From the Guidelines
To assess the respiratory system of a child recently diagnosed with ANCA vasculitis, it is crucial to ask about key symptoms and functional impacts, including cough, shortness of breath, chest pain, wheezing, and recent respiratory infections, as these can indicate upper airway disease, pulmonary capillaritis, or hemorrhage 1. When evaluating the respiratory system, consider the following:
- Cough: frequency, character, timing, and presence of sputum or blood
- Shortness of breath: at rest or with exertion, and impact on daily activities
- Chest pain: location, quality, and triggers
- Wheezing or stridor
- Recent respiratory infections
- Exercise tolerance and sleep disturbances due to breathing difficulties
- Voice changes
- Hemoptysis, as pulmonary hemorrhage is a serious complication of ANCA vasculitis It is also essential to review medication adherence to immunosuppressants, such as cyclophosphamide, rituximab, or corticosteroids, and document environmental exposures that might exacerbate respiratory symptoms 1. The diagnostic evaluation of patients with suspected EGPA, a type of ANCA vasculitis, should always be multidisciplinary and investigate the main disease complications, particularly heart, respiratory, skin, renal, and nervous system involvement, along with ANCA and eosinophilia 1. Given the rarity of pediatric cases and the heterogeneity of the clinical phenotype of EGPA, a thorough assessment of the respiratory system is vital to prevent permanent lung damage and life-threatening complications 1.
From the Research
Assessing the Respirology System in a Kid with ANCA Vasculitis
To assess the respirology system in a kid recently diagnosed with ANCA vasculitis, the following questions can be asked:
- What are the symptoms of respiratory involvement, such as cough, shortness of breath, or chest pain?
- Are there any signs of pulmonary hemorrhage, diffuse pulmonary infiltrates, or respiratory failure?
- What is the severity of the lung involvement, and are there any underlying conditions such as asthma or interstitial lung disease?
- Have there been any previous episodes of respiratory failure or pulmonary infections?
- What are the results of the pulmonary function tests, such as spirometry or diffusion capacity of the lungs for carbon monoxide (DLCO)?
- Are there any findings on chest imaging, such as high-resolution computed tomography (HRCT) or chest X-ray, that suggest lung involvement?
Specific Questions for Different Types of ANCA Vasculitis
- For granulomatosis with polyangiitis (GPA), questions can focus on symptoms of necrotizing granulomatous inflammation, tracheobronchial inflammation, or pulmonary capillaritis 2.
- For microscopic polyangiitis (MPA), questions can focus on symptoms of interstitial lung disease (ILD), pulmonary fibrosis, or diffuse alveolar hemorrhage 3, 2.
- For eosinophilic granulomatosis with polyangiitis (EGPA), questions can focus on symptoms of asthma, allergic rhinitis, or pulmonary eosinophilia 3, 2.
Importance of Early Detection and Treatment
Early detection of ANCA vasculitis and its respiratory involvement is crucial to prevent long-term damage and improve outcomes 4, 2. A comprehensive assessment of the respirology system can help identify the severity of the disease and guide treatment decisions, including the use of immunosuppressive agents, glucocorticoids, or other therapies 5, 6.