Comparison of the Birmingham Vasculitis Activity Score and the Five‐Factor Score to Assess Survival in Antineutrophil Cytoplasmic Antibody–Associated Vasculitis: A Study of 550 Patients From Spain (REVAS Registry)

Soláns-Laqué, Roser; Rodríguez-Carballeira, Mónica; Ríos-Blanco, Juan José; Fraile, G.; Sáez‐Comet, Luis; Martínez-Zapico, Aleida; Frutos, Begoña; Solanich, Xavier; Fonseca‐Aizpuru, Eva; Pasquau‐Liaño, Francisco; Zamora, M.; Oristrell, J; Fanlo, P.; López‐Dupla, Miguel; Abdilla, M.; García‐Sánchez, Isabel‐María; Sopeña, B.; Castillo, M. J.; Perales, Isabel; Callejas‐Rubio, José Luis

doi:10.1002/acr.23912

Cited by 36 publications

(23 citation statements)

References 27 publications

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“…They determined that only the fibrosis-4 index was associated with all-cause mortality during the follow-up in patients with MPA and GPA. BVAS and FFS are known to be useful to predict survival in antineutrophil cytoplasmic antibody-associated vasculitis and our results also support this finding [18,19].…”

Section: Discussionsupporting

confidence: 86%

Clinical features and outcomes of patients with microscopic polyangiitis: experience of two centers

2020

Signa Vitae

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Background and Aim: Microscopic polyangiitis (MPA) is a rare necrotizing vasculitis that involves predominantly the small-caliber blood vessels. In this retrospective study, we aimed to evaluate the clinical findings, prognostic factors, and outcomes of MPA patients followed in two different centers. Material and Method: In this retrospective study, a total of 38 patients diagnosed and followed with MPA in two different centers between January 2005 and December 2019 were included. Medical records of the patients were reviewed retrospectively. The diagnoses were based on the definition of MPA in the Chapel Hill consensus conference (CHCC-2012). In addition to the socio-demographic characteristics, clinical features, laboratory and immunological parameters; time passed for the diagnosis, treatment, outcomes, and follow-up time were recorded for each patient. The Five Factor Scores (FFS; Revised FFS 2009) and Birmingham Vasculitis Activity Score (BVAS; Version 3) were applied in all patients. Results: In a total of 38 patients (10 females, 28 male) were included in the study. The mean age of the participants was 54.52 ± 8.21 (range: 32-74) years. The mean time passed for the diagnosis was 2.93 ± 2.03 (0.50-11) months and the mean follow-up period was 55.0 ± 30.51 (2-124) months. At admission, the mean BVAS was 18.28 ± 5.73 (8-35). The FFS results were as follows; 0 in 4 (10.5%) patients, 1 in 27 (71.1%) patients, 2 in 7 (18.4%) patients. The most common symptoms at admission were dyspnea, cough, and hemoptysis. Renal failure was present in 31 (81.5%) of the patients, at admission. The most common immunologic finding was the p-ANCA positivity in 26 (68.4%) patients. Among all patients, 25 (65.8 %) did not have any other attacks after the diagnosis. In follow-ups, 12 (31.5%) patients were passed away and vasculitis associated mortality rate was 18.4%. The effects of some factors on mortality including age ≥ 65 years, male gender, renal involvement, p-ANCA positivity, decreased oxygen saturation (< 90%), having anemia at admission, having FEV1/FVC < 70 in spirometry, having a FFS score of 2, and having a BVAS > 18 were examined (Table 6). Only having an FFS score of 2 and BVAS > 18 were determined as the factors significantly affecting mortality (P: 0.01). Conclusion: Pulmonary symptoms are more predominant in MPA. Though many patients did not have any other attacks after the diagnosis, the vasculitis associated mortality may reach about 20%. BVAS and FFS are still the best determinants of mortality.

