The Clinical Characteristics of Primary Sjogren's Syndrome With Neuromyelitis Optica Spectrum Disorder in China: A STROBE-Compliant Article

The aim of the present study was to analyze the clinical characteristics of primary Sjogren's syndrome (pSS) with neuromyelitis optica spectrum disorder (NMOSD). We retrospectively reviewed the medical records of 616 patients who were admitted to the Peking Union Medical College Hospital from 1985 to 2013. Of these patients, 43 developed NMOSD. The median duration of symptoms was 60 months and 72% of the patients experienced neurological complications onset in the pSS with NMOSD group. Twenty-one out of 43 patients had neuromyelitis optica (NMO), and 22 exhibited a limited form of NMO. Serum anti-aquaporin-4 (AQP4) antibody positivity was detected in 89.3% of the patients. A total of 60.5% of the patients (26 patients) complained of dry mouth, 72.1% were positive for objective xerostomia, 53.5% complained of dry eyes, and 74.4% had a positive ocular test. Biopsy of the minor salivary glands was performed in 33 patients, 28 of whom (84.8%) had a lymphocytic focus score of ≥1. Anti-Ro/SSA or anti-La/SSB antibodies were detected in 41 patients (95.3%). Compared with the pSS patients without NMOSD, the incidences of xerophthalmia, xerostomia, arthritis, interstitial lung disease, and renal tubular acidosis were significantly lower in the patients with NMOSD. NMOSD is a neurologic complication of pSS. The presence of anti-AQP4 antibody may be a predictor for pSS patients with NMOSD. Neurological manifestations are prominent in these patients. In clinical scenarios involving pSS or NMOSD, rheumatologists and neurologists should be aware of this association and perform the appropriate tests.