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The prevalence of Sjögren syndrome in patients with primary progressive multiple sclerosis
J de Seze1, D Devos, G Castelnovo
1Department of Neurology, CHRU de Lille, France. j-deseze@chru-lille.fr
Objective:
To assess the prevalence of Sjögren syndrome (SS) in patients with primary progressive MS (PPMS).
Background:
SS may be considered in the differential diagnosis of MS. Age at onset and clinical presentation are similar in SS and PPMS. However, occurrence of SS in definite cases of PPMS has been recently reported.
Methods:
Proposed clinical and laboratory diagnostic criteria for SS were systematically assessed in 60 consecutive patients with PPMS. The authors questioned all patients about xerophthalmia and xerostomia, biopsied minor salivary glands, and performed a Schirmer test, a salivary gland scintigraphy, and anti-Ro (SSa) and anti-La (SSb) serologies.
Results:
Ten patients (16.6%) met four or more criteria for SS. This prevalence is higher than in the general population (1 to 5%) and implies that SS can mimic PPMS.
Conclusions:
The authors propose that SS should be screened for systematically in patients with PPMS.
Insights
Sjögren syndrome (SS) affects 16.6% of primary progressive multiple sclerosis (PPMS) patients, a higher prevalence than in the general population. This suggests SS can mimic PPMS and warrants systematic screening.
Area of Science:
- Neurology
- Immunology
- Rheumatology
Background:
- Sjögren syndrome (SS) shares clinical similarities with multiple sclerosis (MS), including age at onset and presentation.
- The differential diagnosis between SS and MS can be challenging due to overlapping symptoms.
- Recent reports indicate SS occurrence in confirmed primary progressive MS (PPMS) cases.
Purpose of the Study:
- To determine the prevalence of Sjögren syndrome (SS) in patients diagnosed with primary progressive multiple sclerosis (PPMS).
- To evaluate the potential for SS to be misdiagnosed as PPMS.
Main Methods:
- Sixty consecutive PPMS patients were assessed using proposed SS diagnostic criteria.
- Methods included patient interviews (xerophthalmia, xerostomia), minor salivary gland biopsy, Schirmer test, salivary gland scintigraphy, and serological testing for anti-Ro (SSa) and anti-La (SSb) antibodies.
Main Results:
- 16.6% of PPMS patients (10 out of 60) met four or more diagnostic criteria for SS.
- This prevalence significantly exceeds that of SS in the general population (1-5%).
- The findings suggest SS can present with symptoms mimicking PPMS.
Conclusions:
- Sjögren syndrome (SS) is more prevalent in PPMS patients than previously recognized.
- Systematic screening for SS is recommended in patients diagnosed with PPMS.
- Early diagnosis of SS can help differentiate it from PPMS and guide appropriate treatment.