Granulomatosis with polyangiitis with head and neck symptoms as the first clinical manifestation: clinical analysis of 28 cases

Abstract

Objective:To investigate the early clinical features and diagnosis of granulomatous polyangiitis（GPA） with head and neck symptoms as the first presentation. Methods:The data of 28 patients with GPA diagnosed in the Second Hospital of Shanxi Medical University from 2014 to 2021, whose first symptoms appeared on the head and neck, were collected. All patients underwent relevant imaging examinations, laboratory tests, endoscopy, and pathological tissue biopsies. Systemic glucocorticoid or combined immunosuppressive therapy was administered and followed up for 1-5 years. Results:Two patients refused treatment and were lost to follow-up; 26 patients were discharged with improved symptoms, complaining of nasal ventilation, resolution of supraorbital swelling, reduced dyspnoea, and renal symptoms. Five patients were repeatedly admitted to the hospital due to recurrent renal involvement. Conclusion:Although GPA often begins with head and neck symptoms, it is non-specific and can easily be confused with chronic inflammatory disease, leading to misdiagnosis. If suspicious cases are identified, they should be combined with endoscopy, pathological tissue biopsy, and special laboratory tests as early as possible to shorten the time to diagnosis, and obtain early diagnosis and treatment.