FRI0492 Eosinophilic Fasciitis (Shulman's Disease): Study of 70 Cases

Background Eosinophilic fasciitis (EF) is a rare sclerodermiform syndrome that was described in 1974 by Shulman. It is characterized by abrupt onset of edema and stiffness accompanied by eosinophilia. Objectives To describe the spectrum of clinical manifestations, treatment and outcome of patients with EF. Methods Observational, retrospective (1983-2014) and multicentric study: 5 urban University Hospitals participated. Inclusion criteria: 1) characteristic skin manifestations, 2) deep biopsy with compatible changes in the muscle fascia. Results A total of 70 patients were included, 41 males (58.6%), mean age: 53.8 years (range: 22-83) and median duration of disease until diagnosis: 4 months (range: 1-38). Previous medical history was relevant, namely: 25 (35.7%) were smokers, 13 (18.6%) had a previous intense physical activity, 9 (12.8%) had changes in their medication and 8 (11.4%) had occupational exposure to chemicals. Prodromal constitutional symptoms were present in 17 cases (24.3%). Skin induration appeared in 68 (97%), with involvement of 4 limbs in 47 (67%) and trunk in 24 (34.2%). Hyperpigmentation was found in 21 (30%), erythema 19 (27%), groove sign in 14 (20%) and “peau d9orange” in 12 (17%). Interstingly 15 patients (21.4%) showed cutaneous localized morphea. The most common extra-cutaneous manifestations were musculoskeletal: arthralgia 36 (51.4%), arthritis 11 (16%), joint contractures 31 (28.6%), myalgia 32 (46%) and weakness 12 (17%). Thirteen patients (18.6%) developed carpal tunnel syndrome, 7 (10%) esophageal dysmotility and 3 (4.3%) restrictive lung impairment. Eosinophilia (>0.5x10 6 /mL) was observed in 58 (83%), hypergammaglobulinemia in 17 (27%), and increased ESR and CRP levels in 37 (60%) and 25 (71.4%), respectively. ANA were positive in 18 (28%) and anti-ENA in 3 (2 anti-Ro, 1 anti-centromere). A First biopsy was negative in 6 cases (8.6%), but diagnosis was confirmed with a second biopsy guided by MRI Glucocorticoids (GC) were administered as initial treatment in 65 (93%) at a mean dose of 50 mg/d (range: 7.5-90), with partial or inadequate response in 42 (65%). Methotrexate (MTX) was the most common immunosuppressant drug (33: 43%), mean dose 15 mg/week. Complete remission was achieved in 15 patients and partial responese in 12. Other drugs used included: anti-H2 (18: 26%), colchicine (10: 14.3%), hydroxychloroquine (8: 11.4%), azathioprine (5: 7%), cyclosporine (2: 3%) and PUVA photochemotherapy (6: 8.6%). Mean follow-up was 84.8 months (range: 1-336). During this period 6 patients developed malignancies (8.5%) and 11 died (16%), 4 of them due to infections, 3 progression of malignancy, 3 cardiovascular disease (CVD) and 1 aplastic anemia. Conclusions The clinical pattern of EF presented in this series is similar to other series. We would like to highlight the high frequency of extra-cutaneous manifestations and the association with morphea. GC were the most common initial treatment, but the response was partial in most cases requiring additional immunosuppressive, being MTX the most frequently choice. During long-term follow-up some patients developed cancer and CVD leading to mortality. Disclosure of Interest None declared