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Vasculitis in children
1Department of Pediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Insights
Pediatric vasculitis, including Henoch-Schonlein purpura (HSP) and childhood polyarteritis nodosa (PAN), presents unique challenges in India. This study highlights severe gastrointestinal and renal issues in HSP and increased central nervous system involvement in PAN.
Area of Science:
- Pediatric Rheumatology
- Pediatric Vasculitis
- Clinical Case Series
Background:
- Vasculitis in children is rare, with limited data from India.
- PGIMER, Chandigarh, has experience managing various pediatric vasculitides.
Purpose of the Study:
- To provide an overview of vasculitides encountered in Indian children.
- To review relevant literature and highlight specific findings.
Main Methods:
- Retrospective review of pediatric vasculitis cases at PGIMER.
- Analysis of clinical presentation, disease types, and outcomes.
- Literature review for comparison.
Main Results:
- Described 8 children with classical Polyarteritis Nodosa (PAN), noting frequent Central Nervous System (CNS) involvement.
- Reported 10 cases of childhood BCPAN with severe peripheral gangrene and auto-amputation in 7.
- Detailed 30 cases of Henoch-Schonlein Purpura (HSP) with high rates of gastrointestinal (86.7%) and severe renal involvement (nephrotic proteinuria 62%, azotemia 50%).
- Observed delayed diagnosis in Kawasaki Disease.
Conclusions:
- Pediatric vasculitis in India presents with distinct features, including severe complications.
- Higher CNS involvement in PAN and severe gangrene in BCPAN noted.
- HSP cases showed significant GI and renal morbidity, sometimes requiring surgical intervention.
- Delayed diagnosis of Kawasaki Disease is a concern.
Abstract:
Vasculitis in children is uncommon and hardly any information is available from India. We, at PGIMER, Chandigarh, have diagnosed and followed many children with vasculitis of different types though not all, which occur in children. In this article, we have given an overview of the vasculitides that we have encountered along-with a review of relevant literature. We have described 8 children with classical PAN and have highlighted a higher frequency of CNS involvement in our patients. Amongst the 10 BCPAN children, as many as 8 had peripheral gangrene which resulted in auto-amputation in 7. Gangrene of such severity has not been previously reported in this condition. We have also included 30 children with HSP. Gastrointestinal involvement was noted in 86.7% of children and in one of these, it was severe enough to result in hypovolemic shock. Such severe bleeding is very rare. Two of our patients with HSP came late to us after having been operated for an 'acute abdomen' elsewhere. Although renal involvement was seen less frequently than reported in the literature, the severity of involvement was greater (nephrotic range proteinuria in 62% and azotemia in 50%). We have only limited experience of Kawasaki Disease but it appears that children with this disorder are probably not being diagnosed in the acute stage in our country.