Abdominal Pain in Systemic Lupus Erythematosus. J Rheumatol 2013; 40:2015-22. [PMID: 24187097 DOI: 10.3899/jrheum.130492] [Citation(s) in RCA: 25] [Impact Index Per Article: 2.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
Abstract
Objective.To evaluate the clinical characteristics of systemic lupus erythematosus (SLE)-induced abdominal pain in a cohort in South China and identify the risk factors for SLE-induced abdominal pain.Methods.This is a retrospective cohort study of SLE patients with complaint of abdominal pain admitted to the first affiliated university hospital of Sun Yat-sen University between 2002 and 2011. Demographic information, clinical features, laboratory findings, SLE Disease Activity Index, and imaging characteristics were documented.Results.Of the 3823 SLE patients reviewed, 213 patients complained of abdominal pain and 132 cases were considered SLE-induced. The most common causes were lupus mesenteric vasculitis (LMV; 73.5%, 97/132) and lupus pancreatitis (LP; 17.4%, 23/132). Other causes included appendicitis, acute gastroenteritis, and peritonitis. Univariate and multivariate logistic regression analysis indicated the European Consensus Lupus Activity Measurement (ECLAM) score was significantly associated with lupus-induced abdominal pain (OR = 1.858, 95% CI: 1.441–2.394, p < 0.001), LMV (OR = 1.713, 95% CI: 1.308-2.244, p < 0.001), and LP (OR = 2.153, 95% CI: 1.282, 3.617, p = 0.004). The serum D-dimer level (OR = 1.004, 95% CI: 1.002-1.005, p < 0.001) was a strongly associated factor for lupus-induced abdominal pain. Moderate and large amounts of ascetic fluid was significantly associated with lupus-induced abdominal pain and LMV. Elevated liver enzymes was a risk factor for LP (OR = 34.605, 95% CI: 3.591-333.472, p = 0.002).Conclusion.LMV and LP were the leading causes of SLE-induced abdominal pain. The serum D-dimer was a strongly associated factor for lupus-induced abdominal pain. ECLAM score was a reliable index in assessment of SLE-associated abdominal pain. Elevated liver enzymes, and moderate or large amounts of ascites, were positively associated with lupus-induced abdominal pain.
Collapse
|
37
|
Retamozo S, Díaz-Lagares C, Bosch X, Bové A, Brito-Zerón P, Gómez ME, Yagüe J, Forns X, Cid MC, Ramos-Casals M. Life-Threatening Cryoglobulinemic Patients With Hepatitis C: Clinical Description and Outcome of 279 Patients. Medicine (Baltimore) 2013; 92:273-284. [PMID: 23974248 PMCID: PMC4553974 DOI: 10.1097/md.0b013e3182a5cf71] [Citation(s) in RCA: 51] [Impact Index Per Article: 4.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/13/2022] Open
Abstract
Cryoglobulinemia is characterized by a wide range of causes, symptoms, and outcomes. Hepatitis C virus (HCV) infection is detected in 30%-100% of patients with cryoglobulins. Although more than half the patients with cryoglobulinemic vasculitis present a relatively benign clinical course, some may present with potentially life-threatening situations. We conducted the current study to analyze the clinical characteristics and outcomes of HCV patients presenting with life-threatening cryoglobulinemic vasculitis. We evaluated 181 admissions from 89 HCV patients diagnosed with cryoglobulinemic vasculitis consecutively admitted to our department between 1995 and 2010. In addition, we performed a systematic analysis of cases reported to date through a MEDLINE search.The following organ involvements were considered to be potentially life-threatening in HCV patients with cryoglobulinemic vasculitis: cryoglobulinemic, biopsy-proven glomerulonephritis presenting with renal failure; gastrointestinal vasculitis; pulmonary hemorrhage; central nervous system (CNS) involvement; and myocardial involvement. A total of 279 patients (30 from our department and 249 from the literature search) fulfilled the inclusion criteria: 205 presented with renal failure, 45 with gastrointestinal vasculitis, 38 with CNS involvement, 18 with pulmonary hemorrhage, and 3 with myocardial involvement; 30 