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Clinicopathological features of multiple mononeuropathy associated with systemic lupus erythematosus: a multicenter study
- Source :
- Journal of neurology. 264(6)
- Publication Year :
- 2017
-
Abstract
- Multiple mononeuropathy (MM) occurs rarely during systemic lupus erythematosus (SLE) but may lead to major disability. The aim of this study was to investigate the clinic-pathological presentations of MM during SLE, as well as long-term outcomes. We conducted a multicentric retrospective study that included patients receiving a diagnosis of MM during SLE. Ten patients were included (8 women and 2 men, median age at MM diagnosis: 40.4 years). SLE was diagnosed before MM in 9/10 patients (median time 8.2 years). When MM occurred, the SLEDAI score was ≥6 for 6/9 patients. Presenting symptoms consisted of sensory deficits (n = 10), neuropathic pain (n = 9), and/or motor deficits (n = 9), sometimes symmetrical, affecting the lower limbs (10/10) and occasionally the upper limbs (5/10). All patients presented with uni- or bilateral damage of the common fibular nerve, with less frequent involvement of the tibial nerve. Serum cryoglobulinemia was positive in 5/9 patients. Electrophysiological studies confirmed the non-symmetrical involvement of multiple nerve trunks in all patients. Neuromuscular biopsy (performed in five patients) showed histological signs of vasculitis in two patients and perivascular lymphocytic inflammatory infiltrates in two others. All patients were treated with glucocorticosteroids combined with cyclophosphamide (n = 6), rituximab (n = 3), or mycophenolate-mofetil (n = 1). The median follow-up was 5 years. Two patients relapsed during follow-up. All patients had motor and/or sensory sequelae upon follow-up. MM associated with SLE is frequently caused by a vasculitis mechanism. Patients improve with steroids and immunosuppressive drugs. Long-term outcomes include frequent clinical sequelae and possible relapses.
- Subjects :
- Adult
Male
medicine.medical_specialty
Cyclophosphamide
Adolescent
Neural Conduction
Gastroenterology
Severity of Illness Index
Mononeuropathy
03 medical and health sciences
Young Adult
0302 clinical medicine
Adrenal Cortex Hormones
Internal medicine
Biopsy
medicine
Humans
Lupus Erythematosus, Systemic
Tibial nerve
Retrospective Studies
030203 arthritis & rheumatology
medicine.diagnostic_test
Mononeuritis Multiplex
business.industry
Electromyography
Mononeuropathies
Middle Aged
medicine.disease
Cryoglobulinemia
Surgery
C-Reactive Protein
Neurology
Rituximab
Female
Neurology (clinical)
business
Vasculitis
030217 neurology & neurosurgery
medicine.drug
Follow-Up Studies
Subjects
Details
- ISSN :
- 14321459
- Volume :
- 264
- Issue :
- 6
- Database :
- OpenAIRE
- Journal :
- Journal of neurology
- Accession number :
- edsair.doi.dedup.....06a013babdc0352a54ec68af71e303ff