Pulmonary Alveolar Phospholipoproteinosis: Experience With 34 Cases and a Review

Abstract

A retrospective review of Mayo Clinic records through 1983 revealed 84 patients (24 male and 10 female; mean age, 41 years) with the diagnosis of pulmonary alveolar phospholipoproteinosis. The major clinical features were dyspnea, cough, fever, and chest pain. Chest roentgenograms usually showed bilateral symmetric alveolar infiltrates, but asymmetric, unilateral, and chronic patchy patterns were also noted. Diagnosis was established by thoracotomy-lung biopsy in 26 patients. Histologic analysis revealed uniform filling of the alveoli by periodic acid-Schiff-positive material and maintenance of normal alveolar architecture. Electron microscopy showed enlarged alveolar macrophages with lamellar osmiophilic inclusions, dense granules, and myeloid bodies. Of the 21 patients who underwent therapeutic bronchoalveolar lavage, 13 had no recurrence of the disease during a mean follow-up of 8.8 years. In patients who underwent pulmonary function testing both before and after lavage, significant restrictive dysfunctions present before the procedure were alleviated afterward. Three deaths occurred among the 34 patients. Pulmonary alveolar phospholipoproteinosis may result from defective clearance of phospholipids by the alveolar macrophages, excessive production of phospholipids by type II pneumocytes, or both. It is likely a nonspecific response to a variety of injuries to the alveolar macrophage or type II pneumocyte or both, including exposure to certain dusts and chemicals and occurrence of hematologic diseases or infections. The uncommon occurrence of this disorder suggests individual susceptibility.

Pulmonary Alveolar Proteinosis

“…9,12,100,129 In addition to the subjective assessment of symptoms, pulmonary function tests, exercise capacity, gas exchange, and serum and bronchoalveolar biomarkers are used to assess disease severity and to guide management decisions.…”

“…98 In some centers, P aO 2 Ͻ 65 mm Hg, P (A-a)O 2 Ͼ 40 mm Hg, or a shunt fraction Ͼ 10 -12% are used as threshold values for therapeutic lung lavage. 129,130 Clinical, 129,130,177 functional, 100,129,177 and radiological 178 improvement are seen in approximately 80% of patients after the first whole-lung lavage, and the median duration of benefit can be as long as 15 months, but the majority require repeat lavage. 12 Approximately 15% require lavage every 6 months, and lack of response is reported in less than 10%.…”

ALVEOLAR PROTEINOSIS (A Case Report)

“…It is believed to be due to disorder of surfactant homeostasis where surfactants cannot be removed by defective alveolar MJAFI. 50: 3, JULY 1994 macrophages [3][4][5]. In many cases no predisposing factors are found and they arc termed "primary".…”

“…In some cases various predisposing factors like consanguinity in marriage, pulmonary alveolar proteinosis in siblings, previous infective processes like tuberculosis, fungal diseases. HIV infection, connective tissue diseases and even use of some drugs are present and this group is called secondary [2,4,5,7].…”

Clinical Biological and Genetic Heterogeneity of the Inborn Errors of Pulmonary Surfactant Metabolism

“…Alveolar proteinosis can be diagnosed at any age, from neonate (42) through to maturity (72 years) (43), the age at the diagnosis being most often between 20 and 50 (44). In the child and adult, the revealing signs are, in more than half of the cases, dyspnea and cough with progressive onset, sometimes with cyanosis, finger clubbing, asthenia, and weight loss.…”