Henoch-Schönlein Purpura in children: not only kidney but also lung

Background Henoch-Schönlein Purpura (HSP) is the most common vasculitis of childhood and affects the small blood vessels. Pulmonary involvement is a rare complication of HSP and diffuse alveolar hemorrhage (DAH) is the most frequent clinical presentation. Little is known about the real incidence of...

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Published in:Pediatric Rheumatology 2019-11, Vol.17 (1), p.1-75, Article 75
Main Authors: Di Pietro, Giada Maria, Castellazzi, Massimo Luca, Mastrangelo, Antonio, Montini, Giovanni, Marchisio, Paola, Tagliabue, Claudia
Format: Article
Language:English
Subjects:
Anemia
Blood
Children
Diffuse alveolar hemorrhage
Hemorrhage
Henoch-Schönlein Purpura
IgA Vasculitis
Intubation
Pathogenesis
Patients
Pediatrics
Pulmonary involvement
Review
Rheumatology
Ventilation
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Summary:Background Henoch-Schönlein Purpura (HSP) is the most common vasculitis of childhood and affects the small blood vessels. Pulmonary involvement is a rare complication of HSP and diffuse alveolar hemorrhage (DAH) is the most frequent clinical presentation. Little is known about the real incidence of lung involvement during HSP in the pediatric age and about its diagnosis, management and outcome. Methods In order to discuss the main clinical findings and the diagnosis and management of lung involvement in children with HSP, we performed a review of the literature of the last 40 years. Results We identified 23 pediatric cases of HSP with lung involvement. DAH was the most frequent clinical presentation of the disease. Although it can be identified by chest x-ray (CXR), bronchoalveolar lavage (BAL) is the gold standard for diagnosis. Pulse methylprednisolone is the first-line of therapy in children with DAH. An immunosuppressive regimen consisting of cyclophosphamide or azathioprine plus corticosteroids is required when respiratory failure occurs. Four of the twenty-three patients died, while 18 children had a resolution of the pulmonary involvement. Conclusions DAH is a life-threatening complication of HSP. Prompt diagnosis and adequate treatment are essential in order to achieve the best outcome.
ISSN:1546-0096
1546-0096
DOI:10.1186/s12969-019-0381-y