Sacco O, Fregonese B, Picco P, Faraci M, Facchetti P, Pistoia V, Rossi G A
Divisione di Pneumologia, G. Gaslini Institute, Genoa, Italy.
Eur Respir J. 1996 Sep;9(9):1958-61. doi: 10.1183/09031936.96.09091958.
Common variable immunodeficiency (CVID) is a primary immunodeficiency disorder characterized by decreased levels of circulating immunoglobulins (Ig) and increased susceptibility to infections. We describe the case of a girl, progressively developing CVID, whose first clinical manifestations were noninfectious diffuse pulmonary infiltrates and rapidly developing hilar and mediastinal lymphadenopathies causing a severe "superior vena caval syndrome". Histological evaluation of surgical samples showed follicular and paracortical hyperplasia of the lymph nodes and poorly organized, non-necrotizing, noninfectious, "reactive" granulomata in lymph nodes and pulmonary tissue. Daily treatment with azathioprine and prednisone induced resolution of the intrathoracic abnormalities but was associated with a progressive decrease of circulating IgG and IgA levels and natural killer (NK) lymphocytes that was not related to treatment. This case demonstrates that granulomatous inflammatory changes may be the first manifestations of common variable immunodeficiency, and that this disorder must be included in the differential diagnosis of lymphoid interstitial pneumonitis and of bilateral mediastinal lymph node enlargement leading to superior vena caval syndrome.
普通可变免疫缺陷（CVID）是一种原发性免疫缺陷疾病，其特征为循环免疫球蛋白（Ig）水平降低以及对感染的易感性增加。我们描述了一名逐渐发展为CVID的女孩的病例，其最初的临床表现为非感染性弥漫性肺浸润以及迅速发展的肺门和纵隔淋巴结病，导致严重的“上腔静脉综合征”。手术样本的组织学评估显示淋巴结的滤泡和副皮质增生，以及淋巴结和肺组织中组织紊乱、非坏死性、非感染性的“反应性”肉芽肿。硫唑嘌呤和泼尼松的每日治疗使胸腔内异常情况得到缓解，但与循环IgG和IgA水平以及自然杀伤（NK）淋巴细胞的逐渐减少有关，这与治疗无关。该病例表明，肉芽肿性炎症变化可能是普通可变免疫缺陷的首发表现，并且在鉴别诊断淋巴间质性肺炎和导致上腔静脉综合征的双侧纵隔淋巴结肿大时必须考虑到这种疾病。
