Alveolar proteinosis: diagnosis and treatment over a 10-year period.

Abstract

Ten years' experience of using bronchoalveolar lavage in the treatment of 10 patients with alveolar proteinosis is reported. The diagnosis was often missed. The interval between onset of symptoms and diagnosis varied from six weeks to six years (median 2 years), so that the start of treatment was often delayed. Some patients experienced severe progressive disability before they had treatment. Whole-lung lavage proved to be a safe, repeatable procedure which provided symptomatic, physiological, and radiological improvement and allowed all 10 patients treated to return to full-time employment.Pulmonary alveolar proteinosis is a disease of unknown aetiology characterised by the accumulation of protein, phospholipid, cholesterol, and free fatty acids within the alveolar spaces. The diagnosis is confirmed by typical electron-microscopic findings in sputum, lung washings, or lung biopsy specimens, without which the condition is often mistaken for interstitial lung disease, especially sarcoidosis. Alveolar proteinosis can be treated effectively by means of whole-lung lavage,' and we report 10 years' experience of the method-53 lavages in 10 patients-to emphasise the hazards of delay in diagnosis, the safety of bronchopulmonary lavage, and the benefit which results. Patients and methodsTen patients (eight men and two women) aged 23-48 years were referred either for diagnosis (five patients) or for further management (five patients).All were dyspnoeic at rest or on mild exercise and only one, with a sedentary occupation, was still able to work. All were current smokers or ex-smokers.Measurements were made of dynamic and static lung volumes, airway resistance, gas transfer factor and the transfer coefficient, the results being expressed as percentages of the means of the predicted normal range.2 Arterial blood gas tensions were

Long-term durable benefit after whole lung lavage in pulmonary alveolar proteinosis

“…In 55 reviewed instances, SEYMOUR and PRESNEILL [5] reported a median duration of clinical benefit of 15 months, with v20% of patients followed beyond 3 yrs remaining free of recurrent PAP manifestations. These results should not be too surprising, taking into account the fact that the cases reviewed were from a rather remote period, ranging late 1960s-1980s, at the dawn of the WLL technique [2,[17][18][19][20]. In contrast, the present series was collected in the 1990s, when the procedure had already improved considerably [4], and the present refinements to the treatment may explain the improvement in the duration of response to WLL.…”

Pulmonary Alveolar Proteinosis

“…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%.…”

Rare Infiltrative Lung Diseases: A Challenge for Clinicians

“…The true prevalence is unknown with current understanding based on fewer than 500 reported cases [5, 9, 10,22,23,24,25]. The peak age of onset is between 30 and 50 years but infants and children may also be affected.…”