Etanercept treatment of cutaneous granulomas in common variable immunodeficiency.
Common variable immunodeficiency (CVID) is a primary immunodeficiency characterized by hypogammaglobulinemia, poor antibody responses, and recurrent bacterial infections, usually of the sinorespiratory tract. A not uncommon complication is granuloma of the lungs, spleen, liver, and/or skin. We report the case of an 18-year-old boy with CVID and chronic granulomas of the left arm (since 13 years of age) refractory to treatment with antibiotics, intravenous immunoglobulin, antifungal agents, systemic and intralesional steroids, IFN-gamma, cyclosporine, methotrexate, hydroxychloroquine, localized radiation therapy, and surgical excision. The lesions improved after treatment with the systemic administration of the TNF-alpha inhibitor etanercept for 1 year. Etanercept prevents soluble TNF from binding to its cell membrane receptor, leading to inhibition of its inflammatory cascade. We recommend further trials of etanercept in patients with CVID with noninfectious recalcitrant granulomas.
Lin
Joann H
JH
Department of Pediatrics, Division of Immunology, Allergy, and Rheumatology, and David Geffen School of Medicine at UCLA, Los Angeles, California 90095, USA. jlin@mednet.ucla.edu
Liebhaber
Myron
M
Roberts
Robert L
RL
Dyer
Zeb
Z
Stiehm
E Richard
ER
D000293:Adolescent; D001132:Arm; D017074:Common Variable Immunodeficiency / Q000150:complications* / Q000276:immunology / Q000628:therapy*; D000068800:Etanercept; D006099:Granuloma / Q000209:etiology* / Q000473:pathology / Q000628:therapy*; D006801:Humans; D007074:Immunoglobulin G / Q000627:therapeutic use*; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008297:Male; D018124:Receptors, Tumor Necrosis Factor / Q000627:therapeutic use*; D012871:Skin Diseases / Q000209:etiology* / Q000473:pathology / Q000628:therapy*; D014409:Tumor Necrosis Factor-alpha / Q000037:antagonists & inhibitors
D007074:Immunoglobulin G; D007166:Immunosuppressive Agents; D018124:Receptors, Tumor Necrosis Factor; D014409:Tumor Necrosis Factor-alpha; D000068800:Etanercept