A fatal case of acute progression of generalized edema and simultaneous flash pulmonary edema in a patient with idiopathic systemic capillary leak syndrome: a case report

Abstract

IntroductionIdiopathic systemic capillary leak syndrome is a rare and fatal disease due to the unexplained episodic attacks of capillary leakage of plasma from the intravascular into the interstitial space. The attack consists of three phases, a prodromal phase, peripheral leak phase and recruitment phase. During the peripheral leak phase, generalized edema, mainly in the trunk and extremities, with hemoconcentration and hypoalbuminemia occurs, while usually the visceral organs like lungs, brain, heart and kidneys seem not to be involved. Treatment of the acute phase is supportive, focusing on adequate but not overzealous fluid resuscitation, because pulmonary edema usually occurs in the recruitment phase.Case presentationA 65-year-old Japanese woman was admitted to our hospital because of severe hypovolemic shock with metabolic acidosis and hemoconcentration and hypoalbuminemia. Although she was considered to be in the peripheral leak phase of idiopathic systemic capillary leak syndrome, which could not be diagnosed during the treatment, the generalized edema worsened further, severe flash pulmonary edema progressed rapidly after fluid resuscitation and she died. The autopsy showed generalized edema, especially alveolar pulmonary edema without endothelial apoptosis.ConclusionsBecause hypovolemic shock and fatal pulmonary edema may progress rapidly together even in the peripheral leak phase of idiopathic systemic capillary leak syndrome, we should keep in mind this rare and fatal disease and recognize the pathophysiology to treat it effectively when the patient has hypovolemia with metabolic acidosis.

Idiopathic systemic capillary leak syndrome (Clarkson disease)

“…Myocardial edema without inflammatory infiltrates was seen in three out of four biopsies reported in the literature, including a post-mortem examination in a patient in the NIH cohort who died of acute SCLS. 13, 14, 33, 56 We have obtained evidence of myocardial edema in active SCLS using cardiovascular MRI (CMRI). 56, 57 Dynamic measurement of extracellular volume (ECV) of heart tissue by CMRI imaging of gadolinium contrast agents is a novel means to detect active vascular leak in vivo.…”

Idiopathic Systemic Capillary Leak Syndrome: A Rare Cause of Refractory Shock Following Influenza B Infection

“… 3 Proteins like albumin, which has a molecular weight <200 kDa extravasate into the interstitial space during the leak phase of ISCLS and may not be effective. 11 As we did not have a definitive diagnosis during the leak phase, our patient was initially stabilized using crystalloids without colloids. According to a report in two ISCLS patients, hemodynamic stabilization can be achieved during the leak phase with 10% pentastarch infusion, due to its greater molecular weight.…”

Capillary leak syndrome presenting as refractory ascites in a patient with lymphoma: A rare case report

“…lung, heart, or intestines) which can confuse the diagnosis and end in a life-threatening situation. [5][6][7] Cancer and chemotherapy are also considered to be important and rare causes of secondary CLS. 8 For example, patients with lymphoma, such as those with diffuse large B-cell lymphoma or T-cell lymphoma, may develop CLS under cytokine therapy (e.g., interleukin (IL)-2, IL-11, interferon), bortezomib, rituximab, CAR-T therapy, and/or autologous stem cell transplantation.…”

“…lung, heart, or intestines) which can confuse the diagnosis and end in a life-threatening situation. 5 – 7 …”