Bronchoalveolar Lavage Findings in a Pediatric Patient With Primary Pulmonary Alveolar Proteinosis

Abstract

Pulmonary alveolar proteinosis (PAP) is an uncommon cause of respiratory failure characterized by abnormal accumulations of surfactant proteins within alveolar spaces. 1 PAP can be due to an autoimmune process or secondary to various systemic diseases including hematopoietic disorders, immune dysregulation, infections, inhalations, lysinuric protein intolerance, iatrogenic, or secondary to drugs. 2 Autoimmune PAP represents 90% to 95% of adult cases of PAP but is more rare in pediatrics, accounting for 5% to 10% of all cases. 2 In this article, we present a case of PAP in the pediatric setting, with serology confirming a primary autoimmune process. A 13-year-old female patient presented with a 6-month history of progressive dyspnea on exertion. On admission, she was found to be hypoxemic, requiring oxygen therapy. Chest computed tomography scan revealed diffuse groundglass opacities accompanied by inter-and intra-lobular septal thickening, a pattern classically described as "crazy paving." She proceeded to whole lung lavage requiring extracorporeal membrane oxygenation (ECMO) for both diagnostic and therapeutic indications. Microscopy demonstrated dense eosinophilic, amorphous globules (Figure 1A), which were periodic acid-Schiff-positive and diastase resistant (Figure 1B), in a background of acellular debris and scant inflammatory cells. Electron microscopy showed these globules to have concentric lamellations characteristic of surfactant bodies (Figure 1C). These pathologic findings were consistent with the clinical suspicion of PAP. 3,4 Further serologic testing demonstrated anti-granulocyte-macrophage colony-stimulating factor (GM-CSF) antibodies, in keeping with autoimmune PAP. Her respiratory status significantly improved following the lavage. She was decannulated from ECMO and successfully extubated. She was discharged home with daily GM-CSF inhalations and nocturnal, supplemental oxygen with outpatient follow-up.

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

“…We cannot exclude the possibility of publication bias. At the onset of the symptoms, the mean age of patients was 12 (±3.04) years; the majority of them were female (64.28%) [ 24 – 36 ].…”

“…[ 25 ] 17 F Exertional SOB Cough No WLL NR USA Shivji et al . [ 24 ] 13 F Exertional SOB No WLL Improved Canada Current report 15 M Exertional SOB Dry cough No WLL Improved Saudi Arabia a Age of patient at onset of symptoms b Disease onset was at age of 11 years. However, the bronchoalveolar lavage fluid was obtained and analyzed at age of 24 years with evidence of elevated level of anti-GM-CSF F female, FFT failure to thrive, M male, NR not reported, rhGM-CSF recombinant human granulocyte-macrophage colony-stimulating factor, SOB shortness of breath, WLL whole lung lavage …”

“…The clinical presentation of aPAP typically follows an insidious and progressive course [ 37 ]. Among the reported cases of pediatric aPAP, the following symptoms were reported (Table 2 ): exertional shortness of breath (78%), cough (dry or productive) (64%), fatigue (14%), fever, weight loss, failure to thrive, and recurrent respiratory infections (7% each) [ 24 – 36 ]. Lung crackles, finger clubbing, cyanosis, and respiratory distress may be noted upon clinical examination [ 7 ].…”