THU0365 Igg4-Related Disease Spanish Register: Description of Treatment and Evolution
Authors: A. Fernández-Codina, F. Martínez-Valle
Journal: Annals of the Rheumatic Diseases (2014)

<h3>Background</h3> IgG4-related disease is a rare entity consisting of inflammation and fibrosis that has been described in many locations. Diagnosis may be challenging and concrete diagnostic criteria have been established recently. <h3>Objectives</h3> To describe the therapeutic experience and the evolution of a series of patients diagnosed with IgG4-related disease (IgG4-RD) in Spain. <h3>Methods</h3> A retrospective multicentric study was performed. A clinical protocol and a joint database were created to constitute the IgG4-related Disease Spanish Register. Patients were enrolled from 14 different centers. Pathologic studies were conducted according to Deshpande et al.<sup>1</sup> diagnostic criteria (histologically highly suggestive or probable IgG4-RD). Patients with insufficient histopathological evidence of IgG4-RD were excluded. <h3>Results</h3> Thirty-five patients were included in the study, 27 of whom (77.1%) were male. Median age at diagnosis was 55 years (IQR 29 years). Eighteen patients (51.4%) presented IgG4-RD with more than one organ affected. There were no differences regarding the treatments received between localized and multi-organic IgG4-RD subjects. Corticosteroids were administered in 30 (85.7%) patients. The median maximum dose was 60 mg/day (IQR 30 mg/day) during 1 month (IQR 0.25 months). Corticosteroid tapering lasted 7.5 months (IQR 8.5 months), although 7 patients kept a low maintenance daily dosage of 5 mg/day (IQR 5 mg/day). In 17 (48.5%) cases, immunosupressors were added due to corticosteroid resistance (sustained disease activity, progression of clinical symptoms or flares when downscaling steroids): mofetil mycophenolate/mycophenolic acid in 6 patients (17.1%), azathioprine in 7 (20%) and cyclophosphamide, azathioprine plus methotrexate, and azathioprine plus cyclophophamide in 1 (2.9%) patient each. Biologic treatment with rituximab was given to 2 (5.7%) patients. Six (17.1%) subjects underwent radical surgery. Median follow up was 22.01 months (IQR 35.79 months). Eleven (31.4%) patients presented a complete response (complete regression of the fibrotic mass/masses) and 22 (62.9%) presented a partial response (&lt;50% of regression). Corticosteroids alone were more frequently given in the complete response group. No patients were refractory to all treatments, and 12 (34.3%) presented recurrences. One patient was asymptomatic and did not require any treatment, and one patient did not have avalable treatment records. During follow-up, one subject (2.9%) died in a car accident, two (6.1%) developed a monoclonal gammopathy of unknown significance and one (3%) developed a prostate cancer. <h3>Conclusions</h3> IgG4 related disease is a rare entity in our country. The most common treatment regime was corticosteroids, initially at high doses, followed by a slow descent of doses, with or without immunosupressors. Rituximab was the only biological drug used. Surgery may be needed occasionally. A partial disappearance of the fibrotic component with resolution of systemic symptoms was the most frequent evolution after treatment. No deceases related to IgG4-RD were reported. More collaborative studies are required in order to assess the treatment response and the evolution of IgG4-RD. <h3>References</h3> Deshpande V, Zen Y, Chan JK, et al. Consensus statement on the pathology of IgG4-related disease. Mod Pathol. 2013;26(8):1150-1. <h3>Disclosure of Interest</h3> : None declared <h3>DOI</h3> 10.1136/annrheumdis-2014-eular.2664