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Thorough Evaluation Essential in Patients with Atypical Presentations of Granulomatosis with Polyangiitis

by Linda Kossoff • October 4, 2024

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CLINICAL QUESTION

Why and how should granulomatosis with polyangiitis (GPA) be considered when assessing patients with sinonasal abnormalities?

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October 2024

BOTTOM LINE

The case study underscores the necessity of a thorough clinical and diagnostic evaluation in patients with atypical presentations of GPA to prevent severe complications.

BACKGROUND: Granulomatosis with polyangiitis is a rare autoimmune vasculitis that presents with variable, systemic manifestations. The hallmark features of GPA include necrotizing granulomatous inflammation, which can cause tissue damage and organ dysfunction. Often, the only signs of the disease are otorhinolaryngological symptoms. Therefore, the diagnosis of GPA requires a multifaceted approach.

STUDY DESIGN: Case study

SETTING: Internal Medicine, Texas Tech University Health Sciences Center, Odessa, Texas

SYNOPSIS: Researchers presented the case of a 68-year-old, morbidly obese woman with a significant history of chronic sinusitis, hypertension, and hyperlipidemia. After noticing a new-onset nose deformity, the woman visited an otorhinolaryngology clinic, where she was diagnosed with an acquired saddle nose deformity and referred to rheumatology for further evaluation. The woman reported no symptoms that might suggest systemic involvement and she denied any drug use. A CT scan ruled out sinus involvement, but laboratory tests revealed positive antineutrophil cytoplasmic antibodies (ANCAs). A biopsy of the nasal septal cartilage revealed necrotizing chronic inflammation, focal vasculitis, and fibrotic scarring—all findings consistent with GPA, even though the more common systemic symptoms, such as renal or pulmonary involvement, were absent. The patient was started on a treatment regimen of methotrexate and folic acid. 

CITATION: Enabi J, Sharif W, Mannem M, et al. Granulomatosis with polyangiitis (GPA): isolated nasal bridge involvement. Cureus. 2024;16:e62911. doi: 10.7759/cureus.62911.

Filed Under: Literature Reviews, Practice Focus, Rhinology, Rhinology Tagged With: GPA, granulomatosis with polyangiitisIssue: October 2024

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