Rheumatology (Oxford, England) · 2011
Eosinophilic fasciitis (Shulman disease): new insights into the therapeutic management from a series of 34 patients.
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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
Аннотация
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.
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.
Предметные рубрики MeSH
AdultAgedAntirheumatic AgentsAzathioprineColchicineDrug Therapy, CombinationEosinophiliaFemaleGlucocorticoidsHumansHydroxychloroquineImmunosuppressive AgentsMaleMethotrexateMethylprednisoloneMiddle AgedPrednisonePrognosisRemission InductionRetrospective StudiesScleroderma, LocalizedSynovitisTreatment Outcome