Details

Autor(en) / Beteiligte

• Lebeaux, David
• Francès, Camille
• Barete, Stéphane
• Wechsler, Bertrand
• Dubourg, Odile
• Renoux, Jérôme
• Maisonobe, Thierry
• Benveniste, Olivier
• Gatfossé, Marc
• Bourgeois, Pierre
• Amoura, Zahir
• Cacoub, Patrice
• Piette, Jean-Charles
• Sène, Damien

Titel

Eosinophilic fasciitis (Shulman disease): new insights into the therapeutic management from a series of 34 patients

Ist Teil von

• Rheumatology (Oxford, England), 2012-03, Vol.51 (3), p.557-561

Ort / Verlag

Oxford: Oxford University Press

Erscheinungsjahr

2012

Link zum Volltext

Quelle

Oxford Journals 2020 Medicine

Beschreibungen/Notizen

• Objective. To analyse therapeutic management of eosinophilic fasciitis (EF). Methods. We reviewed 34 adult patients with biopsy-proven EF. Analyses focused on the therapeutic management, including treatment modalities, responses and associated or predictive factors. Results. Thirty-four patients were included with a diagnosis age of 53 (15) years. They were featured by cutaneous manifestations (88%) including morphoea (41%), myalgia (86%) and hypereosinophilia (85%). Thirty-two patients (94%) were eligible for treatment evaluation and all received CSs as a first-line therapy. Fifteen patients (47%) received methylprednisolone pulses (MPPs) at treatment initiation and 14 patients (44%) received an immunosuppressive drug (ISD), usually MTX (86%), as a second-line therapy. Complete remission was achieved for 69% of patients, remission with disability 19% and failure 12%. A poor outcome was associated with a diagnosis time delay of >6 months [odds ratio (OR) = 14.7] and the lack of MPPs (OR = 12.9). Conclusion. Our study reports new insights into the therapeutic management of EF: (i) CS treatment remains the standard therapy for EF, taken alone or in association with an ISD; (ii) MPPs at initiation of treatment are associated with a better outcome and a lower need of ISD use; (iii) an ISD, usually MTX, might be useful as a second-line therapy, mainly in patients with morphoea-like lesions. Naturally, these practical conclusions should be confirmed by a prospective and multicentre study.