Re-evaluation of IgG4 in systemic fibroinflammatory disease with intracardiac involvement

Takikita-Suzuki, Mikiko; Ishida, Mitsuaki; Okabe, Hidetoshi

doi:10.1016/j.humpath.2009.11.001

Cited by 10 publications

(3 citation statements)

References 4 publications

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“…The results of a previous study found that IgG4 was increased in 60-70% patients with IgG4 related diseases (11). Constrictive pericarditis and pericardial fibrosis combined with retroperitoneal fibrosis is extremely rare and has been depicted only in a few cases in medical literature (12)(13)(14). An elevated level of immunoglobulin (Ig) G4 is characteristic of these diseases and so they are categorized into the hyper IgG4 related disease group.…”

Section: Introductionmentioning

confidence: 99%

Retroperitoneal fibrosis and constrictive pericarditis-IgG4 related diseases: A case report

Terzić

Spasić

Djuric

et al. 2017

Experimental and Therapeutic Medicine

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Abstract. Retroperitoneal fibrosis (RPF) is a rare disease characterized by infiltration of inflammatory cells and deposition of thickened fibrous tissues. The present study presents the case of a 53-year-old patient treated for generalized weakness and fatigue for 1 year prior to hospitalization. A cardiac ultrasound revealed pericardial effusion that required pericardiocentesis, during which 1,400 ml serous fluid with the characteristics of an exudate was aspirated. A pericardiectomy was performed due to persistent effusion and histological examination indicated pericardial fibrosis. A thoracic-abdominal computed tomography scan revealed the presence of retroperitoneal fibrosis. The patient was treated with corticosteroids and azathioprine. Follow-up examinations showed a significant reduction in the amount of abdominal fibrous tissue and no increase in pericardial effusion 1 year following the end of treatment. The patient continues to have regular follow-up control examinations with a cardiologist and nephrologist.

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Section: Introductionmentioning

confidence: 99%

Retroperitoneal fibrosis and constrictive pericarditis-IgG4 related diseases: A case report

Terzić

Spasić

Djuric

et al. 2017

Experimental and Therapeutic Medicine

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“…Darüber hinaus findet sich ein variables Maß an Fibrose sowie oft auch eine obliterative Phlebitis und eine Gewebseosinophilie. Bei der Fibrose findet sich ein charakteristisches storiformes Muster [4,5,15].…”

Section: Histopathologieunclassified

IgG4-assoziierte Erkrankungen

Thiele¹,

Witte²

2022

Arthritis und Rheuma

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ZUSAMMENFASSUNGIgG4-assoziierte Erkrankungen sind fibrosierende entzündliche Systemerkrankungen, die nahezu jedes Organsystem betreffen können. Sie betreffen meistens Männer in einem Alter über 50 Jahre. Zu den Krankheitsmanifestationen zählen u. a. Autoimmunpankreatitis, Lymphadenopathien, retroperitoneale Fibrose, entzündliche Orbitopathie sowie Beteiligungen der Speichel- und Tränendrüsen. Diese können gemeinsam oder isoliert auftreten. In der Diagnostik sind eine histologische Untersuchung sowie die Bestimmung des IgG4 im Serum wichtig. Maligne und andere entzündliche Erkrankungen müssen ausgeschlossen werden. IgG4-assoziierte Erkrankungen sprechen gut auf Glukokortikosteroide an.

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“…Clinical manifestations, actually regarded in the setting IgG4-RD, include broad variety of conditions [3][4][5][13][14][15][16][17][18][19][20][21][22][23][24][25][26][27][28][29][30]. …”

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confidence: 99%

Immunoglobulin G4-Related Disease, Presented with Retroperitoneal Fibrosis and Monoclonal Gammopathy: Case Report and Mini Review

Zakharova¹

2015

UNOAJ

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The process may involve pancreas, bile ducts, gallbladder, liver, thyroid, salivary and lacrimal glands, orbits, lungs, mediastinum, pericardium, aortae and arteries, retroperitoneum, kidneys, prostate and testes, breast, lymph nodes, skin, hypophysis etc. Clinical manifestations, actually regarded in the setting IgG4-RD, include broad variety of conditions [3][4][5][13][14][15][16][17][18][19][20][21][22][23][24][25][26][27][28][29][30]. AbstractImmunoglobulin G4-related disease (IgG4-RD) is a new entity, which comprise a group of conditions, sharing common clinical, serologic and pathologic features -mainly tumor-like swelling of involved organs, lymphoplasmacytic tissue infiltration with the predominance of IgG4 positive plasma cells and CD4 positive T lymphocytes, modest tissue eosinophilia, and so-called "storiform" fibrosis with cartwheel appearance of the arranged fibroblasts and inflammatory cells. The pathogenesis of IgG4-RD is not fully understood, and the pathogenic role of IgG4 is still under discussion. The pathology process may involve pancreas, bile ducts, gallbladder, liver, thyroid, salivary and lacrimal glands, orbits, lungs, mediastinum, pericardium, aortae and arteries, kidneys, prostate and testes, breast, lymph nodes, skin, hypophysis etc., but one of the most common manifestations is retroperitoneal fibrosis. Several case series data showed that IgG4-RD is responsible for a majority of cases of retroperitoneal fibrosis, previously regarded as "idiopathic". The diagnosis of IgG4-RD requires pathology data; lymphoplasmacytic tissue infiltration with mainly IgG4+ plasma cells and lymphocytes is confirmatory. Serum IgG4 levels should be measured and isolated IgG4 elevation is a significant aid in diagnosis, although it is not diagnostic. Elevated IgG4 in patients with IgG4-RD were shown to be usually polyclonal First-line treatment for symptomatic IgG4-RD is prednisone. Here we present a pathology proven case of IgG4-RD, manifested as retroperitoneal fibrosis and monoclonal gammopathy, successfully treated with corticosteroids.

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Re-evaluation of IgG4 in systemic fibroinflammatory disease with intracardiac involvement

Cited by 10 publications

References 4 publications

Retroperitoneal fibrosis and constrictive pericarditis-IgG4 related diseases: A case report

Retroperitoneal fibrosis and constrictive pericarditis-IgG4 related diseases: A case report

IgG4-assoziierte Erkrankungen

Immunoglobulin G4-Related Disease, Presented with Retroperitoneal Fibrosis and Monoclonal Gammopathy: Case Report and Mini Review

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