|
1
|
Tkak H, Sara A, Hamami A, Elouali A, Babakhouya A, Rkain M. Lupus Hepatitis: A Rare Manifestation Revealing Systemic Lupus Erythematosus. Cureus 2024; 16:e54003. [PMID: 38476795 PMCID: PMC10928964 DOI: 10.7759/cureus.54003] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 02/11/2024] [Indexed: 03/14/2024] Open
Abstract
Systemic lupus erythematosus (SLE) is a rare disease in children but is more severe than in adults. SLE may be associated with various non-specific hepatic manifestations, but subacute lupus hepatitis remains unusual and is rarely a mode of revelation. Diagnosis is based on a combination of clinical, laboratory, and histological findings after ruling out other causes of hepatitis, notably autoimmune hepatitis (AIH). We report the case of a young girl with undiagnosed SLE, which first revealed itself as liver involvement and progressed well on corticosteroid therapy. During the course of her illness, she presented with other manifestations that led us to think of SLE with lupus hepatitis.
Collapse
Affiliation(s)
- Hassnae Tkak
- Department of Pediatrics, University Hospital Mohamed VI, Faculty of Medicine and Pharmacy, University Mohamed First, Oujda, MAR
| | - Anane Sara
- Department of Pediatrics, University Hospital Mohamed VI, Faculty of Medicine and Pharmacy, University Mohamed First, Oujda, MAR
| | - Amal Hamami
- Department of Pediatrics, University Hospital Mohamed VI, Faculty of Medicine and Pharmacy, University Mohamed First, Oujda, MAR
| | - Aziza Elouali
- Department of Pediatrics, University Hospital Mohamed VI, Faculty of Medicine and Pharmacy, University Mohamed First,, Oujda, MAR
| | - Abdeladim Babakhouya
- Department of Pediatrics, University Hospital Mohamed VI, Faculty of Medicine and Pharmacy, University Mohamed First, Oujda, MAR
| | - Maria Rkain
- Department of Pediatrics, University Hospital Mohamed VI, Faculty of Medicine and Pharmacy, University Mohamed First, Oujda, MAR
- Department of Pediatric Gastroenterology, University Hospital Mohamed VI, Faculty of Medicine and Pharmacy, University Mohamed First, Oujda, MAR
| |
Collapse
|
|
2
|
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
|
|
3
|
Shekhar, Gupta NY, Harisingani AR. Diagnosis of tuberculosis with autoimmune hepatitis–systemic lupus erythematosus overlap syndrome: a case report. J Med Case Rep 2022; 16:428. [DOI: 10.1186/s13256-022-03572-8] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/13/2021] [Accepted: 08/15/2022] [Indexed: 11/06/2022] Open
Abstract
Abstract
Background
There is ample evidence indicating that immunosuppressive therapy or immune dysregulation in systemic lupus erythematosus increases the risk for tuberculosis. Interestingly, a few case reports suggest that tuberculosis could also be a risk factor for systemic lupus erythematosus and other autoimmune diseases.
Case presentation
We report the case of a 32-year-old Indian patient who was co-diagnosed with tuberculosis, systemic lupus erythematosus, and autoimmune hepatitis without any history of prior immunosuppression. This stresses the complex relationship between tuberculosis and autoimmune diseases.
Conclusion
Further research is warranted in this field to unfold the complex relationship between tuberculosis and systemic lupus erythematosus. It is essential to establish clear guidelines for the management of coexisting tuberculosis and systemic lupus erythematosus to promote individualized treatment.
Collapse
|
|
4
|
Afzal W, Haghi M, Hasni SA, Newman KA. Lupus hepatitis, more than just elevated liver enzymes. Scand J Rheumatol 2020; 49:427-433. [PMID: 32942921 DOI: 10.1080/03009742.2020.1744712] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/26/2022]
Abstract
Systemic lupus erythematosus (SLE), a multisystem autoimmune inflammatory disease, may involve any organs, including the liver. Liver involvement in SLE is not part of the American College of Rheumatology criteria and is relatively rare. Liver disease is usually mild, manifesting as subtle elevation of liver enzymes. Jaundice and hepatomegaly can be seen in some patients; advanced liver disease with cirrhosis is extremely rare. Precise pathology remains obscure. SLE may cause non-specific changes, including hepatocellular, cholestatic, or vascular changes. Alcohol, drugs, viral infections, metabolic disorders, autoimmune hepatitis, and other common causes of liver dysfunction should be excluded. Corticosteroids may expedite the recovery process, but may lead to non-alcoholic fatty liver disease and liver damage. Several large-scale multicentre studies have shown that liver involvement is not the major cause of morbidity and mortality in SLE patients. In this review, we discuss the pathogenesis, diagnosis, differential diagnosis, clinical manifestations, management, complications, and prognosis of lupus hepatitis.
Collapse
Affiliation(s)
- W Afzal
- Sanford School of Medicine, University of South Dakota , Sioux Falls, SD, USA
| | - M Haghi
- Department of Internal Medicine, Coney Island Hospital , Brooklyn, NY, USA
| | - S A Hasni
- National Institute of Arthritis, and Musculoskeletal and Skin Diseases, National Institutes of Health , Bethesda, MD, USA
| | - K A Newman
- School of Medicine, Eisenhower Medical Center, University of California , Rancho Mirage, CA, USA
| |
Collapse
|
|
5
|
Systemic lupus erythematosus, hypoparathyroidism, and hemolytic anemia in a patient with Wilson's disease. Reumatologia 2019; 57:239-242. [PMID: 31548751 PMCID: PMC6753600 DOI: 10.5114/reum.2019.87622] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/18/2019] [Accepted: 07/19/2019] [Indexed: 12/13/2022] Open
Abstract
The authors report a female case of systemic lupus erythematosus (SLE) that was diagnosed as Wilson's disease (WD) when neurological manifestations were evident three years after the first admission. The brain imaging studies showed bilateral symmetrical basal ganglia involvement, slit lamp examination revealed Kayser-Fleischer ring of the cornea, and 24-hour urinary copper and serum ceruloplasmin also confirmed the diagnosis. The patient also had hemolytic anemia and hypoparathyroidism, which are rare presenting features of WD. SLE may be associated with WD, and presence of neurological, behavioral, or liver function abnormalities should raise the suspicion, even without apparent features of WD.
