scholarly journals IgG4 related disease as a cause of isolated retroperitoneal fibrosis with no other organ involvement; Case report

2021 ◽  
Vol 61 ◽  
pp. 69-72
Author(s):  
Almurtada Razok ◽  
Rubab Malik ◽  
Priyanka Cackamvalli ◽  
Muhammad Zahid
Keyword(s):  
Case Report ◽  
Retroperitoneal Fibrosis ◽  
Organ Involvement ◽  
Related Disease ◽  
Igg4 Related Disease

scholarly journals Retroperitoneal Fibrosis as a presentation with masked multiorgan involvement of IgG4‐related disease—demystifying the diagnosis: A case report from Nepal

2021 ◽  
Vol 9 (9) ◽  
Author(s):  
Sangam Shah ◽  
Rajan Chamlagain ◽  
Bikash Baral ◽  
Sanjib Shrestha ◽  
Yagya Raj Adhikari ◽  
...  
Keyword(s):  
Case Report ◽  
Retroperitoneal Fibrosis ◽  
Related Disease ◽  
Igg4 Related Disease

scholarly journals A Case of Immunoglobulin G4-Related Disease with Extensive Multiorgan Involvements

2015 ◽  
Vol 2015 ◽  
pp. 1-9 ◽  
Author(s):  
Kazuhiko Higashioka ◽  
Kenji Yoshida ◽  
Kensuke Oryoji ◽  
Kazuo Kamada ◽  
Shinichi Mizuki ◽  
...  
Keyword(s):  
Retroperitoneal Fibrosis ◽  
Elevated Serum ◽  
Clinical Findings ◽  
Organ Involvement ◽  
Immunoglobulin G4 ◽  
Serum Igg4 Level ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
Igg4 Level

We report a case of IgG4-related disease (IgG4-RD) with multiple ten-organ involvement. This case showed many clinical findings, such as bilateral swelling of salivary and lacrimal glands, autoimmune pancreatitis, interstitial nephritis, retroperitoneal fibrosis, periaortitis, systemic swelling of lymph nodes, pulmonary lesions, splenomegaly, and jejunal lesions. He was suspected as having SLE or malignant lymphoma but diagnosed as having IgG4-RD by the elevated serum IgG4 level and histological findings from kidney and lymph node. We report a case of IgG4-RD with multiple ten-organ involvement that was successfully treated with prednisolone therapy.

scholarly journals Rituximab for retroperitoneal fibrosis due to IgG4-related disease: A case report and literature review

2018 ◽  
Vol 6 (01) ◽  
pp. 4-10 ◽  
Author(s):  
Mohammad Almeqdadi ◽  
Mohammed Al-Dulaimi ◽  
Aleksandr Perepletchikov ◽  
Kevin Tomera ◽  
Bertrand L. Jaber
Keyword(s):  
Case Report ◽  
Literature Review ◽  
Retroperitoneal Fibrosis ◽  
Related Disease ◽  
Igg4 Related Disease

Retroperitoneal Fibrosis Associated With Immunoglobulin IgG4-related Disease in the Differential Diagnosis in Retroperitoneal Tumours. Case Report

2019 ◽  
Vol 15 (5) ◽  
pp. e27-e29
Author(s):  
Gabriela Ruiz Mar ◽  
Óscar E. Cárdenas Serrano ◽  
Jorge Roldan García ◽  
Abraham Cañavera-Constantino ◽  
Víctor M. Menéndez Trejo ◽  
...  
Keyword(s):  
Differential Diagnosis ◽  
Case Report ◽  
Retroperitoneal Fibrosis ◽  
Related Disease ◽  
Igg4 Related Disease

scholarly journals AB1061 2019 ACR/EULAR CLASSIFICATION CRITERIA FOR IGG4-RELATED DISEASE IN RUSSIAN COHORT OF PATIENTS.

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1820.1-1821
Author(s):  
E. Sokol ◽  
S. Palshina ◽  
A. Torgashina ◽  
J. Khvan
Keyword(s):  
Retroperitoneal Fibrosis ◽  
Clinical Symptoms ◽  
Classification Criteria ◽  
The Body ◽  
Cell Counting ◽  
Organ Involvement ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
The Mean

