Research & Literature

A case report of idiopathic systemic capillary leak syndrome that occurred during the postoperative period of abdominoperineal resection for colorectal cancer.

Ozawa T, Yamaguchi H, Kiyomatsu T, et al.

International surgery 2015; (100(1)):58-62 doi:10.9738/INTSURG-D-13-00206.1.

[Idiopathic and secondary capillary leak syndromes: A systematic review of the literature].

Duron L, Delestre F, Amoura Z, Arnaud L

La Revue de medecine interne 2015; (36(6)):386-94.

Clinical and pathological findings of a fatal systemic capillary leak syndrome (Clarkson disease): a case report.

Zancanaro A, Serafini F, Fantin G, et al.

Medicine 2015; (94(9)):e591 doi:10.1097/MD.0000000000000591.

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.

Hirosaki Y, Hayashidani S, Ouchi S, et al.

Journal of medical case reports 2015; (9()):90 doi:10.1186/s13256-015-0544-5.

Systemic capillary leak syndrome and autoimmune diseases: A case series.

Guffroy A, Dervieux B, Gravier S, et al.

Seminars in arthritis and rheumatism 2017; (46(4)):509-512 doi:10.1016/j.semarthrit.2016.08.001.

Compartment Syndrome as a Result of Systemic Capillary Leak Syndrome.

Kyeremanteng K, D'Egidio G, Wan C, et al.

Case reports in critical care 2016; (2016()):4206397 doi:10.1155/2016/4206397.

Idiopathic systemic capillary leak syndrome (Clarkson disease).

Druey KM, Parikh SM

The Journal of allergy and clinical immunology 2017; (140(3)):663-670 doi:10.1016/j.jaci.2016.10.042.

[Angioedema and the role of bradykinins: new treatments and implications in patients with heart failure].

Mansi M, Wu MA, Zanichelli A, Cicardi M

Giornale italiano di cardiologia (2006) 2016; (17(12)):966-972 doi:10.1714/2612.26889.

Capillary leak syndrome: etiologies, pathophysiology, and management.

Siddall E, Khatri M, Radhakrishnan J

Kidney international 2017; (92(1)):37-46 doi:10.1016/j.kint.2016.11.029.

Intravenous Immunoglobulins Improve Survival in Monoclonal Gammopathy-Associated Systemic Capillary-Leak Syndrome.

Pineton de Chambrun M, Gousseff M, Mauhin W, et al.

The American journal of medicine 2017; (130(10)):1219.e19-1219.e27 doi:10.1016/j.amjmed.2017.05.023.

The Clinical Picture of Severe Systemic Capillary-Leak Syndrome Episodes Requiring ICU Admission.

Pineton de Chambrun M, Luyt CE, Beloncle F, et al.

Critical care medicine 2017; (45(7)):1216-1223 doi:10.1097/CCM.0000000000002496.

Clinical Presentation, Management, and Prognostic Factors of Idiopathic Systemic Capillary Leak Syndrome: A Systematic Review.

Eo TS, Chun KJ, Hong SJ, et al.

The journal of allergy and clinical immunology. In practice 2018; (6(2)):609-618 doi:10.1016/j.jaip.2017.07.021.

Adrenomedullin surges are linked to acute episodes of the systemic capillary leak syndrome (Clarkson disease).

Xie Z, Chen WS, Yin Y, et al.

Journal of leukocyte biology 2018; (103(4)):749-759 doi:10.1002/JLB.5A0817-324R.

Recognition and management of idiopathic systemic capillary leak syndrome: an evidence-based review.

Baloch NU, Bikak M, Rehman A, Rahman O

Expert review of cardiovascular therapy 2018; (16(5)):331-340 doi:10.1080/14779072.2018.1456920.

Anesthetic Considerations for a Patient With Chronic Systemic Capillary Leak Syndrome: A Case Report.

Datta R, Panchamia JK

A&A practice 2018; (11(10)):276-278 doi:10.1213/XAA.0000000000000808.

Idiopathic Systemic Capillary Leak Syndrome.

Hajare KR, Patil P, Bansode J

Indian journal of critical care medicine : peer-reviewed, official publication of Indian Society of Critical Care Medicine 2018; (22(5)):369-371 doi:10.4103/ijccm.IJCCM_464_17.

Idiopathic systemic capillary leak syndrome (Clarkson syndrome) in childhood: systematic literature review.

Bozzini MA, Milani GP, Bianchetti MG, et al.

European journal of pediatrics 2018; (177(8)):1149-1154 doi:10.1007/s00431-018-3189-8.

Chronic systemic capillary leak syndrome treatment with intravenous immune globulin: Case report and review of the literature
.

Mullane R, Langewisch E, Florescu M, Plumb T

Clinical nephrology 2019; (91(1)):59-63 doi:10.5414/CN109467.

