title: An unusually dry story.
abstract: We present a middle-aged woman with a prior history of central nervous system (CNS) demyelinating disorder who presented with an acute onset quadriparesis and respiratory failure. The evaluation revealed distal renal tubular acidosis with hypokalemia and medullary nephrocalcinosis. Weakness persisted despite potassium correction, and ongoing evaluation confirmed recurrent CNS and long-segment spinal cord demyelination with anti-aquaporin-4 antibodies. There was no history of dry eyes or dry mouth. Anti-Sjogren's syndrome A antigen antibodies were elevated, and there was reduced salivary flow on scintigraphy. Coexistent antiphospholipid antibody syndrome with inferior vena cava thrombosis was also found on evaluation. The index patient highlights several rare manifestations of primary Sjogren's syndrome (pSS) as the presenting features and highlights the differential diagnosis of the clinical syndromes in which pSS should be considered in the Intensive Care Unit.
authors.affiliation: Department of Medicine, Jawaharlal Institute of Postgraduate Medical Education and Research, Dhanvantri Nagar, Puducherry, India.
authors.forename: Srinivas
authors.initials: S
authors.lastname: Rajagopala
authors.affiliation: Department of Medicine, Jawaharlal Institute of Postgraduate Medical Education and Research, Dhanvantri Nagar, Puducherry, India.
authors.forename: Gurukiran
authors.initials: G
authors.lastname: Danigeti
authors.affiliation: Department of Medicine, Jawaharlal Institute of Postgraduate Medical Education and Research, Dhanvantri Nagar, Puducherry, India.
authors.forename: Dharanipragada
authors.initials: D
authors.lastname: Subrahmanyan
keywords: Acute demyelinating encephalomyelitis; Sjogren's syndrome; distal renal tubular acidosis; hypokalemic paralysis; nephrocalcinosis; neuromyelitis optica