Critical Care: Student/Resident Case Report Poster - Critical Care III |

MODS: A Rare Initial Presentation of MCTD FREE TO VIEW

Joshy Pathiparampil, MD; Bradley Schlussel, MD; Francis Christian, MD; Andrew Kessleman, MD; David Ozeri, MD
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SUNY Downstate, Brooklyn, NY

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;150(4_S):419A. doi:10.1016/j.chest.2016.08.432
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SESSION TITLE: Student/Resident Case Report Poster - Critical Care III

SESSION TYPE: Student/Resident Case Report Poster

PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM

INTRODUCTION: Mixed Connective Tissue Disease (MCTD) is described as an overlap of systemic rheumatologic conditions, specifically systemic lupus erythematosus, systemic sclerosis, polymyositis, dermatomyositis and/or rheumatoid arthritis, in combination with high serum titers of anti-U1 small nuclear anti-ribonucleoprotein (anti-RNP) antibodies. The common clinical manifestations of MCTD are Raynaud’s phenomenon, arthralgia, synovitis, esophageal dysfunction, muscle weakness, and swollen fingers; however, serious disease can cause development of pulmonary, renal, cardiovascular, gastrointestinal and central nervous system manifestations.1 We report the first case of multiple organ dysfunction syndrome (MODS) due to mixed connective tissue disease.

CASE PRESENTATION: 56-year-old woman presents with altered mental status, hypotension, and tachycardia. She was found to have profound hypoglycemia, acute kidney injury, lactic acidosis and leukocytosis. Treatment was initiated with dextrose, fluid resuscitation, stress doses of intravenous steroids, antibiotics, and was admitted to the intensive care unit. Subsequent infectious work up was negative. Computerized tomography of the chest was significant for bilateral interstitial thickening with cystic changes compatible with chronic interstitial lung disease (image 1). Cardiac work up revealed systolic dysfunction with elevated pulmonary artery systolic pressure and clear coronary arteries. Autoimmune work up revealed positive anti-nuclear antibody in a speckled pattern, high anti-RNP titer and low complement. Urine studies were remarkable for nephrotic range proteinuria. Renal biopsy was consistent with Class V lupus nephritis. Significant labs are highlighted in Table 1. Patient responded to steroids and was discharged home.

DISCUSSION: MODS is defined as the development of potentially reversible physiologic derangements involving two or more organ systems not involved in the ICU admission disorder and arising secondary to a potentially life-threatening physiologic insult. MODS is commonly associated with infectious etiology.2 Our patient presented with vasogenic shock deteriorating to MODS in the absence of an underlying infection. Workup suggested a new diagnosis of MCTD which responded to high dose steroids.3 We present the first reported case of MODS as the initial presentation of MCTD.

CONCLUSIONS: MCTD is a rare autoimmune disease that impacts multiple organ systems MCTD should be considered in cases of MODS of unclear etiology

Reference #1: Ortega-Hernandez, Oscar-Danilo, and Yehuda Shoenfeld. “Mixed Connective Tissue Disease: An Overview of Clinical Manifestations, Diagnosis and Treatment.” Best Practice & Research Clinical Rheumatology 26.1 (2012): 61-72.

Reference #2: Udwadia, Farokh. “Chapter-52 Multiple Organ Dysfunction Syndrome.” Principles of Critical Care (2014): 591-606.

Reference #3: Prakash, Udaya B.s. “Respiratory Complications In Mixed Connective Tissue Disease.” Clinics in Chest Medicine 19.4 (1998): 733-46.

DISCLOSURE: The following authors have nothing to disclose: Joshy Pathiparampil, Bradley Schlussel, Francis Christian, Andrew Kessleman, David Ozeri

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