Background Cutaneous polyarteritis nodosa (cPAN) is an immune complex-mediated rare disease that affects small and medium sized vessels in the dermis and subcutaneous tissue.
The clinical course is characterized by periodic exacerbations and remissions that may persist for many years. Most patients respond to NSAIDs and glucocorticoids (GC), whereas some may require DMARDs and/or immunomodulatory therapy.
Objectives To describe the diferrent clinical patterns, laboratory findings and long term outcomes of juvenile cPAN in a tertiary care hospital.
Methods Retrospective observational study, including all patients diagnosed with cPAN between 2002–2016. Diagnosis relied on clinical features confirmed by histological study. Recorded data included clinical features, laboratory results and long-term outcomes.
Results 10 children were included (7 female), mean age at onset was 9.9 years (r:4.1–16.3). Delay from symptoms onset to biopsy confirmed diagnosis was 2±2.3 months; 4 patients underwent a second biopsy due to inconclusive results in the first performed.
Clinical features included cutaneous (100%) and osteomuscular involvement (50%), fever (40%), neuropathy (10%) and weight loss (10%). Reported cutaneous symptoms were 8 patients with nodules, 4 livedo, 4 purpura, 1 ulcer and 1 necrosis. Most lesions were localized in the lower limbs (8), even though it was also reported in upper limbs (3) and trunk (3). Most cases exhibited raised CRP, ESR and leukocyte count with a mean of 26.4±47.9 (mg/L), 27.7±29.7 (mm/h), and 8.6±5.3 (x 109/L) respectively.
As first line therapy, all patients received GC and 6 NSAIDs. 8 were given a DMARD such as HCQ or MTX. Due to persistent activity or relapse, rescue treatment with pulse-GC (20%), MMF (10%) or IVIG (20%) was instituted. Only 1 patient received penicillin prophylaxis due to relapses associated with streptococcal infection.
Mean follow-up was 3.9 years (r: 1.1–10.4). 4 patients had a monophasic disease, six suffered ≥2 relapses. At last follow-up 9 patients were on remission, even though 3 were off-therapy. No complications were reported.
Conclusions Clinical and laboratory findings in our series was similar to previous reports. However, our patients presented a greater number of relapses and DMARDs requirement.
Disclosure of Interest None declared