Collapse
|
|
6
|
Lai WT, Cho WH, Eng HL, Kuo MH, Huang FC. Overlap Syndrome Involving Systemic Lupus Erythematosus and Autoimmune Hepatitis in Children: A Case Report and Literature Review. Front Pediatr 2019; 7:310. [PMID: 31428592 PMCID: PMC6689994 DOI: 10.3389/fped.2019.00310] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/13/2018] [Accepted: 07/09/2019] [Indexed: 01/03/2023] Open
Abstract
Background: The diagnosis of overlap syndrome involving systemic lupus erythematosus (SLE) and autoimmune hepatitis (AIH) is not easily established because of its similar clinical presentations and biochemical features to those of lupus hepatitis. The term overlap syndrome is usually used in the context of overlap of autoimmune hepatitis with PSC (primary sclerosing cholangitis) or PBC (primary biliary cholangitis). Few cases of AIH complicated by SLE have been reported in the literature, and the condition is even rarer in childhood. Case presentation: Here we report the case of a 16-year-old girl with SLE who initially presented with autoimmune (cholestatic) hepatitis. According to American Association for the Study of Liver Diseases practice guidelines, the diagnosis was made based on aggregated scores including female (+2); ALP:AST (or ALT) ratio <1.5(+2); elevated serum IgG level(+3); ANA > 1:80 (+3); negative hepatitis viral markers and drug history (+3, +1); average alcohol intake <25 g/day (+2); and histological interface hepatitis features (+3). She then developed a malar rash, ANA positivity, anti-double-stranded DNA (anti-dsDNA) antibodies, and a low complement level. She met 4 of 17 Systemic Lupus International Collaborating Clinics classification criteria (1) for SLE. Our patient responded very well to corticosteroid at an initial dose of methylprednisolone 40 mg Q12H for 4 days tapering to 1 mg/kg/day according to liver function test results and bilirubin level. No relapse occurred during the 3-year follow-up course. Conclusions: Overlapping of SLE and AIH should be suspected when children with SLE have impaired liver function or AIH patients present with a malar or other skin rash. Liver biopsy plays an important role in establishing the differential diagnosis of SLE with liver impairment or overlap with AIH. The prompt diagnosis and adequate further treatment plans can improve disease outcomes.
Collapse
Affiliation(s)
- Wan-Tz Lai
- Department of Pediatrics, Kaohsiung Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Kaohsiung, Taiwan
| | - Wan-Hua Cho
- Department of Ophthalmology, Kaohsiung Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Kaohsiung, Taiwan
| | - Hock-Liew Eng
- Department of Anatomic Pathology, Kaohsiung Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Kaohsiung, Taiwan
| | - Ming-Hui Kuo
- Department of Pediatrics, Kaohsiung Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Kaohsiung, Taiwan
| | - Fu-Chen Huang
- Department of Pediatrics, Kaohsiung Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Kaohsiung, Taiwan
| |
Collapse
|
|
7
|
Adiga A, Nugent K. Lupus Hepatitis and Autoimmune Hepatitis (Lupoid Hepatitis). Am J Med Sci 2017; 353:329-335. [DOI: 10.1016/j.amjms.2016.10.014] [Citation(s) in RCA: 27] [Impact Index Per Article: 3.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/18/2016] [Revised: 09/23/2016] [Accepted: 10/31/2016] [Indexed: 12/21/2022]
|
|
8
|
Wong GW, Yeong T, Lawrence D, Yeoman AD, Verma S, Heneghan MA. Concurrent extrahepatic autoimmunity in autoimmune hepatitis: implications for diagnosis, clinical course and long-term outcomes. Liver Int 2017; 37:449-457. [PMID: 27541063 DOI: 10.1111/liv.13236] [Citation(s) in RCA: 51] [Impact Index Per Article: 6.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/17/2016] [Accepted: 08/13/2016] [Indexed: 02/13/2023]
Abstract
BACKGROUND Concurrent extrahepatic autoimmune disease (CEHAID) associated with autoimmune hepatitis (AIH) have been incorporated into the diagnostic criteria stipulated by the International Autoimmune Hepatitis Group (IAIHG). Large comprehensive cohort data on the extrahepatic autoimmunity in AIH remain scanty. AIM To systematically assess features and clinical impact of CEHAID on AIH. METHODS Clinical records of 562 patients with AIH from two tertiary centres in the UK were retrospectively reviewed. RESULTS Prevalence of CEHAID in patients with AIH was 42%. Autoimmune thyroid disease was the commonest CEHAID associated with AIH (101/562, 18%). Autoimmune skin diseases were more prevalent in AIH-2 than AIH-1 (21.9% vs 7%, P=.009). Personal history of CEHAID was more commonly found in AIH patients with than without first-degree family history of CEHAID [48/86 (55.8%) vs 169/446 (37.9%), P=.002]. AIH patients with CEHAID were more often women (85.2% vs 76.1%, P=.008), had higher post-treatment IAIHG score (22 vs 20, P<.001), less reactivity to smooth muscle antibodies (49.8% vs 65%, P<.001), more likely to have mild fibrosis at diagnosis (20.9% vs 6.5%, P<.001) and less often had ascites (6.3% vs 13.6%, P=.008) and coagulopathy (1.18 vs 1.27, P=.013) at presentation. Presence of CEHAID, however, did not significantly affect disease progression, prognosis and survival in AIH. CONCLUSIONS Our study confirms the strong association of CEHAID with AIH. Association between personal and familial extrahepatic autoimmunity especially among first-degree relatives was evident. Presence of CEHAID may influence clinical phenotype of AIH at presentation, but without notable impact on the long-term clinical outcomes.
