Patients with systemic vasculitis who require steroids for long periods or who have multiple relapses are likely to have extensive, irreversible organ damage.
Patients with systemic vasculitis who require steroids for long periods or who have multiple relapses are likely to have extensive, irreversible organ damage, say researchers. For every 10 months patients were on corticosteroids the odds ratio for having a Vasculitis Damage Index (VDI) score above 5 was 1.28 (95% CI 1.08 to 1.51, P=0.004), according to Joanna Robson, MBBS, PhD, of the University of Oxford in England, and colleagues.
“And strikingly, the odds ratio was 6.195 [95% CI 1.773 to 21.641, P=0.004] for people who had four or more relapses during follow-up,” she reported at the annual meeting of the British Society for Rheumatology.The multisystem disorders granulomatosis with polyangiitis (formerly referred to as Wegener’s granulomatosis) and microscopic polyangiitis are known collectively as the anti-neutrophil-cytoplasm antibody-associated (ANCA) vasculitides.
The VDI assesses damage in various systems, including musculoskeletal, cardiovascular, skin and mucus membranes, ear/nose/throat, pulmonary, peripheral vascular, gastrointestinal, and renal.”We know that patients with ANCA-associated vasculitis who have more than five items present on the VDI have an increased mortality ratio of 6.4,” Robson said.Previous studies have suggested possible links between steroid use, early disease activity, and relapse with later damage.To more clearly delineate the factors associated with damage, she and her colleagues analyzed data from four trials conducted by the European Vasculitis Study Group and the available long-term follow-up data.The trials included 535 patients, all with biopsy-proven disease, and long-term data were available for 270.Patients’ mean age was 58, and mean disease activity score at baseline was 17. Slightly more than half were men.
More than three-quarters of patients had no damage at baseline, but after a mean of 7.3 years, 34% had five or more items on the VDI. Only 7.9% had no damage at all at final follow-up, Robson noted.The mean duration of steroid use was 40.4 months, and almost half were still on steroids at their last visit.Still being on steroids at the final visit was associated with a higher damage score (P=0.003), as was having multiple relapses (P=0.027), older age (P=0.052), and high baseline creatinine (P=0.002).As expected, high baseline creatinine also was a significant predictor of having five or more items on the VDI during long-term follow-up (OR 1.002 per mmol/L, 95% CI 1.001 to 1.003, P<0.001).
Associations seen for individual VDI items included age at entry for osteoporosis, malignancy, and cerebrovascular events, elevated baseline creatinine was associated with hypertension, angina, and cerebrovascular accidents, and baseline disease activity score was predictive of malignancy.
Associations for duration of corticosteroid use included:
Hypertension, OR 1.03 per month of use (95% CI 1.013 to 1.046, P<0.001)
Cataract, OR 1.032 per month of use (95% CI 1.002 to 1.06, P=0.036)
In addition, associations for the number of relapses included:
Osteoporosis, OR 1.35 for each relapse (95% CI 1.036 to 1.77, P=0.026)
Cerebrovascular event, OR 1.74 for each relapse (95% CI 1.16 to 2.61, P=0.008)
“From this study we can say that prolonged corticosteroid use and high numbers of relapse[s] are associated with increased damage. This indicates that we really need more effective steroid-sparing treatments that can prevent relapse but that also will allow us to reduce the cumulative dose of corticosteroids,” Robson concluded.
Limitations of the study included the fact that the patient population was not an inception cohort and the exclusion of patients older than 80 and those with severe pulmonary hemorrhage from the four clinical trials.
“So we don’t have the full range of patient phenotypes and may be underestimating the damage,” Robson noted.
The authors reported no conflicts of interest.
Robson J, et al. Factors associated with long-term damage in the ANCA-associated vasculitides: an analysis of cohorts from the European Vasculitis Study Group. BSR 2013; Abstract 06