Clinical Characteristics and Outcomes of Polyarteritis Nodosa - an International Study.

To describe the demographics, clinical features, disease course, and survival of polyarteritis nodosa (PAN) through an international collaboration (GLOBAL-PAN). Patients with PAN recruited between 1990 and 2020 from observational cohorts of 9 countries across Europe, Japan, and North America. Eligibility was retrospectively defined using the European Medicines Agency (EMA) classification algorithm. Patients with PAN related to hepatitis B virus (HBV) (n=12) and two monogenic diseases mimicking PAN, deficiency of adenosine deaminase 2 enzyme (n=16) or familial Mediterranean fever (n=11), were excluded. Data regarding organ involvement, relapse, disease-related damage, and survival were analyzed. 358 patients (female/male: 174/184), including systemic PAN (sPAN, n=282) and cutaneous PAN (cPAN, n=76), were included. Twenty-five were pediatric-onset. Mean (SD) age at diagnosis was 44.3 (18.1) years. Constitutional symptoms (71.5%), cutaneous involvement (70.5%), musculoskeletal findings (69.1%), and neurologic features (48.0%) were common manifestations. Among patients with sPAN, gastrointestinal involvement, and proteinuria over 400 mg/day were reported in 52.2% and 11.2%, respectively. During a median (inter-quartile range) 59.6 (99.5) months of follow-up, relapse occurred in 48.5% of patients. One, 5- and 10-year survival rates for sPAN were 97.1%, 94.0%, and 89.0%, respectively. Predictors of mortality for sPAN included age ≥ 65 years at diagnosis, serum creatinine at diagnosis >140 μmol/L, gastrointestinal manifestations, and central nervous system (CNS) involvement . The spectrum of PAN remains a complex, multi-faceted disease. Relapse is common. Age ≥ 65 years and serum creatinine >140 μmol/L at diagnosis, gastrointestinal and CNS involvement are independent predictors of mortality in sPAN.

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