X-linked inhibitor of apoptosis protein (XIAP) deficiency is an inborn error of immunity (IEI). Allogeneic hematopoietic cell transplantation (HCT) is currently the only curative therapy available for XIAP deficiency. Granulomatous and lymphocytic interstitial lung disease (GLILD) is a common immune-related lung complication of IEIs. We present a 6-year-old boy with XIAP deficiency and GLILD. Computed tomography showed lung nodes but no symptoms. Before HCT, GLILD was not managed with immunosuppressive therapy, because he was asymptomatic. The HCT procedure was subsequently performed. The post-HCT course was uneventful; follow-up computed tomography on day 46 showed nodules had disappeared. HCT could potentially ameliorate GLILD like other inflammatory processes associated with the underlying IEIs.

中文翻译：

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同种异体造血细胞移植可改善 XIAP 缺乏症患者的无症状肉芽肿性和淋巴细胞性间质性肺病。

X连锁凋亡抑制蛋白（XIAP）缺陷是一种先天性免疫缺陷（IEI）。同种异体造血细胞移植（HCT）是目前治疗 XIAP 缺陷的唯一疗法。肉芽肿性和淋巴细胞性间质性肺疾病 (GLILD) 是 IEI 常见的免疫相关肺部并发症。我们介绍了一名患有 XIAP 缺陷和 GLILD 的 6 岁男孩。计算机断层扫描显示肺淋巴结，但没有任何症状。在 HCT 之前，GLILD 没有接受免疫抑制治疗，因为他没有症状。随后进行了 HCT 程序。HCT后的课程很顺利；第 46 天的后续计算机断层扫描显示结节已经消失。HCT 可能会像与潜在 IEI 相关的其他炎症过程一样改善 GLILD。