Primary pulmonary alveolar proteinosis.
2012
Introduction. Pulmonary alveolar proteinosis is an uncommon disease
characterized by the accumulation of surfactant proteins and phospholipids
within the alveolar spaces. Acquired disease can be idiopathic (primary) and
secondary. The prevalence of acquired pulmonary alveolar proteinosis is about
0.37 per 100,000 persons. Common symptoms are dyspnea and cough. Chest X-ray
shows bilateral perihilar infiltrates. Open-lung biopsy is the gold standard
for the diagnosis. Treatment includes whole-lung lavage, application of
granulocyte-macrophage colonystimulating factor and lung transplantation.
Case report. We reported a 51 year-old man with primary form of the disease.
It was the second case of this extremely rare disease in the past 30 years in
our clinic. The symptoms were longlasting dry cough, fever and physical
deterioration. Chest Xray revealed bilateral pulmonary infiltrates; computed
tomography showed patchy ground-glass opacification with interlobular
thickening. The diagnosis was established by open lung biopsy. Additional
tests were performed to exclude secondary form of the disease. Conclusion. We
presented a rare clinical entity with typical clinical features and clinical
and radiological course of the disease, in order to improve differential
diagnostic approach to patients with bilateral lung infiltrations. In
patients with pulmonary alveolar proteinosis timely diagnosis and adequate
treatment can improve a prognosis.
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