Meram Faculty of Medicine, Department of Clinical Immunology and Allergy, Necmettin Erbakan University, Konya, 42090, Turkey.
J Clin Immunol. 2015 May;35(4):344-7. doi: 10.1007/s10875-015-0151-7. Epub 2015 Mar 14.
Common variable immunodeficiency is the most common symptomatic primary immune deficiency characterized by hypogammaglobulinemia, recurrent infections, and increased risk of autoimmune disease and malignancy. Secondary amyloidosis develops from chronic inflammatory conditions. The co-existence of CVID (especially in patients with bronchiectasis) and secondary amyloidosis has been reported rarely. We describe the first case of pulmonary hypertension secondary to pulmonary amyloidosis in a patient with CVID.
普通变异性免疫缺陷是最常见的有症状原发性免疫缺陷,其特征为低丙种球蛋白血症、反复感染以及自身免疫性疾病和恶性肿瘤风险增加。继发性淀粉样变性由慢性炎症性疾病引起。CVID(尤其是支气管扩张症患者)和继发性淀粉样变性共存的情况很少见。我们描述了首例 CVID 患者由肺淀粉样变性引起的肺动脉高压病例。