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Nicolosi S, Chernovsky M, Angoni D, Hughes M, Bandini G, McMahan Z, Maggisano M, Salton F, Mondini L, Barbieri M, Screm G, Confalonieri M, Baratella E, Confalonieri P, Ruaro B. Gastrointestinal Manifestations of Sarcoidosis: A State-of-the-Art, Comprehensive Review of the Literature-Practical Clinical Insights and Many Unmet Needs on Diagnosis and Treatment. Pharmaceuticals (Basel) 2024; 17:1106. [PMID: 39338270 PMCID: PMC11435091 DOI: 10.3390/ph17091106] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/30/2024] [Revised: 08/17/2024] [Accepted: 08/20/2024] [Indexed: 09/30/2024] Open
Abstract
This comprehensive literature review explores the involvement of the gastrointestinal (GI) tract in sarcoidosis, a multisystem granulomatous disorder of unknown etiology. GI sarcoidosis presents a diagnostic and therapeutic challenge due to its rarity and nonspecific clinical manifestations, including overlap with other gastrointestinal diseases. We conducted a comprehensive screening of articles addressing the clinical features, diagnostic approaches, and treatment strategies for GI sarcoidosis. Our findings reveal that GI sarcoidosis can affect any part of the gastrointestinal tract, with the stomach and small intestine being the most involved. Clinical presentations range from asymptomatic cases to severe complications such as obstruction and perforation, with reflux being a common symptom. Diagnosis is often delayed due to the nonspecific nature of symptoms and the need for histopathological confirmation. Therapeutic approaches are poorly defined, typically involving corticosteroids as the mainstay of treatment. However, the long-term efficacy and safety of these treatments remain uncertain in this patient group, given the significant risks and complications associated with prolonged glucocorticoid therapy. There is a clear need to develop accurate diagnostic protocols to distinguish GI sarcoidosis from other conditions and to establish standardized therapeutic guidelines to optimize patient outcomes. Further research is essential to enhance our understanding and management of this complex condition.
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Affiliation(s)
- Salvatore Nicolosi
- Pulmonology Unit, Department of Medical Surgical and Health Sciences, University of Trieste, 34149 Trieste, Italy (D.A.); (M.M.)
| | - Maria Chernovsky
- Pulmonology Unit, Department of Medical Surgical and Health Sciences, University of Trieste, 34149 Trieste, Italy (D.A.); (M.M.)
| | - Darina Angoni
- Pulmonology Unit, Department of Medical Surgical and Health Sciences, University of Trieste, 34149 Trieste, Italy (D.A.); (M.M.)
| | - Michael Hughes
- Division of Musculoskeletal and Dermatological Sciences, Faculty of Biology, Medicine and Health, The University of Manchester & Salford Royal NHS Foundation Trust, Manchester M6 8HD, UK
| | - Giulia Bandini
- Department of Experimental and Clinical Medicine, Division of Internal Medicine, Azienda Ospedaliero Universitaria Careggi, University of Florence, 50134 Florence, Italy;
| | - Zsuzsanna McMahan
- McGovern Medical School, The University of Texas Health Science Center at Houston, Houston, TX 77030, USA
| | - Marta Maggisano
- Pulmonology Unit, Department of Medical Surgical and Health Sciences, University of Trieste, 34149 Trieste, Italy (D.A.); (M.M.)
| | - Francesco Salton
- Pulmonology Unit, Department of Medical Surgical and Health Sciences, University of Trieste, 34149 Trieste, Italy (D.A.); (M.M.)
| | - Lucrezia Mondini
- Pulmonology Unit, Department of Medical Surgical and Health Sciences, University of Trieste, 34149 Trieste, Italy (D.A.); (M.M.)
| | - Mariangela Barbieri
- Pulmonology Unit, Department of Medical Surgical and Health Sciences, University of Trieste, 34149 Trieste, Italy (D.A.); (M.M.)
| | - Gianluca Screm
- Pulmonology Unit, Department of Medical Surgical and Health Sciences, University of Trieste, 34149 Trieste, Italy (D.A.); (M.M.)
