Sjögren syndrome and neuromyelitis optica spectrum disorder co-exist in a common autoimmune milieu

Abstract

The relationship between Sjögren's syndrome (SS) and neuromyelitis optica spectrum disorder (NMOSD) is not completely understood. We report two patients with both conditions and review 47 other previously reported cases meeting currently accepted diagnostic criteria, from 17 articles extracted from PubMed. Out of 44 patients whose gender was informed, 42 were females. Mean age at onset of neurological manifestation was 36.2 years (10-74). Serum anti-AQP4-IgG was positive in 32 patients, borderline in 1, and negative in 4. Our Case 1 was seronegative for AQP4-IgG and had no non-organ-specific autoantibodies other than anti-SSB antibodies. Our Case 2 had serum anti-AQP4, anti-SSA/SSB, anti-thyreoglobulin and anti-acethylcholine-receptor antibodies, as well as clinical hypothyreoidism, but no evidence of myasthenia gravis. Our Cases and others, as previously reported in literature, with similar heterogeneous autoimmune response to aquaporin-4, suggest that SS and NMO co-exist in a common autoimmune milieu which is not dependent on aquaporin-4 autoimmunity.Keywords: neuromyelitis optica spectrum disorders, neuromyelitis optica, Sjögren's syndrome, autoimmunity, AQP4-IgG. RESUMOA relação entre síndrome de Sjögren (SS) e espectro da neuromielite óptica (ENMO) ainda não é bem compreendida. Relatamos dois pacientes com ambas as condições e revisamos 47 casos que preenchem critérios diagnósticos das duas doenças, descritos em 17 artigos extraídos da PubMed. Dos 44 pacientes cujo gênero foi informado 42 eram mulheres. A idade média ao início das manifestações neurológicas foi 36,2 anos (10-74). O anticorpo anti-AQP4 foi positivo em 32 dos 37 pacientes, em 1 foi "borderline". Nosso Caso 1 era soronegativo para AQP4-IgG, não tinha autoanticorpos não-órgão específicos, exceto anti-SSB. O Caso 2 era soropositivo para anticorpos anti-AQP4, anti-SSA/SSB, anti-tireoglobulina, e anti-receptor da acetilcolina; apresentava hipotireoidismo, mas não havia evidêncas de miastenia gravis. Nossos casos e outros similares, previamente relatados na literatura, com resposta autoimune heterogênea à aquaporina-4 sugerem que a SS e o ENMO coexistem em meio de autoimunidade não dependente da aquaporina-4.Palavras-chave: espectro de neuromielite óptica, neuromielite óptica, síndrome de Sjögren, autoimunidade, AQP4-IgG.

Crescentic glomerulonephritis in a patient with neuromyelitis optica (Devic´s syndrom)

“…However, our patient may suffer from a somewhat different peculiarity of NMO since we could not prove NMO-specific antibodies. In line with this is the observation that NMO-IgG autoantibodies are not detectable in 20%-30% of NMO patients [19] . Assay sensitivities may differ and thus may explain some negative results [24] .…”

“…After revision of consensus criteria for the diagnosis of NMO [2] the term "NMO spectrum disorders" (NMOSD) was introduced to mirror the various and diverse clinical and radiological manifestations of NMO including those cases of NMO with co-existing systemic autoimmune diseases [19] .…”

“…Commonly patients are tested during onset of clinical symptoms or after initiation of therapy and thus may falsely appear seronegative for NMO autoantibodies [25] . Seronegative patients usually show no gender preference, but a higher proportion of monophasic courses of NMO and often a Caucasian descent [19] . In contrast, seropositive patients exhibit higher frequencies of extensive spinal cord and brain lesions [3] .…”

Primary Sjögren Syndrome in a Child with a Neuromyelitis Optica Spectrum Disorder

“…Interestingly, while there are multiple reports of coexisting SS and NMOSD in adults 9 , to our knowledge, this has not been definitively reported in pediatrics. Several patients with NMOSD have been reported to have …”

Neuromyelitis optica spectrum disorder and Sjögren syndrome are overlapping disorders and participate in the same autoimmunity context?

“…In this number of Arquivos de Neuro-Psiquiatria, Carvalho DC et al revised the literature in search of the relationship among AQP4-IgG serum status, NMOSD and SS 20 . To illustrate their understanding of the coexistence of SS and NMOSD as a predisposition of patients with NMO to multiple autoimmune diseases, they described two cases of primary SS and definite NMO with different AQP4-IgG serum status and other non-organ-specific auto antibodies.…”