patients presented with more than 1 life-threatening cryoglobulinemic manifestation. There were 146 (52%) women and 133 (48%) men, with a mean age at diagnosis of cryoglobulinemia of 54 years (range, 25-87 yr) and a mean age at life-threatening involvement of 55 years (range, 25-87 yr). In 232 (83%) patients, life-threatening involvement was the first clinical manifestation of cryoglobulinemia. Severe involvement appeared a mean of 1.2 years (range, 1-11 yr) after the diagnosis of cryoglobulinemic vasculitis. Patients were followed for a mean of 14 months (range, 3-120 mo) after the diagnosis of life-threatening cryoglobulinemia. Sixty-three patients (22%) died. The main cause of death was sepsis (42%) in patients with glomerulonephritis, and cryoglobulinemic vasculitis itself in patients with gastrointestinal, pulmonary, and CNS involvement (60%, 57%, and 62%, respectively). In conclusion, HCV-related cryoglobulinemia may result in progressive (renal involvement) or acute (pulmonary hemorrhage, gastrointestinal ischemia, CNS involvement) life-threatening organ damage. The mortality rate of these manifestations ranges between 20% and 80%. Unfortunately, this may be the first cryoglobulinemic involvement in almost two-thirds of cases, highlighting the complex management and very elevated mortality of these cases.
Collapse
Affiliation(s)
- Soledad Retamozo
- From Josep Font Laboratory of Autoimmune Diseases (SR, CDL, AB, PBZ, MEG, MRC) and Vasculitis Research Unit (MCC), Department of Autoimmune Diseases; Department of Internal Medicine (XB); Department of Immunology (JY); and Viral Hepatitis Unit (XF), Department of Hepatology; CIBERehd, Hospital Clínic, University of Barcelona, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain
| | | | | | | | | | | | | | | | | | | |
Collapse
|
38
|
Janssens P, Arnaud L, Galicier L, Mathian A, Hie M, Sene D, Haroche J, Veyssier-Belot C, Huynh-Charlier I, Grenier PA, Piette JC, Amoura Z. Lupus enteritis: from clinical findings to therapeutic management. Orphanet J Rare Dis 2013; 8:67. [PMID: 23642042 PMCID: PMC3651279 DOI: 10.1186/1750-1172-8-67] [Citation(s) in RCA: 100] [Impact Index Per Article: 8.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/10/2013] [Accepted: 04/28/2013] [Indexed: 12/15/2022] Open
Abstract
Lupus enteritis is a rare and poorly understood cause of abdominal pain in patients with systemic lupus erythematosus (SLE). In this study, we report a series of 7 new patients with this rare condition who were referred to French tertiary care centers and perform a systematic literature review of SLE cases fulfilling the revised ACR criteria, with evidence for small bowel involvement, excluding those with infectious enteritis. We describe the characteristics of 143 previously published and 7 new cases. Clinical symptoms mostly included abdominal pain (97%), vomiting (42%), diarrhea (32%) and fever (20%). Laboratory features mostly reflected lupus activity: low complement levels (88%), anemia (52%), leukocytopenia or lymphocytopenia (40%) and thrombocytopenia (21%). Median CRP level was 2.0 mg/dL (range 0–8.2 mg/dL). Proteinuria was present in 47% of cases. Imaging studies revealed bowel wall edema (95%), ascites (78%), the characteristic target sign (71%), mesenteric abnormalities (71%) and bowel dilatation (24%). Only 9 patients (6%) had histologically confirmed vasculitis. All patients received corticosteroids as a first-line therapy, with additional immunosuppressants administered either from the initial episode or only in case of relapse (recurrence rate: 25%). Seven percent developed intestinal necrosis or perforation, yielding a mortality rate of 2.7%. Altogether, lupus enteritis is a poorly known cause of abdominal pain in SLE patients, with distinct clinical and therapeutic features. The disease may evolve to intestinal necrosis and perforation if untreated. Adding with this an excellent steroid responsiveness, timely diagnosis becomes primordial for the adequate management of this rare entity.