Background:IgG4-related disease (IgG4-RD) is a systemic immunomediated fibroinflammatory condition that can affect almost any organ in the body. This is the reason for dramatic variety of clinical symptoms and complexity of diagnostics. 2011 Comprehensive diagnostic criteria (CDC) for IgG4-RD are used to establish the diagnosis for all lesions (except autoimmune pancreatitis type 1). In 2019 the new ACR/EULAR classification criteria for IgG4-RD were proposed to facilitate the formation of more homogenous groups of patients primarily for clinical trials inclusion purpose. They also provide a framework for clinicians considering diagnosis of IgG4-RD.Objectives:To evaluate 2019 ACR/EULAR classification criteria for IgG4-RD in Russian cohort of patients with IgG4-RD.Methods:59 patient with IgG4-RD according to CDC with biopsy proven diagnosis were included.Results:The mean number of affected organs was 2.1; 31 patients (52,5%) were women. Majority of patients had sialoadenitis (25 patients) and/or orbital disease (31 patients), 9 had retroperitoneal fibrosis (RPF). Other affected organs were lungs, pancreas, lymph nodes, paranasal sinuses, thyroid and low urinary tract. Twenty five (25) patients (42,4%) had definite, 14 (23,3%) probable and 20 (34,3%) possible diagnosis of IgG4-RD. Twenty three (23) patients (39%) didn’t fulfill the 2019 ACR/EULAR classification criteria for IgG4-RD. Among them were the majority of patients with RPF (7 patients) who were lacking other organ involvement and IgG4 hypersecretion either in the tissue or serum. The majority of excluded cases were due to inadequate pathomorphological evaluation (lacking of the exact number and percentage (if >40%) of IgG4+ cells), lacking of multi-organ involvement or different patterns of involvement, e.g. in case of lungs involvement.Conclusion:The new 2019ACR/EULAR classification criteria for IgG4-RD are very useful in evaluation of typical organ involvement and systemic course of IgG4-RD. It is essential to adjust Russian pathomorphologists’s approach to cell counting and percentage determination for IgG4-RD cases to get suitable protocols.Disclosure of Interests:None declared

scholarly journals A case of giant retroperitoneal lymphangioma and IgG4-positive fibrosis: Causality or coincidence?

2021 ◽  
Vol 9 ◽  
pp. 2050313X2110169
Author(s):  
Kim Rouven Liedtke ◽  
Christoph Käding ◽  
Paula Döring ◽  
Sander Bekeschus ◽  
Anne Susann Glitsch
Keyword(s):  
Retroperitoneal Fibrosis ◽  
Inflammatory Diseases ◽  
Histological Change ◽  
Iliac Vessel ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Cystic Tumors ◽  
Retroperitoneal Cyst ◽  
Retroperitoneal Lymphangioma ◽  
Severe Fibrosis

Several chronic inflammatory diseases have been found to be a subtype of IgG4-related disease, all of which have a typical clinical and histological change, which is based in particular on an overexpression of IgG4 and subsequent fibrosis. At least a part of the retroperitoneal fibrosis, which was originally classified as idiopathic, seems to be assigned to IgG4-related disease. Lymphangiomas are benign, cystic tumors that rarely occur in adults. However, there is no firm association with IgG4-related disease described in the literature to date. This report is about a patient suffering from acute renal failure due to a giant retroperitoneal cyst. Surgical resection remains incomplete in the iliac vessel area due to severe fibrosis and histology revealed features of both lymphangioma and IgG4+ fibrosis. The case description is followed by a brief overview of IgG4-related disease and a consideration of whether lymphangiomas might be assigned to this topic.

scholarly journals Erdheim-Chester Disease: The Importance of Information Integration

2017 ◽  
Vol 10 (2) ◽  
pp. 613-619 ◽  
Author(s):  
Anna Nikonova ◽  
Khashayar Esfahani ◽  
Guillaume Chausse ◽  
Stephan Probst ◽  
Tina Petrogiannis-Haliotis ◽  
...  
Keyword(s):  
Retroperitoneal Fibrosis ◽  
Braf V600e Mutation ◽  
Braf V600e ◽  
High Dose ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Erdheim Chester Disease ◽  
History Of ◽  
Erdheim Chester ◽  
Chester Disease

Background: Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis disorder that utilizes the RAS-RAF-MEK-ERK pathway. It has a highly variable clinical presentation, where virtually any organ can be involved, thus having the potential of posing a great diagnostic challenge. Over half of the reported cases have the BRAF V600E mutation and have shown a remarkable response to vemurafenib. Case Presentation: We describe herein a patient with a history of stroke-like symptoms and retroperitoneal fibrosis that on initial pathology raised the possibility of IgG4-related disease. However, the patient was refractory to high-dose steroids and progressed further, developing an epicardial soft tissue mass and recurrent neurological symptoms. Integration of the above findings with new information at another hospital about a radiological history of symmetrical lower extremities long bone lesions raised the differential diagnosis of ECD. Molecular analysis of formalin-fixed paraffin-embedded tissue of both of the patient’s retroperitoneal biopsies (the second one of which had shown a small focus of foamy histiocytes, CD68+/CD1a–) was positive for BRAF mutation, confirming the diagnosis of ECD. The patient demonstrated a dramatic and sustained metabolic response to vemurafenib on follow-up positron emission tomography scans. Conclusion: This case highlights the need for developing a high index of suspicion for presentations of retroperitoneal fibrosis that could represent IgG4-related disease but fail to respond to steroids. When unusual multisystem involvement occurs, one should consider a diagnosis of a rare histiocytosis. Vemurafenib appears to be an effective treatment for even advanced cases of both ECD and Langerhans histiocytosis bearing the BRAF V600E mutation.

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