Long Term Follow-Up of a Common Variable Immunodeficiency Patient with Infliximab Responsive Granulomatous Disease

Abstract

The best treatment for common variable immunodeficiency (CVID) patients with granulomatous disease remains elusive. We describe the follow up of a CVID patient with infliximab responsive granulomatous disease. We have previously described a now 72 year old white woman diagnosed as CVID at the age of 41. Serum IgG was 112 mg%, IgA 7 mg% and IgM 31 mg%. She was placed on monthly IVIG with good infectious control. From 1999 she developed necrotizing granulomatous nodular lesions affecting her vocal cords, conjunctiva and skin. Some of her skin lesions became ulcerated and painful. She initially improved on high dose prednisone but had severe steroid adverse effects including several vertebral fractures. Cutaneous lesions extended to all limbs, became ulcerated and severely painful. Ciclosporin up to 5 mg/Kg failed to control. In 2008, infliximab 5 mg/Kg was initiated and, after four courses, all but one ulcer were resolved. For the next 11 years, she had immediate skin flares as soon as infliximab was stopped. In 2017 she had an ITP, stopped infliximab and received high dose steroids. When steroids were tapered painful skin ulcers reappeared that were controlled again with infliximab. Laboratory studies showed an increase in IgM levels after placed on infliximab that kept variable high until infliximab was stopped; high circulating CD4+ follicular helper T cells and CD20+ B cells diminishing over time to 1%. Long term follow-up of this infliximab responsive CVID patient shows an unremitting course of this complication and an unexplained relationship between serum IgM with infliximab.