Arthritis & Rheumatism, Volume 63,
November 2011 Abstract Supplement
Abstracts of the American College of
Rheumatology/Association of Rheumatology Health Professionals
Annual Scientific Meeting
Chicago, Illinois November 4-9, 2011.
Eosinophilic Fasciitis (Shulman Disease): New Insights Into the Therapeutic Management From a Series of 34 Patients.
Lebeaux1, David, Frances2, Camille, Barete2, Stéphane, Wechsler1, Bertrand, Dubourg1, Odile, Renoux1, Jérôme, Maisonobe1, Thierry
Eosinophilic fasciitis (EF) is a rare connective tissue disease characterized by a symmetrical and painful swelling with a progressive induration the skin and soft tissues. The therapeutic management of EF is one of the most significant challenges, as there is consensus on the use of steroids (dose and duration) and the interest of immunosuppressive drugs (ISD). The aim of this study was to analyze and report the results of the therapeutic management of 34 patients with a biopsy-proven EF.
We reviewed 34 adult-patients with a biopsy-proven EF. The analyses focused on the clinical, biological and histological features and the therapeutic management, which included the treatment modalities, responses and associated prognostic factors.
1) Clinical, biological and histological features: Thirty-four patients were included with a diagnosis age of 53±15.4 years. They were featured by cutaneous manifestations (88%) including morphea (41%), myalgia (86%) and hypereosinophilia (85%). The inflammatory infiltrates included lymphocytes in all patients (100%) and eosinophils in 26 patients (76.5%). A fascia fibrosis was present in 14 patients (41.2%) and an interstitial myositis in 23 patients (67.6%). A dermal fibrosis (histological morphea) was present in 11/30 patients (36.7%).
2) Therapeutic management and results: Thirty-two patients (94%) were eligible for treatment evaluation and all received corticosteroids as a first line therapy with a prednisone mean daily dose at initiation of 0.77±0.29 mg/kg. Fifteen patients (47%) received methylprednisolone pulses prior to prednisone treatment. At the end of follow-up: (A) 18 patients (56.25%) received steroids alone with a 45±31 month-treatment mean duration; (B) due to the lack of a complete remission, 14 patients (43.75%) required an ISD as a second line therapy in association with the steroid treatment in a 16.9±20.3 month-mean time interval (median=6.5): methotrexate for 12 patients (85.7%) with a 24.7±23.3 month-treatment mean duration and azathioprine for two patients (14.3%).
A complete remission was achieved for 69% of patients, a remission with disability (non progressive sequelae) for 19% and failure for 12%. The lack of complete remission was associated with a diagnosis time delay above 6 months (OR=14.7) and the lack of methylprednisolone pulses (OR=12.9). The relative risk of requiring an ISD was 4.7 times and 4.4 times higher in presence of a morphea and in patients who did not receive methylprednisolone pulses, respectively. EF-associated morphea was associated with a more frequent ISD requirement, mostly methotrexate (OR=64.4)
Our study reports new insights into the therapeutic management of EF: 1) corticosteroids treatment remains the standard therapy for EF, taken alone or in association with an ISD; 2) methylprednisolone pulses at treatment initiation are associated with a better outcome and a lower need of ISD use; 3) an ISD, mostly methotrexate, might useful as a second line therapy, mainly in patients with morphea-like lesions. Naturally, these practical conclusions should be confirmed by a prospective and multicentre study.
To cite this abstract, please use the following information:
Lebeaux, David, Frances, Camille, Barete, Stéphane, Wechsler, Bertrand, Dubourg, Odile, Renoux, Jérôme, et al; Eosinophilic Fasciitis (Shulman Disease): New Insights Into the Therapeutic Management From a Series of 34 Patients. [abstract]. Arthritis Rheum 2011;63 Suppl 10 :1955