Collapse
Affiliation(s)
- Peter Janssens
- Department of internal medicine, French reference centre for Systemic Lupus Erythematosus, AP-HP, Hôpital Pitié-Salpêtrière, F-75013, Paris, France
| | | | | | | | | | | | | | | | | | | | | | | |
Collapse
|
39
|
Ikeda S, Komatsuda A, Togashi M, Yamashita T, Michishita Y, Fujishima M, Fujishima N, Wakui H, Sawada KI. [Case report; Fibrinoid necrotizing angiitis of the gall bladder in a patient with systemic lupus erythematosus]. ACTA ACUST UNITED AC 2012; 101:3229-32. [PMID: 23342596 DOI: 10.2169/naika.101.3229] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/06/2022]
Affiliation(s)
- Sho Ikeda
- Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, Japan
| | | | | | | | | | | | | | | | | |
Collapse
|
40
|
Successful treatment of rectal ulcers in a patient with systemic lupus erythematosus using corticosteroids and tacrolimus. Mod Rheumatol 2012. [DOI: 10.1007/s10165-012-0775-x] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022]
|
41
|
Goh YP, Naidoo P, Ngian GS. Imaging of systemic lupus erythematosus. Part II: gastrointestinal, renal, and musculoskeletal manifestations. Clin Radiol 2012; 68:192-202. [PMID: 22901453 DOI: 10.1016/j.crad.2012.06.109] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/30/2011] [Revised: 05/27/2012] [Accepted: 06/01/2012] [Indexed: 12/22/2022]
Abstract
Systemic lupus erythematosus (SLE) is a chronic, multisystem autoimmune disease that has a relapsing and remitting course. It has a wide range of presentations with various organ manifestations. In this review, we have compiled the radiological findings of gastrointestinal, renal, and musculoskeletal manifestations of SLE.
Collapse
Affiliation(s)
- Y P Goh
- Department of Diagnostic Imaging, Monash Medical Centre, Australia.
| | | | | |
Collapse
|
42
|
Lee J, Jung HS, Nam HC, Kwok SK, Ju JH, Park KS, Kim HY, Park SH. Fulminant amoebic colitis mimicking intestinal vasculitis in a patient with systemic lupus erythematosus. Lupus 2012; 21:1351-5. [PMID: 22570337 DOI: 10.1177/0961203312447669] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/17/2022]
Abstract
Colitis in patients with systemic lupus erythematosus (SLE) is quite rare. It can be caused by intestinal vasculitis, mesenteric vascular thrombosis, concomitant inflammatory bowel disease or infectious colitis. It is important to make an accurate and early diagnosis as the treatments for each condition differ and a delayed diagnosis can result in life-threatening complications. However, non-specific gastrointestinal symptoms make a timely diagnosis challenging. Amoebic colitis is a rare condition in patients with SLE. Here we present a case of fulminant amoebic colitis in a patient with SLE which was initially misdiagnosed as ischemic colitis due to intestinal vasculitis. Her colitis was complicated with multiple intestinal perforations, disseminated intravascular coagulation and acute respiratory distress syndrome; but in the end, the patient was successfully treated with metronidazole and paromomycin.
