Abstract
Pulmonary alveolar proteinosis is a very rare interstitial lung disease caused by abnormal intra-alveolar surfactant accumulation. Usually, it appears as a "crazy-paving" pattern on high-resolution computed tomography. The image is so typical, that together with the characteristic bronchoalveolar lavage examination with presence of Periodic Acid Schiff positive substance is sufficient for establishing diagnosis, without histological confirmation. We present the case of the young woman with severe dyspnoea suspected of acute hypersensitivity pneumonia. The computed tomography showed numerous intralobular nodules uniformly distributed troughout the lungs. Treatment by corticosteroids had no clinical effect and next computed tomography showed progression. Despite the high risk of complications (patient had a respiratory failure), a surgical lung biopsy was performed and the histopathological diagnosis of pulmonary alveolar proteinosis was made. The whole lung lavage procedure performed twice caused regression of radiological lesions and respiratory failure.
Highlights
Pulmonary alveolar proteinosis (PAP) is a rare disease with a prevalence of 3.7–6.2/1 000 000
The image is so typical, that together with the characteristic bronchoalveolar lavage (BAL) examination with presence of Periodic Acid Schiff (PAS) positive substance is sufficient for establishing diagnosis [2]
We present the case of the young woman with severe dyspnoea and radiological image suggesting hypersensitivity pneumonia (HP)
Summary
The final diagnosis of PAP was established basing on the histological examination of surgical lung biopsy specimens. Chest radiograph showed bilateral non-specific disseminated lesions and high resolution computed tomography (HRCT) appeared numerous intralobular nodules uniformly distributed throughout the lungs (Figure 1). At last follow-up after 6 weeks, the patient was asymptomatic and chest radiograph showed regression of the disseminated lesions (Figure 3A, B).
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