A RARE INITIAL PRESENTATION OF MIXED CONNECTIVE TISSUE DISORDER: POSTERIOR REVERSIBLE ENCEPHALOPATHY SYNDROME AND CARDIAC TAMPONADE

Abstract

SESSION TITLE: Medical Student/Resident Critical Care Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Mixed Connective Tissue Disorder (MCTD) is a rheumatic disease with an overlap syndrome characterized by features of systemic lupus erythematosus, scleroderma and polymyositis. It commonly presents as polyarthritis, Raynaud’s phenomenon, sclerodactyly, interstitial lung disease and esophageal dysmotility. We present a young woman who came in with Generalized Tonic Clonic Seizures (GTCS) in the setting of posterior reversible encephalopathy syndrome (PRES),cardiac tamponade as her initial presentation of MCTD. CASE PRESENTATION: 24-year-old African American female with delivery of twins 7 months ago, one previous miscarriage, no other medical history presented to the hospital after being found unresponsive with generalized tonic clonic seizure activity. Vitals significant for blood pressure 210/140, heart rate 97 and was intubated for airway protection. CT head was negative for intracranial bleed with negative urine toxicology. MRI brain revealed increased FLAIR signal in the pons (figure 1), cerebellar peduncles and the midbrain (figure 2) extending into the lateral aspect of thalamus suggestive of PRES. Electroencephalogram showed moderate to severe encephalopathy and CSF analysis was negative for infectious etiology. Blood work significant for BUN/creatinine of 43/2.9 (baseline creatinine 0.6). Echocardiography showed cardiac tamponade (figure 3) and she underwent pericardial window placement. The effusion was hemorrhagic and was negative for malignancy. Autoimmune work up was remarkable for ANA (1:1280, nucleolar), double-stranded DNA 1:40, low C3, C4 levels, positive Ro, RNP antibodies and lupus anticoagulant. The RNA polymerase, SCL-70, beta-2-glycoprotein, and anticardiolipin antibodies were negative. Renal biopsy revealed onion skinning suggestive of scleroderma. She was diagnosed with MCTD and was treated with pulse dose corticosteroids, hydroxychloroquine and mycophenolic acid. She was extubated on day 4 with return of baseline mental status. She required hemodialysis for short term and kidney function improved eventually. DISCUSSION: MCTD is diagnosed based on positive anti-RNP antibodies. Cardiac involvement is seen in 13-65% of the cases with pericarditis being the most common and cardiac tamponade being rare. It is likely secondary to immune complex deposition, inflammation. There has only been one other case report on MCTD presenting as PRES. Endothelial damage leading to vasculopathy along with autonomic dysfunction likely play a role. MCTD can present as either cardiac tamponade or PRES; although rare, one should consider autoimmune etiology whenever either of these conditions are encountered with multisystem involvement, as early diagnoses and treatment is crucial to prevent end-organ damage and death. CONCLUSIONS: Identify PRES and cardiac tamponade as a rare initial presentation of Mixed Connective Tissue Disorder. Discussion on clinical course and management of the same. Reference #1: Rahmanzadeh R, Rahmanzade R, Zabihiyeganeh M. Posterior reversible encephalopathy syndrome in a patient with mixed connective tissue disease: a case report. J Med Case Rep. 2016;10:145 Reference #2: Kumar, M. S., Smith, M. and Pischel, K. D. (2006), Case report and review of cardiac tamponade in mixed connective tissue disease. Arthritis & Rheumatism, 55: 826-830. doi:10.1002/art.22227 Reference #3: Langley RL, Treadwell EL. Cardiac tamponade and pericardial disorders in connective tissue diseases: case report and literature review. J Natl Med Assoc. (1994) 86:149–53 DISCLOSURES: No relevant relationships by Ayesha Azmeen, source=Web Response no disclosure on file for John Magaldi; No relevant relationships by Robert Nardino, source=Web Response No relevant relationships by ayesha shaik, source=Web Response No relevant relationships by Varshitha Thanikonda, source=Web Response