Collapse
Affiliation(s)
- J Lee
- Division of Rheumatology, Department of Internal Medicine, School of Medicine, The Catholic University of Korea, South Korea
| | | | | | | | | | | | | | | |
Collapse
|
43
|
Noguchi K, Takahashi N, Homma S, Kataoka A, Kon Y, Atsumi T, Kamiyama T. Improvement of the Quality of Life of a Patient with Colon Ulceration of Systemic Lupus Erythematosus by Successful Surgical Treatment: Report of a Case. Am Surg 2012. [DOI: 10.1177/000313481207800408] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/15/2022]
Affiliation(s)
- Keita Noguchi
- Department of General Surgery Hokkaido University Graduate School of Medicine Sapporo, Hokkaido, Japan
| | - Norihiko Takahashi
- Department of General Surgery Hokkaido University Graduate School of Medicine Sapporo, Hokkaido, Japan
| | - Shigenori Homma
- Department of General Surgery Hokkaido University Graduate School of Medicine Sapporo, Hokkaido, Japan
| | - Akihiko Kataoka
- Department of General Surgery Hokkaido University Graduate School of Medicine Sapporo, Hokkaido, Japan
| | - Yujiro Kon
- Department of Medicine II Hokkaido University Graduate School of Medicine Sapporo, Hokkaido, Japan
| | - Tatsuya Atsumi
- Department of Medicine II Hokkaido University Graduate School of Medicine Sapporo, Hokkaido, Japan
| | - Toshiya Kamiyama
- Department of General Surgery Hokkaido University Graduate School of Medicine Sapporo, Hokkaido, Japan
| |
Collapse
|
44
|
Abstract
A variety of systemic and extra-abdominal diseases can cause symptoms within the abdominal cavity. Systemic and extra-abdominal diseases may include abdominal symptoms caused by several mechanisms. This article discusses the most important and common of these causes, namely the metabolic/endocrine causes, hematologic causes, inflammatory causes, infectious causes, functional causes, and the neurogenic causes.
Collapse
Affiliation(s)
- J Matthew Fields
- Department of Emergency Medicine, Thomas Jefferson University Hospital, Philadelphia, PA 19107, USA.
| | | |
Collapse
|
45
|
Chattopadhyay P, Abby Philips C, Dhua D, Saha S. Systemic lupus erythematosus presenting as ischaemic proctitis. Lupus 2011; 20:653-655. [PMID: 21300684 DOI: 10.1177/0961203310386277] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/16/2022]
Abstract
Ischaemic colitis is relatively uncommon in systemic lupus erythematosus (SLE), with only very few case reports or case series in world literature. Ischaemic colitis occurs when SLE activity is high and is inarguably due to small vessel vasculitis affecting the large or small intestine. Ischaemic proctitis with rectal ulceration, as a presenting feature in SLE, is even rarer with only two case reports in the world to date. We present the case of a 38-year-old woman, who presented with haematochezia which subsequently proved to be due to ischaemic proctitis with a large rectal ulcer in a case that was subsequently diagnosed as SLE. The clinical course in this patient greatly improved with aggressive management with systemic steroids.
Collapse
Affiliation(s)
- P Chattopadhyay
- Department of Medicine, Nil Ratan Sircar Medical College, 138 AJC Bose Road, Kolkata 700 014, West Bengal, India. drpartha73@gmail
| | | | | | | |
Collapse
|
46
|
Differences in Disease Features Between Childhood-Onset and Adult-Onset Systemic Lupus Erythematosus Patients Presenting with Acute Abdominal Pain. Semin Arthritis Rheum 2011; 40:447-54. [PMID: 20884044 DOI: 10.1016/j.semarthrit.2010.06.011] [Citation(s) in RCA: 28] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/03/2010] [Revised: 06/16/2010] [Accepted: 06/28/2010] [Indexed: 01/12/2023]
|
47
|
Chng HH, Tan BE, Teh CL, Lian TY. Major gastrointestinal manifestations in lupus patients in Asia: lupus enteritis, intestinal pseudo-obstruction, and protein-losing gastroenteropathy. Lupus 2011; 19:1404-13. [PMID: 20947549 DOI: 10.1177/0961203310374337] [Citation(s) in RCA: 43] [Impact Index Per Article: 3.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/02/2023]
Abstract
Gastrointestinal (GI) symptoms are common in patients with systemic lupus erythematosus (SLE) and may be due to the disease itself, side-effects of medications, or non-SLE causes. However, GI manifestations of lupus attract far less attention than the other major organ involvements, are infrequently reviewed and rarely documented in published lupus databases or cohort studies including those from countries in Asia. According to three reports from two countries in Asia, the cumulative prevalence of SLE GI manifestations range from 3.8% to 18%. In this review, we focus on three major GI manifestations in patients from Asian countries: lupus enteritis, intestinal pseudo-obstruction, and protein-losing gastroenteropathy, for which early recognition improves outcome and reduces morbidity and mortality.
