Outcome of refractory to conventional and/or biologic treatment adult Still's disease following canakinumab treatment: Countrywide data in 50 patients.

OBJECTIVE: To assess the efficacy and safety of the IL-1b inhibitor canakinumab in all adults with refractory Still's disease identified from the National Organization For Medicines for off-label drug use. METHODS: In a retrospective longitudinal multicenter cohort of 50 patients (median age 39 years) with active Still's disease despite treatment with corticosteroids (n = 11), conventional and synthetic (n = 34) and/or biologic disease modifying anti-rheumatic drugs (n = 30), we assessed the efficacy of canakinumab 150-300 mg administered every 4 (n = 47) or 8 weeks (n = 3) as combination therapy or monotherapy (n = 7) during a median follow-up of 27 (3-84) months. RESULTS: Α complete response was initially observed in 78% of patients within 3 months (median), irrespective of age at disease onset. A partial response was evident in 20%. One patient had resistant disease. Treatment de-escalation was attempted in 15 of 39 complete responders and a complete drug discontinuation in 21 patients for 8 months (median). Eleven patients (22%) relapsed during treatment, one during de-escalation process, and 11 after treatment discontinuation. Overall, 9 of 11 relapses were successfully treated with canakinumab treatment intensification or re-introduction. At last visit, 18% of patients were off treatment due to remission and 26% due to disease activity. Canakinumab had a significant corticosteroid sparing effect allowing weaning in 21 of 41 cases. Infections (20%, severe 4%) and leucopenia (6%) led to treatment cessation in one patient. CONCLUSION: High rates of sustained remission were observed in this, largest so far, real-life cohort of adult patients with refractory Still's disease treated with canakinumab.

Exacerbation of interstitial granulomatous dermatitis with arthritis by anakinra in a patient with diffuse large B-cell lymphoma.

Interstitial granulomatous dermatitis with arthritis (IGDA) is a rare idiopathic skin disorder with variable cutaneous expression, typically associated with a seronegative arthritis. Histopathology of this disorder reveals a granulomatous infiltrate with foci of collagen degeneration in the deep reticular dermis. We present a case of IGDA in a 56-year-old man with diffuse large B-cell lymphoma, exacerbated by the administration of anakinra.

Extended fungal skin infection due to Aureobasidium pullulans.

Black yeasts are a rare cause of infections especially in Europe, yet their pathological significance is increasing, particularly in cases of immunosuppression. We report a 53-year-old immunocompetent woman with an extensive skin infection due to Aureobasidium pullulans, who responded well to treatment with liposomal amphotericin B.

Evidence-based recommendations for the management of ankylosing spondylitis: results of the Hellenic working group of the 3E Initiative in Rheumatology.

OBJECTIVE: The 3E (Evidence, Experts and Exchange) Initiative is a multi-national effort that involves a large number of experts and practicing rheumatologists addressing specific questions relevant to everyday clinical practice, concerning the management of Ankylosing Spondylitis. Within this multinational group, the Hellenic working group, addressed specific issues complementary to the international ones, and formulated evidence-based recommendations, in order to improve everyday clinical practice for patients with Ankylosing Spondylitis. METHODS: A scientific committee of rheumatologists specializing in AS formulated a set of 7 questions in three domains: diagnosis, monitoring and treatment. Literature search in MedLine for papers published up to August 2006 was conducted. The evidence to support each proposition was evaluated and scored. To avoid any conflict of interest with the sponsor issues related to the use of biologics were not discussed. After extensive discussion among 50 rheumatologists and one Delphi round of votes, the final recommendations were formulated. RESULTS: A literature search resulted in a total of 320 relevant papers of which 29 were evaluated. A total of seven recommendations were formulated: two concerning diagnosis (role of HLA-B27 and MRI) and prognosis, one concerning monitoring for extra-articular manifestations and four concerning treatment (analgesics, disease modifying agents and physical therapy) were made. The level of evidence and the strength of recommendation were reported. The compiled agreement among experts ranged from 90% up to 100%. CONCLUSION: Recommendations for the management of AS were developed using an evidence-based approach followed by physicians' consensus with high level of agreement. These are complementary to existing ones, and address specific domains of everyday clinical practice.

Adenocarcinoma of the colon presenting as polymyositis: case report.

The relationship between polymyositis and malignancy is well known. Several types of tumors can be complicated with myositis and/or more frequently dermatomyositis. It has been suggested that tumors of the large bowel are rarely complicated by myositis. We describe a patient with adenocarcinoma of the colon presenting as polymyositis and review the available literature.