B47 CRITICAL CARE CASE REPORTS: CARDIOVASCULAR DISEASES AND ECHOCARDIOGRAPHY / Thematic Poster

Session / Monday, May 21/9:15 AM-4:15 PM / Area K (Hall A-B2, Ground Level) - San Diego Convention Center

Cardiac Scleromyxedema Causing Cardiogenic Shock and Death

M. C. Runnstrom1, H. Li1, G. Graves2, D. C. Patel2, A. Ataya2; 1Department of Medicine,

University of Florida, Gainesville, FL, United States, 2Department of Medicine, Division of
Pulmonary, Critical Care, and Sleep Medicine, University of Florida, Gainesville, FL, United
States.

Corresponding author's email: martin.runnstrom@medicine.ufl.edu

Introduction: Scleromyxedema is a rare idiopathic disorder characterized by cutaneous eruptions

composed of mucin deposits, fibroblast proliferation and commonly monoclonal gammopathy.
This disorder is distinct from that seen with connective tissue deposits seen in myxedema from
thyroid disease. Systemic involvement can involve most organs. The disease is chronic and no
treatment has been validated to induce long lasting remission. Case report: A 68-year-old female
with a history of hypothyroidism, atrial fibrillation and known scleromyxedema, presented with
acute progressive dyspnea requiring intubation and mechanical ventilation and hypotension
requiring vasopressors. Contrasted computed tomography (CT) of the chest revealed moderate
sized bilateral pleural effusions and compressive atelectasis but no pulmonary embolism. Blood
cultures did not grow any organisms. Over the next two days, she became oliguric and AST and
ALT increased from normal to 5341 and 3550, respectively. The patient was persistently
hypotensive despite being on maximum doses of four vasopressors. Bedside transthoracic
echocardiography showed elevated right ventricular pressures and a new decrease in left
ventricular ejection fraction from recently being normal. Nebulized epoprostenol was started but
despite these interventions, the patient passed away from cardiogenic shock. Postmortem
examination showed extensive eosinophilic deposition within the interstitium of the myocardium,
kidneys and dermis. This eosinophilic deposition was Alcian blue positive and Congo red
negative, consistent with mucin. Exam further revealed cardiac biventricular hypertrophy and
dilation of bilateral atria, as well as congestive hepatopathy with centrilobular necrosis. The
lungs were heavy with diffuse alveolar damage and the main pulmonary arteries were without
thrombus. Discussion: Scleromyxedema, sometimes considered an aggressive form of papular
mucinosis, is a rare idiopathic chronic mucin deposition disorder. It usually appears in middle-
aged men and women and has a poor overall prognosis. Although no treatment has been reliably
validated, intravenous immunoglobulin and corticosteroids have induced temporary lack of
progression of the disease. The optimal treatment is unknown. Bos et al published a review of 17
cases of Autologous Stem Cell Transplantation as the ultimate treatment for scleromyxedema,
with 59 % achieving initial remission, but at a median follow up of >40 months, only 12 % were
still disease free. Our patient likely succumbed secondary to her multi-organ failure as a result of
cardiogenic shock from her mucin deposition disease, demonstrated by the extensive mucin
deposition found on postmortem examination. A rare manifestation of this disease.

This abstract is funded by: no funding

Am J Respir Crit Care Med 2018;197:A3474
Internet address: www.atsjournals.org Online Abstracts Issue