Purpose

Common variable immunodeficiency (CVID) is known to cause infectious, inflammatory, and autoimmune manifestations. Pulmonary hypertension (PH) is an unusual complication of CVID with largely unknown characteristics and mechanisms.

Methods

We report the clinical, functional, hemodynamics, radiologic and histologic characteristics, and outcomes of CVID-associated PH patients from the French PH Network.

Results

Ten patients were identified. The median (range) age at CVID diagnosis was 36.5 (4–49) years and the median delay between CVID and PH diagnosis was 12 (0–30) years. CVID-associated PH affected predominantly women (female-to-male ratio 9:1). Most patients were New York Heart Association functional class III with a severe hemodynamic profile and frequent portal hypertension (n = 6). Pulmonary function tests were almost normal in 70% of patients and showed a mild restrictive syndrome in 30% of patients while the diffusing capacity for carbon monoxide was decreased in all but one patient. High-resolution computed tomography found enlarged mediastinal nodes, mild interstitial infiltration with reticulations and nodules. Two patients had a CIVD-interstitial lung disease, and one presented with bronchiectasis. Pathologic assessment of lymph nodes performed in 5 patients revealed the presence of granulomas (n = 5) and follicular lymphoid hyperplasia (n = 3). At last follow-up (median 24.5 months), 9 patients were alive, and one patient died of Hodgkin disease.

Conclusion

PH is a possible complication of CVID whose pathophysiological mechanisms, while still unclear, would be due to the inflammatory nature of CVID. CVID-associated PH presents as precapillary PH with multiple possible causes, acting in concert in some patients: a portal hypertension, a pulmonary vascular remodeling, sometimes a pulmonary parenchymal involvement and occasionally an extrinsic compression by mediastinal lymphadenopathies, which would be consistent with its classification in group 5 of the current PH classification.

中文翻译：

---

常见可变免疫缺陷患者的肺动脉高压

目的

已知常见可变性免疫缺陷 (CVID) 会导致感染性、炎症性和自身免疫性表现。肺动脉高压 (PH) 是 CVID 的一种不寻常的并发症，其特征和机制在很大程度上是未知的。

方法

我们报告了来自法国 PH 网络的 CVID 相关 PH 患者的临床、功能、血流动力学、放射学和组织学特征以及结果。

结果

确定了十名患者。CVID 诊断的中位（范围）年龄为 36.5（4-49）岁，CVID 和 PH 诊断之间的中位延迟为 12（0-30）年。CVID 相关的 PH 主要影响女性（女性与男性的比例为 9:1）。大多数患者为纽约心脏协会功能 III 级，具有严重的血流动力学特征和频繁的门静脉高压（​​n = 6）。70% 的患者肺功能检查几乎正常，30% 的患者出现轻度限制性综合征，而除 1 名患者外，所有患者的一氧化碳弥散能力均下降。高分辨率计算机断层扫描发现纵隔淋巴结肿大，间质轻度浸润，伴有网状结构和结节。两名患者患有 CIVD 间质性肺病，一名患者出现支气管扩张。对 5 名患者进行的淋巴结病理学评估显示存在肉芽肿（n = 5）和滤泡性淋巴样增生（n = 3）。末次随访（中位24.5个月），9例患者存活，1例患者死于霍奇金病。

结论

PH 是 CVID 的一种可能并发症，其病理生理机制虽然仍不清楚，但可能是由于 CVID 的炎症性质所致。CVID 相关 PH 表现为具有多种可能原因的毛细血管前 PH，在某些患者中协同作用：门静脉高压、肺血管重构、有时肺实质受累，有时是纵隔淋巴结病引起的外在压迫，这与其分类一致在当前 PH 分类的第 5 组中。