Background: Combined immunodeficiency (CID) involves profound defects in B and T lymphocyte development and function. This study examined clinical and immunological phenotypes of CID patients with and without pulmonary manifestations.
Methods: This retrospective multicenter study included 53 CID patients diagnosed between 2009 and 2022 with available thoracic computed tomography scans. Patients were categorized based on pulmonary manifestations presence. Demographic, clinical, and laboratory characteristics were compared using conservative statistical thresholds (P < 0.01). All laboratory parameters were interpreted using age-adjusted pediatric reference ranges.
Results: Among 53 patients (56.6% male), 43 had pulmonary abnormalities on HRCT. Common clinical features included skin lesions (43.4%), failure to thrive (34%), and autoimmunity (32.1%). HRCT revealed pneumonia (28.3%), bronchiectasis (18.9%), interstitial lung disease with BOOP-like pattern (3.8%), and other findings. Using age-adjusted pediatric reference ranges, profound immunological defects were confirmed: absolute lymphocyte count below the 5th percentile in 92% (49/53), CD3 + T cells below the 5th percentile in 94% (47/50 tested), CD4 + T cells below the 5th percentile in 96% (51/53), CD19 + B cells below the 5th percentile in 94% (50/53), and hypogammaglobulinaemia (IgG below the 5th percentile) in 98% (52/53). Patients with abnormal HRCT had significantly lower CD4 + T-cell counts (178 vs. 498 cells/µL; P = 0.008) and CD19 + B-cell counts (42 vs. 189 cells/µL; P = 0.009). Bronchoscopy identified Aspergillus fumigatus, Streptococcus pneumoniae, and multidrug-resistantAcinetobacter baumannii. Deceased patients showed significantly lower baseline platelets (183,000 vs. 266,000 cells/µL; P = 0.009), IgG (380 vs. 720 mg/dL; P = 0.007), and IgE (0.8 vs. 12 IU/mL; P = 0.008).
Conclusion: Pulmonary manifestations affect 81.1% of Iranian CID patients. Low baseline platelets, IgG, and IgE constitute a robust prognostic triad for mortality (P = 0.009, P = 0.007, P = 0.008 respectively). Application of age-adjusted reference ranges revealed profound immunological defects. Systematic HRCT surveillance using low-dose protocols and distinguishing infectious sequelae from immune-mediated lung disease guides targeted management in resource-limited settings.
背景：联合免疫缺陷（CID）涉及B和T淋巴细胞发育和功能的严重缺陷。本研究检查了有和没有肺部表现的CID患者的临床和免疫表型。方法：这项回顾性多中心研究纳入了2009年至2022年间诊断为CID的53例患者，并进行了可用的胸部计算机断层扫描。根据肺部表现对患者进行分类。采用保守统计学阈值比较人口学、临床和实验室特征(P)。结果：53例患者（56.6%为男性）中，43例HRCT表现为肺部异常。常见的临床特征包括皮肤病变（43.4%）、生长失败（34%）和自身免疫（32.1%）。HRCT显示肺炎（28.3%）、支气管扩张（18.9%）、boop样肺间质性疾病（3.8%）及其他表现。使用儿童年龄校正参考范围，证实了严重的免疫缺陷：92%（49/53）的绝对淋巴细胞计数低于第5百分位数，94%（47/50）的CD3 + T细胞低于第5百分位数，96%（51/53）的CD4 + T细胞低于第5百分位数，94%（50/53）的CD19 + B细胞低于第5百分位数，98%（52/53）的低γ -球蛋白血症（IgG低于第5百分位数）。HRCT异常患者CD4 + t细胞计数（178比498细胞/µL， P = 0.008）和CD19 + b细胞计数（42比189细胞/µL， P = 0.009）显著降低。支气管镜检查发现了烟曲霉、肺炎链球菌和耐多药鲍曼不动杆菌。死亡患者的基线血小板（183,000 vs. 266,000细胞/ μ L， P = 0.009）、IgG （380 vs. 720 mg/dL, P = 0.007）和IgE （0.8 vs. 12 IU/mL, P = 0.008）均显著降低。结论：81.1%的伊朗CID患者有肺部表现。低基线血小板、IgG和IgE构成了死亡率的可靠预后三因素（P = 0.009, P = 0.007, P = 0.008）。年龄调整参考值范围的应用揭示了深刻的免疫缺陷。在资源有限的情况下，采用低剂量方案进行系统的HRCT监测，并将传染性后遗症与免疫介导的肺部疾病区分开来，可指导有针对性的管理。
{"title":"Demographic, clinical, and immunological features in combined immunodeficiency patients: a comparative analysis of those with and without pulmonary manifestations - a multicenter study from Iran.","authors":"Ghamartaj Khanbabaee, Matin Pourghasem, Mahnaz Jamee, Seyed Ahmad Tabatabaii, Mitra Khalili, Samin Sharafian, Mehrnaz Mesdaghi, Mahnaz Sadeghi-Shabestari, Armin Shirvani, Saeid Sadr, Arefeh Zahmatkesh, Samaneh Delavari, Narges Eslami, Nazanin Farahbakhsh, Mahboubeh Mansouri, Ebrahim Tabiei, Seyedeh Zalfa Modarresi, Abdolhamid Taghizadeh Behbahani, Golnaz Eslamian, Mazdak Fallahi, Javad Enayat, Shahrzad Fallah, Mahsa Pourghasem, Asghar Aghamohammadi, Zahra Chavoshzadeh","doi":"10.1186/s12890-026-04115-3","DOIUrl":"https://doi.org/10.1186/s12890-026-04115-3","url":null,"abstract":"<p><strong>Background: </strong>Combined immunodeficiency (CID) involves profound defects in B and T lymphocyte development and function. This study examined clinical and immunological phenotypes of CID patients with and without pulmonary manifestations.