Collapse
Affiliation(s)
- Guan-Wee Wong
- Institute of Liver Studies, King's College Hospital, London, UK
| | - Tian Yeong
- Department of Medicine, Brighton and Sussex Medical School, Brighton, UK
| | - David Lawrence
- Department of Gastroenterology and Hepatology, Brighton and Sussex University Hospital, Brighton, UK
| | - Andrew D Yeoman
- Institute of Liver Studies, King's College Hospital, London, UK
| | - Sumita Verma
- Department of Medicine, Brighton and Sussex Medical School, Brighton, UK.,Department of Gastroenterology and Hepatology, Brighton and Sussex University Hospital, Brighton, UK
| | | |
Collapse
|
|
9
|
Shumyak S, Yang LJ, Han S, Zhuang H, Reeves WH. "Lupoid hepatitis" in SLE patients and mice with experimental lupus. Clin Immunol 2016; 172:65-71. [PMID: 27430519 DOI: 10.1016/j.clim.2016.07.007] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/14/2016] [Accepted: 07/10/2016] [Indexed: 12/17/2022]
Abstract
The unusual subset of patients with severe hepatitis, hypergammaglobulinemia, arthritis, and LE cells in the blood reported by Henry Kunkel and others suggested to these investigators that "lupoid" hepatitis might share pathogenic mechanisms with SLE. More than half a century later, the etiology of autoimmune hepatitis remains unclear. The occurrence of autoimmune hepatitis in a small fraction (about 3%) of SLE patients in our lupus cohort and in two mouse models of SLE supports their conclusion that lupoid hepatitis may be share pathogenic mechanisms with SLE. The development of autoimmune hepatitis in mice with pristane-induced lupus provides an opportunity to further explore the potential link between these two autoimmune disorders.
Collapse
Affiliation(s)
- Stepan Shumyak
- Division of Rheumatology & Clinical Immunology, University of Florida, Gainesville, FL 32610. United States
| | - Li-Jun Yang
- Department of Pathology, Immunology, & Laboratory Medicine, University of Florida, Gainesville, FL 32610. United States
| | - Shuhong Han
- Division of Rheumatology & Clinical Immunology, University of Florida, Gainesville, FL 32610. United States
| | - Haoyang Zhuang
- Division of Rheumatology & Clinical Immunology, University of Florida, Gainesville, FL 32610. United States
| | - Westley H Reeves
- Division of Rheumatology & Clinical Immunology, University of Florida, Gainesville, FL 32610. United States.
| |
Collapse
|
|
10
|
Lim DH, Kim YG, Lee D, Min Ahn S, Hong S, Lee CK, Yoo B. Immunoglobulin G Levels as a Prognostic Factor for Autoimmune Hepatitis Combined With Systemic Lupus Erythematosus. Arthritis Care Res (Hoboken) 2016; 68:995-1002. [DOI: 10.1002/acr.22800] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/29/2015] [Revised: 11/04/2015] [Accepted: 11/17/2015] [Indexed: 12/22/2022]
Affiliation(s)
- Doo-Ho Lim
- University of Ulsan College of Medicine; Seoul South Korea
| | - Yong-Gil Kim
- University of Ulsan College of Medicine; Seoul South Korea
| | - Danbi Lee
- University of Ulsan College of Medicine; Seoul South Korea
| | - Soo Min Ahn
- University of Ulsan College of Medicine; Seoul South Korea
| | - Seokchan Hong
- University of Ulsan College of Medicine; Seoul South Korea
| | - Chang-Keun Lee
- University of Ulsan College of Medicine; Seoul South Korea
| | - Bin Yoo
- University of Ulsan College of Medicine; Seoul South Korea
| |
Collapse
|
|
11
|
Autoimmune Hepatitis as an Initial Presentation of SLE. ARCHIVES OF PEDIATRIC INFECTIOUS DISEASES 2016. [DOI: 10.5812/pedinfect.34653] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/16/2022]
|
|
12
|
Muratori P, Fabbri A, Lalanne C, Lenzi M, Muratori L. Autoimmune liver disease and concomitant extrahepatic autoimmune disease. Eur J Gastroenterol Hepatol 2015; 27:1175-1179. [PMID: 26148248 DOI: 10.1097/meg.0000000000000424] [Citation(s) in RCA: 49] [Impact Index Per Article: 4.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/18/2022]
Abstract
AIM To assess the frequency and clinical impact of associated extrahepatic autoimmune diseases (EAD) on autoimmune liver diseases (ALD). PATIENTS AND METHODS We investigated 608 patients with ALD (327 autoimmune hepatitis - AIH and 281 primary biliary cirrhosis - PBC) for concomitant EAD. RESULTS In both AIH and PBC, we observed a high prevalence of EAD (29.9 and 42.3%, respectively); both diseases showed a significant association with autoimmune thyroid disease, followed by autoimmune skin disease, celiac disease, and vasculitis in AIH patients and sicca syndrome, CREST syndrome, and celiac disease in PBC patients. At diagnosis, AIH patients with concurrent EAD were more often asymptomatic than patients with isolated AIH (P<0.01). CONCLUSION Our study confirms the strict association between ALD and EAD, in particular with autoimmune thyroid disease. In the light of our results, all patients with an EAD should be assessed for the concomitant presence of an asymptomatic ALD.
