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Published in: Pediatric Rheumatology 1/2019

Open Access 01-12-2019 | IgA Vasculitis | Review

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

Authors: Giada Maria Di Pietro, Massimo Luca Castellazzi, Antonio Mastrangelo, Giovanni Montini, Paola Marchisio, Claudia Tagliabue

Published in: Pediatric Rheumatology | Issue 1/2019

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Abstract

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.
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Metadata
Title
Henoch-Schönlein Purpura in children: not only kidney but also lung
Authors
Giada Maria Di Pietro
Massimo Luca Castellazzi
Antonio Mastrangelo
Giovanni Montini
Paola Marchisio
Claudia Tagliabue
Publication date
01-12-2019
Publisher
BioMed Central
Published in
Pediatric Rheumatology / Issue 1/2019
Electronic ISSN: 1546-0096
DOI
https://doi.org/10.1186/s12969-019-0381-y

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