Drug-Induced Lupus Erythematosus After 14 Years of Carbamazepine Use in a Patient With Neuromyelitis Optica Spectrum Disorder - A Case Report

Sep 29, 2026

Drug-induced lupus erythematosus developed 14 years after carbamazepine (CBZ) therapy in a 61-year-old woman with aquaporin-4-IgG–positive neuromyelitis optica spectrum disorder (NMOSD). She presented with general fatigue, intermittent fever, night sweats, weight loss, and pleural effusion. Serology showed high antinuclear antibody titers and elevated anti–single-stranded DNA, anti-DNA, and anti-histone antibodies, while imaging excluded malignancy and infection. After CBZ discontinuation, symptoms resolved, inflammatory markers normalized, lupus-related autoantibodies declined, and no recurrence occurred during a 30-month follow-up.

LanguageEnglish

CategoryHealthcare

FormatPDF

Pages6

Reading Time15 min

AccessFree to download

Introduction

Drug-induced lupus erythematosus and related medications

NMOSD background and autoimmune associations

Case Report

Presentation and diagnostic workup

Treatment and follow-up

Conclusions

e-ISSN 1941-5923 © Am J Case Rep, 2025; 26: e949233 DOI: 10. 12659/AJCR.949233 Received: 2025.04.03 Accepted: 2025.10.16 Available online: 2025.11.12 Published: 2025.12.18 Drug-Induced Lupus Erythematosus After 14 Years of Carbamazepine Use in a Patient With Neuromyelitis Optica Spectrum Disorder: A Case Report Authors’ Contribution: Study Design A Data Collection B Statistical Analysis C Data Interpretation D Manuscript Preparation E Literature Search F Funds Collection G ABCDEF 1 ABCDE 2 ABCD 1 ABCDEF 2 Naho Ayuzawa-Takeda Keisuke Saito Hidetoshi Yanagida Tomoko Oeda 1 Department of Rheumatology, National Hospital Organization, Utano National Hospital, Kyoto, Japan 2 Clinical Research Center and Department of Neurology, National Hospital Organization, Utano National Hospital, Kyoto, Japan Corresponding Author: Tomoko Oeda, e-mail: [tomokooeda@gmail.com](tomokooeda@gmail.com)[ ](tomokooeda@gmail.com)[Financial support:](Financial support: None declared)[ None declared](Financial support: None declared) [Conflict of interest:](Conflict of interest: None declared)[ None declared](Conflict of interest: None declared) Patient: Female, 61-year-old Final Diagnosis: Drug induced lupus erythematosus Symptoms: General fatigue • intermittent fever • night sweats • weight loss Clinical Procedure: — Specialty: Rheumatology Objective: Challenging differential diagnosis Background: Carbamazepine (CBZ) is a common therapy for seizures, neuropathic pain, and painful tonic spasms following spinal cord disease; however, it is associated with rare autoimmune complications, including drug-induced lupus erythematosus (DILE). Diagnosis of DILE can be challenging, especially when onset occurs after a prolonged latency period. Neuromyelitis optica spectrum disorder (NMOSD), an autoimmune disease characterized by recurrent inflammation of the central nervous system, frequently coexists with other autoimmune disorders. This report describes a case of DILE in a 61-year-old woman with NMOSD involving the cervical and thoracic spinal cord after 14 years of CBZ therapy. Case Report: A 61-year-old woman with aquaporin-4-IgG-positive NMOSD involving the cervical and thoracic spinal cords had received CBZ for 14 years to control painful tonic spasms. She presented with fatigue, weight loss, intermittent fever, and pleural effusion. Laboratory testing showed a high antinuclear antibody titer (1: 640) and elevated anti–single-stranded DNA, anti-DNA, and anti-histone antibodies. Chest imaging demonstrated pleural effusion and pleural thickening; malignancy and infection were excluded. Discontinuation of CBZ led to resolution of symptoms, normalization of inflammatory markers, and a decline in lupus-related autoantibodies, confirming the diagnosis of DILE. No recurrence was observed during a 30-month follow-up period. Conclusions: This report describes a rare delayed-onset autoimmune adverse effect of CBZ that developed 14 years after treatment initiation. It highlights the importance of clinical awareness of potential autoimmune associations, particularly when CBZ is used in patients with preexisting autoimmune disorders such as NMOSD. Keywords: Carbamazepine • Chemically-Induced Disorders • Lupus Erythematosus, Systemic • Neuromyelitis Optica Full-text PDF: [https://www.amjcaserep.com/abstract/index/idArt/949233](https://www.amjcaserep.com/abstract/index/idArt/949233) 2308 — 3 19 Publisher’s note: All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article, or claim that may be made by its manufacturer, is not guaranteed or endorsed by the publisher This work is licensed under Creative Common AttributionNonCommercial-NoDerivatives 4.0 International (CC BY-NC-ND 4.0) e949233-1 Indexed in: [PMC] [PubMed

FAQs

Why was the diagnosis challenging in this case?

Diagnosis was challenging because drug-induced lupus erythematosus (DILE) can have a delayed onset after prolonged medication latency, and it can mimic systemic lupus erythematosus–like symptoms and laboratory findings.

What findings supported DILE after long-term carbamazepine use?

Laboratory tests showed a high antinuclear antibody titer and elevated anti–single-stranded DNA, anti-DNA, and anti-histone antibodies. Symptoms and serologic abnormalities improved after discontinuing carbamazepine.

What happened after stopping carbamazepine?

Discontinuation led to resolution of symptoms, normalization of inflammatory markers, and a decline in lupus-related autoantibodies, with no recurrence during a 30-month follow-up period.

Sep 29, 2026

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