OBJECTIVE: We aimed to examine the delays in the diagnosis of RA in patients presenting to the Rheumatology Unit, Sarawak General Hospital (SGH).
METHODS: Data on demographics and various delays were collected from the medical records from January 2015 until March 2018. Patient delay is defined as from the time onset of symptom to the first primary care presentation. Primary care delay is defined as from the first primary care presentation to referral to rheumatology. Rheumatology delay is defined as from rheumatology referral to appointment at the rheumatology clinic. Disease modifying anti-rheumatic drugs (DMARDS) delay is defined as from the rheumatology clinic appointment to starting DMARDS. Total delay is from symptom onset to starting DMARDS.
RESULTS: There were 84 new patients diagnosed with rheumatoid arthritis, out of which 66 were females (78.6%). The mean age was 54.1±12.0 years. Only 19 patients (22.6%) were treated with DMARDS within 12 weeks of symptom onset. The median time for patient delay was four weeks (Interquartile range (IQR) 2-20 weeks), while the median time primary care delay was 11 weeks (IQR 4-24 weeks). The median time for rheumatology delay was zero weeks (IQR 0- 1 week) and the DMARDS delay was zero week (IQR 0). The median time from symptom onset to DMARDS initiation was 23.5 weeks (IQR 13.25-51 weeks).
CONCLUSION: The delays in the diagnosis of rheumatoid arthritis were mainly from the patient and primary care.
CASE PRESENTATION: We report the case of a 37-year-old Chinese woman with primary Sjögren syndrome who presented with ataxia over 3 months associated with tremor of the limbs. Magnetic resonance imaging of the brain revealed bilateral cerebellar atrophy. Based on the presence of cerebellar signs with magnetic resonance imaging brain findings, she was diagnosed as cerebellar degeneration secondary to primary Sjögren syndrome. She was treated with methylprednisolone, hydroxychloroquine, and two cycles of monthly intravenous cyclophosphamide. Subsequently, she refused further treatment, and her neurological symptoms remained the same upon the last clinic review. Primary cerebellar degeneration is rarely associated with primary Sjögren syndrome. The pathogenesis of the neurological manifestations in primary Sjögren syndrome is unclear. Treatment involves corticosteroids and immunosuppressive agents with no consensus of a specific therapy for the management of primary Sjögren syndrome with central nervous system involvement.
CONCLUSIONS: Cerebellar degeneration is a rare presentation of primary Sjögren syndrome. Early diagnosis and treatment of this condition is needed to ensure a good outcome.