Evaluation and Management of Acute Compartment Syndrome in the Emergency Department.

Long B, Koyfman A, Gottlieb M

The Journal of emergency medicine 2019; (56(4)):386-397 doi:10.1016/j.jemermed.2018.12.021.

Handling shock in idiopathic systemic capillary leak syndrome (Clarkson's disease): less is more.

Wu MA, Colombo R, Podda GM, Cicardi M

Internal and emergency medicine 2019; (14(5)):723-730 doi:10.1007/s11739-019-02113-4.

Chronic idiopathic systemic capillary leak syndrome: a case report.

Alkhunaizi AM, Kabbani AH, ElTigani MA

Allergy, asthma, and clinical immunology : official journal of the Canadian Society of Allergy and Clinical Immunology 2019; (15()):34 doi:10.1186/s13223-019-0347-0.

Non-occlusive Mesenteric Ischaemia and Acute Kidney Injury: A Case Report of Severe Idiopathic Systemic Capillary Leak Syndrome.

Correia CP, Guiomar V, Coelho F, Almeida J

European journal of case reports in internal medicine 2019; (6(7)):001156 doi:10.12890/2019_001156.

Plasmodium vivax Malaria Presenting with Acute Systemic Capillary Leak Syndrome.

Das BK, Agasti N, Singh YKS, Midya A

The Journal of the Association of Physicians of India 2019; (67(10)):86-87.

Hyperviscosity-related splenic infarction, gastrointestinal ischaemia-reperfusion injury and transient dysarthria in a patient with distributive shock due to idiopathic systemic capillary leak syndrome (Clarkson's syndrome).

van Erven JA, Schrama J, Castelijn DAR

BMJ case reports 2020; (13(1)) doi:10.1136/bcr-2019-232500.

Multiple limb compartment syndrome as a manifestation of capillary leak syndrome secondary to metformin and dipeptidyl peptidase IV inhibitor overdose: A case report.

Kasugai D, Tajima K, Jingushi N, et al.

Medicine 2020; (99(29)):e21202 doi:10.1097/MD.0000000000021202.

Idiopathic systemic capillary leak syndrome (Clarkson's disease) presenting with recurrent hypovolemic shock.

Davies K, Thomas K, Barton L, et al.

Acute medicine 2021; (20(1)):74-77.

[Idiopathic systemic capillary leak syndrome: 2 cases with misleading presentation].

Bichon A, Carvelli J, Bourenne J, et al.

La Revue de medecine interne 2021; (42(9)):660-664 doi:10.1016/j.revmed.2021.03.325.

Capillary leak syndrome: State of the art in 2021.

Bichon A, Bourenne J, Gainnier M, Carvelli J

La Revue de medecine interne 2021; (42(11)):789-796 doi:10.1016/j.revmed.2021.05.012.

Systemic Capillary Leak Syndrome triggered by SARS-CoV2 infection: Case Report and Systematic Review.

Beber A, Dellai F, Abdel Jaber M, et al.

Scandinavian journal of rheumatology 2022; (51(1)):67-69 doi:10.1080/03009742.2021.1917145.

A Case of Systemic Capillary Leak Syndrome With Severe Cardiac Dysfunction After mRNA Vaccination for COVID-19.

Araki T, Morimoto R, Ito R, et al.

CJC open 2022; (4(7)):656-659 doi:10.1016/j.cjco.2022.03.008.

Systemic capillary leak syndrome requiring fasciotomy for limb compartment syndrome: A case report and literature review.

Tanabe M, Hikone M, Sugiyama K, Hamabe Y

Acute medicine & surgery 2022; (9(1)):e756 doi:10.1002/ams2.756.

A Case of Concurrent Leukocytosis and Systemic Capillary Leak Syndrome Due to Pegfilgrastim.

Gotera N, Hasan S, Shrestha P, et al.

Cureus 2022; (14(5)):e24640 doi:10.7759/cureus.24640.

Systemic leak capillary syndrome with myocardial involvement and cardiogenic shock: a case report.

Garatti L, Wu MA, Ammirati E, Sacco A

European heart journal. Case reports 2022; (6(7)):ytac262 doi:10.1093/ehjcr/ytac262.

Denileukin diftitox-induced systemic capillary leak syndrome with acute kidney injury.

Horino T, Okada D, Inotani S, et al.

CEN case reports 2023; (12(1)):63-67 doi:10.1007/s13730-022-00720-3.

Intravenous Immunoglobulins Tapering and Withdrawal in Systemic Capillary Leak Syndrome (Clarkson Disease).

Moyon Q, Pineton de Chambrun M, Gousseff M, et al.

