The Treatment of Lupus Pernio

Long-Term Treatment with Infliximab in Patients with Sarcoidosis

“…Previously designed tools to assess extrapulmonary organ involvement [29,30,31] have not been validated and/or standardized for the European population either, and thus, a validated and standardized tool to assess whether and to what extent an extrapulmonary organ is involved is still elusive. Our observation that 91% of patients with predominantly extrapulmonary sarcoidosis responded to infliximab treatment is in line with previously published data from case reports, retrospective chart analysis and non-randomized studies that demonstrated a high efficacy of infliximab for patients with refractory extrapulmonary sarcoidosis [5,12,19,20,21,23,25,26,27,29,32,33,34,35]. Importantly, our findings are supported by recently published data from a double-blind, randomized study demonstrating that infliximab therapy improved extrapulmonary sarcoidosis compared with placebo [29].…”

Refractory Chronic Cutaneous Sarcoidosis Responsive to Dose Escalation of TNF-Alpha Antagonists

“…In several studies and reports, infliximab and adalimumab were administered based upon the conventional dose regimen for psoriasis. For example, in 54 patients suffering from lupus pernio, a dose regimen of 5 mg/kg infliximab at weeks 0, 2, and 6 followed by an infusion every other month appeared superior to systemic corticosteroids with or without additional agents [7]. In contrast to previous reports, our patient illustrates that the conventional anti-TNF dose regimen may not be sufficient in some patients with cutaneous sarcoidosis.…”

“…In sarcoidosis, high levels of TNF-α are correlated with disease activity and progression [3]. Logically, the efficacy of TNF-α antagonists for the treatment of systemic and cutaneous sarcoidosis has been reported [4,5,6,7,8,9]. Infliximab and adalimumab are monoclonal antibodies directed against TNF-α that bind to free and cell surface TNF-α, while etanercept is a recombinant human TNF receptor fusion protein that antagonizes the effects of endogenous TNF-α by competitively inhibiting its interaction with cell surface receptors.…”

“…The mechanisms underlying the formation, maintenance, and spontaneous resolution of the sarcoid granuloma are not well understood but there are several pieces of evidence that tumor necrosis factor α (TNF-α) plays a crucial role [3]. Several case reports and small series have reported the efficacy of different anti-TNF-α agents in refractory cutaneous and systemic sarcoidosis [4,5,6,7,8,9]. The optimal dosing regimen has still to be defined for this indication before considering difficult-to-treat patients as nonresponders when on standard doses.…”

Sarcoidosis: treatments beyond prednisone and methotrexate

“…Favorable treatment responses in refractory sarcoidosis, sometimes dramatic, have been borne out by subsequent clinical experiences. Cutaneous and neurologic sarcoidosis seem to be especially responsive [25,26].…”