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Successful Treatment of Autoimmune Pulmonary Alveolar Proteinosis in a Pediatric Patient

Management of emergency care, Rare disease, Adverse events of drug therapy , Educational Purpose (only if useful for a systematic review or synthesis)

Mirjana Trukalj, Marija Perica, Željko Ferenčić, Damir Erceg, Marta Navratil, Gzim Redžepi, Boro Nogalo

Croatia Department of Pediatric Allergology and Pulmonology, Children’s Hospital Srebrnjak, Zagreb, Croatia

Am J Case Rep 2016; 17:641-645

DOI: 10.12659/AJCR.897868

Available online:

Published: 2016-09-05


#897868

BACKGROUND: Pulmonary alveolar proteinosis (PAP) is a rare condition characterized by the intra-alveolar accumulation of surfactant-derived material, which impairs gas exchange and results in respiratory insufficiency. Two major subtypes of PAP are autoimmune and non-autoimmune PAP. The diagnosis relies on clinical presentation, ground glass opacities on CT scan, bronchoscopy with PAS stain of BAL fluid (BALF), lung biopsy with PAS-positive material in the alveoli, and the presence of anti GM-CSF antibodies in serum or BALF for an autoimmune subtype. The therapeutic approach to pediatric cases varies according to age and the general clinical state of the child; however, whole lung lavage (WLL) and inhaled or subcutaneous GM-CSF are generally first-line therapy.
CASE REPORT: We report a unique case of an autoimmune type of PAP in a 12-year-old boy, who underwent successful bilateral lung transplantation after inefficacious treatment with GM-CSF, and who developed post-transplant lymphoproliferative disease (PTLD) and was successfully treated with a chemotherapeutic protocol.
CONCLUSIONS: Although lung transplantation is a rarely used therapeutic approach for patients with an autoimmune subtype of PAP, in cases of inefficacious treatment with other modalities, lung transplantation should be considered.

Keywords: Biopsy, Autoimmune Diseases, Bronchoalveolar Lavage - methods, Bronchoscopy, Child, Preschool, Granulocyte-Macrophage Colony-Stimulating Factor - therapeutic use, Lung - diagnostic imaging, Pulmonary Alveolar Proteinosis - therapy, Tomography, X-Ray Computed



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