| | - Marco Confalonieri
- Pulmonology Unit, Department of Medical Surgical and Health Sciences, University of Trieste, 34149 Trieste, Italy (D.A.); (M.M.)
| | - Elisa Baratella
- Radiology Unit, Department of Medical Surgical and Health Sciences, University Hospital of Cattinara, 34149 Trieste, Italy
| | - Paola Confalonieri
- Pulmonology Unit, Department of Medical Surgical and Health Sciences, University of Trieste, 34149 Trieste, Italy (D.A.); (M.M.)
| | - Barbara Ruaro
- Pulmonology Unit, Department of Medical Surgical and Health Sciences, University of Trieste, 34149 Trieste, Italy (D.A.); (M.M.)
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Gezer NS, Başara I, Altay C, Harman M, Rocher L, Karabulut N, Seçil M. Abdominal sarcoidosis: cross-sectional imaging findings. Diagn Interv Radiol 2016; 21:111-7. [PMID: 25512071 DOI: 10.5152/dir.2014.14210] [Citation(s) in RCA: 44] [Impact Index Per Article: 4.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
Abstract
Sarcoidosis is a multisystem inflammatory disease of unknown etiology. The lungs and the lymphoid system are the most commonly involved organs. Extrapulmonary involvement is reported in 30% of patients, and the abdomen is the most common extrapulmonary site with a frequency of 50%-70%. Although intra-abdominal sarcoidosis is usually asymptomatic, its presence may affect the prognosis and treatment options. The lesions are less characteristic and may mimick neoplastic or infectious diseases such as lymphoma, diffuse metastasis, and granulomatous inflammation. The liver and spleen are the most common abdominal sites of involvement. Sarcoidosis of the gastrointestinal system, pancreas, and kidneys are extremely rare. Adenopathy which is most commonly found in the porta hepatis, exudative ascites, and multiple granulomatous nodules studding the peritoneum are the reported manifestations of abdominal sarcoidosis. Since abdominal sarcoidosis is less common and long-standing, unrecognized disease can result in significant morbidity and mortality. Imaging contributes to diagnosis and management of intra-abdominal sarcoidosis. In this report we reviewed the cross-sectional imaging findings of hepatobiliary, gastrointestinal, and genitourinary sarcoidosis.
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Affiliation(s)
- Naciye Sinem Gezer
- Department of Radiology, School of Medicine, Dokuz Eylul University, Izmir, Turkey.
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Abstract
Systemic sarcoidosis that initially presents as a pancreatic mass has rarely been reported. A 47-year-old man presented with idiopathic epigastric abdominal pain. Abdominal CT showed multiple enlarged lymph nodes in the retroperitoneal area and a suspected tumor mass in the pancreatic head region. The preliminary diagnosis was celiac metastasis of a pancreatic malignancy. The definitive diagnosis was systemic sarcoidosis, which was supported by granulomas on histology, clinical radiographic findings, and clinical response to steroids. A review of published reports on sarcoidosis presenting initially as a pancreatic or celiac mass from the Chinese biomedical database (1978-2010) is included.
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Affiliation(s)
| | | | - Jian-Xin Wang
- Prof. Jian-Xin Wang, The Respiratory Diseases Department,, Chinese PLA General Hospital,, 28 Fuxing Rd. 100853, Beijing, China,
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Mayne AIW, Ahmad J, Loughrey M, Taylor MA. Sarcoidosis of the pancreas mimicking adenocarcinoma. BMJ Case Rep 2013; 2013:bcr-2013-009118. [PMID: 23784760 DOI: 10.1136/bcr-2013-009118] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/04/2022] Open
Abstract
Primary sarcoidosis of the pancreas is extremely rare. Clinical presentation is often identical to that of pancreatic adenocarcinoma. Preoperative diagnosis of primary pancreatic sarcoidosis is always challenging. We present a 52-year-old man who developed weight loss and obstructive jaundice. Abdomino-pelvic CT scan showed a mass in the pancreatic head. After hepatopancreaticobiliary MDT discussion, a Whipple's procedure was attempted but the mass was deemed unresectable due to invasion of the superior mesenteric vein. Upon completion of palliative chemotherapy, repeat imaging showed significant mass shrinkage. A reattempt Whipple's procedure was successfully undertaken. Histology showed changes of chronic pancreatitis and peripancreatic granulomatous inflammation with no evidence of malignancy and a diagnosis of sarcoidosis was made. Owing to the devastating nature of pancreatic adenocarcinoma, any mass in the pancreas must be thoroughly investigated before a definitive diagnosis is made.
