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Case Reports
. 2021 Jul 9;15(1):340.
doi: 10.1186/s13256-021-02906-2.

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

Abdalla Mohmed Alasiri  1 , Reem Abdullah Alasbali  2 , Meaad Ali Alaqil  1 , Aishah Marei Alahmari  1 , Nouf Dagash Alshamrani  1 , Rabab Nasir Badri  3
Affiliations
Case Reports

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

Abdalla Mohmed Alasiri et al. J Med Case Rep. .

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.

Keywords: Autoimmune pulmonary alveolar proteinosis; Bronchoalveolar lavage; Case report; Crazy-paving pattern; GM-CSF; Whole lung lavage.

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Conflict of interest statement

The authors declare that they have no competing interests.

Figures

Fig. 1
Fig. 1
Chest X-ray showing extensive bilateral alveolar scattered and coalescent alveolar infiltrate involving both lung fields
Fig. 2
Fig. 2
High-resolution tomography of chest showing extensive diffuse bilateral thickening of the lung interstitium with superimposed interlobular septal thickening consistent with “crazy paving” pattern
Fig. 3
Fig. 3
A milky appearance of the secretions throughout all the airway
Fig. 4.
Fig. 4.
Milky appearance of the bronchoalveolar lavage fluid
Fig. 5
Fig. 5
Bronchoalveolar lavage smears showing many dense globules (arrows) in background of finely granular eosinophilic material using (a) hematoxylin and eosin stain (H&E) ×400 and (b) Papanicolaou stain ×400. Cell block of bronchoalveolar lavage showing granular and globular eosinophilic material that was Periodic acid–Schiff (PAS) positive using (c) H&E stain ×400 and (d) PAS stain ×400
Fig. 6
Fig. 6
Classification of pulmonary alveolar proteinosis based on underlying cause. GM-CSF granulocyte-macrophage colony-stimulating factor
See this image and copyright information in PMC

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