title: Eosinophilic fasciitis (Shulman disease): new insights into the therapeutic management from a series of 34 patients.
abstract: 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).
  
  
  CONCLUSIONS
  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.
authors: Hôpital Pitié-Salpêtrière, Université Pierre et Marie Curie-Paris 6, Paris, France.
authors: David
authors: D
authors: Lebeaux
authors: Camille
authors: C
authors: Francès
authors: Stéphane
authors: S
authors: Barete
authors: Bertrand
authors: B
authors: Wechsler
authors: Odile
authors: O
authors: Dubourg
authors: Jérôme
authors: J
authors: Renoux
authors: Thierry
authors: T
authors: Maisonobe
authors: Olivier
authors: O
authors: Benveniste
authors: Marc
authors: M
authors: Gatfossé
authors: Pierre
authors: P
authors: Bourgeois
authors: Zahir
authors: Z
authors: Amoura
authors: Patrice
authors: P
authors: Cacoub
authors: Jean-Charles
authors: JC
authors: Piette
authors: Damien
authors: D
authors: Sène
mesh_terms: D000328:Adult; D000368:Aged; D018501:Antirheumatic Agents / Q000008:administration & dosage*; D001379:Azathioprine / Q000008:administration & dosage; D003078:Colchicine / Q000008:administration & dosage; D004359:Drug Therapy, Combination; D004802:Eosinophilia; D005260:Female; D005938:Glucocorticoids / Q000008:administration & dosage*; D006801:Humans; D006886:Hydroxychloroquine / Q000008:administration & dosage; D007166:Immunosuppressive Agents / Q000008:administration & dosage*; D008297:Male; D008727:Methotrexate / Q000008:administration & dosage; D008775:Methylprednisolone / Q000008:administration & dosage; D008875:Middle Aged; D011241:Prednisone / Q000008:administration & dosage; D011379:Prognosis; D012074:Remission Induction; D012189:Retrospective Studies; D012594:Scleroderma, Localized / Q000188:drug therapy / Q000209:etiology; D013585:Synovitis / Q000150:complications / Q000188:drug therapy*; D016896:Treatment Outcome
chemical_list: D018501:Antirheumatic Agents; D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D006886:Hydroxychloroquine; D001379:Azathioprine; D003078:Colchicine; D011241:Prednisone; D008775:Methylprednisolone; D008727:Methotrexate