Clinical Characteristics and Outcomes of Polyarteritis Nodosa: An International Study.

Objective: We describe the demographics, clinical features, disease course, and survival of polyarteritis nodosa (PAN) through an international collaboration (GLOBAL‐PAN). Methods: Patients with PAN were recruited between 1990 and 2020 from observational cohorts of nine countries across Europe, Japa...

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Publicado en:Arthritis & Rheumatology Vol. 76; no. 7; pp. 1120 - 1130
Autores principales: Karadag, Omer, Bolek, Ertugrul Cagri, Ayan, Gizem, Mohammad, Aladdin J., Grayson, Peter C., Pagnoux, Christian, Martín‐Nares, Eduardo, Monti, Sara, Abe, Yoshiyuki, Alberici, Federico, Alibaz‐Oner, Fatma, Cuthbertson, David, Dagna, Lorenzo, Direskeneli, Haner, Khalidi, Nader A., Koening, Curry, Langford, Carol A., McAlear, Carol A., Monach, Paul A., Moroni, Luca
Formato: research tables/charts Journal Article
Publicado: Wiley-Blackwell Jul2024
Acceso en línea:Ver este registro en EBSCOhost
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      dt: Jul2024
      vid: 76
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      pub: Wiley-Blackwell
      place: Malden, Massachusetts
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        10.1002/art.42817
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        atl: Clinical Characteristics and Outcomes of Polyarteritis Nodosa: An International Study.
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        au:
          Karadag, Omer
          Bolek, Ertugrul Cagri
          Ayan, Gizem
          Mohammad, Aladdin J.
          Grayson, Peter C.
          Pagnoux, Christian
          Martín‐Nares, Eduardo
          Monti, Sara
          Abe, Yoshiyuki
          Alberici, Federico
          Alibaz‐Oner, Fatma
          Cuthbertson, David
          Dagna, Lorenzo
          Direskeneli, Haner
          Khalidi, Nader A.
          Koening, Curry
          Langford, Carol A.
          McAlear, Carol A.
          Monach, Paul A.
          Moroni, Luca
        affil: Hacettepe University, Ankara, Turkey
      sug:
        subj:
          Polyarteritis Nodosa
          World Health
          Sociodemographic Factors
          Survival Rate Evaluation
          Disease Attributes
          Collaboration
          Human
          Prospective Studies
          Nonexperimental Studies
          Descriptive Statistics
          Male
          Female
          Adult
          Middle Age
          Proteinuria
          Gastrointestinal Diseases
          Age Factors
          Creatinine Blood
          Central Nervous System
          Retrospective Design
          Record Review
          Funding Source
          Adult: 19-44 years
          Middle Aged: 45-64 years
          Male
          Female
      ab: Objective: We describe the demographics, clinical features, disease course, and survival of polyarteritis nodosa (PAN) through an international collaboration (GLOBAL‐PAN). Methods: Patients with PAN were recruited between 1990 and 2020 from observational cohorts of nine countries across Europe, Japan, and North America. Eligibility was retrospectively defined using the European Medicines Agency classification algorithm. Patients with PAN related to hepatitis B virus (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. Results: Three hundred fifty‐eight patients (female:male ratio 174:184), including those with systemic PAN (sPAN, n = 282) and cutaneous PAN (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 (interquartile 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 death for sPAN included age ≥65 years at diagnosis, serum creatinine at diagnosis >140 μmol/L, gastrointestinal manifestations, and central nervous system (CNS) involvement. Conclusion: The spectrum of PAN remains a complex, multifaceted disease. Relapse is common. Age ≥65 years and serum creatinine >140 μmol/L at diagnosis, as well as gastrointestinal and CNS involvement, are independent predictors of death in sPAN.
      pubtype: Academic Journal
      doctype:
        research
        tables/charts
        Journal Article
      ougenre: Article
    language: English
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