A Rare Case of IgG4-related Sclerosing Cholangitis Companied With Miculiczʼs Disease and Lymphadenitis

Abstract

Introduction: IgG4-related sclerosing cholangitis (IgG4-SC) appears to be part of IgG4-related disease (IgG4-RD). We report a rare case of IgG4-SC companied with Miculicz's disease (MD) and lymphadenitis. Methods: Case Report: A 61-year male from Northeast China was admitted for pruritus and jaundice lasting for 6 months. He presented symmetric swelling of the lacrimal glands and right swelling of submandibular gland with slight dysfunction of lacrimal and salivary secretion and a 45-pound weight loss over 3 months. He had a 1-year history of left swelling of submandibular gland and lymphadenopathy. He underwent surgical excision of the swollen lymph node and left submandibular gland one year prior to hospital admission. The patient denied any history of alcohol, tomacco, or illicit drug use. Routine blood test showed the erythrocyte sedimentation rate (ESR) was markedly raised (120 mm/h). Serum biochemical data on admission were as follows: total bilirubin 241 μmol/L, alkaline phosphatase 466 U/L, alanine aminotransferase 131 U/L. Serum immunological testing displayed high levels of IgG4 (31.4 g/L) and IgG (38 g/L), and negative values of anti-SSA and anti-SSB. An ultrasound scan of the neck showed right swollen of submandibular gland (3.5 cm length × 2.5 cm width × 1.5 cm height), suggesting the possibility of chronic sialedenitis. An enhanced CT scan of abdomen revealed the common bile duct wall thickening as well as intra- and extra-hepatic bile duct dilation. The resected specimens from the left submandibular gland and lymph node one year prior to this admission were examined with HE and immunochemical stain which revealed storiform-type fibrosis, and numerous IgG4-positive plasmacytic infiltration with a ratio of IgG4/IgG-positive plasmacytes of more than 40% thus meeting the diagnostic criteria for IgG4-SC companied with MD and lymphadenitis. The patient responded well to corticosteroid therapy and remains healthy with no signs of recurrence at six-month follow-up. Results: No. Conclusion: IgG4-RD is characterized by the enlargement of involved organs, elevated levels of serum IgG4, and abundant infiltration of IgG4-positive plasmacytes in the affected organs. IgG4-SC is the hepatobiliary manifestation of IgG4-RD. Mikulicz's disease (MD) is recently recognized as IgG4-RD distinct from Sjögren's syndrome. We report a rare case of IgG4-SC companied with MD and lymphadenitis. The patient responded well to corticosteroid therapy.