Collapse
Affiliation(s)
- Paolo Muratori
- Department of Medical and Surgical Sciences, Center for the Study and Treatment of Autoimmune Diseases of the Liver and Biliary System, Policlinico di Sant'Orsola, Alma Mater Studiorum, University of Bologna, Bologna, Italy
| | | | | | | | | |
Collapse
|
|
13
|
Iwadate H, Kobayashi H, Shio K, Noguchi E, Watanabe K, Sasajima T, Sekine H, Watanabe H, Ohira H, Obara K, Sato Y. A case of systemic lupus erythematosus complicated by pure red cell aplasia and idiopathic portal hypertension after thymectomy. Mod Rheumatol 2014. [DOI: 10.3109/s10165-006-0465-7] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/21/2023]
|
|
14
|
Cichoż-Lach H, Celiński K. Lesions of alimentary tract in the course of systemic lupus erythematosus. Rheumatology (Oxford) 2013; 51:36-41. [DOI: 10.5114/reum.2013.33392] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 09/13/2023] Open
Abstract
Toczeń rumieniowaty układowy jest przewlekłą, zapalną chorobą tkanki łącznej o podłożu autoimmunologicznym, w przebiegu której mogą być zajęte różne narządy i układy. Objawy ze strony przewodu pokarmowego występują u 25–40% pacjentów z aktywną chorobą. Są one zwykle niespecyficzne i wynikają z istniejącej choroby bądź działań niepożądanych zastosowanej terapii. Zmiany w przewodzie pokarmowym mogą obejmować każdy z jego odcinków, a także dotyczyć wątroby i trzustki. W artykule przedstawiono najczęstsze i najcięższe objawy gastroenterologiczne w przebiegu tocznia rumieniowatego układowego, wśród nich toczniowe zapalenie naczyń krezki, enteropatię białkową, rozedmę pęcherzykową jelita, uszkodzenie wątroby i ostre zapalenie trzustki.
Collapse
|
|
15
|
Luijten RK, Fritsch-Stork RD, Bijlsma JW, Derksen RH. The use of glucocorticoids in Systemic Lupus Erythematosus. After 60years still more an art than science. Autoimmun Rev 2013; 12:617-28. [DOI: 10.1016/j.autrev.2012.12.001] [Citation(s) in RCA: 48] [Impact Index Per Article: 4.0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/15/2012] [Accepted: 12/02/2012] [Indexed: 01/18/2023]
|
|
16
|
Önder FO, Yürekli ÖT, Öztaş E, Kalkan İH, Köksal AŞ, Akdoğan M, Şaşmaz N, Kayaçetin E. Features of systemic lupus erythematosus in patients with autoimmune hepatitis. Rheumatol Int 2012; 33:1581-5. [DOI: 10.1007/s00296-012-2607-x] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/02/2012] [Accepted: 12/08/2012] [Indexed: 10/27/2022]
|
|
17
|
Schiavon LL, Carvalho-Filho RJ, Narciso-Schiavon JL, Lanzoni VP, Ferraz MLG, Silva AEB. Late-onset systemic lupus erythematosus-associated liver disease. Rheumatol Int 2012; 32:2917-2920. [PMID: 20376663 DOI: 10.1007/s00296-010-1492-4] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/28/2010] [Accepted: 03/27/2010] [Indexed: 01/12/2023]
Abstract
Systemic lupus erythematosus (SLE) is a multisystemic autoimmune disease, which predominantly affects women under 50 years old. Although liver disease is not included in the diagnostic criteria, abnormal liver tests are common among patients with SLE and, in a significant proportion of those patients, no other underlying condition can be identified. We described a case of liver involvement in late-onset SLE presenting with a predominantly cholestatic pattern. Other conditions associated with abnormal liver tests were excluded, and the patient showed a prompt response to steroid therapy. The spectrum of the liver involvement in SLE is discussed, with emphasis on the differential diagnosis with autoimmune hepatitis.
Collapse
Affiliation(s)
- Leonardo L Schiavon
- Division of Gastroenterology, Hepatitis Section, Federal University of Sao Paulo, Sao Paulo, Brazil.
| | | | | | | | | | | |
Collapse
|
|
18
|
A patient of lupus presenting with myocarditis and overlapping autoimmune hepatitis. Case Rep Rheumatol 2011; 2011:402483. [PMID: 22937445 PMCID: PMC3420763 DOI: 10.1155/2011/402483] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/08/2011] [Accepted: 09/27/2011] [Indexed: 12/17/2022] Open
Abstract
Systemic lupus erythematosus has myriad presentations. Symptomatic myocarditis and/or symptomatic autoimmune hepatitis associated with lupus are rare at presentation. Here we report a young Asian girl, who presented to us with features of symptomatic myocarditis and overlapping autoimmune hepatitis. She was eventually diagnosed to be harboring systemic lupus erythematosus, in whom rigorous management led to gratuitous recovery.
Collapse
|
|
19
|
Ohira H, Abe K, Takahashi A. Involvement of the liver in rheumatic diseases. Clin J Gastroenterol 2011; 5:9-14. [DOI: 10.1007/s12328-011-0271-4] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/10/2011] [Accepted: 11/10/2011] [Indexed: 01/15/2023]
|
|
20
|
Geri G, Saadoun D, Cacoub P. Manifestations hépatiques des maladies systémiques. Rev Med Interne 2011; 32:486-93. [DOI: 10.1016/j.revmed.2010.07.008] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/27/2009] [Revised: 04/24/2010] [Accepted: 07/19/2010] [Indexed: 12/20/2022]
|
|
21
|
Khalifa M, Benjazia E, Rezgui A, Ghannouchi N, Alaoua A, Braham A, Létaief A, Bahri F. [Lupus hepatitis: a case series of 12 patients]. Rev Med Interne 2010; 32:347-9. [PMID: 21129825 DOI: 10.1016/j.revmed.2010.10.357] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/28/2010] [Revised: 09/25/2010] [Accepted: 10/25/2010] [Indexed: 01/12/2023]
Abstract
PURPOSE Several liver manifestations have been reported in systemic lupus erythematosus (SLE) and are usually non specific. We report on our experience of lupus hepatitis. METHODS A retrospective monocenter study of 73 patients with SLE. The diagnosis of lupus hepatitis was established after exclusion of other causes of hepatitis and hepatic vein thrombosis. RESULTS Liver involvement was noted in 12 patients (16.4%). There were nine female and three male patients; the mean age of these patients was 29 years. In seven patients liver involvement was concurrent with the diagnosis of SLE and it occurred later during an exacerbation of the disease in the five remaining patients. In all patients, liver manifestations were associated with other organ involvement. Clinical manifestations were: hepatomegaly (n=4), jaundice (n=4), abdominal pain (n=3), ascites (n=2), portal hypertension (n=1) and hepatic failure with encephalopathy (n=1). Elevated liver enzyme was noted in 11 cases and liver cholestasis in eight cases. Presence of anti-ribosomal P antibodies was noted in one case. Liver biopsy was performed in five patients, and revealed chronic active hepatitis in three cases, chronic hepatic granulomas in one case and nonspecific inflammation in one case. The outcome was favorable in 11 patients without relapse, and one patient died of encephalopathy and liver failure. CONCLUSION Liver involvement associated with SLE is not uncommon. It is frequently asymptomatic and limited to liver test abnormalities. The role of anti-ribosomal P autoantibodies remains uncertain.
