IgG4-Related Disease and Retroperitoneal Fibrosis

2015 ◽  
Author(s):  
John H. Stone
Keyword(s):  
Retroperitoneal Fibrosis ◽  
Clinical Manifestations ◽  
Organ System ◽  
Idiopathic Retroperitoneal Fibrosis ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Mikulicz Disease ◽  
Histopathologic Findings ◽  
Chronic Periaortitis

IgG4-related disease (IgG4-RD) has been observed to affect almost every organ system, with consistent histopathologic findings across systems. IgG4-RD can mimic malignant, infectious, and inflammatory disorders; accordingly, consideration of the histopathologic features of tissue biopsies and rigorous clinicopathologic correlations are essential to avoid misdiagnosis. Since the early 2000s, IgG4-RD has increasingly been recognized as a cause of what was previously referred to as “idiopathic” retroperitoneal fibrosis (RPF), and this IgG4-related RPF is now considered to comprise an important subset of IgG4-RD. This review includes an overview of IgG4-RD and discusses the pathology, pathophysiology, and clinical manifestations of IgG4. IgG4-related RPF is also discussed in this review, with topics including IgG4-related RPF versus RPF of other causes, the differences between RPF and other subsets of IgG4-RD, and treatment of both IgG4-RD and IgG4-related RPF. Figures show the histopathology features of IgG4-RD, immunostaining of tissue for IgG4, IgG4-related RPF and chronic periaortitis, “Mikulicz disease”, IgG4-RD of the lung, IgG4-related renal disease, and type 1 (IgG4-related) pancreatitis. The table lists conditions known previously by other names that often fall within the spectrum of IgG4-RD. This review contains 7 highly rendered figures, 1 table, and 66 references.

scholarly journals IgG4-Related Disease: A Reminder for Practicing Pathologists

2017 ◽  
Vol 141 (11) ◽  
pp. 1476-1483 ◽  
Author(s):  
Steven C. Weindorf ◽  
John Karl Frederiksen
Keyword(s):  
Autoimmune Pancreatitis ◽  
Plasma Cells ◽  
Clinical Manifestations ◽  
Organ System ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Resection Specimen ◽  
Histopathologic Features

IgG4-related disease (IgG4-RD) is a systemic autoimmune fibroinflammatory disease that produces sclerotic, tumefactive masses containing dense lymphoplasmacytic infiltrates rich in immunoglobulin (Ig) G4+ plasma cells. Initially characterized as a form of autoimmune pancreatitis, the distinctive histopathology of IgG4-RD has now been described in almost every organ system. However, because the clinical manifestations of IgG4-RD are diverse and nonspecific, the disease may go unsuspected until a biopsy or resection specimen is obtained to diagnose a presumed malignancy. Pathologists thus play a key role in the diagnosis of IgG4-RD, and familiarity with its histopathologic features is essential to preventing the irreversible comorbidities associated with this treatable disease. This brief review outlines the epidemiology, clinical manifestations, and histopathology of IgG4-RD, with the aim of furthering pathologists' awareness of and ability to diagnose this disorder.

Is it time to consider IgG4-related disease in idiopathic retroperitoneal fibrosis?

2015 ◽  
Vol 10 (3) ◽  
pp. 222-224
Author(s):  
Luke Teo ◽  
Daniel Jenkin ◽  
Shahir Hamdulay ◽  
Rajesh Kavia
Keyword(s):  
Retroperitoneal Fibrosis ◽  
Idiopathic Retroperitoneal Fibrosis ◽  
Related Disease ◽  
Igg4 Related Disease

scholarly journals An old disease, a new diagnosis; A 49 year-old man with nephrotic syndrome

2019 ◽  
Vol 8 (3) ◽  
pp. 32-32
Author(s):  
Santiago Cedeño ◽  
Ursula Verdalles ◽  
Marian Goicoechea ◽  
Soledad García de Vinuesa ◽  
José Luño
Keyword(s):  
Nephrotic Syndrome ◽  
T Lymphocyte ◽  
Transforming Growth Factor ◽  
Organ System ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Immune Mediated ◽  
Mikulicz Disease ◽  
New Diagnosis ◽  
Regulatory T Lymphocyte

Background: IgG4-related disease (IgG4-RD) is a systemic immune-mediated disease that typically manifests as fibro-inflammatory masses that can affect nearly any organ system. Case Report: We present here a case of a 49-year old man with forgotten old disease (Mikulicz disease) with membranous nephropathy (MN). Conclusions: This entity is currently included within the spectrum of IgG4-related disease. The development of renal disease shortly after the suspension of rituximab suggests another probable pathway involved. To our knowledge the transforming growth factor may be responsible for existing pattern of fibrosis in this disease. The lack of response or at least partial response to rituximab can be explained by greater involvement of regulatory T lymphocyte in the pathophysiology of this entity.

Idiopathic retroperitoneal fibrosis and its overlap with IgG4-related disease

2017 ◽  
Vol 12 (3) ◽  
pp. 287-299 ◽  
Author(s):  
Giovanni Maria Rossi ◽  
Rossana Rocco ◽  
Eugenia Accorsi Buttini ◽  
Chiara Marvisi ◽  
Augusto Vaglio
Keyword(s):  
Retroperitoneal Fibrosis ◽  
Idiopathic 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 A Follow-Up Study of a European IgG4-Related Disease Cohort Treated with Rituximab

2021 ◽  
Vol 10 (6) ◽  
pp. 1329
Author(s):  
Johanna Backhus ◽  
Christian Neumann ◽  
Lukas Perkhofer ◽  
Lucas A Schulte ◽  
Benjamin Mayer ◽  
...  
Keyword(s):  
Disease Activity ◽  
Clinical Activity ◽  
Inflammatory Disorder ◽  
International Consensus ◽  
Steroid Pulse ◽  
Related Disease ◽  
Igg4 Related Disease

Objectives: IgG4-related disease (IgG4-RD) is a chronic fibro-inflammatory disorder affecting virtually any organ. Type 1 autoimmune (type 1 AIP) is its pancreatic manifestation. To date, steroids are considered the first-line pancreatitis treatment. The CD20-binding antibody rituximab (RTX) appears a promising steroid-sparing therapy, although long-term data are lacking. We aimed to bridge this gap with a cohort of IgG4-RD patients treated with RTX and to assess the potential value of the Responder Index (RI) as a discriminatory score for disease activity. Methods: We retrospectively evaluated 46 patients from a tertiary referral centre who were diagnosed with IgG4-RD and/or type 1 AIP according to the International Consensus Diagnostic Criteria or Unifying-AIP criteria between June 2006 and August 2019. Results: Patients resembled previous cohorts in terms of characteristics, diagnosis, and therapeutic response. Thirteen of the 46 patients with IgG4-RD/type 1 AIP were treated with RTX pulse therapy due to relapse, adverse reactions to steroids, or high-risk constellations predicting a severe course of disease with multi-organ involvement. Median follow-up after diagnosis was 52 months for all subjects, and 71 months in IgG4-RD patients treated with RTX. While patients in the RTX group showed no significant response to an initial steroid pulse, clinical activity as measured by the RI significantly decreased in the short-term after RTX induction. Within 16 months, 61% of patients relapsed in the RTX group but responded well to re-induction. Clinical and laboratory parameters improved equally in response to RTX. Conclusion: RTX therapy in patients with IgG4-RD is an effective and safe treatment to induce treatment response and possible long-term remission. Repeated RTX administration after 6–9 months may be of value in reducing the risk of relapse. The RI appears to be a reasonable index to assess disease activity and to identify patients with IgG4-related disease who may benefit from B-cell-depleting therapy.

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