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.
BACKGROUND
Idiopathic 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.


METHODS
A 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.


CONCLUSIONS
Because 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.
Department of Internal Medicine, Japan Community Health Care Organization (JCHO) Kyushu Hospital, 1-8-1 Kishinoura Yahata-Nishiku, Kitakyushu City, Fukuoka Prefecture, 806-8501, Japan. y-hiro@intmed1.med.kyushu.u.ac.jp.
Yuri
Y
Hirosaki
Department of Internal Medicine, Japan Community Health Care Organization (JCHO) Kyushu Hospital, 1-8-1 Kishinoura Yahata-Nishiku, Kitakyushu City, Fukuoka Prefecture, 806-8501, Japan. hayashidani@yahoo.co.jp.
Shunji
S
Hayashidani
Department of Pathology, Japan Community Health Care Organization (JCHO) Kyushu Hospital, 1-8-1 Kishinoura Yahata-Nishiku, Kitakyushu City, Fukuoka Prefecture, 806-8501, Japan. montblanc_510_sayako@yahoo.co.jp.
Sayako
S
Ouchi
Department of Internal Medicine, Japan Community Health Care Organization (JCHO) Kyushu Hospital, 1-8-1 Kishinoura Yahata-Nishiku, Kitakyushu City, Fukuoka Prefecture, 806-8501, Japan. tukasa.ohshima@qkn-hosp.jp.
Tukasa
T
Ohshima
Department of Pathology, Japan Community Health Care Organization (JCHO) Kyushu Hospital, 1-8-1 Kishinoura Yahata-Nishiku, Kitakyushu City, Fukuoka Prefecture, 806-8501, Japan. ryujinakano1@gmail.com.
Ryuji
R
Nakano
Department of Internal Medicine, Japan Community Health Care Organization (JCHO) Kyushu Hospital, 1-8-1 Kishinoura Yahata-Nishiku, Kitakyushu City, Fukuoka Prefecture, 806-8501, Japan. dr.hide4118@gmail.com.
Hideo
H
Yamamoto
D000368:Aged; D019559:Capillary Leak Syndrome / Q000150:complications / Q000175:diagnosis / Q000628:therapy*; D018450:Disease Progression; D004487:Edema / Q000209:etiology*; D017809:Fatal Outcome; D005260:Female; D005440:Fluid Therapy / Q000379:methods; D006801:Humans; D011654:Pulmonary Edema / Q000209:etiology*; D012769:Shock / Q000628:therapy