Neurosjögren: Early Therapy is Associated With Successful Outcomes

Amelia Santosa, MBBS, MMed, MRCP; Anita Y.N. Lim, MBChB, MRCP; Sheila Vasoo, MBBS, FAMS, FRCP(Edin); Tang Ching Lau, MBBS, MMed, MMedSec, FRCP; Gim Gee Teng, MBBS


J Clin Rheumatol. 2012;18(8):389-392. 

In This Article

Abstract and Introduction


Background: Primary Sjögren syndrome (PSS) is a systemic autoimmune condition with an estimated prevalence of 0.6%. The frequency of neurologic manifestations in PSS varies widely from 0% to 60%.

Methods: We report the characteristics of PSS patients with neurologic involvement seen at a single tertiary hospital in Singapore. Eight consecutive women (median age, 51 years [range, 38–67 years]) with neurologic manifestations of PSS seen between March 2009 to June 2011 were followed up for a mean duration of 19 months from the onset of neurologic manifestations.

Results: Six of 8 patients with neurosjögren had their neurologic manifestation at time of PSS diagnosis. The lag times of neurologic manifestations from PSS diagnosis for the remaining 2 patients were 9 and 30 years, respectively. Sicca symptoms were not readily volunteered as a presenting complaint in the majority of patients. All our patients received early aggressive therapy with pulse corticosteroids and intravenously administered cyclophosphamide. The mean duration from initial presentation to initiation of treatment was 11 days (1–26 days). All achieved good recovery regardless of the type or site of neurologic involvement, initial erythrocyte sedimentation rate, immunoglobulin and complement levels.

Conclusions: Neurologic disease, when present, is a strong contributor to disease activity and damage. Confirmatory tests should be conducted early regardless of the presence of sicca symptoms. Vigilance for the development of new neurologic symptoms is imperative even in chronic, apparently stable patients. It is likely that early initiation of treatment contributed to good recovery in our patients.


Primary Sjögren syndrome (PSS) is a systemic autoimmune condition characterized by chronic lymphocytic and plasmacellular infiltration of exocrine glands. Prevalence ranges from 0.09% to 3.5%[1] with a female predilection (ratio of 9:1).[2] Fatigue and joint and muscle pains are common at presentation and occur early in the disease. The hallmark of PSS, ocular and oral dryness, is present in more than 90% of patients,[3] with frequent positivity of antinuclear antibody (ANA), anti-Ro/SS-A, and rheumatoid factor (RF).[2] Prevalence of neurologic manifestations vary from 0% to 60%.[2,4,5] In view of the scarcity of studies on neurosjögren from Asia, we report our cohort of patients with severe neurologic manifestations of PSS and review the literature.