Chest
SELECTED REPORTSPulmonary Interstitial Fibrosis Following Alveolar Proteinosis
Section snippets
Case Report
A black construction worker was well until 1959, when at 45 years of age he experienced an acute illness characterized by cough, shortness of breath, fever and generalized arthralgias. He was hospitalized and treated for “pneumonia.” On a subsequent admission in 1960 an open biopsy of the right lung was interpreted as alveolar proteinosis. From 1961 to 1967 the patient resided in southern California and was asymptomatic without therapy. During the interval he did not visit a doctor,
Discussion
In the original description of PAP by Rosen et al,12 an increase in the number of alveolar septal inflammatory cells was noted in cases 11 and 18. Case 2 from that series, reported in more detail elsewhere, demonstrated mild fibrosis on initial lung biopsy followed by more extensive fibrosis 9½ months later at postmortem.7 The initial lung biopsy from case 7 showed typical PAP and was free from inflammatory reaction or fibrosis.12 Two and one-half years later when the patient was asymptomatic a
ACKNOWLEDGMENT
We thank Dr. Robin T. Vollmer for performing the postmortem examination.
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Cited by (33)
Pulmonary alveolar proteinosis-like change: A fairly common reaction associated with the severity of idiopathic pulmonary fibrosis
2016, Respiratory InvestigationCitation Excerpt :In contrast, secondary PAP occurs as a consequence of various underlying clinical conditions including hematological malignancies, inhalation of toxic dust, and infectious or pharmacological immunosuppression that impairs alveolar macrophage function, resulting in surfactant accumulation [4]. Some reports have indicated an association between PAP and severe pulmonary fibrosis mimicking idiopathic pulmonary fibrosis (IPF) [6–8]. Most of these are case reports that conclude that the fibrosis is a part of PAP [7,8]. However, Hudson et al. [6] have suggested that in some cases, PAP may be a stage of fibrotic disease.
A novel immunodeficiency syndrome as a rare cause of secondary pulmonary alveolar proteinosis: A diagnosis after 5 decades
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