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, Japan,...

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Published inArthritis & Rheumatology Vol. 76; no. 7; pp. 1120 - 1129
Main Authors Ramirez, Giuseppe, Treppo, Elena, Quartuccio, Luca, Pastore, Serena, Martín-Nares, Eduardo, Pagnoux, Christian, Grayson, Peter C., Mohammad, Aladdin J., Ozen, Seza, Emmi, Giacomo, Furuta, Shunsuke, Hinojosa-Azaola, Andrea, Ayan, Gizem, Bolek, Ertugrul Cagri, Hocevar, Alojzija, Warrington, Kenneth J., Karadag, Omer, YAZICI, AYTEN, Seo, Phillip, Kilickap, Saadettin, Kawakami, Tamihiro, Kasifoglu, Timucin, Tufan, Abdurrahman, Karadeniz, Hazan, Kono, Hajime, Springer, Jason, Tezcan, Mehmet Engin, Guido, Jeannin, Gregoni, Gina, Gopaluni, Seerapani, Gazel, Ummugulsum, Forbess, Lindsy, Schiavon, Franco, Felicetti, Mara, Ertenli, Ihsan, Tomsic, Matija, Sánchez-Cubías, Susy Marcela, Conticini, Edoardo, Chung, Sharon, Cefle, Ayse, Bozzola, Enrico, Lopalco, Giuseppe, Vaglio, Augusto, Moreland, Larry, Ugurlu, Serdal, Yazisiz, Veli, Armagan, Berkan, Gercik, Onay, Akar, Servet, Rhee, Rennie, Amudala, Naomi, Merkel, Peter A., Jayne, David, Simonini, Gabriele, Khalidi, Nader A., Padoan, Roberto, Koening, Curry, Langford, Carol A., McAlear, Carol A., Cuthbertson, David, Dagna, Lorenzo, Direskeneli, Haner, Alberici, Federico, Abe, Yoshiyuki, Alibaz-Oner, Fatma, Monti, Sara, Monach, Paul A., Moroni, Luca
Format Journal Article
LanguageEnglish
Published Boston, USA Wiley 01.07.2024
Wiley Periodicals, Inc
Wiley Subscription Services, Inc
Subjects
Online AccessGet full text
ISSN2326-5191
2326-5205
2326-5205
DOI10.1002/art.42817

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Abstract 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.
AbstractList ObjectiveWe describe the demographics, clinical features, disease course, and survival of polyarteritis nodosa (PAN) through an international collaboration (GLOBAL‐PAN).MethodsPatients 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.ResultsThree 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.ConclusionThe 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.
We describe the demographics, clinical features, disease course, and survival of polyarteritis nodosa (PAN) through an international collaboration (GLOBAL-PAN).OBJECTIVEWe describe the demographics, clinical features, disease course, and survival of polyarteritis nodosa (PAN) through an international collaboration (GLOBAL-PAN).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.METHODSPatients 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.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.RESULTSThree 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.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.CONCLUSIONThe 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.
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.
We describe the demographics, clinical features, disease course, and survival of polyarteritis nodosa (PAN) through an international collaboration (GLOBAL-PAN). 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. 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. 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.
Author Treppo, Elena
Gercik, Onay
Furuta, Shunsuke
Ertenli, Ihsan
Jayne, David
Tufan, Abdurrahman
Ozen, Seza
Moroni, Luca
Chung, Sharon
Tomsic, Matija
Vaglio, Augusto
Langford, Carol A.
Kawakami, Tamihiro
Conticini, Edoardo
Forbess, Lindsy
Padoan, Roberto
Kilickap, Saadettin
Seo, Phillip
Cefle, Ayse
Dagna, Lorenzo
Amudala, Naomi
Grayson, Peter C.
Alibaz-Oner, Fatma
Schiavon, Franco
Yazisiz, Veli
Ramirez, Giuseppe
Quartuccio, Luca
McAlear, Carol A.