Collapse
Affiliation(s)
- M Khalifa
- Service de médecine interne, CHU Farhat Hached, Sousse, Tunisia.
| | | | | | | | | | | | | | | |
Collapse
|
|
22
|
Efe C, Ozaslan E, Nasiroglu N, Tunca H, Purnak T, Altiparmak E. The development of autoimmune hepatitis and primary biliary cirrhosis overlap syndrome during the course of connective tissue diseases: report of three cases and review of the literature. Dig Dis Sci 2010; 55:2417-2421. [PMID: 19826950 DOI: 10.1007/s10620-009-0996-9] [Citation(s) in RCA: 26] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/02/2009] [Accepted: 09/15/2009] [Indexed: 12/15/2022]
MESH Headings
- Adult
- Angiotensin-Converting Enzyme Inhibitors
- Cholagogues and Choleretics/therapeutic use
- Cholangitis, Sclerosing/complications
- Cholangitis, Sclerosing/drug therapy
- Female
- Hepatitis, Autoimmune/complications
- Hepatitis, Autoimmune/drug therapy
- Humans
- Immunosuppressive Agents/therapeutic use
- Liver Cirrhosis, Biliary/complications
- Liver Cirrhosis, Biliary/drug therapy
- Lupus Erythematosus, Systemic/complications
- Lupus Erythematosus, Systemic/drug therapy
- Middle Aged
- Scleroderma, Systemic/complications
- Scleroderma, Systemic/drug therapy
- Sjogren's Syndrome/complications
- Sjogren's Syndrome/drug therapy
- Ursodeoxycholic Acid/therapeutic use
Collapse
Affiliation(s)
- Cumali Efe
- Department of Gastroenterology, Numune Education and Training Hospital, Ankara, Turkey.
| | | | | | | | | | | |
Collapse
|
|
23
|
Abstract
BACKGROUND Although the pathomechanisms of autoimmune diseases in various organs remain unresolved, an accumulation of autoimmune diseases in individual patients has been observed. An overlap of autoimmune hepatitis (AIH) and primary biliary cirrhosis (PBC) or primary sclerosing cirrhosis has been well documented. However, the overlap of autoimmune diseases other than PBC or PSC has not yet been investigated in a large cohort. GOAL The goal of our analysis was to investigate the incidence of concurrent autoimmune diseases in patients with AIH. STUDY We analyzed our cohort of 278 patients with AIH for concurrent autoimmune diseases. RESULTS A total of 111 patients (40%) were diagnosed with additional autoimmune diseases. Besides overlap syndromes for PBC and PSC, autoimmune thyroiditis was the most common concurrent disease (28 patients, 10%). Other concurrent autoimmune diseases comprised vitiligo (5 patients), rheumatoid arthritis (5 patients), Sjogren syndrome (4 patients), ulcerative colitis (4 patients), conjunctivitis (4 patients), celiac disease (3 patients), systemic lupus erythematodes (2 patients), type I diabetes (2 patients), multiple sclerosis (2 patients), polymyalgia rheumatica (2 patients), and urticaria (2 patients). One patient each was diagnosed with Crohn's disease, autoimmune gastritis, collagenous colitis, hypophysitis, and sarcoidosis. Investigating 100 patients with polyglandular syndrome and autoimmune thyroid disease for the occurrence of autoantibodies associated with AIH, we identified AIH-associated antibodies only in 1 patient. CONCLUSIONS Concurrent autoimmune diseases are common in patients with AIH and mirror the full range of known autoimmune diseases. Therefore, an extended diagnostic screening for accumulating autoimmune diseases, especially autoimmune thyroiditis, seems reasonable in patients with AIH.
Collapse
|
|
24
|
Sonomoto K, Miyamura T, Watanabe H, Takahama S, Nakamura M, Ando H, Minami R, Yamamoto M, Suematsu E. [A case of systemic lupus erythematosus complicated with autoimmune hepatitis and thrombotic thrombocytic purpura]. ACTA ACUST UNITED AC 2009; 32:110-5. [PMID: 19404009 DOI: 10.2177/jsci.32.110] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/15/2022]
Abstract
A 51-year-old woman was admitted to our hospital because of systemic jaundice, general fatigue in August 24, 2007. She was diagnosed with systemic lupus erythematosus (SLE) as a result of a discoid rash, photosensitivity, lymphocytopenia, elevated serum anti ds-DNA antibody and a positive test for antinuclear antibody. Her laboratory data revealed severe liver dysfunction, suggesting autoimmune hepatitis (AIH). She was also diagnosed with thrombotic thrombocytic purpura (TTP) because of thrombocytopenia, hemolytic anemia, renal dysfunction and decreased ADAM-TS13 activity. The patient was treated by methylprednisolone pulse therapy, fresh frozen plasma infusion and ursodeoxycholic acid. Her symptoms and laboratory data rapidly improved and a liver biopsy was carried out. Interface hepatitis and lymphocyte infiltration were observed in the specimen. A diagnosis of definite AIH was made from her International AIH group score of 20 points. AIH and TTP are rare complications of SLE. The prevalence of the complication of SLE and AIH has been reported as 1.7 approximately 2.7%, and that of SLE and TTP as 1 approximately 4%. We reported here a rare case of SLE complicated with AIH and TTP.
