Mortality in systemic necrotizing vasculitides: A retrospective analysis of the French Vasculitis Study Group registry

Jardel, S.; Puéchal, Xavier; Quellec, A. Le; Pagnoux, Christian; Hamidou, M.; Maurier, F.; Aumaître, O.; Aouba, Achille; Quéméneur, T.; Subra, Jean‐François; Cottin, Vincent; Sibilia, Jean; Godmer, Pascal; Cacoub, P.; Fauchais, Anne Laure; Hachulla, É.; Maucort‐Boulch, Delphine; Guillevin, Loı̈c; Lega, Jean‐Christophe

doi:10.1016/j.autrev.2018.01.022

Cited by 47 publications

(25 citation statements)

References 38 publications

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“…We know that cardiac and non‐cardiac vascular disease is significantly more likely in the presence of extra articular RA . GPA is also associated with a risk of mortality, although more recent evidence shows that despite hospitalizations increasing for this condition, mortality has significantly reduced over the last 15 to 20 years, presumably due to improved immunosuppressive therapy . Mortality linked to RA has also significantly decreased over the last decade .…”

Section: Discussionmentioning

confidence: 99%

“…This trend is mirrored in those patients with systemic necrotising vasculitides. One study found an almost threefold reduction in mortality in those diagnosed before 1980 compared to those diagnosed after 2010 . It seems that mortality is being reduced over time and this could be due to the improved knowledge, detection and management of these autoimmune entities.…”

Section: Discussionmentioning

confidence: 99%

“…Other reported associations include granulomatosis with polyangiitis (GPA, previously Wegener's granulomatosis), relapsing polychondritis, systemic lupus erythematosus (SLE), polyarteritis nodosa and eosinophilic disorders such as Churg‐Strauss syndrome. Other antineutrophil cystoplasmic antibody (ANCA) positive vasculitides are also known to be associated . Finally, infective causes are also described, including fungal and herpes viruses…”

Section: Introductionmentioning

confidence: 99%

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Ocular complications and mortality in peripheral ulcerative keratitis and necrotising scleritis: The role of systemic immunosuppression

Ogra

Sims

McGhee

et al. 2020

Clinical Exper Ophthalmology

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Importance: Inflammatory ocular diseases are associated with systemic disease, ocular morbidity and mortality. Background: To examine clinical characteristics of subjects with peripheral ulcerative keratitis (PUK) and necrotising scleritis. Design: Retrospective case series in a single tertiary centre over 10 years. Participants: All patients presenting with necrotising scleritis, PUK or scleritis with corneal infiltrates. Fifty-two eyes (41 subjects) were identified. Methods: Demographic and clinical data analysis. Main Outcome Measures: Visual loss, ocular complications and mortality. Results: Mean follow up was 7.0 ± 5.3 years, presentation age 51.3 ± 18.6 years, and 23 subjects (56.1%) were female. Overall, 27 eyes presented with PUK, 26 with necrotising scleritis, and 22 with corneal infiltrates associated with active scleritis. Associated systemic diagnosis was observed in 12 subjects at presentation, and in 21 subjects (51.2%) by final follow up. Recurrence occurred in 23 eyes (44.2%): 10 eyes experiencing PUK or necrotising scleritis;13 eyes with non-necrotising scleritis. Perforation occurred in six eyes (11.5%).Moderate vision loss occurred in 10 eyes (19.2%) and severe loss in six eyes (11.5%). Five subjects (12.2%) were deceased by study conclusion, of whom four were not on immunomodulatory therapy (IMT). A marked difference in estimated survival was observed with IMT, with mean estimated survival of 10.7 years without treatment, compared to 24.7 years with IMT (P = .045).Conclusions and Relevance: PUK and necrotising scleritis represent a severe inflammatory disease with high rates of perforation, visual loss and mortality.IMT was associated with a lower rate of mortality and longer estimated survival in this group. K E Y W O R D Scorneal thinning, immunosuppression, inflammation, sclera, ulcerative keratitis

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Ocular complications and mortality in peripheral ulcerative keratitis and necrotising scleritis: The role of systemic immunosuppression

Ogra

Sims

McGhee

et al. 2020

Clinical Exper Ophthalmology

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“…Rokowanie u chorych zaczęło się poprawiać po wdrożeniu do terapii glikokortykosteroidów, co miało miejsce w latach 50. XX wieku [33,34]. Stosowane w monoterapii, wydłużały one czas przeżycia chorych, nie zapobiegały jednak progresji choroby, zwłaszcza w przypadku zajęcia nerek [35].…”