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Wijkstrom M, Bechara RI, Sarmiento JM. A Rare Nonmalignant Mass of the Pancreas: Case Report and Review of Pancreatic Sarcoidosis. Am Surg 2010. [DOI: 10.1177/000313481007600116] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/17/2022]
Abstract
Sarcoidosis is a systemic granulomatous disease of unknown etiology affecting patients from all genetic backgrounds. Pancreatic involvement is rare; the first case was described on autopsy in 1937. We present a case of pancreatic sarcoidosis without a history of the disease presenting as biliary obstruction mimicking pancreatic malignancy. We also review the literature with respect to management and outcomes of similar cases. The patient described here presented with all the signs and symptoms of a pancreatic malignancy, which was confirmed on a CT scan; the positron emission tomography scan and the CA 19-9 level were also confirmatory of the suspected diagnosis. In this setting, if the mass looks resectable, a Whipple procedure would be the next logical step. However, such strategy would be aggressive management for a benign condition that could be palliated with diverting rather than resective procedures without changing the outlook of the disease. We suggest keeping a high index of suspicion in patients with a history of the disease if demographic concordance exists.
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Affiliation(s)
- Martin Wijkstrom
- Departments of Surgery and Emory University School of Medicine, Atlanta, Georgia
| | - Rabih I. Bechara
- Departments of Pulmonary Medicine and Critical Care, Emory University School of Medicine, Atlanta, Georgia
| | - Juan M. Sarmiento
- Departments of Surgery and Emory University School of Medicine, Atlanta, Georgia
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Wellner U, Mattern D, Keck T. Clinical challenges and images in GI. Early peripancreatic and pulmonary sarcoidosis. Gastroenterology 2008; 135:1864, 2159. [PMID: 19013164 DOI: 10.1053/j.gastro.2008.10.068] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/02/2022]
Affiliation(s)
- Ulrich Wellner
- Department of General and Visceral Surgery, University of Freiburg, Freiburg, Germany
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Harder H, Büchler MW, Fröhlich B, Ströbel P, Bergmann F, Neff W, Singer MV. Extrapulmonary sarcoidosis of liver and pancreas: A case report and review of literature. World J Gastroenterol 2007; 13:2504-9. [PMID: 17552036 PMCID: PMC4146771 DOI: 10.3748/wjg.v13.i17.2504] [Citation(s) in RCA: 36] [Impact Index Per Article: 2.0] [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
Sarcoidosis is a chronic multisystemic granulomatous disease of unknown origin, which can involve nearly all organs. In the case of an infrequent gastrointestinal tract involvement in systemic sarcoidosis, granulomas of the liver are most commonly described while isolated pancreatic sarcoid lesions are rarely seen. We report a case of systemic sarcoidosis with exclusive extrapulmonal involvement of the liver and the pancreas in a 71-year-old white man. The diagnosis of liver involvement was confirmed by biopsy. Pancreatic surgery was needed because preoperative evaluation could not exclude pancreatic cancer and for biliary decompression. An extensive literature review of systemic sarcoidosis, focusing on reported cases with unusual presentation of sarcoidosis in the liver and the pancreas, its diagnosis, treatment, and prognosis was made.
