IgG4-related retroperitoneal fibrosis overlapping with primary biliary cirrhosis and primary Sjögren's syndrome: A case report

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.

Figure 1

Contrast-enhanced CT of RPF before treatment. (A, B) Axial nonenhanced CT images show an irregular retroperitoneal mass (arrow) which is isoattenuating to muscle. The mass is located anterior and lateral to the lower abdominal aorta and iliac arteries. (C) Left hydronephrosis is secondary to distal encasement of the ureter by the mass. The renal excretion of contrast material was delayed (arrow). CT = computed tomography, RPF = retroperitoneal fibrosis.

Figure 2

Histopathological findings of RPF and lip biopsy. (A) Hematoxylin and eosin staining of the left parotid gland demonstrates lymphoplasmacytic infiltration, fibrosis and fibrin accumulation. (B) Immunohistochemical staining of IgG4 in RPF reveals no IgG4-positive plasma cells. (C) Hematoxylin and eosin staining of lip biopsy demonstrates a decreased number of gland bubbles and heavy infiltration of lymphocytes. (D) Immunohistochemical staining of IgG4 in lip biopsy reveals no IgG4-positive plasma cells. RPF = retroperitoneal fibrosis.

Figure 3

Contrast-enhanced CT of RPF after treatment. (A, B) Axial nonenhanced CT images reveal a decrease in diffuse retroperitoneal soft tissue (arrow). (C) The renal excretion of contrast material is better than that before treatment (arrow). CT = computed tomography, RPF = retroperitoneal fibrosis.