Case Presentation: A 31-year-old male with a past medical history of opiate use disorder and presumed Granulomatosis with polyangiitis (GPA) on chronic prednisone was admitted for lower extremity edema, sore throat, hemoptysis, and dyspnea. He also reported a progressively worsening mouth ulcer for the past 6 months. He was found to have bilateral lower extremity DVTs and pneumonia on CT chest. He was treated with antibiotics and anticoagulation. CT neck showed large ulcerations and significant changes in the nasal cavity of the pharynx, concerning for mucormycosis. However, the infectious disease and ear, nose, and throat (ENT) specialists found mucormycosis less likely due to the lack of neurological symptoms and slow progression of nasal destruction. Instead, they considered this could be progression of the patient’s possible GPA diagnosis. Rheumatology was consulted and determined the patient did not have a confirmed diagnosis of GPA as his previous tissue biopsy results were inconclusive and he did not complete serology lab work. Despite the inconclusive diagnosis, the patient had been taking prednisone for several months before his hospitalization. Therefore, rheumatology ordered serology and urine studies, and suggested ENT complete a biopsy of the posterior oropharynx. Rheumatologic lab work was unremarkable, and the biopsy showed necroinflammatory debris, fungal spores and pseudohyphae which is consistent with Candida, and importantly no evidence of vasculitis. Exposed bone was also identified during the biopsy, raising concern for osteomyelitis and prompting a neurosurgery consult with a cervical MRI. The images showed C1 osteomyelitis. Biopsy of the C1 spine was unable to be done given its high-risk. Thus, the patient was started on a prolonged antifungal treatment course. Rheumatology concluded the patient did not have GPA due to negative serology and pathology studies and recommended tapering off chronic prednisone. He was stabilized with the antifungal treatments and later discharged.
Discussion: Granulomatosis with polyangiitis (GPA) is a rare small-vessel vasculitis that commonly affects the respiratory tract and kidneys. The first manifestation is often classic otorhinolaryngologic symptoms involving the nasal and paranasal sinuses. Diagnosis is dependent on the combination of clinical, serological, and histopathological signs. This case highlights the need for thorough investigation in confirming diagnosis of GPA.
Conclusions: GPA diagnosis involves a thorough evaluation to avoid unnecessary prolonged steroid use that can lead to immunocompromised states, hypertension, and diabetes, all of which this patient experienced contributing to his complicated hospital course. Although he exhibited clinical signs of GPA including saddle nose deformity and nasal erosions, his intranasal opioid use could also contribute to these manifestations. Intranasal opioid use itself is not typically thought to cause nasopharyngeal ulcers, however street opioids in the region are increasingly found to have xylazine, which is known to cause cutaneous ulcerations with eschar. It was suggested that our patient’s chronic intranasal opioid, with possible xylazine, use and immunocompromised state from chronic prednisone resulted in an invasive fungal infection spreading to the C1 cervical spine. Ultimately, a GPA diagnosis is dependent on serological, pathological, and clinical presentations and appropriate follow up is important for disease management.