A case of granulomatosis with polyangiitis with severe pansi
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A female smoker in her late teens presented with weight loss, epistaxis, rash, and gum bleeding. Exam showed fever, otitis media, and facial nerve weakness, and further evaluation showed elevated rheumatoid factor and C-reactive protein, diffuse sinusitis on sinus CT, and CT chest with multiple nodular lesions and a left upper lobe lesion with cavitation. Biopsy of the lung lesion was consistent with a diagnosis of GPA.

Otolaryngology evaluation shortly after initial diagnosis noted a septal perforation, extensive crusts and drainage, and chronic rhinosinusitis without nasal polyps (CRSsNP). Initial sinus CTs demonstrated opacification of osteomeatal units, near-total opacification of the posterior left ethmoids and left sphenoid sinus, and mucosal thickening involving the maxillary antra. She has had regular sinus debridements throughout her disease course, multiple courses of antibiotics and steroids, and four functional endoscopic sinus surgeries (FESS) for CRSsNP and GPA. Her first FESS involved bilateral anterior and posterior ethmoidectomies, maxillary antrostomies, frontal recess dissection, and sphenoidotomies. Four years after this FESS, marked osteitis of the paranasal sinuses, with complete obliteration of the sphenoid sinus, as well as extensive osteitis of the ethmoids and maxillary sinuses with narrowing of the maxillary sinus lumen bilaterally, were noted on sinus CT. The second FESS involved a left-sided revision Draf IIB. The third was a left endoscopic frontal sinusotomy for an obstructed left frontal ostium.

Her osteitis continued to progress, with her most recent sinus CT ten years after this surgery showing diffuse and severe osteitis, bony erosion leading to the complete loss of the turbinates and nasal septum, obliteration of the ethmoid, maxillary, and sphenoid sinuses, and mucoceles in the bilateral frontal sinuses. Finally, she underwent a revision modified Lothrop for drainage of bilateral frontal mucoceles and connection of the frontal sinus outflow tract into one pathway. Though the patient has experienced numerous GPA exacerbations over her 15-year course, she is now overall doing well with last admission taking place three years ago.

This case illustrates a striking example of GPA progression and possible exacerbation of the disease by CRS and multiple surgeries.

source: https://www.sciencedirect.com/science/article/pii/S2468548820300606