분류 불능형 면역 결핍증(Common Variable Immunodeficiency)과 병발된 쇼그렌 증후군 1예 (A Case of Sjögren’s Syndrome Associated with Common Variable Immunodeficiency)

서지정보

· 발행기관 : 대한내과학회
· 수록지 정보 : 대한내과학회지 / 89권 / 2호 / 254 ~ 258페이지
· 저자명 : 강산하, 조혜정, 홍희진, 홍소담, 김보혜, 정상윤, 최진정

초록

저자들은 분류 불능형 면역 결핍증으로 진단된 환자에서병발된 쇼그렌 증후군 1예를 경험하였기에 이를 보고하는바이다.

영어초록

Common variable immunodeficiency (CVID) is a heterogeneous group of primary immune deficiencies in adults characterized by hypogammaglobulinemia, recurrent bacterial infections, and a higher incidence of autoimmune diseases. More than 25% of CVID patients also have autoimmune diseases such as autoimmune hemolytic anemia, immune thrombocytopenic purpura, rheumatoid arthritis, and systemic lupus erythematosus. However, the pathogenesis of autoimmunity in CVID remains obscure. We report a 56-year-old woman with CVID and Sjögren’s syndrome. In addition to a long history of recurrent upper respiratory infections, acute gastroenteritis, and cellulitis, she has also suffered from persistent xerostomia and xerophthalmia for the past 10 years. Serologic studies revealed hypogammaglobulinemia (low levels of IgG, IgA, and IgM in serum) and the presence of anti-Ro antibodies, and salivary scintigraphy indicated salivary gland involvement. These findings led to a diagnosis of CVID and Sjögren’s syndrome, which was treated by monthly intravenous immunoglobulin therapy.

참고자료

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