| ObjectivePulmonary alveolar proteinosis (PAP) a rare disease and commonly misdiagnosised which has insidious onset and nonspecific clinical symptoms.In order to improve the awareness of PAP, we summarized clinical presentations, laboratory tests, imaging features, treatment and efficacy of 14 cases confirmed by pathology.Methodssystemic review of clinical details and therapeutic efficacy of 14 cases which were confirmed by pathology between 2010.01.01 and 2016.01.01.ResultsAmong the 14 patients,11 cases were males (78.6%) and 3 cases were females (21.4%).Patients’ages ranged from 40 to 65 years old. Most cases had insidious onset and common clinical presentations of 14 cases included progressive short of breath after activity, cough and expectoration.Arterial blood gas analysis revealed different degrees of hypoxemia and pulmonary hyperinflation.Pulmonary function test appeared restrictive ventilation dysfunction and reduced diffusion dysfunction.Chest CT showed both lungs had multiple high dense shadows, including patch, lattice or ground-glass opacity, and some could typically be crazy-paving pattern.Pathological test was the evidence of diagnosis.The clinical presentations, symptoms, arterial blood gas analysis, pulmonary function test and chest CT performance of 8 cases were greatly improved after whole lung lavage.Conclusion1.The clinical presentations of PAP are nonspecific.We should consider PAP when imaging features show both lungs have multiple high dense shadows including patch, lattice, ground-glass opacity or crazy-paving pattern.2.We confirm PAP by PAS-positive BALF or transbronchoscopic lung biopsy clinically while thoracotomy biopsy is the gold standard.3.Whole lung lavage is a well-established classic treatment.Most patients can have a long remission after a whole-lung lavage while a few patients need several lavages.4.It needs to be verified that establishing good compliance of patients and reducing risk factors like dust exposure can relieve symptoms after whole lung lavage. |
