PULMONER ALVEOLER PROTEÝNOZÝS: OLGU SUNUMU

Pulmoner alveoler proteinozis alveollerde lipoproteinoz materyalin birikimi ile karakterize ender görülen bir hastalýktýr. Hastalýðýn ilk belirtisi yavaþ ilerleyen dispnedir. Radyolojik olarak bilateral alveoler infiltrasyonlar görülür ve solunum fonksiyon testlerinde restriktif tipte bozukluk saptanýr. Tanýya sýklýkla biyopsi örneði veya segmental lavaj ile alýnan intraalvoler materyalin incelenmesi ile ulaþýlýr. 43 yaþýndaki olgunun 5 ay önce baþlayan efor dispnesi ve kuru öksürük yakýnmasý mevcuttu. Hastaya idyopatik pulmoner fibrozis tanýsýyla 2 aydýr steroid tedavisi baþlanmýþ idi. Göðüs radyografisinde lezyonlarý devam eden olguya kliniðimizde transbronþiyal forseps biyopsi ile pulmoner alveoler proteinozis tanýsý kondu. Hastanede yattýðý sürede spontan klinik ve radyolojik regresyon izlenen, üçüncü yýl sonunda hala remisyonda olan olgu literatür eþliðinde sunuldu.

PULMONARY ALVEOLAR PROTEINOSIS: CASE REPORT

Pulmonary alveolar proteinosis is a rare disease characterized by the accumulation of lipoproteinaceous material within the alveoli. The first sign of the disease is slowly progressive dispnea. Radiologically bilateral alveolar infiltrations are seen and restrictive type respiratory dysfunction is found. The diagnosis is usually arrived by examination of intraalveolar material obtained either by biopsy specimen or by segmental lavage. 43 year old female had dispnea at effort and dry cough which were started 5 months ago. The patient had been given steroid therapy for two months. With the diagnosis of idiopathic pulmonary fibrosis. The case whose radiological lesions were persistent was diagnosed as pulmonary alveolar proteinosis with transbronchial lung biopsy in our clinic. The case who showed radiological and clinical remission spontaneously during the time she was hospitalized and no relaps seen at the end of the three year represented with related literature.