Background and objective
IgG4-RD is a multisystemic disease, often underdiagnosed, with natural progression to chronic organ damage. We aim to describe clinical features, relapse and chronic organ damage.
Methods
Dynamic ambispective cohort including patients from the Institutional Registry of IgG4-RD at Hospital Italiano de Buenos Aires who met ACR/EULAR 2019 criteria. We analyzed clinical phenotypes, response to treatment, biomarkers and chronic organ damage.
Results
Sixty patients were included; 73% were male and median age at diagnosis was 61 years. Median time to diagnosis was 9 months (IQR 3–21), 10% had >10 years of delay. Multiorgan disease was present at diagnosis in 65% and the most common phenotype was pancreatohepatobiliary (40%). Corticosteroids were used at diagnosis in 77%; seven patients with totally resected disease required no further immunosuppression (median 59 months; IQR 31–144). Relapses occurred in 47%, predominantly in those with multiorgan involvement (89%). Kidneys and the biliary tract were most commonly affected in relapse. Relapsing predictive biomarkers were present in 63% at the onset; multiorgan involvement was associated with relapsing disease (p = 0.002). Chronic organ damage occurred in 71%, in 44% after surgical resection. The most frequent was exocrine pancreatic insufficiency (35%). Patients with baseline damage had a significantly longer diagnostic delay (p = 0.03). After a year of diagnosis, 85% of patients experienced damage.
Conclusion
Baseline organ damage was highly frequent, particularly with delayed diagnosis, while multiorgan involvement predicted relapse, underscoring the need for earlier and multidisciplinary management.
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