Clinical presentations and long term prognosis of childhood onset polyarteritis nodosa in single centre of Korea

Abstract

Childhood-onset polyarteritis nodosa (PAN) is a rare and systemic necrotising vasculitis in children affecting small- to medium-sized arteries. To date, there have been only a few reports because of its rarity. Thus, we aimed to investigate the clinical manifestations, laboratory findings, treatment, and long-term outcomes in patients with childhood-onset PAN and to evaluate the usefulness of the paediatric vasculitis activity score (PVAS). We retrospectively analysed the data of nine patients with childhood-onset PAN from March 2003 to February 2020. The median ages at symptom onset, diagnosis, and follow-up duration were 7.6 (3–17.5), 7.7 (3.5–17.6), and 7.0 (1.6–16.3) years, respectively. All patients had constitutional symptoms and skin manifestations, while five exhibited Raynaud’s phenomenon. Organ involvement was observed in one patient. The median PVAS at diagnosis was 7 (range: 2–32). Prednisolone was initially used for induction in all patients, and other drugs were added in cases refractory to prednisolone. All patients survived, but three patients with high PVAS at diagnosis experienced irreversible sequelae, including intracranial haemorrhage and digital amputation. In conclusion, early diagnosis and treatment may minimise sequelae in patients with childhood-onset PAN. This study suggests that high PVAS score at diagnosis may be associated with poor prognosis.

A Case of Polyarteritis Nodosa with Positive Anti-Phospholipid Antibodies Presenting with Multifocal Myositis

“…Initial blood tests revealed a remarkable elevation in in ammatory markers, including erythrocyte sedimentation rate (ESR), 93 mm/1st hour and 101 mm/2nd hour (ref. [3][4][5][6][7][8][9][10][11][12][13]; white blood cells (WBC), 29,000 cells/µl (ref. [4][5][6][7][8][9][10]; neutrophil cells (NEU), 24,000 cells/µl (ref.…”

“…PAN can present a broad spectrum of manifestations with multiple organ systems, including the skin, gastrointestinal tract, kidney, nervous system, and muscles… [7] [8]. With spare lesions of the lung, the disease most commonly targets the peripheral nervous system and the skin and is distinctive to the antineutrophilic cytoplasmic antibody (ANCA)-associated vasculitis [9] [10]. Musculoskeletal involvement in PAN may present in a limited form or a component of a systemic entity, including myalgia, arthralgia, polymyositis-like syndrome, asymmetric nondeforming polyarthritis, acute leg ischemia, or myopathy [11].…”

Polyarteritis Nodosa: Clinical Cases in Boys 12 and 9 Years Old

“…В настоящее время ведутся разработки препаратов для лечения тяжелых форм ювенильного полиартериита [14,15]. Рассматривается возможность использования препарата ритуксимаб для лечения различных аутоиммунных заболеваний.…”

Polyarteritis nodosa

“…This year, three studies addressed the outcome in cPAN. Lee et al [16] analyzed retrospectively nine patients with cPAN over a period of approximately 17 years in South Korea. They have analyzed the efficacy of the pediatric vasculitis activity score (PVAS) and reported the Five Factor Score (FFS) as 7 (median).…”