Purpose: The aim of the study was to evaluate frequency and clinical relevance of haemorrhagic events associated with primary angiitis of the central nervous system in childhood (cPACNS), a rare but increasingly recognized disease with varying clinical presentations.
Method: A systematic literature review from 1990 onwards was conducted to identify reported cases of cPACNS.
Results: A total of 110 paediatric patients met the inclusion criteria. The median age was 9.5 years. Seven children (7/110, 6.4 %) demonstrated cerebral haemorrhage. Death occurred only in children with cerebral haemorrhage (4/110, 3.6 %); both a sudden and prolonged course of disease was observed.
Conclusion: PACNS is a rare disease and the occurrence of haemorrhage with this condition is even rarer; however, the risk of an unfavourable outcome under these circumstances seems to be increased. PACNS adds to the list of differential diagnoses of intracerebral haemorrhage in the paediatric population.