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Juvenile relapsing periarteritis nodosa and streptococcal infection
J M Tonnelier1, S Ansart, A Tilly-Gentric
1Service de médecine interne et maladies infectieuses, hôpital de la Cavale-Blanche, CHU, Brest, France.
Abstract:
Classic polyarteritis nodosa is a multisystem inflammatory disease associated with necrotizing vasculitis of small and medium arteries. In most cases, the causes of polyarteritis nodosa remain unknown, but viruses (HBV, HCV, HIV) and microbes (especially streptococcus) have been considered as etiologic or contributing factors. A 13-year-old boy was admitted with fever, skin lesions, polyarthritis and muscle involvement. A muscle biopsy demonstrated a necrotizing vasculitis and antistreptolysin titre was tremendously increased. His condition improved following the administration of oral steroids but he experienced relapses 5 and 12 years later when penicillin withdrawal was attempted. The flares were accompanied by a major increase of antistreptolysin titre and response to corticosteroid was obtained. He is currently 38 years old and he remains well on prophylactic penicillin. Polyarteritis nodosa in children may occur after a streptococcal infection. It may be prudent to consider penicillin prophylaxis in patients with periarteritis nodosa when a streptococcal etiology is documented or highly suspected.
Insights
Classic polyarteritis nodosa, a vasculitis affecting medium arteries, can be triggered by streptococcal infections in children. Long-term penicillin prophylaxis may be beneficial for patients with suspected streptococcal etiology.
Area of Science:
- Rheumatology
- Pediatric Vasculitis
- Infectious Disease
Background:
- Polyarteritis nodosa (PAN) is a rare inflammatory condition affecting small and medium arteries.
- Etiology of PAN is often unknown, though viral and microbial factors are implicated.
- Streptococcal infections are considered potential triggers for PAN.
Observation:
- A 13-year-old male presented with fever, skin lesions, polyarthritis, and muscle involvement.
- Muscle biopsy revealed necrotizing vasculitis with elevated antistreptolysin titer.
- The patient experienced relapses upon penicillin withdrawal, with increased antistreptolysin titers.
Findings:
- The patient's condition improved with oral steroids and remained stable with prophylactic penicillin.
- This case suggests a link between streptococcal infection and childhood polyarteritis nodosa.
- Recurrences correlated with elevated antistreptolysin titers, indicating ongoing inflammatory response.
Implications:
- Pediatric PAN may follow streptococcal infections.
- Penicillin prophylaxis should be considered in PAN patients with documented or suspected streptococcal etiology.
- Early diagnosis and management are crucial for long-term outcomes in childhood vasculitis.