Granulomatous disease in common variable immunodeficiency
Küçük Resim Yok
Tarih
2009
Dergi Başlığı
Dergi ISSN
Cilt Başlığı
Yayıncı
Academic Press Inc Elsevier Science
Erişim Hakkı
info:eu-repo/semantics/openAccess
Özet
Granulomatous disease occurs in 8-22% of patients with common variable immunodeficiency (CVID). We examined the clinical and immunologic information of all 37 of 455 (8.1%) CVID subjects with this complication. The median age at diagnosis of CVID was 26 (2-59). 14 had granulomas 1-18 years before diagnosis of CVID. In 6 detection of granulomas coincided with this diagnosis; for 17, granulomas were documented later. 54% had lung granulomas, 43% in lymph nodes and 32% in liver. 54% of the group had had autoimmune diseases, mostly immune thrombocytopenia and hemolytic anemia. 24% had had a splenectomy. Nineteen (51.3%) required steroid treatment for granulomas; other immune suppressants were used in some. Over 25 years 28.5% died (median age 37.5), but not significantly more when compared to our CVID patients without granulomas (19.8%). Those with lung granulomas had similar mortality to those with granulomas in other tissues. (C) 2009 Elsevier Inc. All rights reserved.
Açıklama
Anahtar Kelimeler
Common variable immune deficiency, Granulomatous disease, Mortality, TNF-alpha, Lung disease, Autoimmunity
Kaynak
Clinical Immunology
WoS Q Değeri
Q1
Scopus Q Değeri
Q1
Cilt
133
Sayı
2