Section: Leczenie Ziarniniakowatości Z Zapaleniem Naczyńunclassified

Ziarniniakowatość z zapaleniem naczyń — rys historyczny

Runowska

Majewski

2019

Forum Reumatologiczne

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Zapalenia naczyń to rzadka i bardzo niejednorodna grupa chorób, która charakteryzuje się procesem zapalnym obejmującym ścianę naczynia, prowadzącym do jej uszkodzenia, a w konsekwencji często do niedokrwienia i martwicy zaopatrywanych przez nie tkanek. Obowiązujący system nazewnictwa zapaleń naczyń (na podstawie Chapel Hill Consensus Conference, CHCC 2012) opiera się głównie na dominującej wielkości zajętych naczyń: dużych, średnich lub małych, i obejmuje prawie 30 postaci pierwotnych oraz kilka kategorii postaci wtórnych. Choć szeroko pojęte choroby naczyń krwionośnych były znane już w czasach starożytnych, to terminu "zapalenie naczyń" w kontekście odrębnej jednostki chorobowej używa się dopiero od około 150 lat. Od tego czasu dokonał się niewątpliwie ogromny postęp w zakresie diagnostyki, leczenia i klasyfikacji tych schorzeń, z drugiej jed-nak strony wciąż pozostaje wiele niewiadomych, na przykład w odniesieniu do ich etiologii i patogenezy. Pierwsze opisy przypadków poszczególnych typów zapaleń naczyń dostarczają nie tylko interesującego kontekstu historycznego, ale są także podstawą dla zrozumienia współczesnych kierunków badań. W poniższej pracy przedstawiono rys historyczny ziarniniakowatości z zapaleniem naczyń (GPA, granulomatosis with polyangiitis), należącej do grupy zapaleń małych naczyń związanych z przeciwciałami przeciwko cytoplazmie neutrofilów (AAV, ANCA-associated vasculitis), z uwzględnieniem historii jej odkrycia, leczenia, umiejscowienia w systemie klasyfikacyjnym oraz badań nad znaczeniem przeciwciał ANCA w diagnostyce tej choroby.

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“…Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is the most frequent aetiology of rapidly progressive glomerulonephritis syndrome [1] and the incidence of vasculitis-related mortality is actually 0.10 per 100 persons. [2] Soon after the outspread of ANCA testing a discussion began as to whether biopsy can be avoided in AAV, however, renal biopsy remains the definitive complementary exam and should always be considered in the AAV diagnostic workup. [1] Although percutaneous kidney biopsy (PKB) is a safe procedure, complications can occur and are mainly related to bleeding.…”

Section: Introductionmentioning

confidence: 99%

Biopsy utility in the workup of ANCA-associated vasculitis

Anjo

Campos

Narciso

et al. 2018

CRIM

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ANCA - associated Vasculitis (AAV) positive biopsy is supportive of vasculitis diagnosis, the aim of this manuscript is that renal biopsy is not always compulsory in the diagnosis and management of AAV. 79 years old, Caucasian women, admitted with AAV suspicion, MPO positive. A Microscopic polyangiitis with a pulmonary – renal syndrome was diagnosed after other AAV were excluded. Remission induction and maintenance therapy was made. In 6 months the patient presented a considerable improvement with lower MPO value, ESR and a stabilization of kidney function. Renal biopsy has to be performed in the majority of patients with the suspicion or renal AAV, although in this case it was not performed considering that the patient was on chronic anticoagulation and renal biopsy was not essential for the diagnosis. We suggest that renal biopsy is a valuable method in establishes the aetiology of kidney disease and that it could be dismissed in AAV with suggestive clinical presentation and low suspicion for secondary vasculitis.

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Mortality in systemic necrotizing vasculitides: A retrospective analysis of the French Vasculitis Study Group registry

Cited by 47 publications

References 38 publications

Ocular complications and mortality in peripheral ulcerative keratitis and necrotising scleritis: The role of systemic immunosuppression

Ocular complications and mortality in peripheral ulcerative keratitis and necrotising scleritis: The role of systemic immunosuppression

Ziarniniakowatość z zapaleniem naczyń — rys historyczny

Biopsy utility in the workup of ANCA-associated vasculitis

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