Transient Pulmonary Interstitial Lesions in Aquaporin-4-positive Neuromyelitis Optica Spectrum Disorder

Abstract

We herein report the case of a 76-year old man with aquaporin-4-Immunoglobulin-G(AQP4-IgG)-positive neuromyelitis optica spectrum disorder (NMOSD), in whom transient interstitial pulmonary lesions developed at the early stage of the disease. Chest X-ray showed multiple infiltrative shadows in both upper lung fields, and computed tomography revealed abnormal shadows distributed randomly in the lungs. Surgical lung biopsy showed features of unclassifiable interstitial pneumonia, characterized by various types of air-space organization, which resulted in obscure lung structure. This is the first report to describe the pathological findings of interstitial pneumonia, which may represent a rare extra-central nervous system complication of NMOSD.

Aquaporins in lung health and disease: Emerging roles, regulation, and clinical implications

“…In paraneoplastic NMOSD associated with squamous cell lung carcinoma in a 66-year old female, anti-AQP4 antibodies were present in both the serum and cerebrospinal fluid of the patient but absent on the tumor surface [Reviewed in [127]. AQP4 antibody-positive NMOSD has also been reported to be associated with interstitial pneumonia as another rare extra-CNS complication of this disease [128].…”

Neuromyelitis Optica Spectrum Disorder After BIBP COVID-19 Vaccine: A Case Report

“…Likewise, our patient presented with venous thrombosis of the left lower limb, NMOSD is known to be a risk factor for venous thromboembolism (19). Additionally, she presented an alteration of the pulmonary tomography without clinical translation, an interstitial pulmonary lesion has been described since the Aquaporins4 are found in large quantities at the pulmonary level (20).…”

Neuromyelitis optica with rapid respiratory failure: a case report

“…Although cases exhibiting prolonged respiratory failure with advancement of nerve lesions are not rare, those with acute respiratory failure are very rare. There are some reports of patients with transient interstitial changes in the lungs due to NMO spectrum disorders 7 . At the time of transfer to our hospital, our patient was receiving antibiotic treatment for pneumonia.…”

“…There are some reports of patients with transient interstitial changes in the lungs due to NMO spectrum disorders. 7 At the time of transfer to our hospital, our patient was receiving antibiotic treatment for pneumonia. NMO was suspected due to the rapid emergence of symptoms in the extremities, but there is a great deal of urgency associated with this condition given the fact that it is not well known in the field of critical care and that it progresses rapidly.…”