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.).
Clinical Characteristics and Outcomes of Polyarteritis Nodosa: An International Study / 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; Padoan, Roberto; Seo, Phillip; Warrington, Kenneth J.; Hocevar, Alojzija; Hinojosa‐Azaola, Andrea; Furuta, Shunsuke; Emmi, Giacomo; Ozen, Seza; Jayne, David; Merkel, Peter A.; GLOBAL‐PAN Collaborators: Naomi Amudala, Rennie Rhee, Servet Akar, Onay Gercik, Berkan Armagan, Enrico Bozzola, Ayse Cefle, Ayten Yazici, Sharon Chung, Edoardo Conticini, Susy Marcela Sánchez-Cubías, Ihsan Ertenli, Mara Felicetti, Franco Schiavon, Lindsy Forbess, Ummugulsum Gazel, Seerapani Gopaluni, Gina Gregoni, Jeannin Guido, Hajime Kono, Hazan Karadeniz, Abdurrahman Tufan, Timucin Kasifoglu, Tamihiro Kawakami, Saadettin Kilickap, Giuseppe Lopalco, Larry Moreland, Serena Pastore, Luca Quartuccio, Elena Treppo, Giuseppe Ramirez, Gabriele Simonini, Augusto Vaglio, Jason Springer, Mehmet Engin Tezcan, Matija Tomsic, Serdal Ugurlu, Veli Yazisiz. - In: ARTHRITIS & RHEUMATOLOGY. - ISSN 2326-5191. - STAMPA. - 76:(2024), pp. 1120-1129. [10.1002/art.42817]
Clinical Characteristics and Outcomes of Polyarteritis Nodosa: An International Study
Emmi, Giacomo;Gabriele Simonini;Augusto Vaglio;
2024
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. (Figure presented.).
I documenti in FLORE sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