Collapse
Affiliation(s)
- Koshiro Sonomoto
- Departments of Internal Medicine and Rheumatology, Clinical Research Center, National Hospital Organization, Kyushu Medical Center
| | | | | | | | | | | | | | | | | |
Collapse
|
|
25
|
Conventional oral anticoagulation may not replace prior transesophageal echocardiography for the patients with planned catheter ablation for atrial fibrillation. J Interv Card Electrophysiol 2008; 24:19-26. [PMID: 18982437 DOI: 10.1007/s10840-008-9322-2] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/17/2008] [Accepted: 09/15/2008] [Indexed: 01/31/2023]
Abstract
INSTRUCTION Preablation transesophageal echocardiography (TEE) is dispensable for the patients with planned catheter ablation for atrial fibrillation (AF) and having received at least a 3-week oral anticoagulation therapy according to the recommendations of the Venice Consensus. But the role of prior TEE and the effect of preablation short-term oral anticoagulation drugs (OACs) under the circumstance are still unclear. METHODS AND RESULTS A total of 188 patients with planned catheter ablation for AF and without previous long-term oral anticoagulation, whose duration of AF exceeded 48 h, were randomly divided into receiving 3-week OACs (OACs group) before heparin bridging or receiving no prior OACs (N-OACs group). Follow-up was performed until a TEE had been performed on all the cases before ablation. Consequently, the prevalence of atrial thrombi is 6.3% and 11.7%, respectively (P < 0.05), and the prevalence of minor bleeding is 5.3% and 0%, respectively (P < 0.05), in OACs and N-OACs group. There was no thrombotic event, major hemorrhage, in both groups. CONCLUSION After a 3-week effective oral anticoagulation, atrial thrombi could be resolved partly but not completely in the patients with AF who had not received long-term oral anticoagulation previously. To ensure safety, prior TEE may be necessary for the patients with planned catheter ablation for AF.
Collapse
|
|
26
|
Abstract
Potential causes of abnormal liver function tests include viral hepatitis, alcohol intake, nonalcoholic fatty liver disease, autoimmune liver diseases, hereditary diseases, hepatobiliary malignancies or infection, gallstones and drug-induced liver injury. Moreover, the liver may be involved in systemic diseases that mainly affect other organs. Therefore, in patients without etiology of liver injury by screening serology and diagnostic imaging, but who have systemic diseases, the abnormal liver function test results might be caused by the systemic disease. In most of these patients, the systemic disease should be treated primarily. However, some patients with systemic disease and severe liver injury or fulminant hepatic failure require intensive treatments of the liver.
Collapse
|
|
27
|
Abstract
AIM: To describe the clinical features of type Ⅰ autoimmune hepatitis.
METHODS: One hundred and two cases of autoimmune hepatitis admitted to Tianjin Medical University Hospital from January 2000 to May 2007 were retrospectively analyzed.
RESULTS: There were 85 females and 17 males, with a mean age of 59.73 ± 13.46 years. Clinical manifestations of chronic liver disease were found in 41.18% patients, acute hepatitis manifestations in 18.63% patients, and no symptom in 5.89% patients. Laboratory tests showed that the percentage of abnormal liver function, gamma globulin or immunoglobulin G was 84.33% and 80%, respectively. Negative viral serology was found in 85.29% patients, hyperglycemia occurred in 50.67% patients. The positive rate of antinuclear antibody (ANA), antismooth muscle antibody (SMA) and antimitochondrial antibody (AMA) was 74.51%, 4.90% and 7.84%, respectively. Abdominal ultrasound was abnormal in 97.06% patients. Ultrasonography revealed liver texture asymmetry or decreased density, hepatocirrhosis, hepatoma, bilestone, enlarged abdominal lymph nodes, ascites, etc. Of the 31 patients who received treatment, 18 had improvement in their symptoms, 7 incomplete response, 5 relapse and 7 treatment failure
CONCLUSION: Autoimmune hepatitis has different manifestations. However, it has certain unique features in immunology, imageology, liver histology, which can be useful for its clinical diagnosis and treatment.
Collapse
|
|
28
|
Takahashi A, Rai T, Onizawa M, Monoe K, Kanno Y, Saito H, Abe K, Yokokawa J, Irisawa A, Ohira H. Autoimmune hepatitis complicated by late-onset systemic lupus erythematosus. Hepatol Res 2007; 37:771-4. [PMID: 17506835 DOI: 10.1111/j.1872-034x.2007.00091.x] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/29/2022]
Abstract
A 69-year-old man with autoimmune hepatitis (AIH) was admitted to hospital with high fever and cough. Chest roentgenogram and computed tomography showed pleural and pericardial effusion. Serological tests showed a high titer of antinuclear antibodies and positive anti-DNA antibody and lymphocytopenia. He fulfilled the American College of Rheumatology criteria for systemic lupus erythematosus (SLE). After administration of corticosteroids, his symptoms and liver dysfunction improved. To the authors' knowledge, this is the first male case of overlap between AIH and late-onset SLE.
Collapse
Affiliation(s)
- Atsushi Takahashi
- Second Department of Internal Medicine, School of Medicine, Fukushima Medical University, Fukushima, Japan
| | | | | | | | | | | | | | | | | | | |
Collapse
|
|
29
|
Usta Y, Gurakan F, Akcoren Z, Ozen S. An overlap syndrome involving autoimmune hepatitis and systemic lupus erythematosus in childhood. World J Gastroenterol 2007; 13:2764-7. [PMID: 17569152 PMCID: PMC4147132 DOI: 10.3748/wjg.v13.i19.2764] [Citation(s) in RCA: 24] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/06/2023] Open
Abstract
We report a 12 years old female patient with an overlap syndrome involving autoimmune hepatitis (AIH) and systemic lupus erythematosus (SLE). The patient presented with jaundice, hepatosplenomegaly, malaise, polyarthralgia, arthritis and butterfly rash on the face. Laboratory tests revealed severe liver dysfunction, Coombs positive hemolytic anemia and a positive ANA/anti-dsDNA test. Renal biopsy showed class IIA kidney disease, while liver biopsy showed chronic hepatitis with severe inflammatory activity. The patient satisfied the international criteria for both SLE and AIH. Clinical symptoms and laboratory findings of SLE improved with high dose treatment with corticosteroids and azathioprine, however, remission of the liver disease could not be achieved. Repeat biopsy of the liver after three years of therapy revealed ongoing chronic hepatitis with high level of inflammatory activity. The present case indicates that children with liver dysfunction and SLE should be investigated for AIH. There is much diagnostic and therapeutic dilemma in patients with AIH-SLE overlap syndrome.
