Comparing polyarteritis nodosa in children and adults: a single center study

Kim

2020

Preprint

Background: Polyarteritis nodosa (PAN) is a systematic necrotizing vasculitis involving medium-sized arteries. Childhood-onset PAN ranges from mild to severe systematic disease causing damage and early mortality. As it is a rare disease in children, there is less study on clinical features, diagnosis and treatment than adults. And diagnosis occurs relatively late in most paediatric patients with polyarteritis nodosa. Therefore, this study aimed to investigate clinical manifestations, laboratory findings, treatment strategies, and long-term outcomes among patients with childhood-onset polyarteritis nodosa treated at a single-centre in Korea. We further aimed to evaluate the usefulness of the Birmingham Vasculitis Activity Score (BVAS) and Five-Factor Score (FFS) in paediatric patients.Methods: We retrospectively analysed data collected from patients with childhood-onset PAN treated at our institution from March 2003 to February 2020. Results: Nine patients (6 male and 3 female) were included in the study. The median ages at symptom onset and diagnosis were 7.6 (3–17.5) and 7.7 (3.5–17.6) years, respectively. The median follow-up duration was 7.0 (1.6–16.3) years. Eight patients were diagnosed with systemic PAN, while one was diagnosed with cutaneous PAN. All patients exhibited skin manifestations, while five exhibited Raynaud’s phenomenon. Organ involvement was observed in one patient. Constitutional symptoms such as fever, arthralgia, myalgia, and weight loss were also observed. Antineutrophil cytoplasmic antibodies were absent in all patients. The median BVAS at diagnosis was 8 (range: 2–29), and one patient had an FFS of 1. Prednisolone was initially used for induction in all patients, and other drugs were added in cases refractory to prednisolone. All patients survived despite some complications such as intracranial haemorrhage or digital necrosis requiring amputation.Conclusions: Early diagnosis and treatment may minimise sequelae in patients with childhood-onset PAN. Further multicentre studies are required to clarify the unique characteristics of childhood-onset PAN and establish treatment guidelines, early detection strategies, and biomarkers. Trial registration: Retrospectively registered.

Section: Discussionmentioning

confidence: 73%

“…Previous studies [6,12,20] have reported that skin involvement and arthralgia/arthritis are more common in children than in adults with PAN. In contrast, weight loss and renal, gastrointestinal, and neurologic involvement appear to be more common in adults with PAN.…”

Section: Discussionmentioning

confidence: 96%

Clinical presentations and long-term prognosis of childhood-onset polyarteritis nodosa in a single centre: a retrospective study

Kim

2020

Preprint

“…In children, relapse rate varied widely from 8.9%, up to 75% in the French series with the longest follow‐up . The observed mortality appeared somewhat lower in children, with mortality rates of 0–9.5% in pediatric series, versus 13.9–24.7% in adult series …”

Section: Classical Panmentioning

confidence: 93%

“…It is a systemic, necrotizing medium‐sized vessel vasculitis. It mainly affects adults, but pediatric series have also been published . Classification for both adult and childhood onset PAN have been developed, and the clinical features often overlap with DADA2 (Table ) …”

Section: Classical Panmentioning

confidence: 99%

See 1 more Smart Citation

Diagnosis and management of ADA2 deficient polyarteritis nodosa

Human

Pagnoux

2018

Int J of Rheum Dis

Deficiency of ADA2 (DADA2) is a recently described systemic inflammatory vasculopathy caused by mutations in the CERC1 gene that often, but not always, clinically resembles polyarteritis nodosa (PAN). The condition was originally characterized by livedoid rash, systemic inflammation, variable hypogammaglobulinemia, and early-onset stroke. The phenotypic spectrum has expanded to include patients with immunodeficiency syndromes and bone marrow dysfunction, which are not typical features of PAN. Exploration into the pathogenesis and treatment options of DADA2 has added to our understanding of this condition, but more studies are needed. The purpose of this article is to review the various clinical phenotypes of DADA2, and raise awareness among rheumatologists to consider DADA2 when evaluating patients presenting with PAN-like disease.

Arthritis & Rheumatology

Clinical Characteristics and Outcomes of Polyarteritis Nodosa: An International Study

Karadag,

Bolek,

Ayan

et al. 2024

Self Cite

ObjectiveTo describe the demographics, clinical features, disease course, and survival of polyarteritis nodosa (PAN) through an international collaboration (GLOBAL‐PAN).MethodsPatients 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.Results358 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 .ConclusionThe 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.image