“Rare case of granulomatosis with Polyangiitis, presenting with simultaneous bilateral acute central retinal artery occlusion and profound hearing loss” Journal of Association of chest Physicians,January2019;7:35-7

Granulomatosis with polyangiitis (GPA), previously known as Wegener granulomatosis, is a lethal, uncommon multisystem autoimmune disorder of unknown etiology. Its pathognomonic features include necrotizing granulomatous inflammation and pauci-immune vasculitis in small and medium-sized vessel vessels.[1] Ocular manifestations occur in almost 8% cases. Of all ocular manifestations, bilateral central retinal artery occlusion is very rare. We here report a case of GPA, presenting with acute onset vision loss due to bilateral central retinal artery occlusion (CRAO) and severe auditory impairment along with other manifestations.

Case

A 56-year-old female presented with a history of breathlessness associated with low-grade fever for past 9 months. She was evaluated in a general medicine clinic where a computerised tomography (CT) thorax [Figure 1] was performed which revealed bilateral lung masses of various sizes. A CT-guided transthoracic biopsy showed presence of necrotizing granulomatous inflammation. [Acid Fast Bacilli (AFB) direct smear negative]. Based on the radiological and pathological findings, empirical antitubercular treatment was started by the physician. Patient did not improve and reported to the chest clinic. She was admitted for further workup. Examination findings: During the course of hospital stay, she developed acute onset bilateral visual and hearing loss. Vision was hand movements in both eyes. Her brain magnetic resonance imaging (MRI) was unremarkable except for bilateral maxillary sinusitis. Dilated fundoscopy demonstrated bilateral central retinal artery occlusion [Figure 2]. Pure tone audiogram revealed bilateral mixed hearing loss. Considering all clinical and pathological features, multisystem vasculitis was suspected, and further workup was initiated. Her serum electrolytes, renal parameters, and urine analysis were normal. Her erythrocyte sedimentation rate (ESR) was 120. Further serological tests demonstrated normal antinuclear antigen (ANA) and antiphospholipid antibody titers. Her serum was positive for cytoplasmic antineutrophil cytoplasmic antibody (ANCA, with a positive titer of 1/200). A diagnosis of GPA with involvement of bilateral paranasal sinuses, lungs, retinal arteries, and mixed (sensory neural and conductive) hearing impairment was made. As seen in other cases, renal involvement was not there. Patient was then initiated on immunosuppressive therapy with injection methylprednisolone (1 mg/kg/day). Her visual acuity and hearing loss improved significantly within 5 days of the therapy, objectively assessed on audiometry and visual acuity test.