Abstract

Granulomatosis with polyangiitis (GPA) can involve the skull base or the Eustachian tubes. GPA is diagnosed on the basis of clinical manifestations and serological tests, although it is challenging to discriminate GPA from infectious processes driving skull base osteomyelitis (SBO) and malignant processes such as nasopharyngeal carcinoma (NPC). Moreover, current serological tests have a low sensitivity and cannot distinguish GPA from these other conditions. We hypothesized that certain MRI characteristics would differ significantly among conditions and aimed to evaluate whether the features could differentiate between GPA, SBO, and NPC involving the skull base. We retrospectively evaluated the MRI findings of patients with GPA, SBO, and NPC. We performed univariable logistic regression analyses to identify the predictive variables for differentiating between conditions and evaluated their diagnostic values. We showed, for the first time, that certain MRI findings significantly differed between patients with GPA and those with SBO or NPC, including the lesion morphology and extent, the apparent diffusion coefficient (ADC) values, the contrast enhancement patterns, the presence or absence of necrosis, and retropharyngeal lymphadenopathy. In conclusion, utilizing certain MRI features can improve the diagnostic performance of MRI by differentiating GPA with skull base involvement from other conditions with similar radiologic findings, including SBO and NPC, facilitating treatment plans and, thus, improving patient outcomes.

Highlights

  • Granulomatosis with polyangiitis (GPA), previously known as Wegener’s granulomatosis, is one of the most frequently encountered types of otolaryngological vasculitis [1,2]

  • The nasopharyngeal carcinoma (NPC) group consisted of 16 patients who were at clinical stages T3 (n = 11) or T4 (n = 5)

  • The NPC group was significantly younger than the GPA and skull base osteomyelitis (SBO) groups (p = 0.019 and < 0.001, respectively)

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Summary

Introduction

Granulomatosis with polyangiitis (GPA), previously known as Wegener’s granulomatosis, is one of the most frequently encountered types of otolaryngological vasculitis [1,2]. Otologic impairment in patients with GPA can develop when the disease involves the skull base or the Eustachian tubes, and it is often accompanied by nasopharyngeal manifestations [8]. In such cases, it can be challenging to discriminate GPA from the infectious processes driving skull base osteomyelitis (SBO) and the malignant processes involving the nasopharynx [9,10,11,12,13,14,15,16,17,18]. ANCA testing alone cannot always distinguish between GPA and the other two conditions [14,19]

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