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Published on: August 21, 2017
Neuromyelitis optica--complication or comorbidity in primary Sjögren's syndrome?
Doina Niţescu1, Adriana Nicolau, Simona Caraiola
1Colentina Clinical Hospital, Internal Medicine, Bucharest. doinitescu@yahoo.com
This study highlights two cases of neuromyelitis optica (NMO) linked with primary Sjögren's syndrome (pSS). Early neurological symptoms in NMO patients can mask pSS, delaying diagnosis and impacting outcomes.
Area of Science:
- Neurology
- Immunology
- Rheumatology
Background:
- Neuromyelitis optica (NMO) is a severe demyelinating CNS disorder affecting optic nerves and spinal cord.
- Neurologic involvement is common in primary Sjögren's syndrome (pSS), often preceding diagnosis.
- Co-occurrence of NMO and pSS presents diagnostic challenges due to atypical presentations.
Purpose of the Study:
- To report and compare clinical/laboratory features of two NMO patients with pSS.
- To analyze diagnostic delays and outcomes in NMO-pSS cases.
- To discuss the potential relationship between NMO and pSS, including serological findings.
Main Methods:
- Case report and comparative analysis of two patients with NMO and pSS.
- Review of clinical presentations, laboratory findings, and disease outcomes.
- Analysis of autoantibody profiles, including anti-aquaporin4, anti-Ro, and anti-La antibodies.
Main Results:
- Both patients presented with late pSS diagnoses due to predominant neurological symptoms and initially absent sicca symptoms or specific serology.
- Neuromyelitis optica (NMO) diagnosis was established, with subsequent identification of primary Sjögren's syndrome (pSS).
- Presence of anti-aquaporin4 antibodies alongside anti-Ro and anti-La antibodies was noted, prompting further investigation into disease mechanisms.
Conclusions:
- Systematic screening for pSS is recommended in patients with myelopathy or cranial nerve involvement, especially those with severe outcomes.
- The co-occurrence of NMO and pSS may represent distinct diseases or a variant of Sjögren's syndrome with atypical neurological manifestations.
- Further research is needed to clarify the complex interplay between NMO and pSS and the significance of specific autoantibodies.
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