Myelopathies secondary to Sjögren's syndrome: treatment with monthly intravenous cyclophosphamide associated with corticosteroids.
OBJECTIVE
Central nervous system manifestations in Sjögren's syndrome (SS) include focal deficits, optic neuritis, and myelopathies. Acute and chronic myelopathies are frequently severe and sometimes respond poorly to corticosteroids. The efficacy of intravenous (IV) cyclophosphamide (CYC) has been suggested in single case reports.


METHODS
We describe the potential usefulness of IV CYC in SS patients with severe myelopathies. Fourteen patients [with acute (n = 6) and chronic (n = 8) myelopathies] were treated with monthly CYC infusions (700 mg/m2) in addition to 500 mg of corticosteroids for one year. We evaluated the disability before and after CYC treatment using a walking distance calculation and the Expanded Disability Status Scale (EDSS).


RESULTS
CYC treatment was well tolerated in all cases without serious adverse events. Nine patients (including the 6 with acute myelopathy) were improved after CYC treatment. Three patients were stabilized and 2 patients with chronic myelopathies had moderate progression of disability. The mean walking distance increased from 48.2 m before to 180.4 m after CYC treatment (p < 0.02). Mean EDSS score decreased from 6.6 to 5.7 (not significant). We found a correlation between the length of time before CYC treatment and clinical improvement for both the walking distance (p < 0.02) and the EDSS score (p < 0.05).


CONCLUSIONS
Although a randomized multicenter controlled study is warranted to confirm our findings, IV CYC infusions seem to be useful for the treatment of myelopathies secondary to SS, particularly in acute but also in progressive cases. This treatment should be strongly considered as soon as possible when disease progression is observed.
Department of Neurology, Hôpital R. Salengro, CHRU de Lille, Lille, France. j-deseze@chru-lille.fr
Jerome
J
de Seze
Sophie
S
Delalande
Anne-Laure
AL
Fauchais
Eric
E
Hachulla
Tanya
T
Stojkovic
Didier
D
Ferriby
Pierre-Yves
PY
Hatron
Patrick
P
Vermersch
D000208:Acute Disease; D002908:Chronic Disease; D003520:Cyclophosphamide / Q000008:administration & dosage / Q000627:therapeutic use*; D004185:Disability Evaluation; D004334:Drug Administration Schedule; D004359:Drug Therapy, Combination; D005080:Exercise Test; D005260:Female; D005938:Glucocorticoids / Q000008:administration & dosage / Q000627:therapeutic use*; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage / Q000627:therapeutic use*; D007275:Injections, Intravenous; D008297:Male; D008775:Methylprednisolone / Q000008:administration & dosage / Q000627:therapeutic use*; D008875:Middle Aged; D012189:Retrospective Studies; D012859:Sjogren's Syndrome / Q000150:complications* / Q000188:drug therapy / Q000503:physiopathology; D013118:Spinal Cord Diseases / Q000188:drug therapy / Q000209:etiology*; D013997:Time Factors; D016896:Treatment Outcome; D016138:Walking
D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D008775:Methylprednisolone