PMC6010668_01
a and b: Four weeks after therapy with a rituximab and a prednisone regime.
gr1.jpg
PMC6010668_01_gr1.jpg
fig1
This revealed normal lung volumes, unaffected diffusion capacity and gas exchange and no flow-volume-curve-limitation (Fig. 1a). A correlation to the flow-volume-curve during the maximal burden revealed reduced inspiratory und expiratory flows with a typical oval-shaped curve (Fig. 1b).
CT-scan.
gr2.jpg
PMC6010668_01_gr2.jpg
fig2
a and b: Bronchoscopy showing subglottic stenosis.
gr3.jpg
PMC6010668_01_gr3.jpg
fig3
To exclude malignancy, a bronchoscopy was performed revealing a subglottic stenosis (Fig. 3a).
Subglottic mucosal biopsy with findings consistent with granulomatosis with polyangiitis: Basophilic necrosis (A, HE original magnification 100x) containing abundant nuclear debris (upper part of image B, HE original magnification 400x). The necrosis is bordered by epitheloid histiocytes with the presence of a characteristic giant cell with hyperchromatic, smudged nuclei (arrow). Two small vessels without evidence of vasculitis (lower part of image B). The morphology of the necrosis and the granulomatous inflammation is consistent with granulomatosis with polyangiitis, though a vasculitis was not evident in this biopsy.
gr4.jpg
PMC6010668_01_gr4.jpg
fig4
Though the few sampled small vessels had no signs of inflammation, these findings together with the clinical presentation were consistent with granulomatosis with polyangiitis (GPA) (Fig. 4).
CT scan of the paranasal sinuses.
gr5.jpg
PMC6010668_01_gr5.jpg
fig5
A CT-scan of the paranasal sinuses detected a granuloma in the right sinus maxillaris (Fig. 5).