AI Summary & Key Takeaways
AI InsightAfter one month of treatment clinical improvement was observed during control.
📌 3 Key Takeaways
Abstract
Pulmonary Langerhans’ cell histiocytosis (PLCH) is an uncommon interstitial lung disease with an unknown etiology in smokers. Disease is usually seen with isolated lung involvement and central nervous system involvement is rare.Two PLCH cases, with central diabetes insipidus (DI) determined at diagnosis in one of the cases and during follow-up in the other, is being presented in this report. The patients’ ages were 39 and 24, respectively and they were both male patients with a history of smoking. Widely cystic lesions were viewed at high-resolution computed tomography scans (HRCT) and PLCH was detected at the biopsies obtained by video-assisted thoracoscopic surgery (VATS), in both two cases. Therapy was started with 0.5 mg/kg methyl prednisolone for latter case, because of impairment in pulmonary function tests during follow up. On control, after one month therapy, partial improvement was seen for PLCH and the therapy was continued up to 6 months. At the end of the therapy complete remission was seen and corticotherapy was stopped. Desmopressin was started for both cases at 0.1 mg/day, orally. After one month of treatment clinical improvement was observed during control.
Özet
Pulmoner langerhans hücreli histiyositozis (PLHH), etyolojisi bilinmeyen ve sigara içenlerde görülen, nadir bir interstisyel akciğer hastalığıdır. Hastalıkta genellikle izole akciğer tutulumu vardır ve santral sinir sistemi tutulumu nadirdir. Bu olgu sunumunda, birinde takip, diğerinde ise tanı sırasında santral diabetes insipidus (Dİ) saptanan iki olgu sunulmaktadır. Olgular, yaşları sırasıyla 24 ve 39 olan, sigara içme öyküsü olan erkek hastalardı. İki olgunun da yüksek rezolüsyonlu bilgisayarlı tomografilerinde (YRBT) yaygın kistik lezyonlar izlenmiş olup, video-yardımlı torakoskopik cerrahiyle (VATS) alınan biyopsilerinde PLHH saptandı. Takipte solunum fonksiyonlarının bozulmuş olduğu görülen birinci olguya 0,5 mg/kg metil prednisolon başlandı. Bir ay sonra yapılan kontrolde PLHH için kısmi düzelme gözlenmesi üzerine tedavi 6 aya uzatıldı, tedavi bitiminde tam remisyon saptanarak steroid tedavisi kesildi. İki hastaya da Dİ için oral 0,1 mg/gün desmopressin başlandı, bir ay sonra tam klinik düzelme gözlendi.