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2018 ; 97
(26
): e11303
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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
): e11303
PMID29953016
show 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.
|Aged
[MESH]
|Antibodies, Antinuclear/immunology
[MESH]
|Cholagogues and Choleretics/therapeutic use
[MESH]