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Common variable immunodeficiency treated with a recombinant human IgG, tumour necrosis factor‐α receptor fusion protein
Author(s) -
Smith K.J.,
Skelton H.
Publication year - 2001
Publication title -
british journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.304
H-Index - 179
eISSN - 1365-2133
pISSN - 0007-0963
DOI - 10.1046/j.1365-2133.2001.04092.x
Subject(s) - common variable immunodeficiency , recombinant dna , fusion protein , immunology , medicine , necrosis , virology , tumor necrosis factor receptor , tumor necrosis factor alpha , receptor , antibody , biology , pathology , gene , biochemistry
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.