Collapse
Affiliation(s)
- H H Chng
- Department of Rheumatology, Allergy and Immunology, Tan Tock Seng Hospital, Singapore.
| | | | | | | |
Collapse
|
48
|
Lin HP, Wang YM, Huo AP. Severe, recurrent lupus enteritis as the initial and only presentation of systemic lupus erythematosus in a middle-aged woman. JOURNAL OF MICROBIOLOGY, IMMUNOLOGY, AND INFECTION = WEI MIAN YU GAN RAN ZA ZHI 2011; 44:152-5. [PMID: 21439520 DOI: 10.1016/j.jmii.2009.12.001] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Received: 09/28/2009] [Revised: 10/01/2009] [Accepted: 12/21/2009] [Indexed: 01/29/2023]
Abstract
We describe a previously unreported condition of severe, recurrent lupus enteritis accompanied with severe hypocomplementemia as the initial and only presentation of systemic lupus erythematosus. Systemic lupus erythematosus should be suspected in any patient with computed tomography findings of enteral vasculitis or ischemic enteritis, even without lupus-related symptoms or signs; C3/C4 levels may be helpful in the differential diagnosis. If the symptoms do not improve after medical treatment, such as using steroid or cyclophosphamide pulse therapy, or necrosis and perforation of the intestines are highly suspected, surgical intervention should be considered.
Collapse
Affiliation(s)
- Hsien-Ping Lin
- Division of Gastroenterology, Department of Internal Medicine, Jen-Ai General Hospital, Taichung, Taiwan
| | | | | |
Collapse
|
49
|
Moon SJ, Kwok SK, Park KS, Kim WU, Park SH, Kim HY. Simultaneous presentation of hemophagocytic syndrome and mesenteric vasculitis in a patient with systemic lupus erythematosus. Mod Rheumatol 2011; 21:330-3. [PMID: 21229376 DOI: 10.1007/s10165-010-0401-8] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/12/2010] [Accepted: 11/29/2010] [Indexed: 10/18/2022]
Abstract
We report an 18-year old female patient with systemic lupus erythematosus (SLE), who developed fever, pancytopenia, abdominal pain, and watery diarrhea. Computed tomography (CT) and bone marrow aspirate revealed lupus mesenteric vasculitis (LMV) and hemophagocytic syndrome (HPS). Serologic tests for Epstein-Barr virus (EBV) indicated its reactivation. This case demonstrates that HPS and concomitant LMV associated with viral reactivation can occur as clinical manifestations of SLE flare.
Collapse
Affiliation(s)
- Su-Jin Moon
- Division of Rheumatology, Department of Internal Medicine, School of Medicine, The Catholic University of Korea, Seoul St. Mary's Hospital, 505 Banpo-Dong, Seocho-Gu, Seoul 137-040, South Korea
| | | | | | | | | | | |
Collapse
|
50
|
Matsumoto Y, Wakabayashi H, Otsuka F, Inoue K, Takano M, Sada KE, Makino H. Systemic lupus erythematosus complicated with acute myocardial infarction and ischemic colitis. Intern Med 2011; 50:2669-73. [PMID: 22041378 DOI: 10.2169/internalmedicine.50.5966] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/06/2022] Open
Abstract
Acute myocardial infarction (AMI) is one of the most severe manifestations in patients with systemic lupus erythematosus (SLE). Ischemic colitis, mainly caused by intestinal vasculitis, is also one of the most serious, but uncommon, complications in SLE patients. "SLE vasculitis" simultaneously involving cardiac and gastrointestinal vessels has yet to be reported. This is the first report of SLE accompanying AMI, ischemic colitis and perforation of the digestive tract possibly due to SLE vasculitis, which was dramatically improved by treatment with high-dose glucocorticoid.
Collapse
Affiliation(s)
- Yoshinori Matsumoto
- Department of Medicine and Clinical Science, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Japan.
| | | | | | | | | | | | | |
Collapse
|