Journal List > J Rheum Dis > v.27(2) > 1144357

Lee, Kim, Song, and Park: Combination of Methotrexate and Leflunomide for Adult-onset Still's Disease: A Case Report and Literature Review

Abstract

The treatment of adult-onset Still's disease (AOSD) aims to control systemic inflammation and prevent organ damage. Systemic inflammation can be controlled with corticosteroid (CS) monotherapy in most cases. However, symptoms often flare as CS is tapered, often requiring long-term CS treatment, with its associated risks of infection, cardiovascular disease, and osteoporosis. Disease-modifying antirheumatic drugs (DMARDs) are often used as CS-sparing agents; however, the choice of DMARD has been largely empirical. Methotrexate (MTX) is recommended as the first-line steroid-sparing drug due to its well-known efficacy and safety in rheumatoid arthritis (RA). When MTX treatment is unsuccessful in AOSD, the choice of a second-line drug has not been established. In RA, leflunomide (LEF) has been used as an alternative to or in combination with MTX. To date, there has been no adequate assessment of the combination of LEF and MTX in AOSD. Here, we report a case of refractory chronic AOSD successfully treated with the MTX-LEF combination.

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Figure 1.
Changes in laboratory test results of the patient after a diagnosis of AOSD. Asterisk represents flare. AOSD: adult-onset Still's disease, ESR: erythrocyte sedimentation rate, hs-CRP: high sensitivity C-reactive protein, WBC: white blood cell, LEF: leflunomide, MTX: methotrexate.
jrd-27-116f1.tif
Table 1.
Summary of the literature
Publication No. of cases Age/Sex Clinical features Treatment prior to LEF Treatment with remission
Present report 1 42/male Fever, arthralgia, pericarditis, sore throat, lymphadenopathy, splenomegaly PD 60 mg→20 mg/day, MTX MTX+LEF
Cefle et al., 2005 [5] 1 24/female Fever, arthritis, sore throat, rash, hepatosplenomegaly, lymphadenopathy mPD 1g/day for 3 days, indomethacin, HCQ, MTX, AZA, IV CTX AZA+LEF
Pirildar et al., 2003 [6] 2 Pericardial effusion CS Chloroquine+LEF
Sayarlioglu et al., 2007 [7] 1 19/male Fever, rash, sore throat, polyarthralgia/arthritis, hepatomegaly PD 1 mg/kg/day, MTX, indomethacin, sulfasalazine MTX+LEF

LEF: leflunomide, PD: prednisolone, mPD: methylprednisolone, HCQ: hydroxychloroquine, MTX: methotrexate, AZA: azathioprine, IV: intravenous, CTX: cyclophosphamide, CS: corticosteroid.

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