[Middle-aged man with fever, hypoxia and bilateral alveolar infiltrates].
Pulmonary alveolar proteinosis (PAP) is a rare condition characterized by excessive amounts of surfactant phospholipids and proteins within the pulmonary alveoli. Depending on the amount of surfactant in the alveoli, varying degrees of disturbed gas exchange develop. We report the case of a middle-aged man presenting with fever, dyspnoe, cough and bilateral alveolar infiltrates on chest X-ray. On the basis of pink staining periodic acid-Schiff (PAS) of the milky material recovered from the airways during bronchoscopy, and transbronchial biopsies demonstrating normal interstitial structures, a diagnosis of PAP was established. He has been repeatedly treated with whole lung lavage--totally eight treatments- during a 30-month-period. The effect of therapy was excellent initially, but his symptoms recur, and he now needs treatment with shorter time-intervals. On the basis of this case history, an overview of the pathogenesis, clinical presentation, diagnosis, existing treatment modalities and prognosis of PAP is given.
Aakervik
Bjørn
B
Lungemedisinsk avdeling, Rikshospitalet, 0027 Oslo. anne.nalsund@rikshospitalet.no
Naalsund
Anne
A
Lund
May Brit
MB
Dragsund
Morten
M
Solberg
Steinar
S
Aaløkken
Trond Mogens
TM
Bakke
Per
P
D018893:Bronchoalveolar Lavage; D001992:Bronchoalveolar Lavage Fluid / Q000737:chemistry; D003937:Diagnosis, Differential; D005334:Fever / Q000175:diagnosis*; D006801:Humans; D000860:Hypoxia / Q000175:diagnosis*; D008176:Lung Volume Measurements; D008297:Male; D008875:Middle Aged; D011649:Pulmonary Alveolar Proteinosis / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000628:therapy; D011650:Pulmonary Alveoli / Q000473:pathology; D011859:Radiography