Lung involvement in childhood onset granulomatosis with polyangiitis

Giovanni Filocamo, Sofia Torreggiani, Carlo Agostoni, Susanna Esposito

Research output: Contribution to journalReview article

3 Citations (Scopus)

Abstract

Granulomatosis with polyangiitis is an ANCA-associated systemic vasculitis with a low incidence in the pediatric population. Lung involvement is a common manifestation in children affected by granulomatosis with polyangiitis, both at disease's onset and during flares. Its severity is variable, ranging from asymptomatic pulmonary lesions to dramatic life-threatening clinical presentations such as diffuse alveolar haemorrhage. Several radiologic findings have been described, but the most frequent abnormalities detected are nodular lesions and fixed infiltrates. Interstitial involvement, pleural disease and pulmonary embolism are less common. Histology may show necrotizing or granulomatous vasculitis of small arteries and veins of the lung, but since typical features may be patchy, the site for lung biopsy should be carefully chosen with the help of imaging techniques such as computed tomography. Bronchoalveolar lavage is helpful to confirm the diagnosis of alveolar haemorrhage. Pulmonary function tests are frequently altered, showing a reduction in the diffusion capacity for carbon monoxide, which can be associated with obstructive abnormalities related to airway stenosis. Nodular lung lesions tend to regress with immunosuppressive therapy, but lung disease may also require second line treatments such as plasmapheresis. In cases of massive diffuse alveolar haemorrhage, ventilator support is crucial in the management of the patient.

Original languageEnglish
Article number28
JournalPediatric Rheumatology
Volume15
Issue number1
DOIs
Publication statusPublished - Apr 14 2017

Fingerprint

Granulomatosis with Polyangiitis
Lung
Hemorrhage
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis
Pleural Diseases
Systemic Vasculitis
Plasmapheresis
Respiratory Function Tests
Bronchoalveolar Lavage
Mechanical Ventilators
Carbon Monoxide
Immunosuppressive Agents
Vasculitis
Pulmonary Embolism
Lung Diseases
Veins
Histology
Pathologic Constriction
Arteries
Tomography

Keywords

  • Childhood
  • Granulomatosis with polyangiitis
  • Lung
  • Pulmonary
  • Wegener granulomatosis

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Immunology and Allergy
  • Rheumatology

Cite this

Lung involvement in childhood onset granulomatosis with polyangiitis. / Filocamo, Giovanni; Torreggiani, Sofia; Agostoni, Carlo; Esposito, Susanna.

In: Pediatric Rheumatology, Vol. 15, No. 1, 28, 14.04.2017.

Research output: Contribution to journalReview article

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