Efficacy and Tolerance of Anti–Tumor Necrosis Factor α Agents in Cutaneous Sarcoidosis

Abstract

IMPORTANCEEvidence for the long-term efficacy and safety of anti-tumor necrosis factor α agents (anti-TNF) in treating cutaneous sarcoidosis is lacking.OBJECTIVE To determine the efficacy and safety of anti-TNF in treating cutaneous sarcoidosis in a large observational study.DESIGN, SETTING, AND PARTICIPANTS STAT (Sarcoidosis Treated with Anti-TNF) is a French retrospective and prospective multicenter observational database that receives data from teaching hospitals and referral centers, as well as several pneumology, dermatology, and internal medicine departments. Included patients had histologically proven sarcoidosis and received anti-TNF between January 2004 and January 2016. We extracted data for patients with skin involvement at anti-TNF initiation. MAIN OUTCOMES AND MEASURESResponse to treatment was evaluated for skin and visceral involvement using the ePOST (extra-pulmonary Physician Organ Severity Tool) severity score (from 0 [not affected] to 6 [very severe involvement]). Epidemiological and cutaneous features at baseline, efficacy, steroid-sparing, safety, and relapses were recorded. The overall cutaneous response rate (OCRR) was defined as complete (final cutaneous ePOST score of 0 or 1) or partial response (ePOST drop Ն2 points from baseline but >1 at last follow-up).RESULTS Among 140 patients in the STAT database, 46 had skin involvement. The most frequent lesions were lupus pernio (n = 21 [46%]) and nodules (n = 20 [43%]). The median cutaneous severity score was 5 and/or 6 at baseline. Twenty-one patients were treated for skin involvement and 25 patients for visceral involvement. Reasons for initiating anti-TNF were failure or adverse effects of previous therapy in 42 patients (93%). Most patients received infliximab (n = 40 [87%]), with systemic steroids in 28 cases (61%) and immunosuppressants in 32 cases (69.5%). The median (range) follow-up was 45 (3-103) months. Of the 46 patients with sarcoidosis and skin involvement who were treated with anti-TNF were included, median (range) age was 50 (14-78) years, and 33 patients (72%) were women. The OCRR was 24% after 3 months, 46% after 6 months, and 79% after 12 months. Steroid sparing was significant. Treatment was discontinued because of adverse events in 11 patients (24%), and 21 infectious events occurred in 14 patients (30%). Infections were more frequent in patients treated for visceral involvement than in those treated for skin involvement (n = 12 of 25 [48%] vs n = 2 of 21 [9.5%], respectively; P = .02). The relapse rate was 44% 18 months after discontinuation of treatment. Relapses during treatment occurred in 35% of cases, mostly during anti-TNF or concomitant treatment tapering.CONCLUSIONS AND RELEVANCE Anti-TNF agents are effective but suspensive in cutaneous sarcoidosis. The risk of infectious events must be considered.

Erfolgreiche Therapie einer Sarkoidose mit TNF-alpha-Antagonisten

“…Der langfristige Einsatz von Glukokortikoiden zur Behandlung von Sarkoidose ist mit erheblichen Nebenwirkungen verbunden, daher ist eine kurzfristige Behandlung nur dann angezeigt, wenn klinische Symptome bestehen bzw. die granulomatöse Entzün-dung rasch fortschreitet [12]. Um folgenschwere Nebenwirkungen von langfristigen Glucokortikoidgaben zu vermeiden, sollte bei einer chronischen Sarkoidose eine Kortison-sparende Therapie erwogen werden.…”

Biologic Therapies in Sarcoidosis and Uveitis: A Review

“…Sarcoidosis is a systemic inflammatory disorder wherein the central feature is the formation and propagation of granulomas in affected organs. It has a prevalence of 10 per 100,000 people in the United States, with the incidence and prevalence being higher in African American populations compared to Asians, Hispanics, and Caucasians [ 1 ]. Pulmonary involvement in sarcoidosis is a cardinal feature, however, extra-pulmonary manifestations have been reported in 10-30% of cases [ 2 ].…”

Cutaneous sarcoidosis

“…[ 2 ] The recent studies reported the rate of cutaneous involvement up to 30% in Germany and about 33% in France. [ 3 , 4 ]…”