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[Unusual alveolar proteinosis]
L Boussoffara1, N Boudawara, J Knani
1Service de pneumologie, hôpital Tahar Sfar de Mahdia, Tunisie. leila boussoffara@yahoo.fr
Revue De Pneumologie Clinique
|June 14, 2011
Summary
Pulmonary alveolar proteinosis (PAP) presented unusually with membrane expectoration. This rare lung disease diagnosis was confirmed via bronchoalveolar lavage and biopsy, with physiotherapy leading to a favorable outcome.
Area of Science:
- Pulmonology
- Rare Diseases
- Pathology
Background:
- Pulmonary alveolar proteinosis (PAP) is a rare infiltrative lung disease characterized by surfactant accumulation in alveoli.
- Diagnosis typically involves bronchoalveolar lavage (BAL) and lung biopsy.
- Etiology of PAP remains largely unknown.
Observation:
- A 49-year-old woman presented with chronic expectoration of membranes, an unusual symptom for PAP.
- Imaging revealed alveolar opacities and ground-glass opacities with interlobular thickening.
- BAL showed opaque fluid with periodic acid-Schiff stain-positive material.
Findings:
- Anatomopathology confirmed granular eosinophilic material, excluding neoplasia or hydatid cysts.
- The diagnosis of Pulmonary Alveolar Proteinosis (PAP) was established based on clinical and pathological findings.
- The patient exhibited localized lesions and an atypical presentation.
Implications:
- This case highlights an unusual presentation of PAP, emphasizing membrane expectoration as a potential symptom.
- Physiotherapy alone was effective in managing the condition when no functional deterioration was observed.
- The findings contribute to understanding the diverse clinical manifestations of rare lung diseases like PAP.
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