</p><p><strong>Methods: </strong>This retrospective multicenter study included 53 CID patients diagnosed between 2009 and 2022 with available thoracic computed tomography scans. Patients were categorized based on pulmonary manifestations presence. Demographic, clinical, and laboratory characteristics were compared using conservative statistical thresholds (P < 0.01). All laboratory parameters were interpreted using age-adjusted pediatric reference ranges.</p><p><strong>Results: </strong>Among 53 patients (56.6% male), 43 had pulmonary abnormalities on HRCT. Common clinical features included skin lesions (43.4%), failure to thrive (34%), and autoimmunity (32.1%). HRCT revealed pneumonia (28.3%), bronchiectasis (18.9%), interstitial lung disease with BOOP-like pattern (3.8%), and other findings. Using age-adjusted pediatric reference ranges, profound immunological defects were confirmed: absolute lymphocyte count below the 5th percentile in 92% (49/53), CD3 + T cells below the 5th percentile in 94% (47/50 tested), CD4 + T cells below the 5th percentile in 96% (51/53), CD19 + B cells below the 5th percentile in 94% (50/53), and hypogammaglobulinaemia (IgG below the 5th percentile) in 98% (52/53). Patients with abnormal HRCT had significantly lower CD4 + T-cell counts (178 vs. 498 cells/µL; P = 0.008) and CD19 + B-cell counts (42 vs. 189 cells/µL; P = 0.009). Bronchoscopy identified Aspergillus fumigatus, Streptococcus pneumoniae, and multidrug-resistantAcinetobacter baumannii. Deceased patients showed significantly lower baseline platelets (183,000 vs. 266,000 cells/µL; P = 0.009), IgG (380 vs. 720 mg/dL; P = 0.007), and IgE (0.8 vs. 12 IU/mL; P = 0.008).</p><p><strong>Conclusion: </strong>Pulmonary manifestations affect 81.1% of Iranian CID patients. Low baseline platelets, IgG, and IgE constitute a robust prognostic triad for mortality (P = 0.009, P = 0.007, P = 0.008 respectively). Application of age-adjusted reference ranges revealed profound immunological defects. Systematic HRCT surveillance using low-dose protocols and distinguishing infectious sequelae from immune-mediated lung disease guides targeted management in resource-limited settings.</p>","PeriodicalId":9148,"journal":{"name":"BMC Pulmonary Medicine","volume":" ","pages":""},"PeriodicalIF":2.8,"publicationDate":"2026-01-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146097049","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2026-01-31DOI: 10.1186/s12890-026-04143-z
Marjolein Drent, Lian Trapman, Miranda Hennevelt-Leenen, Jan C Grutters, Anne-Marie Russell
{"title":"Understanding emotional and practical challenges of initiating oxygen therapy in pulmonary fibrosis: insights from a patient-centered survey.","authors":"Marjolein Drent, Lian Trapman, Miranda Hennevelt-Leenen, Jan C Grutters, Anne-Marie Russell","doi":"10.1186/s12890-026-04143-z","DOIUrl":"https://doi.org/10.1186/s12890-026-04143-z","url":null,"abstract":"","PeriodicalId":9148,"journal":{"name":"BMC Pulmonary Medicine","volume":" ","pages":""},"PeriodicalIF":2.8,"publicationDate":"2026-01-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146097080","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2026-01-30DOI: 10.1186/s12890-026-04135-z
Martin Huecker, Ryan Close, Jonathan Mattingly, Haely Studebaker, Craig Zeigler, Daniel O'Brien, Jeremy Thomas
{"title":"Mobile phone auscultation to delineate pneumonia from other respiratory conditions and controls: a prospective cohort study.","authors":"Martin Huecker, Ryan Close, Jonathan Mattingly, Haely Studebaker, Craig Zeigler, Daniel O'Brien, Jeremy Thomas","doi":"10.1186/s12890-026-04135-z","DOIUrl":"https://doi.org/10.1186/s12890-026-04135-z","url":null,"abstract":"","PeriodicalId":9148,"journal":{"name":"BMC Pulmonary Medicine","volume":" ","pages":""},"PeriodicalIF":2.8,"publicationDate":"2026-01-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146084210","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2026-01-29DOI: 10.1186/s12890-025-04076-z
Yorusaliem Abrham, Siyang Zeng, Wendy Lin, Colin Lo, Alexander Beckert, Laurel Evans, Michelle Dunn, Brian Giang, Krish Thakkar, Julian Roman, Paul D Blanc, Mehrdad Arjomandi
