IgG4-Related Disease: Results From a Multicenter Spanish Registry

Abstract

IgG4-related disease (IgG4-RD) is a rare entity consisting of inflammation and fibrosis that has been described in multiple organs. Concrete diagnostic criteria have been established recently and there is a lack of large series of patients.To describe the clinical presentation, histopathological characteristics, treatment and evolution of a series of IgG4-RD Spanish patients.A retrospective multicenter study was performed. Twelve hospitals across Spain included patients meeting the current 2012 consensus criteria on IgG4-RD diagnosis.Fifty-five patients were included in the study, 38 of whom (69.1%) were male. Median age at diagnosis was 53 years. Thirty (54.5%) patients were included in the Histologically Highly Suggestive IgG4-RD group and 25 (45.5%) in the probable IgG4-RD group. Twenty-six (47.3%) patients had more than 1 organ affected at presentation. The most frequently affected organs were: retroperitoneum, orbital pseudotumor, pancreas, salivary and lachrymal glands, and maxillary sinuses.Corticosteroids were the mainstay of treatment (46 patients, 83.6%). Eighteen patients (32.7%) required additional immunosuppressive agents. Twenty-four (43.6%) patients achieved a complete response and 26 (43.7%) presented a partial response (<50% of regression) after 22 months of follow-up. No deaths were attributed directly to IgG4-RD and malignancy was infrequent.This is the largest IgG4-RD series reported in Europe. Patients were middle-aged males, with histologically probable IgG4-RD. The systemic form of the disease was frequent, involving mainly sites of the head and abdomen. Corticosteroids were an effective first line treatment, sometimes combined with immunosuppressive agents. Neither fatalities nor malignancies were attributed to IgG4-RD.

FIGURE 1

Microscopic samples of IgG4-related disease. Panel A shows immunostaining for IgG4-positive plasma cell infiltrate in a retroperitoneal mass, at 400×. Panel B illustrates an important lymphoplasmacytic infiltrate in a retroperitoneal mass sample, stained with hematoxylin and eosin at 200×. Finally, Panel C, stained with Masson's trichrome, depicts the storiform fibrosis at 100× in a sample of pleura.

FIGURE 2

Distribution by organs involved in IgG4-related disease Spanish patients. Total recount by organs: retroperitoneum 15; orbitary pseudotumor 12; pancreas and salivary glands 9; maxillary and lachrymal glands 8; aorta and lung, kidney and mesentery 4; gallbladder 3; pachymeninges, pleura and biliary ducts 2; thyroid, extraocular muscles, breast, pericardium, adenopathic and mediastinum 1; hypophysis, prostate, liver, skin, and arteries 0.