Disease flares, damage accrual and survival in ANCA-associated vasculitis in Western Australia
Journal Title: International Journal of Clinical Rheumatology - Year 2019, Vol 14, Issue 1
Abstract
Background: ANCA-associated vasculitis (AAV) is a potentially life-threatening condition for which limited Australian data are available. We investigated the clinical and epidemiological outcomes of AAV in the last decade in a tertiary setting in Western Australia (WA). Methods and findings: A single centre observational study of patients fulfilling classification criteria for granulomatous polyangiitis (GPA), eosinophilic polyangiitis (EGPA) and microscopic polyangiitis (MPA) between 2007-2016. Clinical data collected at baseline, flares and last follow-up included disease activity (BVAS), treatment regimen and damage (VDI) scores. Non-parametric tests and Kaplan Meyer survival estimates were used in analysis. Results: During the study period there were 54 incident and 13 prevalent cases for an annual AAV incidence rate of 10.1/million (95% CI: 7.6-13.8) with rates for GPA (n=35), MPA (n=12) and EGPA (n=7) at 6.5, 2.2 and 1.3 per million respectively. Point prevalence per 1/1/2016 was 116.7/million. Age and gender distribution were similar between subtypes, but baseline BVAS scores were significantly higher in MPA patients (16 vs 10 vs 10, p<0.01). The relapse rate (0.4 per patient-year) was equivalent across AAV subtypes with less than 30% of patients in remission and damage accrual present in 93% at last followup. Patient survival (93.2% at 5 years) was negatively impacted especially by damage accrual with VDI >3. Conclusion: The incidence of especially GPA in WA is lower than in surrounding regions. Although current management of AAV leads to good short-term survival, it associates with frequent development of organ damage, which impacts on survival.
Authors and Affiliations
Shereen Paramalingam, Warren D Raymond, Chanakya Sharma, Gursharan Dogra, Andrew Mclean-Tooke, Johannes Nossent
Keywords
Related Articles
- EP ID EP614580
- DOI -
- Views 43
- Downloads 0
The role of miRNAs in autoimmune inflammatory diseases
MicroRNAs (miRNAs) are abundant short 21- 23 nucleotides-long non-coding RNAs that modulate virtually all eukaryotic gene expression processes during development and are crucial for sustaining optimal cell functions and...
Risk of upper gastrointestinal adverse events in Malaysian rheumatic patients on long-term non-steroidal antiinflammatory drugs
Background: Non-steroidal anti-inflammatory drug (NSAID)-induced upper gastrointestinal (GI) adverse events are well-described in the Western population but data is lacking in Asian patients. This study aims to describe...
Body image in lupus: Is it disease activity, physical function, depression, pain, fatigue, sleep, fibromyalgia or stress?
Patients with systemic lupus erythematosus (SLE) frequently experience poor body image (BI), an important issue, though not well researched or understood thus far. BI is perception of one’s own body. The effects of disea...
Antiphospholipid antibodies and single nucleotide polymorphisms in patients with venous ulcer in the population of Latvia
Background: Chronic venous insufficiency (CVI) may lead to leg ulceration (CVI stage C6) (Nicolaides A.N. et al., 2000). Some authors published that genetic risk factors are observed more frequently in patients with CVI...
Lower ratings of pain intensity in older adults lead to underestimation of disease activity in patients with rheumatoid arthritis
Background: To investigate the influence of age on the components of the 28-joint Disease Activity Score (DAS28)-C-Reactive Protein (CRP) in patients with Rheumatoid Arthritis (RA) and whether DAS28-CRP can be equally in...