Autoimmune Pulmonary Alveolar Proteinosis (APAP) is an acquired condition associated with a concurrent autoimmune disease. The diagnosis may be delayed due to its infrequency.
Clinical features:
(1) onset of dyspnea and cough
(2) presence of pulmonary alveolar proteinosis
(3) evidence of an autoimmune disease (rheumatoid arthritis, SLE, systemic sclerosis, sarcoidosis, other)
(4) presence of autoantibodies to GM-CSF (granulocyte/macrophage colony stimulating factor)
(5) exclusion of other mechanisms for pulmonary alveolar proteinosis
A patient with autoimmune pulmonary alveolar proteinosis may develop interstitial fibrosis.
Therapy may include:
(1) rituximab
(2) exogenous GM-CSF
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