# Severe relapse after cessation of immunosuppressive therapy in a patient with co‐occurrence of neuromyelitis optica spectrum disorder and Sjögren's syndrome: A case report

## TL;DR

A 14-year-old girl with two autoimmune disorders improved with treatment but had a severe relapse after stopping her medication.

## Contribution

Highlights the rare co-occurrence of NMOSD and SS in a young patient and the risks of discontinuing immunosuppressive therapy.

## Key findings

- The patient showed significant improvement with immunosuppressive therapy.
- Severe relapse occurred after voluntary discontinuation of treatment.
- Long-term treatment is crucial for managing NMOSD and SS in young patients.

## Abstract

Neuromyelitis optica spectrum disorder (NMOSD) is a group of autoimmune disorders characterized by inflammatory involvement of the optic nerve, spinal cord, and central nervous system. NMOSD is often associated with other autoimmune disorders, including Sjogren's syndrome (SS). While NMOSD typically occurs at a peak in young or older individuals, the coexistence of NMOSD and SS in a youngster is rare. Here, we presented a case of a 14‐year‐old girl with NMOSD and SS who responded well to immunosuppressive therapy but experienced a severe relapse after discontinuation of therapy. We described the clinical course of a case over 8 years, underscoring the importance of long‐term treatment for NMOSD and SS. This case, along with the review of relevant literature, will raise awareness of this type of disease and facilitate early diagnosis and treatment to avoid serious sequelae.

We report a girl with a diagnosis of “neuromyelitis optica spectrum disorder comorbid Sjogren's syndrome” whose condition showed significant improvement after treatment with corticosteroids and immunosuppressive drugs (prednisolone, hydroxychloroquine, and mycophenolate mofetil). However, she developed a relapse and developed a more severe disease due to voluntary discontinuation of the immunosuppressive drugs.