López Ana Laura, Fernández Romero Diego Santiago
Servicio de Alergia e Inmunología, Hospital Británico de Buenos Aires, Argentina.
Unidad Inmunología e Histocompatibilidad, Hospital Dr. C.G. Durand, Buenos Aires, Argentina.
Eur J Case Rep Intern Med. 2025 Jul 29;12(9):005632. doi: 10.12890/2025_005632. eCollection 2025.
Interstitial lung disease is a major complication in patients with common variable immunodeficiency. There are some publications that try to shed light on the pathophysiology of this non-infectious complication, most of them highlight the role of follicular T cells and CD21 B cells. Moreover, there are no guidelines based on randomized controlled studies on the treatment of patients with interstitial lung disease and the published case series or small uncontrolled studies describe a wide range of response rates to treatment. In this scenario, the decisions are based on expert opinion rather than on robust scientific evidence. This could lead to potential biases associated with expert opinion based on a small number of patients.
We present the case of an adult patient with common variable immunodeficiency and interstitial lung disease. He did not receive rituximab due to an adverse reaction and underwent treatment with azathioprine, achieving prolonged remission after treatment discontinuation.
If T-cell involvement could be the main underlying cause of this complication, treatment targeting T-cells may possibly be an effective therapeutic option. Our case, complemented with a physiopathology review, suggests a beneficial effect of azathioprine that warrants further research.
It is important to keep in mind that patients with common variable immunodeficiency are a heterogeneous group, so no single treatment will be the best for all of them. It is necessary to review the rationale for the increasing use of anti-CD20 drugs in non-infectious complications in patients with common variable immunodeficiency. Such use is based on expert recommendations and not on randomized, controlled studies with quantitative endpoints.
There are no guidelines based on randomized controlled studies on the treatment of patients with common variable immunodeficiency and interstitial lung disease.The use of rituximab in the treatment of various non-infectious manifestations in patients with common variable immunodeficiency, including interstitial lung disease, could be the result of potential biases associated with expert opinion based on a small number of patients.T-cell involvement could be the main underlying cause of this complication so treatment targeting T-cells may possibly be an effective therapeutic option.
间质性肺病是常见可变免疫缺陷患者的主要并发症。有一些出版物试图阐明这种非感染性并发症的病理生理学，其中大多数强调滤泡性T细胞和CD21+B细胞的作用。此外，目前尚无基于随机对照研究的间质性肺病患者治疗指南，已发表的病例系列或小型非对照研究描述了广泛的治疗反应率。在这种情况下，治疗决策基于专家意见而非有力的科学证据。这可能导致基于少数患者的专家意见产生潜在偏差。
我们报告一例患有常见可变免疫缺陷和间质性肺病的成年患者。由于不良反应，他未接受利妥昔单抗治疗，而是接受了硫唑嘌呤治疗，停药后病情获得长期缓解。
如果T细胞参与可能是该并发症的主要潜在原因，那么针对T细胞的治疗可能是一种有效的治疗选择。我们的病例结合病理生理学综述表明，硫唑嘌呤具有有益作用，值得进一步研究。
必须牢记，常见可变免疫缺陷患者是一个异质性群体，因此没有一种单一治疗方法对所有患者都是最佳的。有必要重新审视在常见可变免疫缺陷患者的非感染性并发症中越来越多地使用抗CD20药物的理由。这种使用是基于专家建议，而非基于具有定量终点的随机对照研究。
目前尚无基于随机对照研究的常见可变免疫缺陷和间质性肺病患者治疗指南。在常见可变免疫缺陷患者的各种非感染性表现（包括间质性肺病）的治疗中使用利妥昔单抗，可能是基于少数患者的专家意见产生潜在偏差的结果。T细胞参与可能是该并发症的主要潜在原因，因此针对T细胞的治疗可能是一种有效的治疗选择。
