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Record W3116551151 · doi:10.1186/s13023-020-01621-3

French recommendations for the management of systemic necrotizing vasculitides (polyarteritis nodosa and ANCA-associated vasculitides)

2020· review· en· W3116551151 on OpenAlexaff
Benjamin Terrier, Raphaël Darbon, Cécile‐Audrey Durel, É. Hachulla, Alexandre Karras, Hélène Maillard, T. Papo, Xavier Puéchal, G. Pugnet, T. Quéméneur, M. Samson, Camille Taillé, Loı̈c Guillevin, Vincent Audard, O. Aumaître, Karine Briot, P. Cacoub, P. Cathébras, Dominique Chauveau, O. Chosidow, Laurent Chouchana, Vincent Cottin, Divi Cornec, Éric Daugas, Élisabeth Diot, N. Dupin, Khalil El Karoui, Olivier Fain, P. Gobert, Philippe Guilpain, M. Hamidou, Aurélie Hummel, M. Jachiet, S. Jouneau, Noémie Jourde‐Chiche, C. Landron, Claire Le Jeunne, Jean‐Christophe Lega, Xavier Mariette, Christian Pagnoux, Philippe Rémy, Frédéric Vandergheynst

Bibliographic record

VenueOrphanet Journal of Rare Diseases · 2020
Typereview
Languageen
FieldMedicine
TopicVasculitis and related conditions
Canadian institutionsMount Sinai Hospital
Fundersnot available
KeywordsMicroscopic polyangiitisMedicinePolyarteritis nodosaGranulomatosis with polyangiitisEosinophilicVasculitisDiseaseSystemic vasculitisNecrotizing VasculitisAnti-neutrophil cytoplasmic antibodyDermatologyIntensive care medicinePediatricsInternal medicinePathology

Abstract

fetched live from OpenAlex

Systemic necrotizing vasculitis comprises a group of diseases resembling polyarteritis nodosa and anti-neutrophil cytoplasmic antibody-associated vasculitis (ANCA): granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, and microscopic polyangiitis. The definitive diagnosis is made in cooperation with a reference center for autoimmune diseases and rare systemic diseases or a competency center. The management goals are: to obtain remission and, in the long term, healing; to reduce the risk of relapses; to limit and reduce the sequelae linked to the disease; to limit the side effects and the sequelae linked to the treatments; to improve or at least maintain the best possible quality of life; and to maintain socio-professional integration and/or allow a rapid return to school and/or professional activity. Information and therapeutic education of the patients and those around them are an integral part of the care. All health professionals and patients should be informed of the existence of patient associations. The treatment of vasculitis is based on variable combinations of glucocorticoids and immunosuppressants, chosen and adapted according to the disease concerned, the severity and/or extent of the disease, and the underlying factors (age, kidney function, etc.). Follow-up clinical and paraclinical examinations must be carried out regularly to clarify the progression of the disease, detect and manage treatment failures and possible relapses early on, and limit sequelae and complications (early then late) related to the disease or treatment. A distinction is made between the induction therapy, lasting approximately 3-6 months and aimed at putting the disease into remission, and the maintenance treatment, lasting 12-48 months, or even longer. The role of the increase or testing positive again for ANCA as a predictor of a relapse, which has long been controversial, now seems to have greater consensus: Anti-myeloperoxidase ANCAs are less often associated with a relapse of vasculitis than anti-PR3 ANCA.

Fetched live from OpenAlex and de-inverted. Abstracts are not stored in this database: the inverted indexes are 8.6 GB of the frame’s 9.3 GB of text, and the host has 13 GB free.

How this classification was reachedexpand

Full frame machine prediction

Teacher imitation

Not calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.

metaresearch head score (Codex)0.006
metaresearch head score (Gemma)0.010
Version: metacan-v3-hybrid-931329e0061cValidation status: machine_predicted_unvalidated
Candidate categoriesnone
Consensus categoriesnone
DomainCandidate signal: none · Consensus signal: none
Study designCandidate signal: Not applicable · Consensus signal: Not applicable
GenreCandidate signal: Review · Consensus signal: Review
Teacher disagreement score0.072
Threshold uncertainty score0.144

Distilled classifier scores by category (both heads)

CategoryCodexGemma
Metaresearch0.0060.010
Meta-epidemiology (narrow)0.0010.000
Meta-epidemiology (broad)0.0010.003
Bibliometrics0.0040.002
Science and technology studies0.0010.001
Scholarly communication0.0020.001
Open science0.0030.001
Research integrity0.0070.005
Insufficient payload (model declined to judge)0.0170.008

Machine scores (provisional)

The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.

Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.

Opus teacher head0.031
GPT teacher head0.302
Teacher spread0.271 · how far apart the two teachers sit on this one work
Validation statusscore_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from it

Classification

machine, unvalidated

Machine predicted; a candidate call from one source (direct Gemma or distilled Codex), not a consensus.

The models applied no category: nothing in the taxonomy fit this work.
Study designNot applicable
Domainnot available
GenreReview

How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".

Quick stats

Citations74
Published2020
Admission routes1
Has abstractyes

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