Autoimmune pulmonary alveolar proteinosis with progressive fibrosis refractory to treatment with whole lung lavage, inhaled GM-CSF and rituximab

Abstract

Pulmonary fibrosis occurs in up to 30% of patients with pulmonary alveolar proteinosis, and pulmonary hypertension is rarely identified. A previously healthy, highly active, asymptomatic 59-year-old male was diagnosed with autoimmune pulmonary alveolar proteinosis. He was followed clinically for five years until he developed disease progression with dyspnea on exertion. In the interim, he had developed pulmonary fibrosis, and was later found to have pulmonary hypertension. He continued to decline despite aggressive treatment with whole-lung lavage, inhaled granulocyte-macrophage colony-stimulating factor, and rituximab and now has very limited activity, despite use of supplemental oxygen. Pulmonary fibrosis and pulmonary hypertension are uncommon and unpredictable complications of pulmonary alveolar proteinosis. Current treatment regimens for pulmonary alveolar proteinosis may not benefit patients once fibrosis is established. It is not known whether early aggressive treatment can stabilize or delay disease progression.