Lung involvement in childhood onset granulomatosis with polyangiitis

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.

Lung biopsy in the diagnosis of pediatric ANCA‐associated vasculitis

“…8 Typical lung histology described in GPA include signs of vasculitis, pulmonary capillaritis, fibrinoid necrosis and granulomatous inflammation. 11 Our findings were consistent with these reports. In MPA, lung histology is less well described, but vasculitis and pulmonary capillaritis are typical, especially in the context of DAH.…”

Coughing up clues: 16-year-old girl with acute haemoptysis

“…However, recent data from adult studies suggest that rituximab may be an effective alternative therapy for the use of cyclophosphamide. This is usually followed by a remission-maintenance phase with tapering doses of corticosteroids combined with other agents over a period of months to years in order to reduce the chances of disease flares 18. In severe cases of pulmonary haemorrhage, plasma exchange is also used in addition to standard induction treatment 19.…”

“…In severe cases of pulmonary haemorrhage, plasma exchange is also used in addition to standard induction treatment 19. In the case of our patient, who was a 16-year-old girl, rituximab was selected as an induction agent instead of cyclophosphamide because its efficacy in achieving remission induction is non-inferior, and it does not have the same deleterious infertility side effect profile 18…”

Onset of granulomatosis with polyangiitis obscured by heart disease in an elderly man

“…The appearance of a nodule in a few days is frequent in this pathology––it represents the “granuloma,” that is the extra vasal inflammation and necrosis, typical manifestation of GPA, and the most common pulmonary radiographic and CT finding. In fact, in adults the most common radiological appearance is represented by multiple nodules, randomly distributed throughout the lung [3]––they can range from few millimeters to more than 10 cm in maximum diameter and become cavitated. The cavities usually have thickened walls and are characterized by an irregular inner margin and the absence of calcifications [17].…”

“…There is no sex predilection––males and females are equally involved. The typical age of onset is between 40 and 60 years and the average age of 45 years [1,2], is rare in children (3.3%-7%) [3] and elderly and it's most frequently reported in men of 45-65 years of age [4].…”