Granulomatosis with polyangiitis presenting with unilateral facial nerve palsy and nasal septum perforation

Abstract

Granulomatosis with polyangiitis (GPA) is a necrotising vasculitis of unknown cause that has several systemic manifestations. The disease is characterised by the classical triad involving acute inflammation of the upper and lower respiratory tracts with renal involvement. However, the disease pathology can involve the central nervous system. This case report presents a case of GPA with facial nerve palsy as the first manifestation of the disease, which has been rarely reported in the medical literature.

An atypical presentation of granulomatosis with polyangiitis: A case report

“…There are cases reported with episcleritis and conjunctivitis as the initial clinical features of GPA [16 , 17] . A case of septal perforation associated with unilateral facial nerve palsy has also been reported [18] . This case is a rare presentation of GPA where a patient has been mistakenly diagnosed with sinonasal polyposis due to sinonasal disease seen on CT scans and overlapping clinical features.…”

A Case Report of Pseudomonas Infection in a Patient With Nasal Septum Perforation, Cocaine Use Disorder, and a Perinuclear Anti-neutrophil Cytoplasmic Antibody (p-ANCA)-Positive Assay

“…ANCA-positive vasculitides like granulomatosis with polyangiitis are systemic diseases that affect multiple organs within the body. Granulomatosis with polyangiitis often presents initially with ear-nose-throat involvement (e.g., rhinitis, sinusitis, mastoiditis, saddle nose deformity, epistaxis) and can also include lung involvement (e.g., pulmonary nodules, pleural effusion) causing cough, dyspnea, wheezing, and hemoptysis [ 7 ]. A study by Cannady et al reported NSP in 33% of their patients with granulomatosis with polyangiitis, commonly presenting as part of the initial symptomology [ 8 ].…”

“…Renal involvement is not a common primary presentation; however, 77-85% of patients ultimately develop kidney disease with signs including proteinuria and red blood cell casts. This can rapidly evolve to end-stage renal disease [ 7 ].…”

Granulomatosis with polyangiitis presenting headache: A case report and review of literature

“…[ 1 ] Neurological manifestations are present in 20% to 50% of GPA cases, with central nervous system involvement in 10% of patients. [ 2 , 3 ] In hypertrophic dura meningitis, GPA with headache is typical. [ 4 ] In this case, cerebral magnetic resonance (MR) enhancement revealed no meningeal thickening, to our knowledge, this manifestation had not been found previously.…”