Abstract
IntroductionDermatomyositis is a chronic inflammatory disorder characterized by muscular and dermatologic symptoms with variable internal organ involvement. This is the first report on a patient with acute dermatomyositis and fulminant systemic capillary leak syndrome.Case presentationA 69-year-old Caucasian woman with chronic dermatomyositis presented with clinical signs of severe hypovolemic shock and pronounced hemoconcentration (hematocrit, 69%). Her colloid osmotic pressure was 4.6mmHg. Following a bolus dose of prednisolone (500mg), fluid resuscitation was initiated. During volume loading, anasarca and acute respiratory distress rapidly developed. Echocardiography revealed an underfilled, hypokinetic, diastolic dysfunctional left ventricle with pericardial effusion but no signs of tamponade. Despite continued fluid resuscitation and high-dosed catecholamine therapy, the patient died from refractory shock 12 hours after intensive care unit admission. A laboratory analysis of her complement system suggested the presence of C1 inhibitor deficiency as the cause for systemic capillary leakage. The post-mortem examination revealed bilateral pleural, pericardial and peritoneal effusions as well as left ventricular hypertrophy with patchy myocardial fibrosis. Different patterns of endomysial/perimysial lymphocytic infiltrations adjacent to degenerated cardiomyocytes in her myocardium and necrotic muscle fibers in her right psoas major muscle were found in the histological examination.ConclusionsThis case report indicates that acute exacerbation of chronic dermatomyositis can result in a fulminant systemic capillary leak syndrome with intense hemoconcentration, hypovolemic shock and acute heart failure. In the presented patient, the cause for diffuse capillary leakage was most probably acquired angioedema, a condition that has been associated with both lymphoproliferative and autoimmunologic disorders.
Highlights
Dermatomyositis is a chronic inflammatory disorder characterized by muscular and dermatologic symptoms with variable internal organ involvement
This case report indicates that acute exacerbation of chronic dermatomyositis can result in a fulminant systemic capillary leak syndrome with intense hemoconcentration, hypovolemic shock and acute heart failure
The disease progresses with intermittent episodes, most acute dermatomyositis exacerbations do not result in critical illness
Summary
This case report indicates that acute exacerbation of chronic dermatomyositis can result in fulminant systemic capillary leak syndrome with intense hemoconcentration, hypovolemic shock and acute heart failure. The cause for diffuse capillary leakage was most probably acquired angioedema, a condition that has been associated with both lymphoproliferative and autoimmunologic disorders. Consent Written informed consent was obtained from the next-ofkin (husband) of the deceased patient for publication of this case report and accompanying images. Author details 1Department of Anesthesiology, Perioperative Medicine and General Intensive Care, Paracelsus Private Medical University and Salzburg General Hospital, Müllner Hauptstrasse 48, 5020 Salzburg, Austria. Author details 1Department of Anesthesiology, Perioperative Medicine and General Intensive Care, Paracelsus Private Medical University and Salzburg General Hospital, Müllner Hauptstrasse 48, 5020 Salzburg, Austria. 2Institute of Pathology, Paracelsus Private Medical University and Salzburg General Hospital, 5020 Salzburg, Austria. 3Internal Medicine I, Paracelsus Private Medical University and Salzburg General Hospital, 5020 Salzburg, Austria
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