Comparative study of clinical, pathological and HRCT findings of primary alveolar proteinosis and silicoproteinosis

ObjectiveTo compare the clinical, high-resolution computed tomography (HRCT) and pathological findings of primary alveolar proteinosis (PAP) and silicoproteinosis.Material and methodsThe study included 15 patients with PAP (6 women, 9 men, mean age 31 years) and 13 with silicoproteinosis (13 men, mean age 29.5 years). PAP was diagnosed by lung biopsy in 13 and bronchoalveolar lavage in two patients and silicoproteinosis by bronchoalveolar lavage in 10 and autopsy in three cases. HRCT images were reviewed by two chest radiologists with consensus for the presence, extent and distribution of ground-glass opacities, septal thickening, consolidation and nodules. Radiological–pathological correlation was performed by one radiologist and one chest pathologist.ResultsSeven (46%) patients with PAP were asymptomatic; the remainder presented slowly progressive dyspnea and dry cough. All silicoproteinosis patients had dry cough and rapidly progressive dyspnea. The most common HRCT finding on PAP was the crazy-paving pattern (93%). All cases had areas of geographic sparing in the affected lung. The most common finding in silicoproteinosis (92%) was dependent consolidation with calcification in 83%. Centrilobular nodules were common (85%). On pathology, both diseases demonstrated intra-alveolar accumulation of PAS material, thickening of interlobular septae and alveolar walls and no evidence of fibrosis. A few silica particles were seen in silicoproteinosis.ConclusionDespite the pathological similarities, PAP and silicoproteinosis have distinct clinical and imaging features and prognosis. Bilateral crazy-paving pattern with areas of geographic sparing is characteristic for PAP. Silicoproteinosis presents with bilateral dependent consolidation often with areas of calcification. The crazy-paving pattern is not seen in silicoproteinosis.