Infliximab treatment for severe granulomatous disease in common variable immunodeficiency: a case report and review of the literature

Fever of Unknown Origin in a Patient with Common Variable Immunodeficiency Associated with Multisystemic Granulomatous Disease

“…Prolonged corticotherapy has been the most widely studied treatment option, and appears helpful in reducing lymph node enlargement and splenomegaly, improving uveitis and hematological abnormalities (6)(7)(8)20). In most published cases of granulomatous CVID, the use of steroids was not associated with increased frequency of infections or worsening immunological function in patients on IVIG (6 (20,21), although further studies are needed to confirm the real usefulness of these new biological therapies.…”

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).…”

Chronic granulomatous disease associated with common variable immunodeficiency – 2 clinical cases

“…6 A recent hypothesis is that high levels of tumor necrosis factor (TNF) might be related to the development of granulomas in these patients. 6,12 Non-caseous granulomatous lesions that affect CVID patients are histologically indistinguishable from those of sarcoidosis. The patient's age and sex, diminished cellular immunity, the systemic involvement, and high levels of angiotensin-converting enzyme are common features for the two pathologies, so a previous story of recurrent/persistent bacterial infections should raise suspicion of CVID.…”

“…However, the therapeutic strategy toward this granulomatous disease has diverged. Although corticotherapy is not universally accepted as a mandatory treatment, 12,15 it was prescribed for the first patient because of the systemic involvement, namely, the cytopenia secondary to the bone marrow involvement. The second patient did not receive any therapy directed at the GD.…”

“…However, both of them improved in clinical, analytical, and radiological terms, which is in line with the favorable evolution reported by Bouvry et al 1 The appropriate treatment for GD-CVID patients has not yet been established. 6,7,12,15 There are no protocols regarding diagnosis, treatment and follow-up, nor prognosis for GD-CVID patients. Many of these patients are followed individually and in a nonstandardized way.…”