Lung involvement in childhood onset granulomatosis with polyangiitis
#MMPMID28410589
Filocamo G
; Torreggiani S
; Agostoni C
; Esposito S
Pediatr Rheumatol Online J
2017[Apr]; 15
(1
): 28
PMID28410589
show ga
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.
|Adolescent
[MESH]
|Age of Onset
[MESH]
|Airway Obstruction
[MESH]
|Biopsy
[MESH]
|Bronchoalveolar Lavage
[MESH]
|Child
[MESH]
|Granulomatosis with Polyangiitis/complications/pathology/*physiopathology/therapy
[MESH]
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