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2016 ; 95
(40
): e5096
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Diagnosing ANCA-associated vasculitis in ANCA positive patients: A retrospective
analysis on the role of clinical symptoms and the ANCA titre
#MMPMID27749588
Houben E
; Bax WA
; van Dam B
; Slieker WAT
; Verhave G
; Frerichs FCP
; van Eijk IC
; Boersma WG
; de Kuyper GTM
; Penne EL
Medicine (Baltimore)
2016[Oct]; 95
(40
): e5096
PMID27749588
show ga
Currently no validated diagnostic system for antineutrophil cytoplasmic antibody
(ANCA)-associated vasculitis (AAV) is available. Therefore, diagnosing AAV is
often challenging. We aimed to identify factors that lead to a clinical diagnosis
AAV in ANCA positive patients in a teaching hospital in The Netherlands.In this
study, all patients that tested positive for ANCA proteinase 3 (PR3) and/or
myeloperoxidase (MPO) between 2005 and 2015 were analysed. Patients with a
clinical diagnosis of AAV were compared with patients without a clinical
diagnosis of AAV. Clinical symptoms and laboratory variables at presentation,
including the ANCA titre, were collected for both patients with and without AAV.
Clinical and laboratory variables related with AAV were investigated, using
multivariable logistic regression.Two hundred thirty seven consecutive patients
with a positive ANCA were included, of whom 119 were clinically diagnosed with
AAV. Of the 118 ANCA positive patients without AAV, 87 patients had an
alternative diagnosis, including inflammatory bowel disease (n?=?24), other
rheumatic diseases (n?=?23), infection (n?=?11), malignancy (n?=?4), and other
diagnoses (n?=?25). In a multivariable regression model, a high ANCA titre (odds
ratio [OR] 14.16, 95% confidence interval [CI] 6.93-28.94) and a high number of
affected organ systems (OR 7.67, 95% CI 3.69-15.94) were associated with AAV.MPO
and PR3 ANCA can be positive in a variety of diseases that mimic AAV. A higher
ANCA titre and multiple affected organ systems may help to discriminate between
AAV and other systemic illnesses in anti-PR3 and anti-MPO positive patients. A
diagnostic scoring system incorporating these factors should be considered.