An Unusual Case of Voice Hoarseness as Herald of Granulomatosis with Polyangiitis

Introduction: Granulomatosis with polyangiitis (GPA) is a necrotizing vasculitis of small and medium sized arteries. We report a case of ANCA associated vasculitis (AAV) with initial presentation as hoarseness of voice. Case Description: A 60-year-old female patient with history of hypothyroidism, and osteoporosis presented with complaints of hoarseness of voice, cough, shortness of breath, pleuritic chest pain and fatigue for one week. She reported bilateral shoulder and knee pain with onset three months ago followed by the occurrence of a raised non-pruritic erythematous rash involving upper and lower extremities. She was treated with Valacyclovir for possible herpes zoster without improvement. She developed worsening shoulder pain, fever and hoarseness of voice two weeks ago and had biopsy of the skin rash consistent with palisading neutrophilic dermatitis versus possible granulomatous dermatitis. On examination, joints were without synovitis and the rash has begun to fade on extremities. Labs were significant for worsening anemia (11.5->9.3 g/dL), elevated inflammatory markers (ESR 108-> 75, CRP 148.7 U/L), ANA 1:80, urinalysis with large blood, 300 mg proteinuria, and >100 RBC, Creatinine 0.69 mg/dL, LDH 311 U/L, procalcitonin 0.03, UPCR 286 mg/g. CT angiogram showed dense bilateral consolidations throughout all lobes and she was treated with antibiotics for multifocal pneumonia. Workup was sent for autoimmune and infectious etiologies. MPO antibodies were found to be positive and the possibility of AAV was considered. Patient underwent bronchoscopy with bronchoalveolar lavage which did not show diffuse alveolar hemorrhage. She underwent renal biopsy which showed focal necrotizing and crescentic glomerulonephritis, pauci immune type, consistent with GPA and received pulse dose steroids and Rituximab. She was discharged on oral Prednisone taper. Patient reported improvement in hoarseness of voice with treatment for AAV. Discussion: Laryngeal manifestations of GPA include subglottic or glottic disease. Subglottic disease presents commonly with airway symptoms while glottic disease causes voice changes by causing reduced vocal fold motion. Our patient had hoarseness of voice which improved upon treatment of GPA. Clinicians should be aware of the possibility of hoarseness of voice as a presenting diagnosis of GPA.