Immunodeficiencies in childhood interstitial lung diseases: First data from the Turkish National chILD registry
Bildiriler > Özet Bildiri
Yazarlar: Tuğba Şişmanlar Eyüboğlu, Handan Kekeç, Melih Hangül, Gökçen KARTAL ÖZTÜRK, Mina Hızal, Fazılcan Zirek, Mervenur Tekin, Ayşe Tana Aslan, Figen GÜLEN, İsmail Güzelkaş, Ayça Kıykım, Sanem Eryılmaz Polat, Sinem Can Oksay, Gökçen Ünal, Ali Ersoy, Erdem Başaran, Ela Erdem Eralp, Beste Özsezen, Tuğba Ramaslı Gürsoy, Halime Nayır Büyükşahin, Füsun Ünal, Cansu Yılmaz Yeğit, Nazan Çobanoğlu, Nagehan Emiralioğlu, Ebru Güneş Yalçın, Ayşe Ayzıt Kılınç, Güzin Cinel, Saniye Girit, Sevgi Pekcan, Yasemin Gökdemir, Ahmet Cevdet Ceylan, Diclehan Orhan, Berna Oğuz, Nural Kiper
Rare ILD/DPLD, , , Uluslararası, Özet Bildiri, ss PA4299-PA4299
DOI: 10.1183/13993003.congress-2024.pa4299
Özet
Objective: 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. Method: 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. Results: 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. Discussion: 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