Immunodeficiencies in childhood interstitial lung diseases: First data from the Turkish National chILD registry

<bold>Objective:</bold> Immunodeficiencies are associated with different Childhood Interstitial Lung Diseases (chILD) patterns, but data in this area are limited. We aimed to reveal the diagnoses, lung involvement, and clinical characteristics of immunodeficiency patients diagnosed with chILD. <bold>Method:</bold> All patients diagnosed with immunodeficiency(Diagnosis code:B3) registered in the Turkish Registry of chILD in 2023 were included. Age at presentation, age at diagnosis, type of immunodeficiency, clinical, laboratory, and radiological findings, and clinical status of patients were noted. <bold>Results:</bold> A total of 667 patients were recorded into the registry, and 114 (17%) had immunodeficiency. The mean age of patients with immunodeficiency was 12,4±6.1 years and 47% of them were female. Among the patients, 60% had primary immunodeficiency; the others were secondary, mainly secondary to hematopoietic stem cell transplantation (HSCT). The most common radiological finding was ground glass opacity, nodules, bronchial thickness, and linear opacities, respectively. The most common diagnosis was bronchiolitis obliterans after HSCT and chILD without any specific diagnosis. However, there are many different diagnoses, such as GLILD, PAP, pleuropulmonary fibroelastosis, hypersensitive pneumonitis, etc. The mean FEV1 % was 58.5±28.7,and FVC 61.3±27%. Seven of them died during follow-up. <bold>Discussion:</bold> Immunodeficiencies are an important cause of chILD. Patients may present with different types of involvement. Mortality and morbidity may be common in children with immunodeficiency and chILD. As registries become more widespread, knowledge on immunodeficiency and chILD will also increase

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Published
2024-09-14
DOI
https://doi.org/10.1183/13993003.congress-2024.pa4299
Primary Topic
Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
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article
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article

Immunodeficiencies in childhood interstitial lung diseases: First data from the Turkish National chILD registry

Tuğba Şişmanlar Eyüboğlu, Gökçen Kartal Öztürk, Saniye Girit, Erdem Başaran et al.
Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
article

Immunodeficiencies in childhood interstitial lung diseases: First data from the Turkish National chILD registry

Tuğba Şişmanlar Eyüboğlu, Gökçen Kartal Öztürk, Saniye Girit, Erdem Başaran, Halime Nayır Büyükşahin, Mina Hızal, Nagehan Emiralioğlu, İsmail Güzelkaş, Figen Gülen, Ayça Aslan Kıykım, Güzin Cinel, Tuğba Ramaslı Gürsoy, Ahmet Cevdet Ceylan, Ayşe Ayzıt Kılınç, Yasemi̇n Gökdemi̇r, Dıclehan Orhan, Gökçen Ünal, Nural Ki̇per, Melih Hangül, Ela Erdem Eralp, Sinem Can Oksay, Nazan Çobanoğlu, Sanem Eryılmaz Polat, Cansu Yılmaz Yeğit, Fazılcan Zirek, Ali Özgür Ersoy, Füsun Ünal, Beste Özsezen, Berna Oğuz, Ayşe Tana Aslan, Sevgi Pekcan, Handan Kekeç, E. Güneş Yalçın, Mervenur Tekin
article en

Abstract

<bold>Objective:</bold> Immunodeficiencies are associated with different Childhood Interstitial Lung Diseases (chILD) patterns, but data in this area are limited. We aimed to reveal the diagnoses, lung involvement, and clinical characteristics of immunodeficiency patients diagnosed with chILD. <bold>Method:</bold> All patients diagnosed with immunodeficiency(Diagnosis code:B3) registered in the Turkish Registry of chILD in 2023 were included. Age at presentation, age at diagnosis, type of immunodeficiency, clinical, laboratory, and radiological findings, and clinical status of patients were noted. <bold>Results:</bold> A total of 667 patients were recorded into the registry, and 114 (17%) had immunodeficiency. The mean age of patients with immunodeficiency was 12,4±6.1 years and 47% of them were female. Among the patients, 60% had primary immunodeficiency; the others were secondary, mainly secondary to hematopoietic stem cell transplantation (HSCT). The most common radiological finding was ground glass opacity, nodules, bronchial thickness, and linear opacities, respectively. The most common diagnosis was bronchiolitis obliterans after HSCT and chILD without any specific diagnosis. However, there are many different diagnoses, such as GLILD, PAP, pleuropulmonary fibroelastosis, hypersensitive pneumonitis, etc. The mean FEV1 % was 58.5±28.7,and FVC 61.3±27%. Seven of them died during follow-up. <bold>Discussion:</bold> Immunodeficiencies are an important cause of chILD. Patients may present with different types of involvement. Mortality and morbidity may be common in children with immunodeficiency and chILD. As registries become more widespread, knowledge on immunodeficiency and chILD will also increase

Ministry of Health (TR), Izmir University (TR), Ankara University (TR), Akdeniz University (TR), Mardin Artuklu University (TR), Istanbul Medipol University (TR), Ege University (TR), Turkish Society of Hematology (TR), Memorial Ankara Hospital (TR), Necmettin Erbakan University (TR), Istanbul University-Cerrahpaşa (TR), Samsun University (TR), Education Training And Research (US), Kayseri Eğitim ve Araştırma Hastanesi (TR), Istanbul Medeniyet University (TR), Hacettepe University (TR), Marmara University (TR), Erciyes University (TR), Gazi University (TR)
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Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
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