Cavitating Lung Lesions with Encephalopathy in a Patient with Long Standing Sjogren's Syndrome: An Elusive Cause

A known case of primary Sjogren's syndrome with chronic kidney disease presented with respiratory symptoms and subsequent altered sensorium. Chest imaging suggested cavitating lung lesions in both the lungs. Serum c-ANCA was positive without any upper respiratory tract involvement or active urinary sediments. Treatment with appropriate anti-microbials produced no improvement in the respiratory or neurological parameters. MRI brain revealed tell-tale signs of CNS vascultis. A diagnosis of GPA was made as per European Medical Agency algorithm and the patient was started on immune-suppressants to which there was dramatic response. Her respiratory and neurological parameters started recovering steadily. However her stay at hospital was complicated by severe hospital acquired pneumonia to which she succumbed. We review the intricacies of the case, discussing the diagnostic conundrum and treatment dilemma we faced, as well as the systematic approach employed to manage the patient.