{"title":"Correction: Self‑report underestimates the frequency of the acute respiratory exacerbations of COPD but is associated with BAL neutrophilia and lymphocytosis: an observational study.","authors":"Yorusaliem Abrham, Siyang Zeng, Wendy Lin, Colin Lo, Alexander Beckert, Laurel Evans, Michelle Dunn, Brian Giang, Krish Thakkar, Julian Roman, Paul D Blanc, Mehrdad Arjomandi","doi":"10.1186/s12890-025-04076-z","DOIUrl":"10.1186/s12890-025-04076-z","url":null,"abstract":"","PeriodicalId":9148,"journal":{"name":"BMC Pulmonary Medicine","volume":"26 1","pages":"31"},"PeriodicalIF":2.8,"publicationDate":"2026-01-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12853590/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146084273","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2026-01-29DOI: 10.1186/s12890-026-04141-1
Yuzhen Jin, Lijie Qin, Peng Liu, Zhaoxia Song, Mengjie Li
Background: Voriconazole is a first-line antifungal agent for invasive fungal infections. Reports of voriconazole-induced interstitial pulmonary edema are exceedingly rare.
Case presentation: We describe a 74-year-old woman with pneumonia who developed facial edema, dyspnea, and pink frothy sputum after four days of voriconazole therapy. Chest computed tomography (CT) revealed bilateral pleural effusions and interlobular septal thickening. She was initially misdiagnosed with acute heart failure, but failed to respond to diuretics. Both B-type natriuretic peptide (BNP) levels and echocardiography were unremarkable. The diagnosis of voriconazole-induced acute interstitial pulmonary edema was established. Voriconazole withdrawal and corticosteroid therapy resulted in rapid clinical resolution.
Conclusion: Voriconazole hypersensitivity may manifest as acute interstitial pulmonary edema with concomitant angioedema, a presentation that can mimic cardiogenic pulmonary edema. Early recognition and timely corticosteroid intervention are crucial for favorable outcomes.
{"title":"Voriconazole-Induced acute interstitial pulmonary edema with angioedema: A case report.","authors":"Yuzhen Jin, Lijie Qin, Peng Liu, Zhaoxia Song, Mengjie Li","doi":"10.1186/s12890-026-04141-1","DOIUrl":"https://doi.org/10.1186/s12890-026-04141-1","url":null,"abstract":"<p><strong>Background: </strong>Voriconazole is a first-line antifungal agent for invasive fungal infections. Reports of voriconazole-induced interstitial pulmonary edema are exceedingly rare.</p><p><strong>Case presentation: </strong>We describe a 74-year-old woman with pneumonia who developed facial edema, dyspnea, and pink frothy sputum after four days of voriconazole therapy. Chest computed tomography (CT) revealed bilateral pleural effusions and interlobular septal thickening. She was initially misdiagnosed with acute heart failure, but failed to respond to diuretics. Both B-type natriuretic peptide (BNP) levels and echocardiography were unremarkable. The diagnosis of voriconazole-induced acute interstitial pulmonary edema was established. Voriconazole withdrawal and corticosteroid therapy resulted in rapid clinical resolution.</p><p><strong>Conclusion: </strong>Voriconazole hypersensitivity may manifest as acute interstitial pulmonary edema with concomitant angioedema, a presentation that can mimic cardiogenic pulmonary edema. Early recognition and timely corticosteroid intervention are crucial for favorable outcomes.</p>","PeriodicalId":9148,"journal":{"name":"BMC Pulmonary Medicine","volume":" ","pages":""},"PeriodicalIF":2.8,"publicationDate":"2026-01-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146084227","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2026-01-29DOI: 10.1186/s12890-026-04118-0
Zeyang Dong, Mengyao Zhao, Xixi Sun, Xianting Yan, Sihui Zheng, Jian Ye, Bin Huang, Jin Ge
{"title":"Prognostic assessment of the COPD patient: it is time to consider respiratory muscle ultrasound.","authors":"Zeyang Dong, Mengyao Zhao, Xixi Sun, Xianting Yan, Sihui Zheng, Jian Ye, Bin Huang, Jin Ge","doi":"10.1186/s12890-026-04118-0","DOIUrl":"https://doi.org/10.1186/s12890-026-04118-0","url":null,"abstract":"","PeriodicalId":9148,"journal":{"name":"BMC Pulmonary Medicine","volume":" ","pages":""},"PeriodicalIF":2.8,"publicationDate":"2026-01-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146084204","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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