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10.1097/MD.0000000000011303

http://scihub22266oqcxt.onion/10.1097/MD.0000000000011303
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C6039603!6039603!29953016
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suck abstract from ncbi


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pmid29953016      Medicine+(Baltimore) 2018 ; 97 (26): ä
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  • IgG4-related retroperitoneal fibrosis overlapping with primary biliary cirrhosis and primary Sjögren s syndrome: A case report #MMPMID29953016
  • Huang X; Lu B; Li M; Fan Y; Zhang L
  • Medicine (Baltimore) 2018[Jun]; 97 (26): ä PMID29953016show ga
  • 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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