Refractory multisystem sarcoidosis responding to infliximab therapy

Abstract

Chronic progressive multisystem granulomatous disease is seen in 10-30% of patients with sarcoidosis and can result in end organ damage. Corticosteroids are the mainstay of treatment with the addition of cytotoxic agents in severe cases. Some patients are refractory to such treatment and, therefore, management is a challenge. There is currently limited evidence for biological agents such as infliximab, a monoclonal anti-tumor necrosis factor-α antibody in the treatment of multisystem sarcoidosis. We report outcomes of three patients with extensive multisystem sarcoidosis refractory to conventional treatment and treated at our center. Clinical assessment and radiographic imaging were used to assess the response to infliximab treatment. Infliximab therapy induced clinical remission in all three patients, and this clinical response correlated with radiographic evidence of the resolution of granulomatous disease. Serum ACE level was reduced in all cases, and daily steroid dosage was reduced. We propose that infliximab can be an effective treatment in patients with multisystem complex sarcoidosis refractory to conventional drug therapy and can result in sustained clinical remission. Our experience supports the urgent need for randomized controlled clinical trials of anti-TNF therapy in refractory systemic sarcoidosis.

Association of the TNF-α G-308A polymorphism with TNF-inhibitor response in sarcoidosis

“…The results of our study are in line with other case series demonstrating efficacy of infliximab in 64-100% of sarcoidosis patients treated [12,30,31]. In the present study, uveitis demonstrated the best response rate (74%), followed by RFI (57%) and SFN (54%).…”

Refractory multisystem sarcoidosis involving pelvic bone responding to infliximab

“…There is currently limited evidence for biological agents such as infliximab, a monoclonal anti–TNF α antibody in the treatment of multisystem sarcoidosis [1]. Our case supports the need for randomized controlled clinical trials of anti-TNF therapy in refractory systemic sarcoidosis.…”

“…Chronic progressive multisystem granulomatous disease is seen in 10–30% of patients with sarcoidosis and can result in end organ damage [1]. Corticosteroids are the mainstay of treatment with the addition of cytotoxic agents in severe cases.…”

Infliximab in recalcitrant granuloma annulare

“…Mainly interferon-c and interleukin-2 are secreted, and TNF-a production is increased through macrophage activation. By analogy with the treatment used in other granulomatous diseases (sarcoidosis, necrobiosis lipoidica), treatment with anti-TNF-a was considered to represent a reasonable option in GA. 5 instances of the treatment of GA with anti-TNF have been reported, including one case with etanercept, 6 four with adalimumab, 7,8 and two with infliximab. 9,10 The present case is remarkable because clinical improvement was observed after one infusion only, and the complete disappearance of lesions was apparent after the fourth infusion.…”