Merkel, Peter A.
Karadag, Omer
Akar, Servet
Tezcan, Mehmet Engin
Kasifoglu, Timucin
Kono, Hajime
Emmi, Giacomo
Armagan, Berkan
Felicetti, Mara
Pastore, Serena
Karadeniz, Hazan
Gregoni, Gina
Mohammad, Aladdin J.
Monti, Sara
Pagnoux, Christian
Martín-Nares, Eduardo
Bozzola, Enrico
Simonini, Gabriele
Direskeneli, Haner
Hinojosa-Azaola, Andrea
Ugurlu, Serdal
Gazel, Ummugulsum
Sánchez-Cubías, Susy Marcela
Abe, Yoshiyuki
YAZICI, AYTEN
Khalidi, Nader A.
Hocevar, Alojzija
Gopaluni, Seerapani
Koening, Curry
Monach, Paul A.
Warrington, Kenneth J.
Lopalco, Giuseppe
Springer, Jason
Rhee, Rennie
Bolek, Ertugrul Cagri
Guido,
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ContentType Journal Article
Contributor Treppo, Elena
Felicetti, Mara
Gercik, Onay
Ertenli, Ihsan
Gregoni, Gina
Karadeniz, Hazan
Tufan, Abdurrahman
Pastore, Serena
Bozzola, Enrico
Simonini, Gabriele
Ugurlu, Serdal
Chung, Sharon
Sánchez-Cubías, Susy Marcela
Gazel, Ummugulsum
Vaglio, Augusto
Tomsic, Matija
Kawakami, Tamihiro
Conticini, Edoardo
Forbess, Lindsy
Kilickap, Saadettin
Cefle, Ayse
Gopaluni, Seerapani
Amudala, Naomi
Schiavon, Franco
Yazisiz, Veli
Ramirez, Giuseppe
Quartuccio, Luca
Lopalco, Giuseppe
Springer, Jason
Rhee, Rennie
Yazici, Ayten
Akar, Servet
Tezcan, Mehmet Engin
Kasifoglu, Timucin
Guido, Jeannin
Kono, Hajime
Armagan, Berkan
Moreland, Larry
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Copyright 2024 American College of Rheumatology
2024 American College of Rheumatology.
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Notes Author disclosures and graphical abstract are available at
https://acrjournals.onlinelibrary.wiley.com/doi/10.1002/art.42817
https://onlinelibrary.wiley.com/doi/10.1002/art.42817
Supported by the Vasculitis Clinical Research Consortium, which received funding from the National Center for Advancing Translational Science, the National Institute of Arthritis and Musculoskeletal and Skin Diseases (grant U54‐AR‐057319), and the National Center for Research Resources (grant U54‐RR‐019497) of the NIH. Dr Jayne's work was supported by the National Institute for Health Research Cambridge Biomedical Research Centre.
.
Additional supplementary information cited in this article can be found online in the Supporting Information section
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Snippet Objective We describe the demographics, clinical features, disease course, and survival of polyarteritis nodosa (PAN) through an international collaboration...
We describe the demographics, clinical features, disease course, and survival of polyarteritis nodosa (PAN) through an international collaboration...
ObjectiveWe describe the demographics, clinical features, disease course, and survival of polyarteritis nodosa (PAN) through an international collaboration...
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SubjectTerms Adenosine
Adenosine deaminase
Adult
Age
Aged
Algorithms
Central nervous system
Creatinine
Demographics
Diagnosis
Disease
Europe - epidemiology
Familial Mediterranean fever
Female
Hepatitis B
Humans
International cooperation
International studies
Japan - epidemiology
Male
Middle Aged
North America - epidemiology
Patients
Pediatrics
Polyarteritis Nodosa
Proteinuria
Proteinuria - etiology
Recurrence
Retrospective Studies
Signs and symptoms
Survival
Survival Rate
Young Adult
Title Clinical Characteristics and Outcomes of Polyarteritis Nodosa: An International Study
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