A paradigm of diagnostic criteria for polyarteritis nodosa: Analysis of a series of 949 patients with vasculitides

Abstract

Objective. To establish a set of clinical and paraclinical criteria potentially useful as a diagnostic screening tool for polyarteritis nodosa (PAN).Methods. The abilities of individual descriptive items to predict a diagnosis of PAN were evaluated by screening available data from 949 patients from the French Vasculitis Study Group database, including 262 with PAN and 687 with control vasculitides. Selected items were tested in a logistic regression model to establish a minimal set of nonredundant PANpredictive criteria. The discriminative accuracy of these items and of the American College of Rheumatology (ACR) 1990 criteria were assessed by reapplying them to the initial patient sample and a subgroup restricted to PAN and microscopic polyangiitis (MPA) patients. A computer simulation procedure was conducted on artificially generated patient data to evaluate the usefulness of these criteria in predicting a diagnosis of PAN.Results. The analysis resulted in the retention of The systemic vasculitides are a heterogeneous group of diseases that have blood vessel inflammation as a common trait. In 1990, relying on many previous efforts, the American College of Rheumatology (ACR) proposed a set of classification criteria that were selected by a panel of experts based on an analysis of multicenter patient data (1). Seven distinct entities were thus characterized, including polyarteritis nodosa (PAN) (2). Although the primary purpose of the ACR 1990 criteria was to standardize the classification of vasculitis patients to facilitate communication among researchers (3), their good discriminative accuracy indicated by the initial assessments, with sensitivities of 71-94% and specificities of 87-92% for different types of vasculitides,

Polyarteritis Nodosa

“…13,14 Diagnostic criteria have also been attempted and through a survey of 949 patients in the FVSG, HBV positivity, peripheral neuropathy, arteriographic abnormalities, as well as the absence of ANCA, asthma, ear nose and throat signs, glomerulopathy, and cryoglobulinemia were predictive of PAN diagnosis. 15 Despite these efforts, criteria for systemic vasculitides, particularly for PAN, still remain unsatisfactory, and there is a large international collaborative effort aimed to establish better diagnostic and classification criteria. 16,17 EPIDEMIOLOGY/RISK FACTORS PAN is a rare disease that affects all racial groups, affecting men more often than women, and occurring primarily in patients in their 40s to 60s.…”

Vasculitis related to viral and other microbial agents

“…primary systemic vasculitis [3]. However, !30% of PAN cases are secondary to HBV infection [4e6], with HBV positivity being the single most important criterion in confirming the diagnosis of PAN, in the setting of compatible clinical and angiographic features [6]. In recent decades, the incidence of PAN has significantly declined in the context of HBV vaccination programs and blood transfusion screening [7].…”

Classification des vascularites de l’enfant

“…L'algorithme de Watts permet, en combinant de faç on séquentielle les classifications de l'ACR et de Chapel-Hill, de classer plus de 95 % des patients avec une bonne reproductibilité [10]. De plus, le groupe franç ais d'étude des vascularites a proposé des critères de périar-térite noueuse (PAN) [11] qui reposent en partie sur l'exclusion des signes les plus caractéristiques des autres vascularites. Ainsi, la tendance moderne est de définir une vascularite systémique primitive par ce qu'elle est autant que par ce qu'elle n'est pas.…”