IgG4-related cholangitis (IRC) is a key hepatobiliary manifestation of IgG4-related disease, a systemic fibroinflammatory condition that often affects multiple organs. Diagnosing IRC can be challenging, as its clinical and imaging features may overlap with malignancy and other biliary disorders. A new retrospective study provides one of the largest single-centre descriptions to date of IRC, offering valuable insight into its presentation, treatment response, and long-term prognosis.
The analysis included 107 patients treated over a 10-year period at a tertiary referral centre, aiming to clarify real-world diagnostic patterns and outcomes while highlighting factors associated with relapse.
Typical Presentation and Systemic Involvement
Patients were predominantly male, with a mean age of just over 60 years. Jaundice was the most common presenting symptom, affecting more than three-quarters of patients, followed closely by weight loss. Nearly all patients had elevated serum IgG4 levels, reinforcing its diagnostic value when interpreted alongside clinical and imaging findings.
Systemic involvement was common, with autoimmune pancreatitis present in over 96% of cases, underscoring the strong association between IRC and pancreatic manifestations of IgG4-related disease. Imaging most frequently revealed isolated stenosis of the lower common bile duct, consistent with type 1 IRC.
These findings highlight the importance of comprehensive evaluation, as reliance on imaging alone may raise concern for biliary malignancy and lead to unnecessary invasive procedures.
Steroids Effective but Relapse Common
Glucocorticosteroids formed the cornerstone of treatment, with almost 90% of patients receiving steroid therapy. All treated patients demonstrated an excellent initial response, confirming the high steroid sensitivity of IRC.
During a median follow-up of more than five years, relapse occurred in 37.5% of patients. Importantly, nearly half of relapses developed after steroid withdrawal. Statistical analyses identified steroid maintenance duration as the only independent risk factor for relapse, with longer maintenance therapy associated with a reduced risk. These findings suggest that careful consideration of tapering schedules and maintenance strategies may be crucial in long-term disease control.
Excellent Long-Term Prognosis
Despite the relatively high relapse rate, long-term outcomes were favourable. Only four patients died during follow-up, and none developed cholangiocarcinoma, a feared complication often considered during initial diagnostic work-up. Five- and ten-year overall survival rates exceeded 95%, and disease-specific survival was 100%.
The results confirm that IgG4-related cholangitis is a benign condition with an excellent prognosis when appropriately treated. While relapse remains a management challenge, it did not adversely affect survival, reinforcing the effectiveness of steroid therapy and the importance of long-term monitoring rather than aggressive intervention.
Reference
Sun J et al. Clinical features, responses to therapy and prognosis of 107 patients with IgG4-related cholangitis: a retrospective study. BMC Gastroenterol. 2026;DOI: 10.1186/s12876-025-04598-0.
