Idiopathic systemic capillary leak syndrome: a case report
Abstract Background Idiopathic systemic capillary leak syndrome (ISCLS) is a rare disease characterized by recurrent episodes of acute life-threatening attacks of shock, hemoconcentration, and hypoalbuminemia. Increase in capillary permeability results in reversible plasma movement into the intersti...
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| Format: | Article |
| Language: | English |
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BMC
2023-03-01
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| Series: | BMC Nephrology |
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| Online Access: | https://doi.org/10.1186/s12882-023-03122-4 |
| _version_ | 1797860047837986816 |
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| author | Hyang-yun Lee Jungho Shin Su-Hyun Kim Jin Ho Hwang |
| author_facet | Hyang-yun Lee Jungho Shin Su-Hyun Kim Jin Ho Hwang |
| author_sort | Hyang-yun Lee |
| collection | DOAJ |
| description | Abstract Background Idiopathic systemic capillary leak syndrome (ISCLS) is a rare disease characterized by recurrent episodes of acute life-threatening attacks of shock, hemoconcentration, and hypoalbuminemia. Increase in capillary permeability results in reversible plasma movement into the interstitial spaces followed by appearance of related symptoms or complications, including renal failure. This condition can be potentially life-threatening; however, it is easily misdiagnosed. Case presentation A 47-year-old man with no previous medical history presented to the emergency department after experiencing general weakness and abdominal pain. He developed hypovolemic shock within 3 h of presentation and initial laboratory tests showed hemoconcentration, hypoalbuminemia and acute kidney injury. Following vigorous fluid therapy and supportive care, the patient recovered, but a similar episode recurred after 4 months without any specific trigger. Based on the combined clinical manifestations and laboratory findings of both the attacks, he was diagnosed with ISCLS. Symptomatic relief was achieved via oxygen supplementation and massive volume replacement using normal saline and the patient was prescribed bambuterol 10 mg and theophylline 400 mg once-a-day. He was discharged from the hospital on day 5 of hospitalization. Thereafter, the patient has been followed for 5 years without any symptoms or recurrence of ISCLS even in the situation of COVID-19 infection. Conclusions ISCLS is an extremely infrequent and commonly misdiagnosed disease. However, early diagnosis, treatment and prophylaxis through accumulated clinical data can prevent ISCLS recurrence and the development of related fatal complications. Therefore, clinicians need to be well aware of the variety of clinical characteristics and treatment options of this disease. |
| first_indexed | 2024-04-09T21:39:25Z |
| format | Article |
| id | doaj.art-c2953cf5cc8b4a75a552f7642a99bc3e |
| institution | Directory Open Access Journal |
| issn | 1471-2369 |
| language | English |
| last_indexed | 2024-04-09T21:39:25Z |
| publishDate | 2023-03-01 |
| publisher | BMC |
| record_format | Article |
| series | BMC Nephrology |
| spelling | doaj.art-c2953cf5cc8b4a75a552f7642a99bc3e2023-03-26T11:07:50ZengBMCBMC Nephrology1471-23692023-03-012411710.1186/s12882-023-03122-4Idiopathic systemic capillary leak syndrome: a case reportHyang-yun Lee0Jungho Shin1Su-Hyun Kim2Jin Ho Hwang3Division of Nephrology, Department of Internal Medicine, Chung-Ang University HospitalDivision of Nephrology, Department of Internal Medicine, Chung-Ang University HospitalDepartment of Internal Medicine, Chung-Ang University College of Medicine, Chung-Ang University HospitalDivision of Nephrology, Department of Internal Medicine, Chung-Ang University HospitalAbstract Background Idiopathic systemic capillary leak syndrome (ISCLS) is a rare disease characterized by recurrent episodes of acute life-threatening attacks of shock, hemoconcentration, and hypoalbuminemia. Increase in capillary permeability results in reversible plasma movement into the interstitial spaces followed by appearance of related symptoms or complications, including renal failure. This condition can be potentially life-threatening; however, it is easily misdiagnosed. Case presentation A 47-year-old man with no previous medical history presented to the emergency department after experiencing general weakness and abdominal pain. He developed hypovolemic shock within 3 h of presentation and initial laboratory tests showed hemoconcentration, hypoalbuminemia and acute kidney injury. Following vigorous fluid therapy and supportive care, the patient recovered, but a similar episode recurred after 4 months without any specific trigger. Based on the combined clinical manifestations and laboratory findings of both the attacks, he was diagnosed with ISCLS. Symptomatic relief was achieved via oxygen supplementation and massive volume replacement using normal saline and the patient was prescribed bambuterol 10 mg and theophylline 400 mg once-a-day. He was discharged from the hospital on day 5 of hospitalization. Thereafter, the patient has been followed for 5 years without any symptoms or recurrence of ISCLS even in the situation of COVID-19 infection. Conclusions ISCLS is an extremely infrequent and commonly misdiagnosed disease. However, early diagnosis, treatment and prophylaxis through accumulated clinical data can prevent ISCLS recurrence and the development of related fatal complications. Therefore, clinicians need to be well aware of the variety of clinical characteristics and treatment options of this disease.https://doi.org/10.1186/s12882-023-03122-4Acute kidney injuryCase reportHemoconcentrationHypoalbuminemiaIdiopathicSystemic capillary leak syndrome |
| spellingShingle | Hyang-yun Lee Jungho Shin Su-Hyun Kim Jin Ho Hwang Idiopathic systemic capillary leak syndrome: a case report BMC Nephrology Acute kidney injury Case report Hemoconcentration Hypoalbuminemia Idiopathic Systemic capillary leak syndrome |
| title | Idiopathic systemic capillary leak syndrome: a case report |
| title_full | Idiopathic systemic capillary leak syndrome: a case report |
| title_fullStr | Idiopathic systemic capillary leak syndrome: a case report |
| title_full_unstemmed | Idiopathic systemic capillary leak syndrome: a case report |
| title_short | Idiopathic systemic capillary leak syndrome: a case report |
| title_sort | idiopathic systemic capillary leak syndrome a case report |
| topic | Acute kidney injury Case report Hemoconcentration Hypoalbuminemia Idiopathic Systemic capillary leak syndrome |
| url | https://doi.org/10.1186/s12882-023-03122-4 |
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