Patient Characteristics, Treatment and Prognosis in Patients with Neuromyelitis OpticaSpectrum Disorder Coexisting with Systemic Lupus Erythematosus or Sjögren's Syndrome: An Analysis Using Japanese Inpatient Database

Hiroshi Shiba¹, Mikio Nakajima², Kaito Nakamura³, Yusuke Sasabuchi⁴, Hiroyuki Ohbe⁵, Yuya Kimura⁶, Tadahiro Goto⁷, Hiroki Matsui⁸, Kiyohide Fushimi⁹ and Hideo Yasunaga⁵, ¹Suwa central hospital, Chino, Japan, ²Emergency and Critical Care Center, Tokyo Metropolitan Hiroo Hospital, Hiroo, Tokyo, Japan, ³Department of Rheumatology, Dokkyo Medical University, Shimotsuga, Tochigi, Japan, ⁴Department of Real-world Evidence, Graduate School of Medicine, The University of Tokyo, Bunkyo-ku, Tokyo, Japan, ⁵Department of Clinical Epidemiology and Health Economics, School of Public Health, The University of Tokyo, Bunkyo-ku, Tokyo, Japan, ⁶Department of Health Services Research, Graduate School of Medicine, The University of Tokyo, Bunkyo-ku, Tokyo, Japan, ⁷Department of Health Data Science, Graduate School of Data Science, Yokohama City University, Yokohama, Kanagawa, Japan, ⁸Department of Clinical Epidemiology and Health Economics, School of Public Health, Bunkyo-ku, Tokyo, Japan, ⁹Department of Health Policy and Informatics, Institute of Science Tokyo, Bunkyo-ku, Tokyo, Japan

Meeting: ACR Convergence 2025

Keywords: neurology, Sjögren's syndrome, Systemic lupus erythematosus (SLE)

Session Information

Date: Tuesday, October 28, 2025

Title: (2437–2469) Systemic Lupus Erythematosus – Treatment Poster III

Session Type: Poster Session C

Session Time: 10:30AM-12:30PM

Background/Purpose: Neuromyelitis optica spectrum disorder (NMOSD) can coexist with systemic lupus erythematosus (SLE) and Sjögren’s syndrome (SS). Due to overlapping clinical presentations and the rarity of these conditions, specific treatment strategies for acute-phase NMOSD with SLE (NMOSD/SLE) and NMOSD with SS (NMOSD/SS) are lacking. This study aimed to investigate the clinical characteristics, treatment and prognosis of patients with NMOSD/SLE and NMOSD/SS.

Methods: This retrospective descriptive study utilized the Diagnosis Procedure Combination (DPC) database, a nationwide inpatient database in Japan. We identified patients with NMOSD (ICD-10 codes, G360) between 2010 and 2023. Patients were categorized into three groups: NMOSD alone, NMOSD/SLE and NMOSD/SS. We extracted clinical data during hospitalization and compared patient characteristics, treatments and outcomes between these groups.

Results: A total of 8,029 patients were included: NMOSD alone (n=7,507), NMOSD/SLE (n=217), and NMOSD/SS (n=305). The median age was 55 years and 76% were female. NMOSD/SLE patients had a higher burden of comorbidities compared to those with NMOSD alone or NMOSD/SS. The treatment regimen included more frequent use of plasma exchange and cyclophosphamide, and a wider use of immunosuppressive agents in NMOSD/SLE patients. The percentage of plasma exchange were: NMOSD alone 31.1%, NMOSD/SLE 44.2%, NMOSD/SS 36.1% (p< 0.001). The percentage of intravenous cyclophosphamide administration were: NMOSD alone 0.4%, NMOSD/SLE 4.1%, NMOSD/SS 2.3% (p< 0.001). The percentage of rituximab administration were: NMOSD alone 0.4%, NMOSD/SLE 1.4%, NMOSD/SS 0.3% (p=0.056). The most common disease-modifying antirheumatic drug was azathioprine (NMOSD alone 11.3%, NMOSD/SLE 46.1%, NMOSD/SS 18.7%, p< 0.001), followed by tacrolimus, cyclosporine, and mycophenolate mofetil. Crude in-hospital mortality was 1.3%. There was no significant difference in in-hospital mortality across all groups; however, NMOSD/SLE patients had better activities of daily living scores and were more likely to be discharged to home. The proportion of patients with a Barthel index of 100 (total independent) in the three groups were: NMOSD alone 57.7%, NMOSD/SLE 60.8%, NMOSD/SS 50.2% (p=0.006). The percentage of discharge to home were: NMOSD alone 74.1%, NMOSD/SLE 83.4%, NMOSD/SS 69.2% (p=0.001).

Conclusion: Although NMOSD/SLE patients had a greater burden of comorbidities, they received a broader range of treatments during the acute phase and showed favorable outcomes in terms of functional status and discharge to home.

Disclosures: H. Shiba: None; M. Nakajima: None; K. Nakamura: None; Y. Sasabuchi: None; H. Ohbe: None; Y. Kimura: None; T. Goto: None; H. Matsui: None; K. Fushimi: None; H. Yasunaga: None.

To cite this abstract in AMA style:

Shiba H, Nakajima M, Nakamura K, Sasabuchi Y, Ohbe H, Kimura Y, Goto T, Matsui H, Fushimi K, Yasunaga H. Patient Characteristics, Treatment and Prognosis in Patients with Neuromyelitis OpticaSpectrum Disorder Coexisting with Systemic Lupus Erythematosus or Sjögren’s Syndrome: An Analysis Using Japanese Inpatient Database [abstract]. Arthritis Rheumatol. 2025; 77 (suppl 9). https://acrabstracts.org/abstract/patient-characteristics-treatment-and-prognosis-in-patients-with-neuromyelitis-opticaspectrum-disorder-coexisting-with-systemic-lupus-erythematosus-or-sjogrens-syndrome-an-analysis-using-japanese/. Accessed .

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/patient-characteristics-treatment-and-prognosis-in-patients-with-neuromyelitis-opticaspectrum-disorder-coexisting-with-systemic-lupus-erythematosus-or-sjogrens-syndrome-an-analysis-using-japanese/