Endobronchial Involvement in Granulomatosis with Polyangiitis

Granulomatosis with Polyangiitis (GPA), previously known as Wegener's Granulomatosis, is a vasculitis associated with anti-neutrophil cytoplasmic antibodies (ANCAs). Radiologically, a wide spectrum of pulmonary manifestations can be observed.Including nodules, cavitary lesions, interstitial involvement, alveolar hemorrhage, and tracheobronchial stenosis. While pulmonary involvement is commonly observed, endobronchial involvement is relatively less frequent. Major symptoms in cases of GPA with endobronchial involvement include shortness of breath, cough, bloody sputum, and wheezing. Subglottic stenosis, frequently observed in the early stages, is a significant finding. Visualizing the airways endoscopically/bronchoscopically continues to be the primary diagnostic procedure for evaluating airway disease (1). There are very few studies investigating the significance of endobronchial involvement in GPA. It has been reported that the majority of cases of GPA with active endobronchial lesions (EBL) may have involvement of more organs and systems, primarily including the ear, nose, and throat (2). In a recent study focusing on the evaluation of EBL in GPA using fiberoptic bronchoscopy (FOB), it was found that EBL was detected in 19% of cases. In terms of frequency, mucosal inflammation, hemorrhage, ulcers, mass-like lesions, and inflammatory strictures have been reported in descending order. In this study, the authors emphasized the necessity of performing FOB in cases with respiratory symptoms to rule out alveolar hemorrhage and potential infections (3).

Wong MD et al (4) emphasized the importance of early diagnosis and treatment in cases of GPA with endobronchial involvement. They highlighted that the endobronchial involvement could be a sign of acute pulmonary capillaritis, which can lead to fatal respiratory failure due to widespread alveolar hemorrhage.