Etanercept treatment of cutaneous granulomas in common variable immunodeficiency

Common variable immunodeficiency-associated myelitis

“…Like other antibody deficiency states, CVID may present with recurrent bacterial infections but it may also be accompanied by unexplained inflammatory disease often characterized by granulomas. Noncaseating granulomas similar to those seen in sarcoidosis can be seen in 5 to 10 % of patients with CVID [1,4,6,9,[12][13][14]; caseating granulomas have rarely been reported [5, 15, 16]. Patients with granulomatous complications may have increased serum TNF-α levels [17].…”

“…A rare complication of CVID is neurological involvement presenting as a myelopathy [3]. There are increasing reports of refractory CVID being treated with TNF-α inhibitors [4][5][6][7][8][9][10]. In none of these reports has CNS involvement been the primary and the plantar response was extensor.…”

A review on guidelines for management and treatment of common variable immunodeficiency

“…+ cells and lack of CD4 + cells has already been shown to be associated with the granuloma formation in tissues [132][133][134]. Genetic predisposition may have a role in different aspects of granulomatous diseases in CVID patients.…”

“…All mentioned therapeutic modalities are used in case reports or limited studies and their results for extrapolation to CVID should be tested in clinical trials. Monoclonal antibodies as TNF-α inhibitors, such as etanercept [132,142] and infliximab [143][144][145][146], can be used in the case of generalized granulomatous lesions combined with autoimmunity, but no systematic clinical trials have investigated their efficacy and safety. Opportunistic infections like Pneumocystis jirovecii pneumonia may result when patients are under treatment for lymphoproliferative diseases [52,115].…”

Enhanced formation of giant cells in common variable immunodeficiency: Relation to granulomatous disease

“…A high proportion of CVID individuals with granulomatous disease have a TNF receptor mutation that may predispose to inflammatory disease (Mullighan et al, 1997). Anti-TNF treatment can reduce granulomatous lesions in CVID (Lin et al, 2006;Thatayatikom et al, 2005) and classical sarcoid disease (Wells et al, 2008). In vitro anti- predominantly reduces inflammatory responses (Sewell et al,1999;Ballow et al, 1989) and suppressed the production of IFN and IL12p70 from B cells (Bayry et al, 2011).…”