Unusual presentation of a mass and “cobble-stone like” changes in the bronchus of a patient with granulomatosis with polyangitis
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Ping Fan
A 44-year-old male was diagnosed with granulomatosis with polyangiitis (GPA) 1 year ago because of intermittent cough, short of breath, epistaxis, hearing loss and a positive protein-ase-3 anti-neutrophilic cytoplasmatic antibody (PR3-ANCA) test. After being treated with oral prednisone, cyclophosphamide for a year, he was hospitalized due to reappearance of cough and epistaxis. The nasopharyngoscopy showed extensive necrosis of the left nasal cavity. The biopsy of nasal cavity mucosa revealed fibrinoid necrotizing vasculitis (Figure 1A). His chest computed tomography (CT) showed multiple nodules and cavities (Figure 1B). Bronchoscopy showed diffuse edematous hypermic mucosal changes and cobble-stone like lesions (Figure 1C, 1D). A narrowed tracheobronchus could be seen and a whitish mass which moved back and forth with breathing could be observed in the bronchi (Figure 1E). Bronchoscopic biopsy was performed. The pathology showed nonspecific inflammation (Figure 1F). Bronchial lavage fluid were negative for bacterial and fungal cultures both. After being treated with 500 mg methylprednisolone intravenously for 3 days, the patient's cough and epistaxis disappeared.
(A) Pathological changes of the nasal cavity mucosa biopsy: inflammatory exudates, granulomas formation with necrosis, and fibrinoid necrosis of a small vascular walls. (B) Chest CT: multiple nodules and cavities. (C, D and E) Bronchoscopic observations: the mucosa of bronchus was edema, hyperemia (blue arrow in Figure C), narrowing of the middle lobe of the right lung (black arrow in Figure C), “cobble-stone” changes of the mucosa (blue arrow in Figure D), whitish mass in the lumen of upper lobe of the left lung (blue arrow in Figure E). (F) HE staining with transbronchoscopic biopsy of the “cobble-stone like” changes of the bronchial mucosa: chronic mucosal inflammation with necrosis, granulation formation with squamous metaplasia (X 200). CT, computed tomography; HE, hematoxylin-eosin.
Bronchoscopic examination is thought to be useful in detecting the abnormal bronchial findings of GPA.[1] The most common airway abnormalities in GPA are mucosal edema, erythema, thickening, and granularity of mucosal surface could be seen. Persistent mucosal inflammation followed by fibrosis is responsible for bronchial stenosis. GPA can also cause tracheal or bronchial mass lesions, called “inflammatory pseudotumor”. In general, “cobble-stone like” mucosa or inflammatory pseudotumor in airway indicate active disease.[2] The quick treatment response of this patient suggested that the changes of this patient was acute inflammation and could be reversed.
Funding
This article received no external funding.
Author Contributions
Not applicable.
Informed Consent
The patient has given the consent for his images and other clinical information to be reported in the journal.
Ethical Statement
Not applicable.
Conflict of Interest
All authors declare no conflict of interest.
Reference
[1] Nishiuma T, Ohnishi H, Yoshimura S, et al. A case of granulomatosis with polyangiitis (Wegener's granulomatosis) presenting with marked inflamed tracheobronchial mucosa. Case Rep Med. 2013;2013:208194.Suche in Google Scholar
[2] Polychronopoulos VS, Prakash UB, Golbin JM, et al. Airway involvement in Wegener's granulomatosis. Rheum Dis Clin North Am. 2007;33:755–775.Suche in Google Scholar
© 2023 Ping Fan et al., published by De Gruyter
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Artikel in diesem Heft
- Editorial
- The challenge of ocular inflammation in systemic vasculitis: How to address inequalities of care?
- Review
- Animal models for large vessel vasculitis – The unmet need
- Pathogenesis of anti-neutrophil cytoplasmic antibody-associated vasculitis
- Original Article
- Clinical and vascular features of stroke in Takayasu's arteritis: A 24-year retrospective study
- Development and internal validation of a model to predict long-term survival of ANCA associated vasculitis
- Case Report
- Intermittent fever and cough in a 56-year-old patient: Relapsing polychondritis and extranodal NK/T-cell lymphoma
- Images
- Unusual presentation of a mass and “cobble-stone like” changes in the bronchus of a patient with granulomatosis with polyangitis
- Letter to the Editor
- COVID-19 vaccine-associated autoimmune disorders: Comments
