Researchers discovered a rare case of neuromyelitis optica spectrum disorder (NMOSD) associated with cryptogenic organizing pneumonia (COP) in a young patient, as published in the European Journal of Case Reports in Internal Medicine. The case appears to be unique given that the only 2 prior reports of this association were in elderly patients.

“This case is unusual and interesting as this young girl presented with symptoms of early-onset NMOSD along with COP,” the authors wrote. “Since the pathogenesis of NMSOD involves the development of antibodies against aquaporin-4 [AQ4], which is also expressed in lung tissue, it is likely that this occurrence is a true association rather than a coincidence.”

The presence of AQ4 antibodies distinguishes NMOSD from multiple sclerosis and other CNS disorders, and its presence in lung tissue and COP, a chronic inflammatory lung disease, suggests a link between the 2 conditions.

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AQ4 is also present in a number of other conditions including acute lung injury, chronic obstructive pulmonary disease, and asthma. However, the underlying role played by AQ4 in these conditions is not yet known.

The differential diagnosis of NMOSD involves neuroimaging, AQ4 antibody detection, and an analysis of cerebrospinal fluid. In this case, involving a 14-year old girl, the neuroimaging and the finding of AQ4 antibodies confirmed her diagnosis.

Although she had symptoms of COP and ground-glass opacities on chest computed tomography (CT), due to her age and lack of radiological findings the team decided against a lung biopsy to confirm that diagnosis. The patient was treated for NMOSD with rituximab and corticosteroids and given that immunosuppression and glucocorticoid therapy are also standard for COP, this treatment was continued.

The authors noted that despite persistent COP findings on CT and evidence of ongoing neurological disease, the patient had completely normal lung function. The team emphasizes the fact that understanding the association between COP and NMOSD is essential for optimal diagnoses and management of these patients.


Shafiq I, Wahla AS, Uzbeck MH, Khan N, Zoumot Z. Neuromyelitis optica spectrum disorder associated with cryptogenic organizing pneumonia in a young patient. EJCRIM. 2022;9. doi:10.12890/2021_003105