Demyelinating syndrome in SLE encompasses different subtypes: Do we need new classification criteria? Pooled results from systematic literature review and monocentric cohort analysis.
OBJECTIVE
To describe features of demyelinating syndrome (DS) in systemic lupus erythematosus (SLE).


METHODS
A systematic review using a combination of Mesh terms in PubMed and a retrospective analysis of 343 adult patients with SLE were carried out to identify patients with DS. Retrieved cases were classified as affected with DS according to 1999 ACR nomenclature and attributed to SLE by applying the 2015 algorithm. DS defined according to the clinical but not temporal 1999 ACR criteria was classified as clinically isolated syndrome (CIS).


RESULTS
Estimated prevalence of DS (including CIS) in the SLE cohort was 1.3% and incidence rate was 1.5 cases per 1000 patient-years. Overall, 100 cases from literature review and 4 from SLE cohort were identified and are presented as a whole: 49 (47.1%) were classified as neuromyelitis optica spectrum disorders (NMOSD), 29 (27.9%) as CIS, 14 (13.5%) as NMO, 7 (6.7%) as DS prominently involving the brainstem and 5 (4.8%) as DS prominently involving the brain. DS was the SLE onset manifestation in 41 (39.4%) patients. Longitudinally extensive transverse myelitis was the most frequent manifestations being present in 73 (70.2%) patients (37 NMOSD, 21 CIS, 14 NMO, 1 DSB). Methylprednisolone (79.8%) and cyclophosphamide (55.8%) pulses, but also plasma-exchange (16.3%) and rituximab (7.6%) in relapsing-refractory cases, were mostly prescribed. Complete recovery rate ranged between 62% in CIS to 7% in NMO.


CONCLUSIONS
DS in SLE is rare (1%) and encompasses different subtypes including CIS. Timely diagnosis and early treatment are recommended to minimize complications.
Chair of Rheumatology and Rheumatology Unit, University Clinic AOU of Cagliari, Italy. Electronic address: matteopiga@alice.it.
Matteo
M
Piga
Chair of Rheumatology and Rheumatology Unit, University Clinic AOU of Cagliari, Italy.
Elisabetta
E
Chessa
Radiology Department, "G. Brotzu" General Hospital, Cagliari, Italy.
Maria Teresa
MT
Peltz
Chair of Rheumatology and Rheumatology Unit, University Clinic AOU of Cagliari, Italy.
Alberto
A
Floris
Chair of Rheumatology and Rheumatology Unit, University Clinic AOU of Cagliari, Italy.
Alessandro
A
Mathieu
Chair of Rheumatology and Rheumatology Unit, University Clinic AOU of Cagliari, Italy.
Alberto
A
Cauli
D015331:Cohort Studies; D003711:Demyelinating Diseases / Q000209:etiology*; D005260:Female; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000150:complications*; D009364:Neoplasm Recurrence, Local; D012189:Retrospective Studies
Attribution algorithm; Classification criteria; Clinically isolated syndrome; Demyelinating syndrome; MRI; Multiple sclerosis; Neuropsychiatric disorder; Systemic lupus erythematosus