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THU0543 IGG4-Related Disease in Italy. Clinical Features and Outcomes of a Large Cohort of Patients
  1. C. Campochiaro,
  2. G. Ramirez,
  3. E. Bozzolo,
  4. M. Lanzillotta,
  5. A. Berti,
  6. E. Baldissera,
  7. L. Dagna,
  8. L. Praderio,
  9. R. Scotti,
  10. M. Tresoldi,
  11. M.G. Sabbadini,
  12. E. Della Torre
  1. Internal Medicine and Clinical Immunology, IRCCS San Raffaele, Milan, Italy

Abstract

Background IgG4-related disease (IgG4-RD) is a rare fibro-inflammatory condition characterized by expansive lesions, serum IgG4 elevation in the majority of cases, and a prompt response to glucocorticoids (GCs) [1]. The diagnosis of IgG4-RD might be challenging because clinical manifestations are largely non-specific and overlap with other inflammatory and neoplastic conditions. The current gold standard for the diagnosis of IgG4-RD is the identification of characteristic histological features that are strikingly similar across the full range of organs affected. These features include a dense tissue fibrosis with a storiform pattern, a diffuse lymphoplasmacytic infiltrate with abundant IgG4-positive plasma cells, mild to moderate eosinophilia, and obliterative phlebitis [2]. GCs represent the first-line treatment for induction of remission, and typically lead to dramatic clinical responses in the majority of cases with both pancreatic and extra-pancreatic disease [3].

Objectives To describe the clinical features, treatment response and follow-up of a large cohort of Italian patients with IgG4-related disease (IgG4-RD) referred to a single tertiary care centre.

Methods 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 (14 females, 27 males) were included in this study, 20 with “definite” IgG4-RD, and 21 with “possible” IgG4-RD. The mean age at disease onset was 62 years. The mean follow-up was 34 months (range 6-83 months). A history of atopy was present in 30% of patients. Pancreas, retroperitoneum, and major salivary glands were the most frequently involved organs. Clinical characteristics of the patients' cohort at IgG4-RD onset are summerized in table 1. Serum IgG4 were elevated in 68% of cases. Thirty-six patients were initially treated with glucocorticoids for inducing remission. IgG4-RD RI decreased from a mean of 7.8 at baseline to 2.9 after 1 month of therapy. Relapse occurred in 19/36 patients (53%) and required additional immunosuppressive drugs to maintain long-term remission. No further relapses were observed. A single case of orbital pseudotumor 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.

References

  1. Stone JH, Zen Y, Deshpande V. IgG4-related disease. N Engl J Med 2012;366:539–51.

  2. Deshpande V, Zen Y, Chan JK, et al. Consensus statement on the pathology of IgG4-related disease. Mod Pathol 2012;25(9):1181-92.

  3. Kamisawa T, Okazaki K, Kawa S, et al. Japanese consensus guidelines for management of autoimmune pancreatitis. III. Treatment and prognosis of AIP. J Gastroenterol 2010;45:471-7.

Disclosure of Interest None declared

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