Simple, readily available clinical indices predict early and late mortality among patients with ANCA-associated vasculitis

Haris, Ágnes; Polner, Kálmán; Arányi, József; Braunitzer, Henrik; Kaszás, Ilona; Rosivall, László; Kökény, Gábor; Mucsi, István

doi:10.1186/s12882-017-0491-z

Cited by 16 publications

(11 citation statements)

References 27 publications

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“…Most patients had ≥1 comorbidity at diagnosis confirming results of previous studies [3,17,18] who also found that majority of patients had comorbid diseases including important cardiovascular diseases [18]. Some studies have demonstrated comorbidity at diagnosis is one factor determining long term survival [17,18] but another study suggested the patients functional status, which would be influenced by comorbidity is more predictive [3].…”

Section: Response To Induction Therapysupporting

confidence: 81%

Treatment practices, response to therapy and adverse events in ANCA-associated vasculitis patients in Europe: top-line findings from a retrospective observational study

Rutherford

Goette

2019

Preprint

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Background ANCA-associated vasculitis patient outcome data in the real world setting is scarce. This study measures key clinical outcomes and adverse effects over the first 12 months of remission induction therapy.Methods This was a retrospective study of 929 newly diagnosed [ND] and 268 relapsing patients [RP] conducted online by 399 clinicians. Each clinician completed a survey for 3 patients meeting the following criteria: initiated remission induction treatment for new or relapsing disease between Nov 2014 and Feb 2017, ≥ 6 months of therapy including ≥ 1 course of induction therapy, under continuous care for ≥12 months. Data were collected relating to baseline presentation and at 1, 3, 6, and 12 months.Results 58% were >55 years old with more granulomatosis with polyangiitis (GPA, 54%) versus microscopic polyangiitis (MPA, 46%), and <20% of patients had Birmingham Vasculitis Activity Scoring (BVAS) performed. Median symptom duration prior to diagnosis was 6 to 7 weeks. Presenting symptoms were similar between ND and RP, noted differences (≥ 5%) were more fever, rash, and neuropathy, and less renal disease in RP. The majority (68% ND and 84% RP) had at least one comorbidity at diagnosis, with a similar distribution. Glucocorticoids (GC) were used by 83% ND and 76% RP; >50% were still receiving GC at 12 months. Most common treatments were cyclophosphamide+GC for ND (59%) and rituximab+GC for RP (44%). Many patients had slow and/or partial response to therapy, by 12 months >60% had a full response. 81% of patients with response by month 1 maintained full response through month 12. Adverse events and infections were common, especially during the first 3 months when GC use is highest.Conclusions Real world data show that both ND and RP ANCA-associated vasculitis patients respond variably to induction remission treatment and many experience adverse events and infections over the first 12 months of treatment. The presence of comorbidities at treatment initiation in most patients compounded the adverse impacts of disease and treatment. This study improves our understanding of the reality of clinical outcomes in ANCA-associated vasculitis and the need for targeted therapeutic approaches.

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Section: Response To Induction Therapysupporting

confidence: 81%

Treatment practices, response to therapy and adverse events in ANCA-associated vasculitis patients in Europe: top-line findings from a retrospective observational study

Rutherford

Goette

2019

Preprint

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“…17 Resolution of hematuria was also considered criteria for remission. 15 Relapse was defined as an increase in serum creatinine level accompanied with GFR decline, new RBC cast above 5 cells, new proteinuria above 500 mg over 24 hours not explained by any other secondary causes, worsening or new extrarenal manifestations attributable to active vasculitis after a time of remission, with or without increase in ANCA titers. 12 Complications encountered (namely, infection required hospitalization, thrombotic events, malignancy and infertility) and disease outcomes were also recorded.…”

Section: Data Collectionmentioning

confidence: 99%

“…3,13 We hypothesized that the initial BVAS might be a valid independent measure that can estimate the future relapse as only few articles have evaluated the predictive value of BVAS for future relapses with ANCA associated vasculitis. 14,15 Birmingham Vasculitis Activity Score (BVAS) is a well-established and validated measurement tool for disease activity and extension, as BVAS=0 defines disease remission and BVAS≥1 defines active disease. 16 Consequently, the present retrospective analysis would explore the characters of AAV patients, with the correlates for increased relapses and complications, in addition to the ability of BVAS to a marker of impending disease relapse.…”

Section: Introductionmentioning

confidence: 99%

ANCA-Associated Vasculitis Clinical Presentation and Clinical Predictors of Relapse in Saudi Arabia

