Retrospective analysis of IgG4-related disease cases at a tertiary care centre in India.

ABSTRACT

Objectives: IgG4-related disease (IgG4-RD) is a chronic inflammatory disorder with prominent fibrosis. This retrospective analysis was undertaken to study the clinical, laboratory, and radiological characteristics of patients with extra-pancreatic IgG4-RD and their response to treatment at a tertiary care centre located in northern India. Material and methods: Patient data from our centre between January 2017 and January 2021 were reviewed. Probable/definite IgG4-RD cases were included in the analysis. Results: A total of 14 cases were identified with a median age of 39 years (range 19-56 years). There were 10 males and 4 females. All patients presented with slowly progressive soft tissue swellings with pain/discomfort related to local mass effect. The median delay in diagnosis was 9.5 months (range 2-72 months). Cross-sectional imaging showed soft tissue masses in all cases. All contrast-enhanced studies (n = 7) showed enhancement on computed tomography and magnetic resonance imaging scan. F-18 fluorodeoxyglucose-avidity was observed in 8 of 9 (88.9%) cases. Biopsies performed in 12 of these were classified as definite in 8 and possible IgG4-RD in 4 cases. Patients were treated with a median dose of 1 mg/kg/day (range 0.5-1 mg/kg/day) prednisolone. Steroids were successfully tapered in all 12 cases with 41.6% (5 of 12) being off corticosteroids at a median follow-up of 10 months (range 0-18 months). Two patients were lost to follow-up. Conclusions: IgG4-related disease is a chronic illness with a wide spectrum of manifestations, in which the diagnosis is often delayed, but it shows an excellent response to treatment. Efforts must be made to increase awareness among physicians about this disease to institute appropriate treatment as early as possible.