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Largest Series Yet Describes IgG4-Related Disease

A report from Japan describes 235 patients with immunoglobulin G4-related disease, which is characterized by masses in various organs and infiltration of plasma cells.

Inoue D, Yoshida K, Yoneda N, et al. IgG4-Related Disease: Dataset of 235 Consecutive Patients. Medicine. 2015;94(15):e680.  doi: 10.1097/MD.0000000000000680

The largest reported cohort for immunoglobin G4-related disease (IgG4-RD), this paper characterizes 235 patients from eight general hospitals in Kobe, Japan.

The most common manifestation was pancreatitis, in 142 patients (60%), followed by sialadenitis (34%), tubulointerstitial nephritis (23%), dacryoadenitis (23%), and periaortitis (20%). Periaortitis was more common in males and sialodacryoadenitis was more common in females.

Patients were identified by searching the hospital radiology database, since IgG4-RD patients are supposed to undergo imaging at least once.

Inclusion criteria were histology-proven IgG4 according to the Pathology Consensus Statement, or definitive type 1 autoimmune pancreatitis meeting the International Consensus Diagnostic Criteria, or both.

Serum IgG4 was elevated ≤135 mg/dL above normal in 208 patients (88%) and >270 mg/dL in 67 (71%). IgG4 was higher in patients with multiorgan involvement (median 629 mg/dL). The IgG4/IgG ratio was >10% in 218 patients with IgG values available.

Corticosteroids were effective, but the relapse rate was 24% in the study period (median 37 months).

The male:female ratio was 4:1. Median age was 67 years.

Cancer incidence was similar to that in the Japanese population.