Collapse
Affiliation(s)
- Yusuf Usta
- Hacettepe University, Faculty of Medicine, Department of Pediatrics, Section of Gastroenterology, Hepatology and Nutrition, Ankara 06100, Turkey.
| | | | | | | |
Collapse
|
|
30
|
Branger S, Schleinitz N, Veit V, Martaresche C, Bourlière M, Roblin X, Garcia S, San Marco M, Camoin L, Durand JM, Harlé JR. Étude de l'association hépatite auto-immune et antiphospholipides. Rev Med Interne 2007; 28:218-24. [PMID: 17331625 DOI: 10.1016/j.revmed.2006.12.005] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/30/2006] [Revised: 12/01/2006] [Accepted: 12/11/2006] [Indexed: 02/06/2023]
Abstract
INTRODUCTION Only few series have reported the association of autoimmune hepatitis with antiphospholipid antibodies. The aim of our study is to investigate the frequency of these antibodies in a series of autoimmune hepatitis and to search for a correlation with clinical, biological or histological characteristics. MATERIAL AND METHODS Antiphospholipid were investigated in 24 patients with well defined autoimmune hepatitis. Characteristics were compared between antiphopholipids positive and negative patients. Characteristics of our patients were also compared toward cases collected in a literature review. RESULTS The frequency of antiphospholipid antibodies is of 70.8% in our series. Four patients had a well defined antiphospholid syndrome. Seven patients had a systemic lupus erythematosus in the antiphospholipid group whereas none in the antiphospholipid negative group. The frequency of the different antiphopholipid antibodies was: IgG ACL (52.9%), IgM APE (52.9%), ACC (43.7%), IgG Abeta2GP1 (41.2%). We found no correlation between hypergammaglobulinemia and the presence or the isotype of antiphospholipid antibodies. Clinical presentation and outcome as biological and histological parameters were similar in both groups. CONCLUSION Our study report a high frequency of antiphospholipids antibodies in autoimmune hepatitis patients. However we found no clinical, biological or histological correlation with the presence of antiphospholipids. Further longitudinal studies on larger cohorts should clarify the association between antiphospholipid antibodies and autoimmune hepatitis and potential therapeutic issues.
Collapse
Affiliation(s)
- S Branger
- Service de médecine interne du Professeur-Harlé, CHU de La-Conception, 147, boulevard Baille, 13005 Marseille, France.
| | | | | | | | | | | | | | | | | | | | | |
Collapse
|
|
31
|
Nakayama S, Mukae H, Morisaki T, Sakamoto N, Ohba K, Abe K, Takeshima F, Mizuta Y, Ida H, Ozono Y, Kohno S. Sarcoidosis accompanied by systemic lupus erythematosus and autoimmune hepatitis. Intern Med 2007; 46:1657-61. [PMID: 17917329 DOI: 10.2169/internalmedicine.46.0201] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/06/2022] Open
Abstract
A 52-year-old woman was admitted to our hospital for further examination of blurred vision, abnormal lung shadows and an elevated level of angiotensin-converting enzyme. Sarcoidosis was suspected, however, careful history taking revealed the existence of photosensitivity and polyarthralgia. Laboratory tests showed lymphocytopenia, liver dysfunction, hypergammaglobulinemia, and positive anti-nuclear, anti-double stranded DNA and anti-smooth muscle antibodies. Liver biopsy examination showed chronic active hepatitis. She was diagnosed with the triplex of sarcoidosis, systemic lupus erythematosus and autoimmune hepatitis. Marked improvement was noted after corticosteroid therapy.
Collapse
Affiliation(s)
- Seiko Nakayama
- Second Department of Internal Medicine, Nagasaki University School of Medicine, Nagasaki
| | | | | | | | | | | | | | | | | | | | | |
Collapse
|
|
32
|
West M, Jasin HE, Medhekar S. The development of connective tissue diseases in patients with autoimmune hepatitis: a case series. Semin Arthritis Rheum 2006; 35:344-8. [PMID: 16765710 DOI: 10.1016/j.semarthrit.2005.12.001] [Citation(s) in RCA: 18] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/24/2022]
Abstract
OBJECTIVES To study the prevalence of connective tissue diseases (CTD) in patients with autoimmune hepatitis (AIH). METHODS We identified 11 cases of AIH over the past 7 years at our institution, through a systematic chart review of patients with this diagnosis. Their charts were reviewed for the development of systemic CTD. RESULTS Three of the 11 patients with a definitive diagnosis of AIH developed systemic CTD. All were white: 2 women and 1 man, with an age range of 33 to 62 years, and with disease duration of 1 to 7 years. One patient developed systemic lupus erythematosus (SLE) with vasculitis and peripheral neuropathy. The second developed limited scleroderma and the third developed undifferentiated connective tissue disease (UCTD) and interstitial lung disease. There appear to be shared susceptibility alleles for AIH and CTD in addition to the shared positive autoantibodies. CONCLUSIONS Patients with AIH may be at increased risk for developing systemic CTD. Conversely, a review of the literature reveals that patients with systemic CTD may be at increased risk of developing AIH. Patients with either AIH or CTD should be monitored for further development of concurrent autoimmune diseases.
