Case Report: Organizing pneumonia associated with AQP4-antibody neuromyelitis optica spectrum disorder in children

TL;DR

A child with a rare neurological disorder also developed lung inflammation, which improved with treatment and showed antibodies in lung fluid.

Contribution

Demonstrates a rare pulmonary manifestation of AQP4-Ab NMOSD and suggests bronchoalveolar lavage antibody testing as a diagnostic aid.

Findings

An 8-year-old girl with AQP4-Ab NMOSD developed organizing pneumonia.

Anti-AQP4-IgG antibodies were detected in bronchoalveolar lavage fluid.

Lung injury improved with immunotherapy and spontaneously.

Abstract

Aquaporin-4-antibody Neuromyelitis Optica Spectrum Disorder (AQP4-Ab NMOSD) is an immune-mediated inflammatory disorder of the central nervous system, characterized by predominant involvement of the optic nerves and spinal cord. Pulmonary involvement, particularly in the form of organizing pneumonia, remains an uncommon manifestation of AQP4-Ab NMOSD.

We present the case of an 8-year-old girl with AQP4-Ab NMOSD who developed organizing pneumonia concurrent with the onset of her neurological disease. The initial lung injury resolved following immunotherapy, while the subsequent injury improved spontaneously. Anti-AQP4-IgG Antibody was detected positive in the bronchoalveolar lavage fluid.

When lung injury occurs in patients with AQP4-Ab NMOSD, detection of anti-AQP4-IgG antibodies in bronchoalveolar lavage fluid may serve as an adjunctive diagnostic tool for the organizing pneumonia associated with AQP4-Ab NMOSD.

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