The epidemiology of Wegener's granulomatosis and microscopic polyangiitis in a Southern Hemisphere region

Gibson, Andrew; Stamp, Lisa K.; Chapman, Peter T.; O’Donnell, John L.

doi:10.1093/rheumatology/kei259

“…The average age at diagnosis in the present study (42 years) was similar to that observed in studies from São Paulo (43.4 years) and the U.S. (41 years) [1,13], but GPA patients from Chile (50.8 years), Australia (55 years), New Zealand (66 years) and Italy (58.8 years) were significantly older [10,11,15,26], possibly due to differences in geographical variables and/or in methodology. In fact, populational studies suggest GPA is more frequent in older individuals whereas case studies conducted at hospitals show a lower age at diagnosis [5,27,28].…”

Section: Discussionsupporting

confidence: 86%

“…Compared to the international literature, the upper respiratory tract and the lungs were less frequently affected in our patients from Northeastern Brazil [11,13,25]. The most frequent disease extension was generalized, and time to diagnosis was relatively long (in one case, 18 years from the onset of symptoms).…”

Section: Discussioncontrasting

confidence: 65%

“…For example, the female gender was predominant in a study from Southern Europe while the male gender was predominant in a study from Northern Europe [5,10]. Within the southern hemisphere, the male gender is predominant in New Zealand (43°S) (M/F ratio: 1.2) and Argentina (31°S) (M/F ratio: 1.2), while the female gender is predominant in Chile (33°S) (M/F ratio: 0.8) and São Paulo (23°S) (M/F ratio: 0.9) [11,13,15,16]. In short, available data suggest a tendency for female predominance among GPA patients at low latitudes in both hemispheres [10].…”

Section: Discussionmentioning

confidence: 95%

“…Epidemiological studies from the northern hemisphere also support the notion of environmental risk, as reflected in regional differences in prevalence and incidence and the existence of a North-South negative gradient (the incidence of GPA is higher in Northern Europe than in Southern Europe) [5,9,10]. Genetic and geographical factors may explain these differences [10][11][12].…”

Section: Introductionmentioning

confidence: 83%

“…-ENT involvement was less frequent in Northeastern Brazil (n = 16; 64%) than in São Paulo (n = 115; 85.8%) and New Zealand (n = 63; 86%) [11,13]. -Pulmonary involvement was less frequent in Northeastern Brazil (n = 15; 60%) than in the U.K.…”

Section: Frequency Of Clinical Manifestations: Northeastern Brazil Vsmentioning

confidence: 92%

See 3 more Smart Citations

Granulomatosis with polyangiitis in Northeastern Brazil: study of 25 cases and review of the literature

Azevedo

¹

,

Lima

²

,

Carvalho

³

et al. 2018

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Background: Little has been published about the epidemiology of Granulomatosis with polyangiitis (GPA) in South America, especially in the intertropical zone, and no epidemiological data from Brazil are available. The purpose of the present study was to draw a clinical and demographic profile of GPA patients living in Northeastern Brazil based on laboratory, histological and imaging findings, and evaluate the frequency of organic involvement.Methods: Clinical, epidemiological and treatment data of GPA patients were collected retrospectively and compared with the literature.Results: The cohort included 25 GPA patients (84% female) aged 45.8 ± 16.1 years. Renal and ear-nose-throat (ENT) manifestations were the most common (both 64%). One third (32%) of the patients had 24-h proteinuria > 1 g, 50% had creatinine clearance < 50 mL/min at the time of diagnosis, and 33% had recurrent kidney damage during disease progress. The affected organs included lungs (60%), joints (44%), skin (32%), peripheral nervous system (28%), eyes (28%) and heart (16%). ENT involvement (n = 16/64%) was less frequent in our region than in São Paulo (n = 115/85.8%). Renal (n = 16/64%) and pulmonary (n = 15/60%) involvement was less frequent in our region than in the U.K. (renal n = 30/90%; pulmonary n = 28/84.8%).