Alahmari

Daajani²,

Alsayed

et al. 2021

OARRR

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Background: Anti-neutrophil cytoplasm antibodies-associated vasculitis (AAV) is a rare autoimmune condition with high-relapsing rate and incidence of complications, resulting in increased morbidity and mortality. Characters of patients with anti-neutrophil cytoplasm antibodies-associated vasculitis in Saudi Arabia require further exploration. Objective: To evaluate the clinical profile, relapse rate and disease-related complications among patients with AAV at a tertiary hospital in Saudi Arabia. To estimate the role of BVAS score at the time of presentation in predicting relapse during the disease course. Design and Setting: This retrospective cohort study was performed through data collection from patients' records who had AAV, who visited the rheumatology clinic. The collected data involved the demographics of patients and their investigations, medications, and outcomes of treatment. Statistical analysis was executed through SPSS version 26. Results: Fifty-two patients were eligible for inclusion, while 48 patients were analyzed because of missing data. Females represented 60.4%. Half of the patients were more than 50 years old, and 68.8% had comorbidities. As for diagnosis, 62.5% had granulomatosis with polyangiitis, 25% had eosinophilic granulomatosis with polyangiitis, and 12.5% had microscopic polyangiitis. The rate of relapse was 31.3%, while the remission rate was 68.8%. Additionally, 66.7% had lower respiratory involvement, and 43.8% had renal involvement. More than half of the patients had BVAS score below 14.5 points. The study did not explore a positive correlation between the disease relapse and high BVAS at the first presentation. Conclusion: Early prediction of relapse and such intervention is of paramount importance in order to avoid accrual of organ damage with treatments that prevent further relapses. BVAS score was not found to be a potential predictor in our study. Future studies are highly endorsed, with prospective design and large sample size to achieve statistical significance for the incidence of relapses and complications.

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“…A previous study found that comorbidities were associated with mortality, and this relationship remained independent of other important clinical characteristics [17]. We also analysed the relationship between comorbidities and the prognosis and renal outcome, while comorbidities were not independent predictors of death or ESRD or the doubling of serum creatinine.…”

Section: Discussionmentioning

confidence: 94%

“…MPA is a multiple-system disease, involving primarily the kidneys and lungs, and it is characterized by necrotizing glomerulonephritis and pulmonary capillaritis [11]. Many clinical indexes have been reported to have prognostic value in AAV patients, such as age [6, 12, 13], haemoglobin level [8], albumin level [14], Birmingham Vasculitis Activity Score (BVAS) [15, 16] and comorbidities [17]. Many studies have shown that AAV patients with worse renal function have a poorer prognosis [11, 13, 15, 18], including patients with MPA [14, 19].…”

Section: Introductionmentioning

confidence: 99%

Clinical characteristics and outcomes in microscopic polyangiitis patients with renal involvement: a study of 124 Chinese patients

Shi

Shen

2019

BMC Nephrol

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Background Microscopic polyangiitis (MPA) is a systemic autoimmune disease, and renal involvement is frequently present in MPA. MPA patients with renal involvement may have a worse prognosis. In this study, we aimed to evaluate the prognostic factors associated with all-cause death and renal survival in MPA patients with renal involvement. Methods A retrospective observational cohort study was performed. One hundred twenty-four patients newly diagnosed with MPA with renal involvement excluding end-stage renal disease (ESRD) who were hospitalized at the First Affiliated Hospital of Chongqing Medical University from January 2012 to July 2017 were included. All the survivors were followed up with until July 2018. The clinical and laboratory data at the time of the initial MPA diagnosis were collected, and the predictive values of the variables for mortality and renal outcome were analysed. Results Among the 124 patients, 52 were men (41.9%) and 72 were women (58.1%), and the age range was from 25 to 85 years (63.9±10.6 years). Seventy-six patients (61.3%) had pulmonary involvement. Multivariate Cox analysis revealed that age≥65 years (HR: 2.437; P =0.021), serum creatinine≥500 μmol/L (HR=2.207; P =0.009) and interstitial lung disease (ILD) (HR=2.366; P =0.013) were associated with mortality. Cox multivariate analysis identified that serum creatinine≥500 μmol/L (HR=8.236; P <0.001) and ILD (HR=2.649; P =0.001) were independent detrimental factors for renal survival, and immunosuppressive treatment was a protective factor for renal survival (HR=0.349; P =0.001). The area under the ROC curve (AUC) of the serum creatinine level at diagnosis was 0.705 for mortality and 0.870 for progression to ESRD or the doubling of serum creatinine. Conclusions Age, serum creatinine level at diagnosis and ILD were independent predictors of mortality in MPA patients with renal involvement. Serum creatinine level at diagnosis, ILD and immunosuppressive treatment were independently related to renal survival.

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Simple, readily available clinical indices predict early and late mortality among patients with ANCA-associated vasculitis

Cited by 16 publications

References 27 publications

Treatment practices, response to therapy and adverse events in ANCA-associated vasculitis patients in Europe: top-line findings from a retrospective observational study

Treatment practices, response to therapy and adverse events in ANCA-associated vasculitis patients in Europe: top-line findings from a retrospective observational study

ANCA-Associated Vasculitis Clinical Presentation and Clinical Predictors of Relapse in Saudi Arabia

Clinical characteristics and outcomes in microscopic polyangiitis patients with renal involvement: a study of 124 Chinese patients

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