Background Sarcoidosis is a systemic granulomatous autoimmune disease. Clinically it may range from a mild to a severe life-threatening disease.
Objectives To assess the efficacy of anti-TNFα therapy in refractory and severe sarcoidosis.
Methods 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.
Results 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 In 1 patient etanercept (dose 50mg/ew) was initially used, in this case it had to been replaced by infliximab due to inefficacy.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 P. jirovecii, septic shock by P. aeruginosa and VV Zoster infection.
Conclusions Infliximab and adalimumab appear to be effective and safe drugs in the management of severe and refractory sarcoidosis. Further controlled studies are warranted.
Disclosure of Interest None Declared