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Anti-cytokine targeted therapies for ANCA-associated vasculitis

Barnsley

¹

,

Buchbinder

²

,

Riminton

³

2010

Cochrane Database of Systematic Reviews

1

0

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BackgroundAnti-neutrophilic cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) are a group of rare auto-inflammatory diseases that a ects mainly small vessels. AAV includes: granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA). Anti-cytokine targeted therapy uses biological agents capable of specifically targeting and neutralising cytokine mediators of the inflammatory response. ObjectivesTo assess the benefits and harms of anti-cytokine targeted therapy for adults with AAV. Search methodsWe searched the Cochrane Central Register of Controlled Trials (2019, Issue 7), MEDLINE and Embase up to 16 August 2019. We also examined reference lists of articles, clinical trial registries, websites of regulatory agencies and contacted manufacturers. Selection criteriaRandomised controlled trials (RCTs) or controlled clinical trials of targeted anti-cytokine therapy in adults (18 years or older) with AAV compared with placebo, standard therapy or another modality and anti-cytokine therapy of di erent type or dose. Data collection and analysisWe used standard methodological procedures expected by Cochrane. Main resultsWe included four RCTs with a total of 440 participants (mean age 48 to 56 years). We analysed the studies in three groups: 1) mepolizumab (300 mg; three separate injections every four weeks for 52 weeks) versus placebo in participants with relapsing or refractory EGPA; 2) belimumab (10 mg/kg on days 0, 14, 28 and every 28 days therea er until 12 months a er the last participant was randomised) or etanercept (25 mg twice a week) with standard therapy (median 25 months) versus placebo with standard therapy (median 19 months) in participants with GPA/MPA; and 3) infliximab (3 mg/kg on days 1 and 14, before the response assessment on day 42) versus rituximab (0.375g/m 2 on days 1, 8, 15 and 22) in participants with refractory GPA for up to 12 months. None of the studies were assessed as low risk Anti-cytokine targeted therapies for ANCA-associated vasculitis (Review)

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Anti-cytokine targeted therapies for ANCA-associated vasculitis

Bała

¹

,

Małecka‐Massalska

²

,

Koperny

³

et al. 2020

Cochrane Database of Systematic Reviews

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BackgroundAnti-neutrophilic cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) are a group of rare auto-inflammatory diseases that a ects mainly small vessels. AAV includes: granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA). Anti-cytokine targeted therapy uses biological agents capable of specifically targeting and neutralising cytokine mediators of the inflammatory response. ObjectivesTo assess the benefits and harms of anti-cytokine targeted therapy for adults with AAV. Search methodsWe searched the Cochrane Central Register of Controlled Trials (2019, Issue 7), MEDLINE and Embase up to 16 August 2019. We also examined reference lists of articles, clinical trial registries, websites of regulatory agencies and contacted manufacturers. Selection criteriaRandomised controlled trials (RCTs) or controlled clinical trials of targeted anti-cytokine therapy in adults (18 years or older) with AAV compared with placebo, standard therapy or another modality and anti-cytokine therapy of di erent type or dose. Data collection and analysisWe used standard methodological procedures expected by Cochrane. Main resultsWe included four RCTs with a total of 440 participants (mean age 48 to 56 years). We analysed the studies in three groups: 1) mepolizumab (300 mg; three separate injections every four weeks for 52 weeks) versus placebo in participants with relapsing or refractory EGPA; 2) belimumab (10 mg/kg on days 0, 14, 28 and every 28 days therea er until 12 months a er the last participant was randomised) or etanercept (25 mg twice a week) with standard therapy (median 25 months) versus placebo with standard therapy (median 19 months) in participants with GPA/MPA; and 3) infliximab (3 mg/kg on days 1 and 14, before the response assessment on day 42) versus rituximab (0.375g/m 2 on days 1, 8, 15 and 22) in participants with refractory GPA for up to 12 months. None of the studies were assessed as low risk Anti-cytokine targeted therapies for ANCA-associated vasculitis (Review)

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The epidemiology of Wegener's granulomatosis and microscopic polyangiitis in a Southern Hemisphere region

Cited by 90 publications

References 11 publications

Granulomatosis with polyangiitis in Northeastern Brazil: study of 25 cases and review of the literature

Granulomatosis with polyangiitis in Northeastern Brazil: study of 25 cases and review of the literature

Anti-cytokine targeted therapies for ANCA-associated vasculitis

Anti-cytokine targeted therapies for ANCA-associated vasculitis

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