Collapse
Affiliation(s)
- Margaret West
- Division of Rheumatology and Clinical Immunology, Department of Internal Medicine, University of Arkansas for Medical Sciences and Central Arkansas Veterans Hospital System, Little Rock, AR 72205, USA
| | | | | |
Collapse
|
|
33
|
Iwadate H, Kobayashi H, Shio K, Noguchi E, Watanabe K, Sasajima T, Sekine H, Watanabe H, Ohira H, Obara K, Sato Y. A case of systemic lupus erythematosus complicated by pure red cell aplasia and idiopathic portal hypertension after thymectomy. Mod Rheumatol 2006; 16:109-12. [PMID: 16633932 DOI: 10.1007/s10165-006-0465-7] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/17/2005] [Accepted: 02/21/2006] [Indexed: 10/24/2022]
Abstract
We describe a 49-year-old woman who presented in 2002 with pure red cell aplasia (PRCA), systemic lupus erythematosus (SLE), and idiopathic portal hypertension (IPH) that developed following a thymectomy. She underwent a thymectomy at 40 years of age to treat myasthenia gravis. PRCA developed 3 years after the thymectomy and she was successfully treated with cyclosporin. Systemic lupus erythematosus and IPH were diagnosed 6 years later. We conclude that immunological dysfunction resulting from the thymectomy contributed significantly to the subsequent development of PRCA, SLE, and IPH in this patient. This is the first report to describe this extremely rare occurrence.
Collapse
Affiliation(s)
- Haruyo Iwadate
- Department of Internal Medicine II, Fukushima Medical University School of Medicine, 1 Hikarigaoka, Fukushima, 960-1295, Japan.
| | | | | | | | | | | | | | | | | | | | | |
Collapse
|
|
34
|
Luth S, Birklein F, Schramm C, Herkel J, Hennes E, Muller-Forell W, Galle PR, Lohse AW. Multiplex neuritis in a patient with autoimmune hepatitis: A case report. World J Gastroenterol 2006; 12:5396-8. [PMID: 16981276 PMCID: PMC4088213 DOI: 10.3748/wjg.v12.i33.5396] [Citation(s) in RCA: 11] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/06/2023] Open
Abstract
A 37-year old woman presented with a 9-year history of hepatitis of unknown origin and aminotransferases within a 3-fold upper limit of normal. Autoimmune hepatitis (AIH) was diagnosed on the basis of elevated aminotransferases, soluble liver antigen/liver pancreas (SLA/LP) autoantibodies and characteristic histology. Immunosuppressive therapy led to rapid normalization of aminotransferases. Two years later, the patient developed left sided hemisensory deficits under maintenance therapy of prednisolone and azathioprine (AZT). Later she developed right foot drop and paraesthesia in the ulnar innervation territory on both sides. Magnetic resonance imaging (MRI) and cerebral panangiography suggested cerebral vasculitis. Neurological investigation and electromyography disclosed multiplex neuritis (MN) probably due to vasculitis. Consistent with this diagnosis, autoantibodies to extractable nuclear antigens were detectable in serum. Immunosuppression was changed to oral 150 mg cyclophosphamide (CPM0) per day. Prednisolone was increased to 40 mg/d and then gradually tapered to 5 mg. Oral CPM was administered up to a total dose of 40 g and then substituted by 6 times of an intervall infusion therapy of CPM (600 mg/m2). Almost complete motoric remission was achieved after 3 mo of CPM. Sensibility remained reduced in the right peroneal innervation territory. Follow-up of cranial MRI provided stable findings without any new or progressive lesions. This is the first report of multiplex neuritis in a patient with autoimmune hepatitis.
Collapse
Affiliation(s)
- S Luth
- Department of Medicine I, University Medical Centre Hamburg-Eppendorf, Martinistr. 52, Hamburg 20246, Germany
| | | | | | | | | | | | | | | |
Collapse
|
|
35
|
Cazanave C, Rakotondravelo S, Morlat P, Blanco P, Bonnet F, Beylot J. [Autoimmune hepatitis in a HIV-HCV co-infected patient: diagnostic ant therapeutic difficulties]. Rev Med Interne 2006; 27:414-9. [PMID: 16545501 DOI: 10.1016/j.revmed.2006.01.016] [Citation(s) in RCA: 16] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/20/2005] [Accepted: 01/23/2006] [Indexed: 12/12/2022]
Abstract
INTRODUCTION Autoimmune hepatitis (AIH) is a chronic inflammatory hepatic disorder, characterized by hypergammaglobulinemia and autoantibodies. In some cases, AIH can be associated with another liver disease; such as the hepatitis C-AIH overlap syndrome, which diagnosis and treatment may be delicate. EXEGESE We report a type 1 AIH case in a HIV-HCV co-infected woman. AIH remission and HCV eradication were obtained with prednisone and interferon plus ribavirine. AIH relapse appeared with corticosteroid withdrawal and a new remission was obtained with immunosuppressive treatment associating prednisone and azathioprine, without opportunistic infection. CONCLUSION This case illustrates diagnostic and therapeutic difficulties of hepatitis C-AIH overlap syndromes in an HIV-infected patient. To our knowledge, it is the first AIH case report in a HIV-HCV co-infected patient.
Collapse
Affiliation(s)
- C Cazanave
- Service de Médecine Interne et Maladies Infectieuses, Hôpital Saint-André, 1, rue Jean-Burguet, CHU de Bordeaux, 33075 Bordeaux cedex, France.
| | | | | | | | | | | |
Collapse
|
|
36
|
Abstract
There are multiple relations between rheumatic diseases and the liver, nevertheless the liver is extremely rare involved in rheumatic diseases. "Elevated liver enzymes" are quite often found in patients who are under the medication with immunosuppressive drugs or/and non-steroidal antirheumatics. The most frequent cause for "elevated liver enzymes" are toxic and allergic side effects of drugs; however, in rare cases it might be extremely helpful to examine, whether an independent liver disease exists. Underlying liver diseases which might be associated with the rheumatic disorder or exist accidentally may change the therapeutic management of the patient. If the liver disease present can cause the rheumatic disorder (e. g. virus-induced vasculitis, hemochromatosis), a specific hepatological therapy should precede the immunosuppression.
Collapse
Affiliation(s)
- C Wiegard
- I. Medizinische Klinik, Universitätsklinikum Hamburg-Eppendorf, Martinistr. 52, 20246 Hamburg, Germany
| | | |
Collapse
|