The edition · Rheumatology
Sjögren is not a dry-eye disease
Over 25 years of linked Western Australian data, primary Sjögren disease carried 1.8 times the mortality of matched controls, and Sjögren with another connective tissue disease 3.1 times. The excess deaths were infection, ischaemic heart disease, lymphoid malignancy and interstitial lung disease - four things a review appointment can act on. Plus updated COVID vaccines, plasma exchange in childhood vasculitis, two numbers that stratify scleroderma, and a patch that works by day three.
The edition in brief
Today's rheumatology desk opens with mortality in Sjögren disease. Linked Western Australian registry data from 1980 to 2013 compared 457 patients with primary Sjögren disease, 218 with Sjögren plus rheumatoid arthritis and 77 with Sjögren plus another connective tissue disease against 3,716 age and sex matched controls. Mortality risk was raised in all three groups - hazard ratios of 1.8, 2.1 and 3.1 respectively - with 25-year survival in primary disease of 77%. The excess was driven by infection, ischaemic heart disease, lymphoid malignancy and interstitial lung disease. In 60,980 adults with autoimmune rheumatic conditions and incident COVID-19, adjusted odds of COVID-related hospitalisation fell progressively with vaccination: 0.59 after the initial series, 0.29 after a booster and 0.31 after the updated 2023-2024 vaccine, an absolute risk reduction of 5.6% for boosted or updated over initial series alone. Only 10% had received the updated vaccine. A multicentre retrospective series of 26 children with ANCA-associated vasculitis found therapeutic plasma exchange used in the 11 sickest, mainly for severe kidney involvement, with Pediatric Vasculitis Activity Score falling from 12.0 to 4.5 but renal abnormalities frequently persisting, and one death from catheter-related sepsis. In 227 patients with systemic sclerosis followed for a median 38 months, baseline active disease on the modified disease activity index (hazard ratio 2.21) and lower forced vital capacity independently predicted complications or death. The edition closes on 89 patients with symptomatic knee osteoarthritis, where adding a topical flurbiprofen patch to oral celecoxib produced faster pain and function improvement by day 3, with the pain advantage gone by day 14.
Four causes account for the excess mortality in Sjögren disease
Treat Sjögren disease review as a systemic risk assessment - vaccination, cardiovascular risk, lymphoma red flags and lungs - not only a symptom consultation.
Nine in ten of these patients had not had the updated vaccine
Ask every patient on immunosuppression whether they have had a booster or updated COVID vaccine - only one in ten had.
Plasma exchange in childhood ANCA vasculitis: used on the sickest, and the kidneys still did badly
Plasma exchange remains a selective adjunct in severe paediatric ANCA vasculitis, and will not recover kidneys already badly damaged at presentation.
Two baseline numbers stratify systemic sclerosis beyond the lungs
Record the modified disease activity index and forced vital capacity at baseline in systemic sclerosis, and let an active score raise surveillance across all organs, not just the lungs.
The Sjögren lymphoma questions take ninety seconds
Run the salivary gland, complement and purpura checks at every Sjögren review, and write down the negatives.
Adding a topical patch to oral celecoxib bought three days, not two weeks
Use a topical NSAID alongside an oral one to settle a knee osteoarthritis flare faster, and stop it once the flare settles rather than continuing both.
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