Abstract
BackgroundPulmonary 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 presentationA-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.ConclusionThe 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.
Highlights
Pulmonary alveolar proteinosis is a rare interstitial lung disease characterized by accumulating surfactant materials in the alveoli
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
Summary
PAP is a rare interstitial lung disease with multiple types and clinical presentations. aPAP is not the usual form in children and adolescents. APAP is not the usual form in children and adolescents. PAP is a rare interstitial lung disease with multiple types and clinical presentations. It should be considered in the differential diagnosis after excluding more common causes such as congenital and secondary forms. WLL should be the first-line treatment with or without inhaled rhGM-CSF
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