Abstract
Pulmonary alveolar proteinosis is a rare disease of alveolar surfactant accumulation. Primary, autoimmune etiology accounts for 90% of cases. This report presents a case of secondary alveolar proteinosis, a metal worker with a pulmonary infection who was subsequently diagnosed with alveolar proteinosis based on the results of bronchoalveolar lavage and lung biopsy. He underwent complete resolution of the alveolar proteinosis after whole lung lavage and change of his workplace. Long term follow-up hasn’t shown any sequelae and he has suffered no relapse. This favorable disease course is representative of secondary alveolar proteinosis with a reversible causative agent.
Highlights
Pulmonary alveolar proteinosis (PAP) is a rare disease with an estimated incidence of 0, 36 per million [1]
We present a case of secondary PAP, due to occupational exposure to mineral dust and the long-term follow-up after treatment with whole lung lavage
The acquired or idiopathic form represents 90% of all PAP cases and is an autoimmune disorder with autoantibodies against the granulocyte-macrophage colony stimulating factor (GM-CSF) that reduce the number of alveolar macrophages in the lungs [4,5]
Summary
Pulmonary alveolar proteinosis (PAP) is a rare disease with an estimated incidence of 0, 36 per million [1]. The followup chest X-ray one month later showed interstitial changes and he was referred to our Department for Pulmonary diseases and Allergy for evaluation On examination, his physical status was normal with a pulse rate of 84/min, blood pressure 120/80, respiratory rate of 14/min and oxygen saturation of 96%, without abnormal respiratory phenomena. His physical status was normal with a pulse rate of 84/min, blood pressure 120/80, respiratory rate of 14/min and oxygen saturation of 96%, without abnormal respiratory phenomena He had a repeat chest X-ray that showed diffuse reticulonodular opacities, especially in the lower lobes, more on the right side than on the left (Figure 2). Bronchoscopy with Bronchial Alveolar Lavage (BAL) and a transbronchial lung biopsy of the right lower lobe were performed. The patient is in good physical condition, pulmonary function tests performed in 2014 have shown no deterioration (Table 1)
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