THU0391 Anti-TNF therapy in severe and refractory sarcoidosis: Multicenter study of 25 patients
Authors: I. Pérez-Martín, R. Blanco, N. Ortego, V. Llorens-Bellés, A. Adán, A. Olivé, W.A. Sifuentes Giraldo, S. González, C. Bejerano, M.C. González-Vela, M.A. González-Lόpez, H. Fernández-Llaca, A. Oterino, M.J. Sedano, M. Agudo, A. Corrales, V.M. Martínez-Taboada, M.A. Gόnzalez-Gay
Journal: Annals of the Rheumatic Diseases (2013)

<h3>Background</h3> Sarcoidosis is a systemic granulomatous autoimmune disease. Clinically it may range from a mild to a severe life-threatening disease. <h3>Objectives</h3> To assess the efficacy of anti-TNFα therapy in refractory and severe sarcoidosis. <h3>Methods</h3> We reviewed the medical records of patients seen at several hospitals that were diagnosed as having sarcoidosis and treated with anti-TNFα therapy because of disease severity. <h3>Results</h3> We assessed 25 patients (14 women/11 men); mean age at the beginning of anti-TNFα therapy: 47±14 years (range: 27-69); mean duration of sarcoidosis before the onset of anti-TNFα therapy 66±74 months (range 3-360). The main clinical complications that made necessary the use of anti-TNFα agents were: uveitis (9 patients), neurosarcoidosis (6 patients), arthritis (3 patients), skin-lupus pernio (2 patients), lung disease (2 patients), bone marrow involvement-pancitopenia (1 case), systemic-myopathy (1 case), and aortitis (1 case). Most patients had been refractory to oral corticosteroids and at least one immunosuppressive drug. However, in 3 patients with severe neurosarcoidosis anti-TNFα agents were prescribed along with corticosteroids as the initial therapy. In the remaining 22 patients, besides high-dose prednisone, previously to anti-TNFα therapy they had received the following drugs: i.v. methylprednisolone (500-1000 mg for 3 consecutive days) (4 cases), methotrexate (16 cases), azathioprine (3 cases), hydroxychloroquine (2 cases), cyclosporine (1 case), and sulphasalazine (1 cases). Anti-TNFα drugs were usually associated to an immunosuppressive agent (methotrexate, or azathioprine). Infliximab was the most commonly initial anti-TNFα drug used in this series- in 16 cases (3-5 mg/kg/i.v. at 0, 2, 6 and then every 4-8 weeks). Adalimumab was initially administered in the other 8 patients (40 mg/sc EOW or EW if necessary). In 1 patient etanercept (dose 50mg/ew) was initially used, in this case it had to been replaced by infliximab due to inefficacy. Infliximab was discontinued in 5 cases because of inefficacy and in another 2 due to adverse events (severe rash and gastrointestinal intolerance, respectively). In these 5 cases, infliximab was switched to adalimumab. Adalimumab was discontinued in 1 of 8 cases because of development of a lupus-like syndrome. After a mean time of anti-TNFα therapy of 19,8±19,8 months (range 1-72), complete clinical remission was achieved in 14 cases and partial improvement in 6 patients. Of major importance, in most patients anti-TNFα treatment allowed the withdrawal or a significant reduction of corticosteroid therapy. The most common adverse events were infections. Three of them were severe: pneumonia by <i>P. jirovecii</i>, septic shock by <i>P. aeruginosa</i> and VV Zoster infection. <h3>Conclusions</h3> Infliximab and adalimumab appear to be effective and safe drugs in the management of severe and refractory sarcoidosis. Further controlled studies are warranted. <h3>Disclosure of Interest</h3> None Declared