IgG4-related retroperitoneal fibrosis overlapping with primary biliary cirrhosis and primary Sjögren's syndrome

Abstract

Rationale:IgG4-related disease (IgG4-RD) is a chronic fibro-inflammatory disorder which is characterized by elevated levels of serum IgG4 and infiltration of IgG4-bearing plasma cells in the involved organs. Primary biliary cirrhosis (PBC) and Primary Sjögren's syndrome (pSS) are both distinct from IgG4-related disease. We herein describe a Chinese patient with IgG4-related RPF overlapping with PBC and pSS.Patient concerns:We report a case of 69-year-old male with recurrent lower abdominal pain for 10 months. Laboratory data showed elevated erythrocyte sedimentation rate and hepatobiliary enzymes, renal dysfunction, high titers of antinuclear antibody, anti-SS-A antibody and anti-mitochondrial type 2, high immunoglobulin (Ig) G levels and elevated serum IgG4 (9 g/L). Contrast-enhanced computed tomography and magnetic resonance imaging were suggestive of retroperitoneal fibrosis and unilateral ureteral occlusion. Immunohistochemical staining for IgG4 did not demonstrate infiltration of IgG4-positive plasma cells in the retroperitoneal mass, but revealed significant infiltration of lymphocytoplasma cells as well as fibrosis and fibrin accumulation.Diagnoses:The patient was diagnosed with IgG4-related retroperitoneal fibrosis based on the International Consensus Diagnostic Criteria. He was also diagnosed with primary biliary cirrhosis and primary Sjögren's syndrome.Interventions:250 mg ursodeoxycholic acid was administered twice daily, and prednisolone was initiated at a dose of 40 mg/day and then tapered to 25 mg after 45 days.Outcomes:The size of the retroperitoneal soft tissue mass gradually reduced and the abnormal laboratory parameters were restored to normal.Lessons:This rare clinical condition has seldom been reported in the literature, which suggests that common immunogenetic factors may be involved in the development of IgG-related RPF, PBC and pSS.

A Case Report of a Prevertebral Mass in an Elderly Male Post Hodgkin's Lymphoma

“…Tfh cells are also increased in affected tissues, and these cells participate in B cell development, proliferation, and class switches to IgG4. The IgG4 found in increased levels in the affected tissues resulting from the plasma cell activation is not a cause of inflammation and fibrosis [ 19 ].…”

Presenting IgG4-related disease: an emerging entity in Pakistan

“…In a study on patients (n=125) with biopsy-proven disease at Massachusetts General Hospital Boston, multi-organ involvement was seen in 38% of the patients (20). There are two case reports on retroperitoneal brosis with coexistent primary biliary cholangitis (21,22). Timely diagnosis of IgG4-RD, with non-speci c clinical symptomatology and multi-organ multisystem involvement is challenging may require interdisciplinary cooperation of correct identi cation and management of this disorder.…”

An Overlapping Case of IgG4-related Disease and Klinefelter Syndrome with Lupus-like Serological and Neurological Features: A Case Report and Literature Review

“…As there were no detectable similarities in the clinical features in the five patients, the complications observed are likely to be occult. However, there are several reports on overlapping cases of IgG4-RD and Sjögren syndrome, ANCA-associated vasculitis and relapsing polychondritis, suggesting that IgG4-RD can share some heterogenous autoimmune conditions (13)(14)(15)(16). Moreover, GWAS conducted for IgG4-RD recently revealed that this condition shares several disease susceptibility genes with SLE, such as human leukocyte antigen (HLA)-DRB1 (p = 1.1×10 -11 for IgG4-RD; odds ratio of 2.4 for SLE) and FCGR2B (p = 2.0×10 -8 for IgG4-RD, odds ratio of 2.3-2.45 for SLE) (23,24,(27)(28)(29), thereby suggesting the common genetic factors associated with the development of the two diseases, which may be independent of KS because these two genes are not located on X chromosome.…”

“…It is characterized by elevated serum IgG4 levels, infiltration of IgG4-positive plasma cells, obliterative phlebitis, and tissue fibrosis of the affected organs ( 8 ). Its association with autoimmune diseases such as SLE ( 9 - 12 ), Sjögren syndrome ( 13 , 14 ), antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis ( 15 ), and relapsing polychondritis ( 16 ) is suggestive of a possible similarity in the immune-disturbances observed in these diseases. We herein report an interesting overlapping case of IgG4-RD and KS with lupus-like serological and neurological features.…”