Primary biliary cirrhosis and idiopathic retroperitoneal fibrosis: a rare association

Tang, Kwok; Schofield, J. B.; Powell-Jackson, P R

doi:10.1097/00042737-200207000-00013

Cited by 11 publications

(5 citation statements)

References 13 publications

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“…However, clinical features of an overlap of IgG4-RD and PBC remain unknown because of its rarity. All five PBC patients with AIP or idiopathic retroperitoneal fibrosis previously reported were male (6,7,(16)(17)(18). The proportions of women are 87% and 17-38% in PBC and IgG4-RD, respectively (1,19), thus suggesting that gender may be associated with the occurrence of IgG4-RD in PBC.…”

Section: Discussionmentioning

confidence: 86%

“…Considering that two cases of an overlap of IgG4-SC and PBC with concurrent AIP have been reported (6,7), some PBC patients may have AIP. In addition, some PBC patients have been reported to have idiopathic retroperitoneal fibrosis (16)(17)(18), which is one of the manifestations of IgG4-RD (2). Therefore, a small subset of PBC patients may have IgG4-RD.…”

Section: Discussionmentioning

confidence: 99%

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Overlap of IgG4-related Sclerosing Cholangitis and Primary Biliary Cirrhosis

et al. 2014

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We herein present a case of an overlap of immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) and primary biliary cirrhosis (PBC). A 61-year-old man was diagnosed with PBC due to abnormal liver biochemical tests and positivity for serum anti-M2 antibody. However, his response to bezafibrate and ursodeoxycholic acid was insufficient. Five years later, his serum IgG4 level was found to increase. His liver biopsy specimens showed features of nonsuppurative destructive cholangitis in some portal tracts and periductal fibrosis with dense infiltration of IgG4-positive cells in other portal tracts. This case demonstrates that the serum IgG4 level may be worth measuring in patients with PBC refractory to conventional treatment.

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Section: Discussionmentioning

confidence: 86%

Section: Discussionmentioning

confidence: 99%

Overlap of IgG4-related Sclerosing Cholangitis and Primary Biliary Cirrhosis

et al. 2014

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“…To date, a few cases of overlapping PBC and idiopathic RPF have been reported. [ 24 , 25 ] It is suggested that a small subset of PBC patients may have IgG4-related RPF. However, the clinical features of overlapping IgG4-RD and PBC are unknown due to its rarity.…”

Section: Discussionmentioning

confidence: 99%

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

Huang

Lü²,

Li³

et al. 2018

Medicine

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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.

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“…Recently, a significant number of CECs have been detected in different diseases, such as acute coronary syndrome, sickle cell anemia, rickettsia and cytomegalovirus infection, SLE, ANCA-associated small-vessel vasculitis, Kawasaki disease, and SSc [14,[10][11][12][13][14][15][16][17][18][19][20][21][22][23][24][25][26][27]. In addition, in a large group of patients with SLE, we found a number of CECs higher than in normal controls [28], but lower with respect to that observed in patients with CP in the present study (mean values in SLE patients 44.5/mL, 95% CI 38.…”

Section: Discussionmentioning

confidence: 99%

Increased levels of circulating endothelial cells in chronic periaortitis as a marker of active disease

Moroni¹,

Papa²,

Moronetti³

et al. 2005

Kidney International

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Primary biliary cirrhosis and idiopathic retroperitoneal fibrosis: a rare association

Abstract: Primary biliary cirrhosis has rarely been reported to be associated with idiopathic retroperitoneal fibrosis. We describe the case of a 57-year-old man who was confirmed to have both conditions, reflecting the likely autoimmune aetiopathogenesis common to both disorders.

Cited by 11 publications

References 13 publications

Overlap of IgG4-related Sclerosing Cholangitis and Primary Biliary Cirrhosis

Overlap of IgG4-related Sclerosing Cholangitis and Primary Biliary Cirrhosis

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

Increased levels of circulating endothelial cells in chronic periaortitis as a marker of active disease

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