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Section: Discussionsupporting

confidence: 86%

Clinical features and outcomes of patients with microscopic polyangiitis: experience of two centers

2020

Signa Vitae

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“…PR3 ANCA positive 56%-83% 8,14 ANCA Negative 11%-31% 14,25 ENT involvement 70%-99% 13,26 Rhinologic Sinus involvement/Rhinitis 47%-72% 8,25 Recurrent epistaxis/nasal crusting 28%-83% 25,27 Nasal cavity adhesions 19%-26% 18,28 Rhinorrhoea 35%-54% 25,28 Mucosal ulcerations/granulations 10%-27% 18,28 Nasal septal perforation 8%-33% 27,29 External nasal/Saddle deformity 2%-22% 27,29 Epiphora 13% 29…”

Section: Prevalence Of Ent Features Of Granulomatosis With Polyangiitismentioning

confidence: 99%

“…Retro-orbital granulomata 3%-10% 25,29 Otologic Hearing loss (Audiometric) 23%-37% 8,28 OME/CHL 10%-31% 26,28 SNHL 8%-19% 26,30 Tinnitus 24%-30% 18,28 Head and neck/Laryngologic Hoarseness (including Laryngeal oedema/ Chronic laryngitis/VC palsy/Laryngeal ulceration)…”

Section: Prevalence Of Ent Features Of Granulomatosis With Polyangiitismentioning

confidence: 99%

“…ANCA positive 31%-58% 26,37 MPO ANCA positive 25%-50% 26,47 ENT involvement 48%-96% 37,38 Rhinologic CRSwNP 42%-51% 37,38 Change in sense of smell 40% 47 Nasal crusting 8%-43% 47,48 Otologic OME/CHL 3%-12% 26 59 although the importance of abstinence from cocaine for a good outcome cannot be overstated.…”

Section: Prevalence Of Ent Features Of Eosinophilic Granulomatosis Wimentioning

confidence: 99%

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Updates in antineutrophil cytoplasmic antibody (ANCA)‐associated vasculitis for the ENT surgeon

Coates

Pero

2020

Clinical Otolaryngology

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ENT involvement is common in ANCA-associated vasculitis (AAV), particularly in GPA and EGPA. Early recognition and treatment is important for good outcomes, yet evidence suggests that UK ENT surgeons may not consistently recognise the early features of AAV, despite a similar incidence to vestibular schwannoma. AAV is a rapidly advancing field, with significant developments in the understanding of its pathogenesis, classification and treatment over the past decade. Relevant vasculitis mimics are also discussed with a particular focus on the increasing prevalence of vasculitis mimics driven by an increase in recreational cocaine use, as well as the emergence and reclassification of several other vasculitis mimics in the head and neck. This article reviews key recent updates in the vasculitis literature, with a particular focus on those relevant to recognition and diagnosis of AAV for the ENT surgeon. Strengths and limitations of relevant diagnostic testing are discussed, and a method of evaluation of patients with features of AAV presenting to ENT services is outlined. | 317

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“…They survey takes only a few minutes to complete but does require training and it is essential to only score acute/ongoing symptoms and signs, not fixed damage from vasculitis or comorbidity. The BVAS scoring system is the gold standard for assessing vasculitis disease activity in clinical trials and has been to shown to have prognostic value and correlate with mortality risk assessment [21,22]. It was hypothesized that this was not used routinely in clinical practice and this was confirmed since <20% had BVAS scores reported.…”

Section: Response Ratesmentioning

confidence: 99%

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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Comparison of the Birmingham Vasculitis Activity Score and the Five‐Factor Score to Assess Survival in Antineutrophil Cytoplasmic Antibody–Associated Vasculitis: A Study of 550 Patients From Spain (REVAS Registry)

Cited by 36 publications

References 27 publications

Clinical features and outcomes of patients with microscopic polyangiitis: experience of two centers

Clinical features and outcomes of patients with microscopic polyangiitis: experience of two centers

Updates in antineutrophil cytoplasmic antibody (ANCA)‐associated vasculitis for the ENT surgeon

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

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