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Prompt therapy provided successful outcomes for patients with neurosjögren
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Aggressive early therapy with pulse corticosteroids and cyclophosphamide in patients presenting with neurosjögren resulted in successful recoveries in a recent study.
In a retrospective chart review, researchers in Singapore followed eight women (median age, 51 years) with primary Sjögren’s syndrome (PSS), including neurologic involvement, for a mean of 19 months since manifestation onset. Six patients displayed neurologic symptoms at PSS diagnosis; the other two presented at 9 and 30 years after diagnosis. Manifestations included longitudinally extensive transverse myelitis, ataxic sensory neuropathy, axonal sensimotor neuropathy, cranial neuropathy and organic brain syndrome.
The majority of patients did not voluntary offer sicca symptoms during consultation, though when directly questioned, some reported slight ocular dryness and others cited difficulty in swallowing that required fluid intake. After a mean of 11 days post-consultation, all patients began intravenously administered or oral methylprednisolone, and six were given monthly intravenous cyclophosphamide at a median cumulative dose of 5.1 g.
At 19-month follow-up, five patients displayed complete recovery from neurologic symptoms, with the others achieving “good recovery” regardless of type or site involvement, immunoglobulin and complement levels or erythrocyte sedimentation rate. Two patients with peripheral neuropathy exhibited mild paresthesias, while those with transverse myelitis showed quicker therapy response and full recovery at 5 months.
“Neurologic disease, when present, is a strong contributor to disease activity and damage [in patients with PSS],” the researchers concluded. “Vigilance is needed when female patients present with new-onset unexplained neurologic syndromes, as the typical sicca complex may be absent.
“Seemingly benign PSS may evolve over time to affect the neurologic system. Continued follow-up and awareness for new neurologic symptoms facilitate early intervention. It is likely that early initiation of treatment contributed to good recovery in our patients.”
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