A Chinese Girl with NMOSD and Sjögren’s: case report

Case Report: A Chinese Girl with NMOSD and Sjögren’s Disease

This case involves a 14-year-old Chinese girl diagnosed with two rare autoimmune diseases: neuromyelitis optica spectrum disorder (NMOSD) and primary Sjögren’s. NMOSD is a condition where the immune system attacks the central nervous system, particularly the optic nerves and spinal cord, causing symptoms like vision loss and pain. Sjögren’s is another autoimmune disorder that typically affects moisture-producing glands, like those for saliva and tears, but can also impact other organs.

What Happened?

- At age 11, the girl experienced sudden headaches, painful eye movements, and vision loss in her right eye. Tests showed she had NMOSD, confirmed by the presence of specific antibodies (AQP4-IgG) and MRI findings showing inflammation in her optic nerve.

- She was treated with strong anti-inflammatory drugs (methylprednisolone) and immune system support (immunoglobulin), which helped restore her vision. She was then put on long-term medications (prednisone and mycophenolate mofetil) to prevent further attacks.

- Later, tests showed she had elevated liver enzymes (ALT and AST), which led to further investigation. A biopsy of her salivary gland revealed signs of Sjögren’s, confirmed by specific antibodies (anti-Ro/SSA and anti-La/SSB). She met the official criteria for primary Sjogren's diagnosis.

- Because her liver enzymes remained high, her medication was switched from mycophenolate mofetil to tacrolimus, which normalized her liver function. She has not had any NMOSD relapses since the initial episode.

Why Is This Important?

- NMOSD and primary Sjögren’s are rare in children, and having both together is even rarer. This case shows the importance of checking for other autoimmune diseases in kids with NMOSD, as they can have additional conditions like Sjögren’s that need specific treatment.

- Regular testing for antibodies (like ANA or others) can help doctors catch these conditions early, improving treatment and quality of life. If someone with Sjögren’sdevelops neurological symptoms, testing for NMOSD is critical to avoid missing the diagnosis.

SOURCE: A Chinese girl with neuromyelitis optica spectrum disorder coexisting with primary Sjogren’s syndrome: a case report and literature review.