AN INTERESTING CASE OF GRANULOMATOSIS WITH POLYANGIITIS (WEGENER'S GRANULOMATOSIS) MASQUERADING AS A MULTISYSTEM DISORDER

Dr. Sadananth S.*, Dr. Sivasubramanian MD Dr. Rathnakumar MD

ABSTRACT

Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis, is a small-vessel, antineutrophil cytoplasmic antibody (ANCA)-associated necrotising vasculitis that classically presents with a triad of upper respiratory, pulmonary, and renal involvement. We report a 53-year-old woman with a 15-year history of type 2 diabetes mellitus, systemic hypertension, and hypothyroidism who presented with a one-week history of bilateral ear discharge, cough, and rapidly progressive breathlessness, together with oral ulcers, decreased hearing, giddiness, and headache. Examination revealed bilateral perforated tympanic membranes with otorrhoea, oral ulceration, tachypnoea, and bilateral lower motor neuron facial palsy with absent taste sensation, together with a left-sided glossopharyngeal and vagal palsy. Laboratory evaluation showed marked leucocytosis, a strikingly elevated ESR and CRP, preserved renal function, and a negative autoimmune and infective screen, while imaging revealed bilateral paranasal sinusitis, bilateral acute-on-chronic otomastoiditis, and a thick-walled cavitating right lower lobe lesion with multiple bilateral pulmonary nodules. A bronchial wash grew Aspergillus fumigatus, presumed to represent saprophytic colonisation of a necrotic cavity, while transbronchial lung biopsy showed only an organised abscess without diagnostic granulomatous vasculitis. A strongly positive cytoplasmic ANCA (cANCA, proteinase-3 pattern), interpreted together with the ENT, pulmonary, and cranial neuropathic findings, established a diagnosis of GPA. Nerve conduction studies additionally revealed a subclinical demyelinating-axonal motor polyradiculoneuropathy despite an unremarkable bedside limb examination. The patient was treated with pulse intravenous methylprednisolone followed by an oral steroid taper; systemic symptoms improved, but bilateral facial palsy and hearing loss persisted as residual deficits at follow-up. This case illustrates the value of ANCA serology and multisystem pattern recognition in securing a diagnosis of GPA even when histopathology is non-diagnostic, and highlights cranial mononeuropathy as an under-recognised, potentially irreversible manifestation of the disease.

Keywords: Granulomatosis with polyangiitis; Wegener's granulomatosis; cytoplasmic ANCA; cranial neuropathy; facial nerve palsy; pulmonary cavitation.