Common variable immunodeficiency treated with a recombinant human IgG, tumour necrosis factor-α receptor fusion protein

Authors

  • K.J. Smith,

    1. Department of Dermatology and Pathology, National Naval Medical Center, Bethesda, MD 20889-5600, U.S.A.
      *Laboratory Corporation of America, Herndon, VA, U.S.A.
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  • H. Skelton

    1. Department of Dermatology and Pathology, National Naval Medical Center, Bethesda, MD 20889-5600, U.S.A.
      *Laboratory Corporation of America, Herndon, VA, U.S.A.
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Kathleen J.Smith COL, MC USA. E-mail: HSkelIII@aol.comThe opinions or assertions contained herein are the private views of the authors and should not be construed as official or as reflecting the views of the Department of the Army, Department of the Navy, or Department of Defense.

Abstract

Common variable immunodeficiency (CVI) is characterized by a failure in B-cell differentiation and impaired immunoglobulin secretion, but with a variable clinical presentation, including the development of sarcoidal granulomas and autoimmune diseases, as well as an increased incidence of malignancies. We present a 21-year-old white man who carried a diagnosis of juvenile rheumatoid arthritis and presented 6 years later with scarring alopecia showing sarcoidal granulomas. Further work confirmed the diagnosis of CVI, and with increasing systemic symptoms, it was elected to treat the patient with a tumour necrosis factor (TNF)-α antagonist, a TNF-α receptor IgG1 fusion protein. The patient showed improvement in his systemic symptoms and some hair regrowth after 3 months of therapy, and continued improvement in his systemic disease with only mild scalp hair thinning in the areas of prior involvement after almost 1 year of therapy. CVI and sarcoid may have overlapping clinical and immunological findings. Previous therapies for CVI, including intravenous immunoglobulin, have not altered the mortality of the disease. TNF-α is a primary cytokine and is elevated in CVI, and specific inhibition of TNF-α in this patient was effective in moderating his disease, including his skin disease.

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