TY - JOUR
T1 - Clinical Characteristics and Outcomes of Polyarteritis Nodosa
T2 - An International Study
AU - Karadag, Omer
AU - Bolek, Ertugrul Cagri
AU - Ayan, Gizem
AU - Mohammad, Aladdin J.
AU - Grayson, Peter C.
AU - Pagnoux, Christian
AU - Martín-Nares, Eduardo
AU - Monti, Sara
AU - Abe, Yoshiyuki
AU - Alberici, Federico
AU - Alibaz-Oner, Fatma
AU - Cuthbertson, David
AU - Dagna, Lorenzo
AU - Direskeneli, Haner
AU - Khalidi, Nader A.
AU - Koening, Curry
AU - Langford, Carol A.
AU - McAlear, Carol A.
AU - Monach, Paul A.
AU - Moroni, Luca
AU - Padoan, Roberto
AU - Seo, Phillip
AU - Warrington, Kenneth J.
AU - Hocevar, Alojzija
AU - Hinojosa-Azaola, Andrea
AU - Furuta, Shunsuke
AU - Emmi, Giacomo
AU - Ozen, Seza
AU - Jayne, David
AU - Merkel, Peter A.
N1 - Publisher Copyright:
© 2024 American College of Rheumatology.
PY - 2024
Y1 - 2024
N2 - 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. (Figure presented.).
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. (Figure presented.).
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U2 - 10.1002/art.42817
DO - 10.1002/art.42817
M3 - Article
C2 - 38343337
AN - SCOPUS:85186458087
SN - 2326-5191
JO - Arthritis and Rheumatology
JF - Arthritis and Rheumatology
ER -