SESSION TYPE: Interesting Topics in ILD
PRESENTED ON: Tuesday, October 23, 2012 at 02:45 PM - 04:15 PM
PURPOSE: Pulmonary alveolar proteinosis (PAP) is a rare disease in which lipoproteinaceous material accumulates within alveoli. Clinical characteristics and outcomes of PAP were studied to facilitate the diagnosis and treatment of this rare entity.
METHODS: We analyzed the clinical manifestations, radiologic findings,pathological ,treatment and follow-up data of 25 patients with PAP from Shanghai Pulmonary Hospital since Oct 1998 to Oct 2011. and a thorough review of medical literature was also made.
RESULTS: There were 17 males and 8 females with a mean age 43.19 ± 7.37 years. Exertional dyspnea and cough was the most common symptoms. Chest X-ray showed that bilateral extensive patchy lesions with flaky invasive foci, which were more prominent in the middle and lower lung fields with significant hilar infiltration mimicking cardiogenic pulmonary edema. Chest HRCT showed characteristic “Geographic” or “Crazy-Paving” signs. pulmonary restrictive ventilatory defect and diffuse dysfunction was found in 12 cases, the other 7 cases were normal. 7 cases underwent bronchoscopic lung biopsy (TBLB) and bronchoalveolar lavage (BALF), 3 cases had alveolar lavage and pathological analysis of PAS staining,15 cases did thoracoscopic lung biopsy. 13 cases underwent pulmonary lavage therapy under general anesthesia, and follow-up visits showed that their clinical symptoms and chest radiographic lesions were significantly improved. Three cases could not tolerate pulmonary lavage therapy and switched to receive the treatment of subcutaneous recombinant human granulocyte - macrophage colony stimulating factor (rHuGM-CSF). After a follow-up period of for 5 , 7 and 19 months , their symptoms significantly improved and chest CT scan proved that lesions significantly resolved. The other 9 cases with milder disease were followed-up alone. After follow-up of 1 to 9 years, the patients were still in a stable condition.
CONCLUSIONS: PAP has some special imaging features such as : diffuse bilateral lung disease, pulmonary edema-liking findings, and Crazy-Paving or Geographic sign. TBLB, BALF or thoracoscopic lung biopsy is helpful to establish a pathologic diagnosis. Pulmonary Lavage therapy proved to be an effective method; in patients with milder disease, observation is also an option. For patients with severe disease, subcutaneous rHuGM-CSF shows efficacy.
CLINICAL IMPLICATIONS: It will be helpful for clinicians to further understand the clinical features and outcomes of pulmonary alveolar proteinosis.
DISCLOSURE: The following authors have nothing to disclose: Haiqing Chu, Jinming Liu, Lan Zhao, Tao Gui
No Product/Research Disclosure InformationShanghai Pulmonary Hospital, Shanghai, China