Department of Laboratory Medicine and Pathology, Mayo Clinic, Scottsdale, AZ.
Department of Laboratory Medicine and Pathology.
Am J Surg Pathol. 2020 Aug;44(8):1073-1081. doi: 10.1097/PAS.0000000000001479.
Common variable immunodeficiency (CVID) and selective immunoglobulin A deficiency (IgAD) often cause chronic lung disease, but the pulmonary pathologic features of these systemic diseases are poorly recognized by pathologists. It has been claimed that CVID cases show a characteristic combination of noncaseating granulomas-lymphoid proliferations termed granulomatous-lymphocytic interstitial lung disease (GLILD). We present 34 surgical lung biopsy cases of CVID and 4 of IgAD. Noncaseating granulomas were seen in 23/34 (68%) CVID and 2/4 (50%) IgAD cases. A statistically identical pattern of benign lymphoid proliferation was found in CVID and IgAD whether or not granulomas were present. Organizing pneumonia, sometimes considered a part of GLILD, was seen in 25/34 (74%) CVID and 2/4 (50%) IgAD cases and did not correlate with the presence of granulomas. On follow-up, 3 CVID patients died (only 1 of pulmonary disease), while 21 others are alive at 1 to 300 months with no difference by presence or absence of granulomas. Three IgAD patients with follow-up are alive. We conclude that CVID and IgAD are indistinguishable in surgical lung biopsies and a subset of both show patterns that would qualify as GLILD, while other cases lack granulomas but have identical patterns of lymphoid infiltration and organizing pneumonia. We suggest that GLILD is neither a specific nor a useful entity, and biopsies from CVID and IgAD patients should be diagnosed simply by microscopic pattern(s) observed. The prognosis of CVID with lymphoid infiltrates with or without granulomas in this series was good, contrary to claims in the literature about GLILD.
普通变异型免疫缺陷（CVID）和选择性免疫球蛋白 A 缺乏症（IgAD）常导致慢性肺部疾病，但这些系统性疾病的肺部病理特征并未被病理学家所认识。据称，CVID 病例表现为一种非干酪性肉芽肿-淋巴样增生的特征性组合，称为肉芽肿性淋巴样间质肺病（GLILD）。我们报告了 34 例 CVID 和 4 例 IgAD 的手术肺活检病例。23/34（68%）例 CVID 和 2/4（50%）例 IgAD 中可见非干酪性肉芽肿。无论是否存在肉芽肿，在 CVID 和 IgAD 中都发现了具有统计学意义的良性淋巴样增生模式。机化性肺炎，有时被认为是 GLILD 的一部分，在 25/34（74%）例 CVID 和 2/4（50%）例 IgAD 中可见，与肉芽肿的存在无关。在随访中，3 例 CVID 患者死亡（只有 1 例死于肺部疾病），而其余 21 例患者在 1 至 300 个月时存活，且有无肉芽肿的生存时间无差异。有随访的 3 例 IgAD 患者仍存活。我们得出结论，在手术肺活检中，CVID 和 IgAD 无法区分，两者的亚组表现符合 GLILD 的标准，而其他病例缺乏肉芽肿，但具有相同的淋巴样浸润和机化性肺炎模式。我们认为，GLILD 既不是一种特定的也不是一种有用的实体，CVID 和 IgAD 患者的活检应仅根据观察到的微观模式进行诊断。在本系列中，伴有或不伴有肉芽肿的 CVID 伴淋巴样浸润的预后良好，与文献中关于 GLILD 的说法相反。
