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Objectives: The main distinguishing feature between MPA ( microscopic polyangiitis ) and PAN (polyarteritis nodosa ) is the presence of oligoimmune vasculitis in arterioles, venules, or capillaries in MPA .
PAN is a disease limited to medium vessels, and MPA is a mainly small vessel vasculitis that can also involve medium vessels .
The purpose of this article is to develop and validate classification criteria for microscopic polyangiitis (MPA) .
Immune PAN is limited to medium-vessel disease, and MPA is a predominantly small-vessel disease that can also involve medium-vessel disease .
The purpose of this article is to develop and validate classification criteria for microscopic polyangiitis (MPA) .
Methods: Patients with vasculitis or comparative disease were recruited into an international cohort
.
The study was conducted in five phases : ( 1 ) identification of candidates using a consensus approach, ( 2 ) prospective collection of candidates at diagnosis , ( 3 ) data-driven reduction of the number of candidates, and ( 4 ) case review by an expert panel to determine Score-based risk scores for disease classification were derived in the development set using least absolute shrinkage and selection operator logistic regression with reference to diagnosis and (5) , and performance was subsequently validated in an independent set of characteristic cases and comparative diseases .
Methods: Patients with vasculitis or comparative disease were recruited into an international cohort
RESULTS: The development dataset for MPA included 149 MPA and 408 comparative cases .
The validation set consisted of an additional 142 MPAs and 414 comparison cases .
From the 91 candidate items, regression analysis identified 10 MPA items , of which 6 were retained .
Final criteria and their weights were as follows: perinuclear anti-neutrophil cytoplasmic antibody ( ANCA ) or anti-myeloperoxidase- ANCA positive ( +6 ), oligoimmune glomerulonephritis ( +3 ), pulmonary fibrosis or Isolate lung disease ( +3 ), sino- nasal symptoms or signs (-3) , cytoplasmic ANCA or antiprotease 3 ANCA positivity (-1) and eosinophil count ≥1 × 109/L (-4)
Conclusions: The 2022 American College of Rheumatology / Consortium of European Associations of Rheumatology MPA classification criteria are now validated and available for clinical research
Source:
Suppiah R, Robson JC, Grayson PC THE DCVAS INVESTIGATORS , et al .
2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for microscopic polyangiitis.
Annals of the Rheumatic Diseases 2022; 81: 321-326.
Suppiah R, Robson JC, Grayson PC THE DCVAS INVESTIGATORS , et al .
2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for microscopic polyangiitis.
, et al Annals of the Rheumatic Diseases Annals of the Rheumatic Diseases 2022; 81: 321-326.
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