Protein-A immunoadsorption combined with immunosuppressive treatment in refractory primary Sjögren's syndrome coexisting with NMOSD: a case report and literature review.

The treatment of primary Sjögren's syndrome (pSS) coexisting with neuromyelitis optica spectrum disorder (NMOSD) using protein-A immunoadsorption combined with immunosuppressive therapy has rarely been reported. Herein, we present the case of a 35-year-old female diagnosed with pSS concomitant with NMOSD (pSS-NMOSD) who demonstrated a positive response to protein-A immunoadsorption after failing to respond to therapy comprising high-dose intravenous methylprednisolone (IVMP) and intravenous immunoglobulin (IVIG). Within one week of receiving three sessions of immunoadsorption combined with immunosuppressive treatment, the patient's clinical symptoms (blurred vision, paraparesis, and dysfunctional proprioception) significantly improved. Additionally, a rapid decrease in the circulating levels of Aquaporin-4 immunoglobulin G antibodies (AQP4-IgG), immunoglobulin (Ig) A, IgG, IgM, erythrocyte sedimentation rate (ESR), and rheumatoid factor (RF) were observed. Magnetic resonance imaging (MRI) further revealed a significant reduction in the lesions associated with longitudinal extensive transverse myelitis. During the follow-up period, prednisolone was gradually tapered to a maintenance dose of 5-10 mg/day, whereas mycophenolate mofetil (MMF) was maintained at 1.0-1.5 g/day. The patient's condition has remained stable for four years, with no signs of recurrence or progression observed on imaging examination. Therefore, this case suggests that protein A immunoadsorption may represent a potentially effective therapeutic option for patients with pSS-NMOSD who are refractory to conventional treatments.

Fan W ，Chen X ，Xiao P ，Wei B ，Zhang Y ，Huang J ，Wu S ，Lu L ... -  《Frontiers in Immunology》

Overlap syndrome of anti-aquaporin 4 positive neuromyelitis optica spectrum disorder and primary Sjögren's syndrome: a systematic review of individual patient data.

Central nervous system (CNS) involvement can occur in primary Sjögren's syndrome (pSS) due to co-existing neuromyelitis optica spectrum disorder (NMOSD) which has a highly relapsing course requiring indefinite immunosuppression, and if not diagnosed early, damage accrual occurs over time leading to permanent disability and morbidity. In this review, we describe and outline the clinical course and outcomes of anti-aquaporin 4 (AQP4) antibody seropositive NMOSD with pSS overlap cases. To investigate the co-existence of AQP4 + NMOSD with pSS, we conducted a review of individual patient data from case reports and case series found in major databases. The study extracted clinico-demographic features, imaging and laboratory profiles, treatment approaches, and outcomes of these patients. Inclusion criteria for the review required patients to have positivity for anti-AQP4 or NMO-IgG autoantibodies in the blood and/or cerebrospinal fluid (CSF) and exhibit at least one manifestation of both pSS and NMOSD. In this overlap between AQP4 + NMOSD and pSS, 44 patients were included of whom 41 (93.2%) were females. The mean age of pSS onset was 44.8 ± 18.4 years and NMOSD onset was 43.2 ± 19.8 years. In 20 (45.5%) patients, NMOSD preceded pSS onset, 13 (29.5%) NMOSD occurred after pSS onset, and 11 (25%) patients had a simultaneous presentation. 31 (70.5%) patients experienced acute transverse myelitis, 21 (47.7%) optic neuritis, 14 (31.8%) cerebral syndrome, 10 (22.7%) acute brainstem syndrome, 5 (11.4%) area postrema syndrome, and 2 (4.5%) diencephalic clinical syndromes. For the treatment of acute phase, 40 (90.9%) patients received intravenous methylprednisolone, 15 (34.1%) received plasma exchange, and 10 (22.7%) received intravenous immunoglobulin; and for the induction/maintenance therapy, 16 (36.4%) patients received cyclophosphamide, 6 (13.6%) received rituximab, 16 (36.4%) received azathioprine, and 10 (22.7%) received mycophenolate mofetil. Disease course was monophasic in 2 (4.5%) and relapsing in 27 (61.4%) patients. At median (IQR) follow-up duration of 2.4 (6) years, 39 (88.6%) patients showed improvement, 3 (6.8%) showed stabilization and 2 (4.5%) showed worsening of their NMOSD manifestations. In this overlap syndrome of AQP4 + NMOSD and pSS, patients have a neurologically disabling disorder that can mimic neurological manifestations of pSS, frequently occurs prior to the onset of pSS, has a relapsing course, responds well to immunosuppressants, and necessitates indefinite treatment. Collaborative multicentre studies are needed to clarify the natural history and outcomes of this rare overlap syndrome.

Prasad CB ，Kopp CR ，Naidu G ，Sharma V ，Misra DP ，Agarwal V ，Sharma A ... -  《-》

Neuromyelitis optica spectrum disorder as an initial presentation of primary Sjögren's syndrome.

Longitudinal myelitis in patients with Sjögren's syndrome (SS) is a rarely reported occurrence. Here, we present a patient with longitudinal myelitis who was found to have both primary SS and a positive antibody to aquaporin-4 (NMO-IgG). We review the recent literature concerning the overlap between primary SS-associated myelitis and the presence of NMO-IgG, suggestive of a neuromyelitis optica spectrum disorder (NMOSD). A patient with longitudinal myelitis, SS, and a positive NMO-IgG is presented. A review of the relevant English literature based on a PubMed and Embase search is then discussed. The keywords used were Sjögren's syndrome, myelitis (longitudinal and transverse), neuromyelitis optica, and Devic's disease. Our patient fulfills the criteria for both primary SS and NMOSD. Several small studies have shown that most SS patients with longitudinal myelitis are positive for the antibody to aquaporin-4. Additionally, minor salivary gland biopsies of patients with NMO or NMOSD have evidence of lymphocytic inflammation, suggesting that there may be an overlap in the mechanism of NMOSD and longitudinal myelitis secondary to primary SS. This overlap in mechanism may have important ramifications with regard to prognosis and treatment of SS-related myelitis. The presentation of longitudinal myelitis in a patient with SS should be considered a possible NMOSD. Checking for the presence antiaquaporin-4 antibodies provides important prognostic information and may help to guide treatment decisions.

Kahlenberg JM 《-》

Aquaporin-4-IgG positive neuromyelitis optica spectrum disorder and systemic autoimmune diseases overlap syndrome: a single-center experience.

Martín-Nares E ，Hernandez-Molina G ，Fragoso-Loyo H 《-》

Sjögren's syndrome and neuromyelitis optica spectrum disorders (NMOSD)--a case report and review of literature.

Jayarangaiah A ，Sehgal R ，Epperla N 《BMC Neurology》