Although current management of ANCA-associated vasculitis (AAV) leads to good short-term survival, there are still too many patients with residual complications including organ damage, a WA study shows.
A retrospective study of 67 adult patients with Anti-neutrophil cytoplasmic antibody (ANCA) vasculitis seen at the Royal Perth Hospital between 2007 and 2016 determined the annual incidence rate for AAV was 10.1 per million.
Thirty-five patients were diagnosed with granulomatous polyangiitis (GPA, incidence 0.5 per million), 12 with microscopic polyangiitis (MPA, incidence 2.2 per million) and seven with eosinophilic polyangiitis (EGPA, incidence 1.3 per million).
“Two patients were lost to follow-up and during a median follow-up of 4 years, 45/65 patients (69.2%) experienced 106 disease flares (range 1-8 per patient),” the study authors wrote in the International Journal of Clinical Rheumatology.
“Few patients remained free of organ damage with high ENT damage accrual damage seen in GPA, renal damage in MPA and neuropathy in EGPA, all consistent with previous reports.”
Overall patient survival at one year was 98.5% and 93.2% at five years however severe organ damage was a predictor of poorer survival.
Lead researcher Professor Hans Nossent from the University of Western Australia told the limbic that severe infections were the most common complication due to immunosuppression treatment.
“Recent advances have been the introduction of B cell depletion treatment as the less toxic option, and the reduction in steroid treatment and the reduction in the use of cyclophosphamide as well.”
“But still some of these conditions, GPA especially have a tendency to flare up quite often despite treatment.”
He added there was a lot of interest in the development of biologics against complement factors.
“While they are very expensive, they appear to be effective in stopping the inflammatory process without an overly high rate of infections.”
Professor Nossent said improved awareness of the admittedly rare conditions, faster diagnosis and early initiation of treatment were required. Many patients currently had quite a long diagnostic delay
“For example, GPA will go along as chronic sinusitis for a few years even before further investigations are being done. Or chronic cough with EGPA.”
“And it can also progress quite quickly – a lot of people will end up with renal failure if not treated because a very necrotising glomerulonephritis will develop. In terms of pulmonary damage, they will have severe pulmonary fibrosis, pulmonary hypertension, or even pulmonary bleeding.”
“One of the big developments in Australia and New Zealand has been the formation of a special interest group for vasculitis last year. Hopefully we will be able to compile a registry and conduct studies similar to the large ones currently ongoing in Europe.”