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Affiliation(s)
- Hermann Harder
- Department of Medicine II (Gastroenterology, Hepatology and Infectious Diseases), University Hospital of Heidelberg at Mannheim, Theodor-Kutzer-Ufer 1-3, D-68167 Mannheim, Germany
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Caceres M, Sabbaghian MS, Braud R, Wilks S, Boyle M. Pancreatic sarcoidosis: unusual presentation resembling a periampullary malignancy. ACTA ACUST UNITED AC 2006; 63:179-85. [PMID: 16757369 DOI: 10.1016/j.cursur.2005.06.011] [Citation(s) in RCA: 24] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/13/2004] [Revised: 05/22/2005] [Accepted: 06/08/2005] [Indexed: 01/29/2023]
Abstract
Sarcoidosis involving the pancreas is a rare occurrence. Isolated cases of localized or diffuse involvement of the pancreas have been reported in the literature. The preoperative diagnosis of this entity is a clinical challenge, and surgical intervention is usually needed to make a definitive diagnosis. We report a patient that presents with a preoperative evaluation suggestive of cholangiocarcinoma. Surgical management involved pancreaticoduodenectomy, which revealed pancreatic sarcoidosis with regional lymph node involvement. An extensive literature review of sarcoidosis of the pancreas is provided, which cites all reported cases in which the presentation warranted surgical intervention.
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Affiliation(s)
- Manuel Caceres
- Department of Surgery, Louisiana State University Health Sciences Center, New Orleans, Louisiana, USA.
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Baroni RH, Pedrosa I, Tavernaraki E, Goldsmith J, Rofsky NM. Pancreatic sarcoidosis: MRI features. J Magn Reson Imaging 2005; 20:889-93. [PMID: 15503350 DOI: 10.1002/jmri.20199] [Citation(s) in RCA: 12] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/08/2022] Open
Abstract
Pancreatic sarcoidosis is a rare entity, and few reports in the literature describe the CT aspects of the disease. We report the MR features of a pathologically-confirmed case of pancreatic sarcoidosis, which presented as multiple pancreatic masses with low signal intensity on T1-weighted images, mild high signal intensity on T2-weighted images, and decreased enhancement compared to the normal pancreas after administration of gadolinium. Pancreatic sarcoidosis should therefore be included in the differential diagnosis of multiple pancreatic masses with these MRI features.
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Affiliation(s)
- Ronaldo H Baroni
- Department of Radiology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, Massachusetts 02115, USA
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Abstract
A 61-year-old female patient is described who presented with weight loss, steatorrhoea and enlargement of the pancreatic head. Surgical exploration for suspected pancreatic cancer revealed multiple peritoneal white spots, initially suggestive for peritoneal metastases or tuberculosis but finally identified as peritoneal sarcoidosis. Pancreatic insufficiency could not be proven in further studies. We found pancreas divisum as an additional cause for the pancreatic head mass, and steatorrhoea was due to late-onset oligosymptomatic coeliac disease. This case demonstrates diagnostic pitfalls when several rare disorders are manifest in a single patient. Coeliac disease and sarcoidosis might be sequels of similar immune responses to certain antigens.
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Affiliation(s)
- Karlheinz Kiehne
- Department of Internal Medicine, Universitätsklinikum Schleswig Holstein, Campus Kiel, Kiel, Germany.
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Romboli E, Campana D, Piscitelli L, Brocchi E, Barbara G, D'Errico A, Fusaroli P, Corinaldesi R, Pezzilli R. Pancreatic involvement in systemic sarcoidosis. A case report. Dig Liver Dis 2004; 36:222-7. [PMID: 15046194 DOI: 10.1016/j.dld.2003.09.020] [Citation(s) in RCA: 11] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/11/2022]
Abstract
We report a case of serum pancreatic hyperenzymemia as a primary manifestation of sarcoidosis; pancreatic involvement was confirmed by endoscopic ultrasonography which revealed a notable glandular fibrosis of the pancreas. It is important that patients with systemic sarcoidosis who have increased serum levels of amylase and lipase be checked in order to detect the presence of possible pancreatic involvement. Endoscopic ultrasonography represents the technique of choice used to confirm the clinical suspicion of pancreatic inflammation.
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Affiliation(s)
- E Romboli
- Department of Internal Medicine and Gastroenterology, Sant'Orsola Hospital, Alma Mater Studiorum University of Bologna, Via Massarenti 9, 40138 Bologna, Italy
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12
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Affiliation(s)
- David M Warshauer
- Both authors: Department of Radiology, University of North Carolina School of Medicine, Manning Dr., Old Clinic Bldg., Rm. 2016, Box 7510, Chapel Hill, NC 27599-7510, USA
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