Sjögren’s syndrome myelopathy: spinal cord involvement in Sjögren’s syndrome might be a manifestation of neuromyelitis optica

Abstract

All patients with SSM had poor prognosis with high mean annual relapse rates, and most seemed to have the clinical and immunological characteristics of NMO. Early aggressive immune therapies should be considered in patients with SSM irrespective of the presence or absence of optic neuritis.

Neuromyelitis optica spectrum disorder in patients with connective tissue disease and myelitis

“…These findings raise the question of whether an association exists between NMO and the CNS disease seen in SS. The small number of patients in our series precludes firm conclusions; however, additional studies have reported a similar association (8,30,39). Definitive evidence for an association between NMO and SS has not yet been established, and mechanistic studies are needed to explain the potential connection between the 2 diseases.…”

“…LM is a characteristic finding in NMO/NMOSD and its presence may be used to differentiate from myelitis in classic MS, which rarely extends beyond a single vertebral segment (33). Longitudinal cord involvement has been reported in SLE and SS as well (7,8,14,17,22,23,30,34,35). These reports have identified a high prevalence of the NMO-IgG antibody and additional features consistent with concurrent NMO/NMOSD in these patients.…”

“…Our cohort adds 5 cases of overlap SS/NMO. To our knowledge, this represents the largest collection of such patients described in the literature, matching those described by Kim et al (8). Additional at-risk patients (based on features such as LM or NMO-IgG positivity) were identified but were not included based on insufficient data to diagnose NMO according to the 2006 revised criteria.…”

“…An association between the NMO-IgG antibody and LM in patients with systemic lupus erythematosus (SLE) and Sjö gren's syndrome (SS) has been reported in the literature (7,8), and has spurred interest in NMO/NMOSD from the field of rheumatology. Herein we describe a cohort of patients with autoimmune myelitis and discuss the implications of coexistent NMO/NMOSD for subsequent clinical management.…”

Acute inflammatory myelopathies

“…Additional cases describing the co-occurrence of Sj€ ogren syndrome with NMO were reported prior to the general availability of the anti-AQP4 antibody assay (Mochizuki et al, 2000;Arabshahi et al, 2006;Gokcay et al, 2007). Subsequent to the commercial availability of the anti-AQP4 antibody test, multiple cases of Sj€ ogren syndrome-associated myelopathy have tested seropositive for the anti-AQP4 antibody (Hamnik et al, 2008;Javed et al, 2008;Sofat and Venables, 2008;Kim et al, 2009;Min et al, 2009;Wandinger et al, 2010;Kahlenberg, 2011). As with SLE, there appears to be overlap between NMO and Sj€ ogren syndrome.…”