Neurology -- Correspondence for Hummers et al., 62 (1) 147-149

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Correspondence published:

Reply to Petereit: Laura K. Hummers, Fredrick M. Wigely, Antony Rosen, Livia Casciola-Rosen, Douglas Kerr, Chitra Krishnan, James Mahoney and Steven Morris (18 February 2004)

Recurrent transverse myelitis associates with anti-Ro (SSA) autoantibodies: Hela F Petereit, Andreas Perniok (18 February 2004)

Reply to Petereit 18 February 2004

Laura K. Hummers,
Johns Hopkins University
5501 Hopkins Bayview Circle, Room 1B.32 Baltimore, MD 21224,
Fredrick M. Wigely, Antony Rosen, Livia Casciola-Rosen, Douglas Kerr, Chitra Krishnan, James Mahoney and Steven Morris
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Re: Reply to Petereit

lhummers{at}jhmi.edu Laura K. Hummers, et al.

We thank Dr. Petereit for her interest and comments on our article. The prevalence of central nervous system (CNS) disease among patients with a definite diagnosis of primary Sjogren’s syndrome is fiercely debated with ranges from 0-20% in different published series [2,5,6]. Our study focused on cases that presented with recurrent inflammatory transverse myelitis, a clinical problem that can occur in the absence of a clearly defined underlying cause. None of our patients had symptoms or signs of the sicca complex (dry mouth or keratoconjunctivitis) and therefore these patients did not meet the currently accepted criteria for primary Sjogren’s syndrome. Our patients did have an inflammatory lesion of the spinal cord associated with the presence of anti-Ro antibodies. The presence of Ro autoantibodies is not specific to primary Sjogren’s syndrome and is associated with other autoimmune diseases such as lupus and congenital heart block. The presence of anti-Ro antibodies in patients presenting with recurrent inflammatory transverse myelitis in the absence of other signs or symptoms suggest that an occult autoimmune process may be causing this CNS disease. An autoimmune process may cause transverse myelitis and immunosuppressive therapy may be helpful in preventing relapses.
References
1. Dafni UG, Tzioufas AG, Staikos P, et al. Prevalence of Sjögren´s syndrome in a closed rural community. Ann Rheum Dis 1997; 56:521-525.
2. Alexander EL. Neurological disease in Sjögren´s syndrome: Mononuclear inflammatory vasculopathy affecting central/peripheral nervous system and muscle. Rheum Dis Clin North Am 1993; 19:869-908.
3. Hietaharju A, Yli-Kerttula U, Häkkinen V, et al. Nervous system manifestation in Sjögren´s syndrome. Acta Neurol Scand 1990; 81:144-152.
4. Vitali C, Bombardieri S, Jonsson R, et al. Classification criteria for Sjögren´s syndrome: a revised version of the European criteria proposed by the American-European Consensus Group. Ann Rheum Dis 2002; 61:554-558.
5. Andonopoulos, A.P., et al., Neurologic involvement in primary Sjogren's syndrome: a preliminary report. J Autoimmun, 1989. 2(4): p. 485-488.
6. Anaya JM, V.L., Restrepo L, Molina JF, Mantilla RD, Vargas S, Central Nervous System Compromise in Primary Sjogren's Syndrome. J Clin Rheumatol, 2002. 8: p. 189-196.

Recurrent transverse myelitis associates with anti-Ro (SSA) autoantibodies 18 February 2004

Hela F Petereit,
Department of Neurology, University of Cologne
Josef-Stelzmann-Str. 9, D-50924 Köln,
Andreas Perniok
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Re: Recurrent transverse myelitis associates with anti-Ro (SSA) autoantibodies

hela.petereit{at}medizin.uni-koeln.de Hela F Petereit, et al.

The authors describe a series of 13 patients with recurrent transverse myelitis in whom they found serum anti-Ro antibodies significantly more often than in an age- and sex-matched control group. We propose that these patients might suffer from Sjögren´s syndrome with mildly symptomatic sicca symptoms and involvement of the nervous system.
With a prevalence of 2-3 %, Sjögren syndrome is one of the most frequent autoimmune diseases oftenly underdiagnosed because of mild symptoms. [1,2] Central and peripheral nervous system disease is seen in about 20% of cases. [2] Transverse myelitis is a well known CNS involvement in Sjögren´s syndrome. [3] Interestingly, many individuals are unaware of the disease and do not complain about typical sicca symptoms like dry eyes and dry mouth. [2] This observation has been made by several other groups by screening for sicca symptoms using surveys and careful rheumatological examination and by work- up of mothers of children with Ro antibody associated congenital heart block. Diagnosis in suspected cases should be ascertained by an experienced rheumatologist with the help of further tests including measurement of lacrimation, search for a salivary gland involvement and sometimes biopsy of the salivary gland according to recent diagnostic criteria. [4]
Although Hummer et al state that none of the patients had clinical signs of a connective tissue disease, it is not mentioned if these tests were performed. It may have been helpful to do the full range of examinations for Sjögren´s syndrome in the patients with recurrent transverse myelitis since in case of a proven Sjögren´s syndrome, an immunosuppressive treatment might be effective in preventing neurological relapses or deterioration.
References
1. Dafni UG, Tzioufas AG, Staikos P, et al. Prevalence of Sjögren´s syndrome in a closed rural community. Ann Rheum Dis 1997;56:521-525.
2. Alexander EL. Neurological disease in Sjögren´s syndrome: Mononuclear inflammatory vasculopathy affecting central/peripheral nervous system and muscle. Rheum Dis Clin North Am 1993;19:869-908.
3. Hietaharju A, Yli-Kerttula U, Häkkinen V, et al. Nervous system manifestation in Sjögren´s syndrome. Acta Neurol Scand 1990;81:144-152.
4. Vitali C, Bombardieri S, Jonsson R, et al. Classification criteria for Sjögren´s syndrome: a revised version of the European criteria proposed by the American-European Consensus Group. Ann Rheum Dis 2002;61:554-558.