Myelit bulguları ile prezente olan ve sicca semptomları olmayan sjögren vakası

Sjögren sendromu (SS) egzokrin bezlerin kronik, inflamatuar ve otoimmün hastalığıdır. SS' da hastaların %20' sinde nörolojik bulgular gelişir. Nörolojik bulgular göz ve ağız kuruluğu gibi sicca semptomları ortaya çıkmadan önce de görülebilir. Periferik sinir sistemi tutulumu (PSS) %15, santral sinir sistemi (SSS) tutulumu %5 oranında görülür. SSS tutulumu, PSS tutulumuna göre çok daha az görülür. Bu vakada, transvers myelit ile prezente olan, serum anti Ro-SSA/anti La-SSB antikorları negatif olan ve sicca bulguları olmayan bir sjögren sendromu ele alınmıştır.

Anahtar Kelimeler:

Sjögren sendromu, Myelit, Sicca semptomları

A Sjögren's syndrome case presenting with myelitis and without sicca symptoms

Sjögren’s syndrome (SS) is a chronic inflammatory disorder of exocrine glands with autoimmune etiology. Neurological symptoms occur in approximately 20% of patients with Sjögren’s syndrome. Neurological symptoms may occur before the onset of sicca symptoms such as; dry eyes and dry mouth. The prevalence of peripheral and central neurological manifestations in Sjögren syndrome are about 15% and 5% respectively. CNS involvement is much less common than peripheral nervous system involvement. In this case, our patient presented symptoms of transverse myelitis; however, the serum Anti-Ro/SSA and anti-La/SSB antibodies were negative and there were no dry eyes and dry mouth.

Keywords:

Sjögren’s syndrome, Myelitis, Sicca symptoms,

PDF

___

1. Delalande S, de Seze J, Fauchais AL, Hachulla E, Stojkovic T, Ferriby D, Dubucquoi S, Pruvo JP, Vermersch P, Hatron PY. Neurologic manifestations in primary Sjögren syndrome: a study of 82 patients. Medicine (Baltimore). 2004;83(5):280-91.
2. Tobón GJ, Pers JO, Devauchelle-Pensec V, Youinou P. Neurological Disorders in Primary Sjögren's Syndrome.Autoimmune Dis. 2012;2012:645967.
3. Gono T, Kawaguchi Y, Katsumata Y, Takagi K, Tochimoto A, Baba S, Okamoto Y, Ota Y, Yamanaka H. Clinical manifestations of neurological involvement in primary Sjögren’s syndrome. Clin Rheumatol. 2011;30(4):485-90.
4. Fayyaz A, Kurien BT, Scofield RH. Autoantibodies in Sjögren's Syndrome. Rheum Dis Clin North Am. 2016;42(3):419-34.
5. Berkowitz AL, Samuels MA. The neurology of sjögren’s syndrome and the rheumatology of peripheral neuropathy and myelitis . Pract Neurol 2014;14:14-22.
6. Carvajal Alegria G, Guellec D, Mariette X, Gottenberg JE, Dernis E, Dubost JJ, Trouvin AP, Hachulla E, Larroche C, Le Guern V, Cornec D, Devauchelle-Pensec V, Saraux A. Epidemiology of neurological manifestations in Sjögren's syndrome: data from the French ASSESS Cohort. RMD Open. 2016 Apr 20;2(1):e000179.
7. Birnbaum J. Peripheral nervous system manifestations of Sjögren syndrome: clinical patterns, diagnostic paradigms, etiopathogenesis, and therapeutic strategies.Neurologist. 2010;16(5):287-97.