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Lung involvement in childhood onset granulomatosis with polyangiitis
Giovanni Filocamo1, Sofia Torreggiani2, Carlo Agostoni2
1Pediatric Rheumatology, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, via Commenda 9, 20122, Milan, Italy. giovanni.filocamo@policlinico.mi.it.
Insights
Granulomatosis with polyangiitis commonly affects children's lungs, presenting varied symptoms from mild lesions to severe alveolar hemorrhage. Early diagnosis and treatment, including immunosuppressants and supportive care, are vital for managing pediatric lung involvement.
Area of Science:
- Pediatric Rheumatology
- Pulmonology
- Immunology
Background:
- Granulomatosis with polyangiitis (GPA) is a rare ANCA-associated vasculitis in children.
- Lung involvement is frequent in pediatric GPA, manifesting at onset and during flares.
- Severity ranges from asymptomatic lesions to life-threatening diffuse alveolar hemorrhage.
Purpose of the Study:
- To summarize the key aspects of lung involvement in pediatric granulomatosis with polyangiitis.
- To highlight diagnostic challenges and therapeutic strategies for GPA in children.
Main Methods:
- Review of clinical presentations, radiologic findings, and diagnostic methods for GPA-related lung disease in pediatric patients.
- Discussion of treatment approaches, including immunosuppression, plasmapheresis, and supportive care.
Main Results:
- Common radiologic findings include nodular lesions and fixed infiltrates; interstitial disease and pulmonary embolism are less frequent.
- Pulmonary function tests often show reduced diffusion capacity for carbon monoxide and obstructive abnormalities.
- Histopathology may reveal necrotizing or granulomatous vasculitis, necessitating careful biopsy site selection guided by imaging.
Conclusions:
- Pediatric GPA lung disease requires a multidisciplinary approach for diagnosis and management.
- Prompt recognition and treatment are crucial to prevent severe complications and improve outcomes in affected children.
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.
