Autoimmune pulmonary alveolar proteinosis successfully treated with lung lavage in an adolescent patient: a case report

Abstract

Background Pulmonary alveolar proteinosis is a rare interstitial lung disease characterized by accumulating surfactant materials in the alveoli. The autoimmune form is by far the most common in adults, while in the pediatric age group, the vast majority of cases are congenital. We report a case of an adolescent patient diagnosed with autoimmune pulmonary alveolar proteinosis, which is unusual in this age group. Case presentation A-15 year-old Saudi male presented to the emergency department with a history of shortness of breath and low oxygen saturation. High-resolution computed tomography of his chest showed a global crazy-paving pattern. Autoantibodies against granulocyte-macrophage colony-stimulating factor were detected in his serum. A diagnosis of the autoimmune form of pulmonary alveolar proteinosis was confirmed after excluding other possible causes. The patient improved after he underwent whole lung lavage under general anesthesia, and he was independent of oxygen therapy after 6 months of follow-up. Conclusion The autoimmune form of pulmonary alveolar proteinosis is rare in the pediatric age group and should be considered when no apparent cause of this disease was found. Whole lung lavage should be the first treatment modality offered in this setting with close follow-up and monitoring.

Alveolar Proteinosis: Bronchoalveolar Lavage Experience and Current Literature Review

“…There was a dramatic improvement was observed in dyspnea symptoms and oxygen demand, after lavage with 10 L of saline for both the left and right lung respectively, in 2 sessions performed 72 hours apart, lasting approximately 4 hours. After a 6-month follow-up, it was reported that the patient's room air oxygen saturation was 93% and his symptoms had completely resolved [15]. The studies shared in the literature review and their characteristics are summarized in Table-2.…”

Autoimmune pulmonary alveolar proteinosis in children

“…Whereas in adults autoantibodies against GM-CSF are the principal cause of PAP, in children this cause is rarely identified [ 2 ]. Until now, <10 cases have been published [ 3 – 9 ], whereas a genetically caused interruption of signal transduction of the GM-CSF receptor due to mutations in downstream α- or β-subunit of the receptor has been more frequently described [ 2 ].…”