The journal of allergy and clinical immunology. In practice 2022; (10(11)):2889-2895 doi:10.1016/j.jaip.2022.07.006.

Massive vulvar edema revealing idiopathic systemic capillary leak syndrome.

Belfeki N, Postic G, Hadji NA, et al.

Archives of gynecology and obstetrics 2022; (306(5)):1795-1797 doi:10.1007/s00404-022-06740-9.

Idiopathic systemic capillary leak syndrome, a unique complement and interferon mediated endotheliopathy syndrome: The role of the normal skin biopsy in establishing the diagnosis and elucidating pathogenetic mechanisms.

Magro CM, Mo JH, Pecker MS

Annals of diagnostic pathology 2022; (61()):152028 doi:10.1016/j.anndiagpath.2022.152028.

Management of Acute Episodes of Clarkson Disease (Monoclonal Gammopathy-Associated Systemic Capillary Leak Syndrome) With Intravenous Immunoglobulins.

Pecker MS, Hammudi M, Melchio R, et al.

Annals of internal medicine. Clinical cases 2022; (1(6)) doi:10.7326/aimcc.2022.0496.

Idiopathic systemic capillary leak syndrome: a case report.

Lee HY, Shin J, Kim SH, Hwang JH

BMC nephrology 2023; (24(1)):72 doi:10.1186/s12882-023-03122-4.

Systemic capillary leak syndrome secondary to decompression sickness.

Mathavan A, Mathavan A, Jones K, Ataya A

BMJ case reports 2023; (16(7)) doi:10.1136/bcr-2022-253045.

Chronic systemic capillary leak syndrome with lymphatic capillaries involvement and MYOF mutation: case report and literature review.

Gao D, Zhong W, Zhang W, et al.

Frontiers in genetics 2023; (14()):1282711 doi:10.3389/fgene.2023.1282711.

Renal Replacement Therapy in Idiopathic Systemic Capillary Leak Syndrome: A Case Report.

Tokutake M, Nakazawa A, Ota M

Cureus 2024; (16(2)):e53982 doi:10.7759/cureus.53982.

Intrinsic endothelial hyperresponsiveness to inflammatory mediators drives acute episodes in models of Clarkson disease.

Ablooglu AJ, Chen WS, Xie Z, et al.

The Journal of clinical investigation 2024; (134(10)).

Idiopathic Systemic Capillary Leak Syndrome: Report of a Pediatric Case.

Vasco J, Albuquerque F, Costa F, et al.

Acta medica portuguesa 2024; (37(11)):802-804 doi:10.20344/amp.21531.

[Hypotension and Generalized Edema Due to Plasma Leakage: A Case Report].

Gatto S, Di Maria A, Bonesso C, et al.

Giornale italiano di nefrologia : organo ufficiale della Societa italiana di nefrologia 2024; (41(5)) doi:10.69097/41-05-2024-07.

Systemic capillary leak syndrome.

Druey KM, Arnaud L, Parikh SM

Nature reviews. Disease primers 2024; (10(1)):86 doi:10.1038/s41572-024-00571-5.

A case of frequent of prerenal acute kidney injury attacks: importance of recognizing systemic capillary leak syndrome: a case report.

Miyazawa R, Nakamura H, Kumagai M, et al.

The Journal of international medical research 2024; (52(11)):3000605241301863 doi:10.1177/03000605241301863.

Systemic capillary leak syndrome: a nosological entity that the nephrologist must be aware of.

Basile C

Journal of nephrology 2025; (38(6)):1533-1540 doi:10.1007/s40620-025-02250-4.

Clarkson disease in critically and non-critically ill patients: insights from the Italian IRIS-CLS registry.

Colombo R, Montomoli J, Lanzi T, et al.

Internal and emergency medicine 2025; (20(4)):991-1001 doi:10.1007/s11739-025-03890-x.

Systemic capillary leak syndrome complicated by lower extremity compartment syndrome: a case report.

Ishihara A, Sagishima K, Ejima T, et al.

JA clinical reports 2025; (11(1)):31 doi:10.1186/s40981-025-00795-8.

A rare cause of shock in cases of refractory hypotension, hypoproteinaemia and haemoconcentration.

Elhassan O, Williams S, Joseph F

Clinical medicine (London, England) 2026; (26(2)):100550 doi:10.1016/j.clinme.2025.100550.

Idiopathic systemic capillary leak syndrome and related shock in PICU: an underdiagnosed disease?

Piastra M, Portaccio I, Dondi A, et al.

Journal of anesthesia, analgesia and critical care 2026; (6(1)):22 doi:10.1186/s44158-026-00337-3.

Extremity compartment syndrome: What you need to know.

Stahel PF, Burns BL, Ziran N,

The journal of trauma and acute care surgery 2026; doi:10.1097/TA.0000000000005169.