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

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 PAN-predictive 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 3 positive predictive parameters (hepatitis B virus antigen and/or DNA in serum, arteriographic anomalies, and mononeuropathy or polyneuropathy) and 5 negative predictive parameters (indirect immunofluorescence detection of antineutrophil cytoplasmic antibody; asthma; ear, nose, and throat signs; glomerulopathy; and cryoglobulinemia) for the criteria set. These criteria yielded 70.6% sensitivity for all control vasculitides and 89.7% for MPA controls, with 92.3% specificity for all controls and 83.1% for MPA controls. The discriminant abilities of this set of items outperformed the ACR 1990 criteria in all analytical situations, showing better robustness to variations in the prevalence of individual vasculitides. CONCLUSION The use of positive and negative discriminant criteria could constitute a sound basis for developing a diagnostic tool for PAN to be used by clinicians. Further prospective analyses and validations in different populations are needed to confirm these items as satisfactory diagnostic criteria.