IgG4-related disease in Italy: Clinical features and outcomes of a large cohort of patients

C. Campochiaro, G. A. Ramirez, E. P. Bozzolo, M. Lanzillotta, A. Berti, E. Baldissera, L. Dagna, L. Praderio, R. Scotti, M. Tresoldi, L. Roveri, A. Mariani, G. Balzano, R. Castoldi, C. Doglioni, M. G. Sabbadini, E. Della-Torre

Research output: Contribution to journalArticlepeer-review

Abstract

Objectives: To describe the clinical features, treatment response, and follow-up of a large cohort of Italian patients with immunoglobulin (Ig)G4-related disease (IgG4-RD) referred to a single tertiary care centre.Method: Clinical, laboratory, histological, and imaging features were retrospectively reviewed. IgG4-RD was classified as definite or possible according to international consensus guidelines and comprehensive diagnostic criteria for IgG4-RD. Disease activity was assessed by means of the IgG4-RD Responder Index (IgG4-RD RI).Results: Forty-one patients (15 females, 26 males) were included in this study: 26 with definite IgG4-RD and 15 with possible IgG4-RD. The median age at diagnosis was 62 years. The median follow-up was 36 months (IQR 24-51). A history of atopy was present in 30% of patients. The pancreas, retroperitoneum, and major salivary glands were the most frequently involved organs. Serum IgG4 levels were elevated in 68% of cases. Thirty-six patients were initially treated with glucocorticoids (GCs) to induce remission. IgG4-RD RI decreased from a median of 7.8 at baseline to 2.9 after 1 month of therapy. Relapse occurred in 19/41 patients (46%) and required additional immunosuppressive drugs to maintain long-term remission. Multiple flares occurred in a minority of patients. A single case of orbital pseudotumour did not respond to medical therapy and underwent surgical debulking.Conclusions: IgG4-RD is an elusive inflammatory disease to be considered in the differential diagnosis of isolated or multiple tumefactive lesions. Long-term disease control can be achieved with corticosteroids and immunosuppressive drugs in the majority of cases.

Original languageEnglish
Pages (from-to)135-145
Number of pages11
JournalScandinavian Journal of Rheumatology
Volume45
Issue number2
DOIs
Publication statusPublished - Mar 3 2016

ASJC Scopus subject areas

  • Rheumatology
  • Immunology
  • Immunology and Allergy

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