Fulminant systemic capillary leak syndrome due to C1 inhibitor deficiency complicating acute dermatomyositis: a case report

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.

Peripartum heart failure caused by left ventricular diastolic dysfunction: a case report

“…Gradwohl‐Matis et al. reported that a C1 inhibitor deficiency caused an acute combined systolic and diastolic heart failure due to systemic capillary leakage . Therefore, it is possible that low levels of C1 inhibitor may have been associated with acute diastolic dysfunction in our case.…”

Angioedema‐like presentation as the presenting finding of juvenile myositis and juvenile dermatomyositis in 2 patients

“…Both hereditary angioedema (HAE) and acquired angioedema (AAE) with C1 inhibitor deficiency have previously been reported in patients with dermatomyositis 31,32 . Although the cause and effect relationship between C1 inhibitor deficiency and dermatomyositis is not clear, C1 inhibitor deficiency will result in loss of inhibition of the classical complement pathway, leading to low serum C4 levels as a result of excessive consumption, and an activated complement system can induce inflammatory myopathy by deposition of the membrane attack complex in muscle tissues 33 .…”

“…Both hereditary angioedema (HAE) and acquired angioedema (AAE) with C1 inhibitor deficiency have previously been reported in patients with dermatomyositis. 31,32 Although the cause and effect…”

Management of Post-Partum Pulmonary edema in an Intensive Care Unit (ICU) of a peripheral district hospital: A Case Report

“…In Kakogawa et al study an elevated level of serum prolactin was found, as the 16-kDa cleavage product of prolactin as well as C1 inhibitor deficiency is a major contributor to PPCM [8,10]. The deficiency of C1 inhibitor led to the onset of acute heart failure, marked by a combined dysfunction in both systolic and diastolic phases, attributed to the leakage of capillaries throughout the body [11]. However, we are unable to test serum prolactin and C1 inhibitor due to limited resources and unavailability of test in peripheral setting.…”