Trigeminal Neuralgia in Systemic Sclerosis

Nancy Maltez¹, Marie Hudson², Yves Troyanov³, May Choi⁴, Mianbo Wang⁵, Marvin J. Fritzler⁶, Murray Baron⁷ and Doug Smith⁸, ¹Rheumatology, The Ottawa Hospital, Ottawa, ON, Canada, ²Division of Rheumatology, Jewish General Hospital, Lady David Institute for Medical Research, Montreal, QC, Canada, ³Rheumatology, Hôpital du Sacré-Coeur de Montréal, Montréal, QC, Canada, ⁴University of Calgary, Calgary, AB, Canada, ⁵Lady Davis Institute for Medical Research, Montreal, QC, Canada, ⁶Medicine, University of Calgary, Calgary, AB, Canada, ⁷Rheumatology, McGill University, Jewish General Hospital, Montreal, QC, Canada, ⁸Division of Rheumatology, Department of Medicine, The Ottawa Hospital - University of Ottawa, Ottawa, ON, Canada

Meeting: 2017 ACR/ARHP Annual Meeting

Date of first publication: September 18, 2017

Keywords: Systemic sclerosis

Session Information

Date: Tuesday, November 7, 2017

Title: Systemic Sclerosis, Fibrosing Syndromes and Raynaud's – Clinical Aspects and Therapeutics Poster III

Session Type: ACR Poster Session C

Session Time: 9:00AM-11:00AM

Background/Purpose:

Trigeminal neuralgia (TN) is characterized by pain and spasms affecting one or more divisions of the fifth cranial nerve. Of note, TN is one of the peripheral nervous system manifestations of systemic sclerosis (SSc), reported to be present in approximately 4% of patients. Proposed pathophysiologic mechanisms in this context have included nerve entrapment and compression from mandibular bone resorption, a phenomenon seen in SSc due to pressure ischemia from overlying tight sclerotic skin compromising blood supply to the bone. Previous studies of TN identified an association with overlap syndromes notably in patients with inflammatory myositis (IM), arthritis and interstitial lung disease (ILD). It has also been suggested that facial numbness can precede or follow other manifestations of SSc. However, since there is a paucity of evidence concerning TN in SSc. we undertook a nested case-control study to identify associations between SSc and TN in a multi-centered SSc cohort.

Methods:

Data were retrieved from the Canadian Scleroderma Research Group (CSRG) registry, an open cohort of 1652 SSc subjects enrolled since 2004. Subjects with a physician-reported diagnosis of TN were identified at the baseline study visit (prevalent cases) and during follow-up (incident cases). Four SSc subjects without TN and matched to each case on study visit were identified as controls for either prevalent or incident cases. Sociodemographic, clinical and serological characteristics of cases and controls were compared. P values < 0.05 were considered statistically significant.

Results:

43 (43/1652; 2.6%) prevalent and 36 incident (36/6193 total person-years follow-up; incidence rate 5.8 per 1000 person-years) TN cases were identified and matched to 144 and 172 controls, respectively. There were no significant differences in mean age, gender distribution and mean disease duration between cases and controls. Compared to controls, prevalent cases had more IM (24.4% vs. 5.2%, p<0.001) and arthritis (46.5% vs. 30.2%, p=0.043). Similarly, incident cases also had more IM (19.4% vs. 6.3%, p=0.033) and arthritis (50.0% vs. 16.2%, p<0.001) compared to controls. There was a trend towards more ILD in prevalent (32.6% vs 23.8%, p=0.241) and incident (55.6% vs 40.6%, p=0.105) cases compared to controls. U1RNP was numerically more frequent in cases versus controls (prevalent cases vs controls 9.4% vs 5.4%; incident cases vs controls 17.1% vs 5.8%). Pm/Scl antibodies were infrequent and similar in prevalent (2.9% vs 3.5%) and incident (5.9% vs 5.7%) cases compared to controls.

Conclusion:

This study provides novel evidence for a syndrome linking TN, IM, arthritis and possibly ILD. We propose that TN could be a consequence of active inflammation and represent a sign of disease activity in SSc.

Disclosure: N. Maltez, None; M. Hudson, None; Y. Troyanov, None; M. Choi, None; M. Wang, None; M. J. Fritzler, None; M. Baron, None; D. Smith, None.

To cite this abstract in AMA style:

Maltez N, Hudson M, Troyanov Y, Choi M, Wang M, Fritzler MJ, Baron M, Smith D. Trigeminal Neuralgia in Systemic Sclerosis [abstract]. Arthritis Rheumatol. 2017; 69 (suppl 10). https://acrabstracts.org/abstract/trigeminal-neuralgia-in-systemic-sclerosis/. Accessed .

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