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Hemorrhagic Shock From Spontaneous Retroperitoneal Bleed in a Patient With Dermatomyositis FREE TO VIEW

Dharmi Patel, MD; Nader Kamangar, MD
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Olive View-UCLA Medical Center, Sylmar, CA

Chest. 2013;144(4_MeetingAbstracts):330A. doi:10.1378/chest.1705311
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SESSION TITLE: Critical Care Student/Resident Case Report Posters II

SESSION TYPE: Medical Student/Resident Case Report

PRESENTED ON: Tuesday, October 29, 2013 at 01:30 PM - 02:30 PM

INTRODUCTION: Spontaneous retroperitoneal bleed is a rare complication associated with dermatomyositis, with few reported cases in the literature. We describe a case of this complication in order to raise awareness of this rare yet potentially fatal entity.

CASE PRESENTATION: A 50 year-old male with no past medical history presented with 3 weeks of arthralgias, myalgias, odynophagia, rash, and weight loss. Initial physical exam was significant for Gottron’s papules, periungual erythema, and a subtle heliotrope rash. He underwent extensive workup that was most consistent with a diagnosis of dermatomyositis. He was subsequently treated with methyprednisolone as well as intravenous immunoglubulin, however he continued to complain of right hip pain. On hospital day number 18, the patient acutely became tachycardic and hypotensive, requiring transfer to the ICU. He was found to be in hypovolemic shock secondary to a large spontaneous retroperitoneal bleed in the right lower abdominal area with evidence of hemorrhage within the iliacus and psoas muscles (Figures 1 and 2). He required massive transfusions with over 20 units of blood products over a course of hours. Thereafter, his course was complicated by multi-organ failure culminating in PEA arrest. A thorough post-mortem analysis was done, including muscle biopsy that was consistent with severe rhabdomyolysis and foci of red cell extravasation. An extensive myositis panel was then sent to a reference lab and revealed a positive anti-Ro 52 antibody, supporting the diagnosis of dermatomyositis.

DISCUSSION: Hemorrhagic infiltration between the muscle bundles in dermatomyositis has been described as early as 1903, however only a few cases of spontaneous retroperitoneal bleed have been reported in the literature. The majority of these cases were secondary to the spontaneous rupture of an aneurysm and consisted of hemorrhage into the iliacus and psoas muscles. The underlying mechanism is still uncertain, however is presumed to be secondary to microvascular injury from a B-cell-mediated inflammatory response. Unlike the patient in our case who complained of persistent hip pain, the reported cases of spontaneous hemorrhage in dermatomyositis were noted to have abdominal pain, which led to prompt imaging and diagnosis.

CONCLUSIONS: Due to the fatal consequences, it is important for the critical care specialist to be aware of this rare complication so that the appropriate investigation and management can be promptly initiated.

Reference #1: Kinney TD and Maher MM. Dermatomyositis: a study of five cases. Am J Pathol. 1940 September; 16(5): 561-594.9.

Reference #2: Miwa Y, Mizuho M, Takahashi R, et al. Dermatomyositis complicated with hemorrhagic shock of the iliopsoas muscle on both sides and the thigh muscle. 2010;20(4): 420-422.

Reference #3: Yamagishi M, Tajima S, Suetake A, et al. Dermatomyositis with hemorrhagic myositis. Rheumatology international. 2009;29(11):1363-1366.

DISCLOSURE: The following authors have nothing to disclose: Dharmi Patel, Nader Kamangar

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