Efficacy of Anakinra in Refractory Adult-Onset Still's Disease: Multicenter Study of 41 Patients and Literature Review.

Adult-onset Still's disease (AOSD) is often refractory to standard therapy. Anakinra (ANK), an interleukin-1 receptor antagonist, has demonstrated efficacy in single cases and small series of AOSD. We assessed the efficacy of ANK in a series of AOSD patients. Multicenter retrospective open-label study. ANK was used due to lack of efficacy to standard synthetic immunosuppressive drugs and in some cases also to at least 1 biologic agent. Forty-one patients (26 women/15 men) were recruited. They had a mean age of 34.4 ± 14 years and a median [interquartile range (IQR)] AOSD duration of 3.5 [2-6] years before ANK onset. At that time the most common clinical features were joint manifestations 87.8%, fever 78%, and cutaneous rash 58.5%. ANK yielded rapid and maintained clinical and laboratory improvement. After 1 year of therapy, the frequency of joint and cutaneous manifestations had decreased to 41.5% and to 7.3% respectively, fever from 78% to 14.6%, anemia from 56.1% to 9.8%, and lymphadenopathy from 26.8% to 4.9%. A dramatic improvement of laboratory parameters was also achieved. The median [IQR] prednisone dose was also reduced from 20 [11.3-47.5] mg/day at ANK onset to 5 [0-10] at 12 months. After a median [IQR] follow-up of 16 [5-50] months, the most important side effects were cutaneous manifestations (n = 8), mild leukopenia (n = 3), myopathy (n = 1), and infections (n = 5). ANK is associated with rapid and maintained clinical and laboratory improvement, even in nonresponders to other biologic agents. However, joint manifestations are more refractory than the systemic manifestations.

Clinical features and predictive factors in psoriatic arthritis-related uveitis.

OBJECTIVES: To analyze the clinical features of uveitis in psoriatic arthritis (PsA) and to investigate the factors predicting its appearance. PATIENTS AND METHODS: Retrospective cohort study (1991-2000) of 71 patients diagnosed with PsA according to the criteria of Moll and Wright. All patients were studied according to a standard protocol. The group was divided into 3 articular categories: axial, oligoarticular, and polyarticular. Human leukocyte antigen (HLA)-Cw typing was performed by the polymerase chain reaction-sequence specific oligonucleotide probes (PCR-SSOP) method in 65 patients and in 177 healthy donors. HLA-DR typing was done by serologic methods in the 71 patients and in 82 healthy donors from the same racial and geographic origin. The HLA-B27 allele also was tested among the study population. All subjects with possible inflammatory ocular disease received a complete ophthalmologic examination at the Ophthalmology Department of our hospital. Only patients with uveitis were analyzed. Univariate and multivariate analyses were applied. RESULTS: Thirteen patients had uveitis (18% of this series), 4 (31%) had an insidious onset, and the remaining had acute-onset uveitis. Five cases (39%) had bilateral-simultaneous uveitis. Ten (77%) presented with anterior uveitis only, 2 with anterior and posterior pole involvement, and only 1 case with isolated posterior pole involvement. Four patients needed oral corticosteroids; 2 of them also used immunosuppresive drugs. None of our patients developed sequelae or complications. In univariate analysis, uveitis was associated with inflammatory back pain (P =.02), sacroiliac pain (P =.001), syndesmophytes (P =.001), bilateral sacroileitis (P =.0001), HLA-DR13 (P =.002), and HLA-B27 (P =.026). In multivariate analysis, the predictive factors for uveitis were bilateral sacroileitis (OR 17, 95% CI: 3.7-76, P =.0002), HLA-DR13 (OR 24, 95% CI: 3.78-150, P =.0056), and syndesmophytes (OR 9.7, 95% CI: 0.97-97, P =.05). CONCLUSIONS: Insidious onset, bilaterality, posterior pole involvement, and chronicity are common in PsA patients with uveitis. In this study, extensive axial involvement (bilateral sacroileitis and syndesmophytes), and the HLA-DR13 antigen were the best predictors for the appearance of uveitis.