Long-term patient survival in a Swedish population-based cohort of patients with ANCA-associated vasculitis

Abstract

ObjectivesPatients with antineutrophil cytoplasmatic antibodies-associated vasculitides (AAV) exhibit higher mortality than the general population. In the current study, we assessed whether cluster affiliation based on clinical presentation might predict mortality.MethodsWith case record review, the outcomes for a population-based cohort of patients diagnosed with AAV in southern Sweden (catchment area of 0.7 million inhabitants) between 1997 and 2010 were assessed. Based on organ involvement at presentation, the cohort was stratified into the following clusters: gastrointestinal, cardiovascular, non-renal, renal with proteinase 3 (PR3) and renal without PR3. Cluster affiliation, demographics, clinical and laboratory values at entry were tested as prognostic factors for survival in multivariable models.Results195 patients (98 female) with a median age of 69 years (IQR 55–77) at diagnosis were included in the cohort. The median time of follow-up was 4 years for the 98 patients (50%) who died during follow-up and 11 years for those alive at end of follow-up. The 1-year, 2-year, 5-year and 10-year survival was 87%, 82%, 70% and 55%, respectively. Prognostic factors for survival were sex, age, renal function and cluster affiliation. The mortality of patients with AAV was significantly increased compared with the general population except in the non-renal cluster. The cardiovascular and gastrointestinal clusters showed the highest mortality.ConclusionEven though the mortality in patients with AAV is increased compared with the general population this does not apply to patients without gastrointestinal, cardiovascular or renal involvement at diagnosis. We suggest that the initial clinical presentation is an important predictor for survival.

Clinical characteristics and potential biomarkers for disease activity of patients with ANCA associated vasculitis: a monocenter study in China

“…A low hemoglobin was reported to be imparted an increased risk of death, possibly as markers of the severity of the systemic inflammation. Moreover, low hemoglobin had been considered to be a negative prognostic factor of AAV [5,33]. Interestingly, our observations revealed that hemoglobin levels were correlated with disease activity and also as an independent predictor for activity, in accordance with other studies who described lower hemoglobin levels were associated with severe AAV [33].…”

“…Our findings concur with previous reports that ENT involvement was predominant in GPA but rare in EGPA and MPA, renal involvement was almost constant in MPA but exceptional in EGPA; neurological involvement was notably more prevalent in EGPA than in GPA and MPA [5,6,17,18,20]. However, these clinical characteristics cannot distinguish different types of AAV, just like laboratory tests, they are also not specific.…”

“…The clinical manifestations of the AAV are diverse and can be confined to one organ, or multiple organs and even lifethreatening and prompt immunosuppressive treatment is crucial to reduce death by major-organ failure as well as long-term morbidity. Nevertheless, even if the diagnosis is correct and immunomodulatory therapy has been initiated, AAV tend to progress and the individual treatment response remains challenging to predict [5,6] Birmingham Vasculitis Activity Score (BVAS) had been used for assessing the activity and severity of AAV [5,7], and it was reportedly associated with the poor prognosis of AAV. Besides BVAS, large-scale biomarker studies had been conducted to identify an ideal biomarker reflecting disease activity.…”

Clinical characteristics of Polish patients with ANCA-associated vasculitides—retrospective analysis of POLVAS registry

“…This corroborated with a higher rate of kidney and lower respiratory tract involvement and lower rate of ENT involvement-all factors identified as independently related to poor prognosis. An increase in mortality during the first 2 years of AAV was already observed among Swedish AAV patients [33]. Interestingly, delay from the first symptoms of disease to diagnosis was in males shorter than in females.…”

Risk of opportunistic infections in patients with antineutrophil cytoplasmic antibody‐associated vasculitis, using a Japanese health insurance database

“…Prognosis in patients with AAV has recently been improved by remission‐induction treatment using immunosuppressive agents such as cyclophosphamide and high‐dose corticosteroids (CS) . However, it has been reported that patients with AAV have significantly higher mortality than the general population, with the most common cause of death being infection . Infection is therefore 1 of the most clinically important complications among patients with AAV, and a challenge to overcome for physicians treating AAV.…”