Cerebral Involvement in Systemic Capillary Leak Syndrome

Yamagami, Keiko; Miyaichi, Toshinori; Kanki, Rie

doi:10.2169/internalmedicine.6122-20

Cited by 3 publications

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“…Rhabdomyolysis from muscle edema can also lead to renal tubular injury causing AKI [26]. SCLS can also affect the neurological system by increasing vascular permeability in the brain [27][28] (Table 1).…”

Section: Introductionmentioning

confidence: 99%

Systemic Capillary Leak Syndrome (SCLS) Presentation in Patients Receiving Anti-cancer Treatments

Anipindi¹,

Kacarow²,

Bitetto³

2023

Cureus

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Systemic capillary leak syndrome (SCLS) is due to increased capillary permeability to proteins and fluid extravasation from blood vessels into surrounding tissues and body cavities. This fluid extravasation leads to hypotension, generalized anasarca, pleural effusions, and pericardial effusions --the more severe cases of SCLS can cause multiorgan dysfunction, including cardiovascular collapse, shock, and death. The treatment includes corticosteroids, diuretics, albumin, immunoglobulins, and crystalloids. SCLS is potentially fatal. Recognizing signs and symptoms early and treating the patients is essential as this condition is fatal. It sometimes is a diagnosis of exclusion, being very challenging to diagnose and treat. The lack of understanding of the underlying mechanisms causing SCLS and proper treatment guidelines, especially in cancer patients, made diagnosing and treating this condition hard. Reports show that many cancers and anti-cancer treatments, including newer immunotherapy, cause SCLS. The mortality rate of SCLS associated with cytotoxic chemotherapy is 24% at five years. This review focuses on the cancers and anti-cancer drugs causing SCLS, treating acute SCLS, and available preventive regimens. The fundamental purpose of this review is to help clinicians recognize SCLS early to avoid delays in diagnosis and treatment. We also would like to elaborate on the fact that research on cancer-related SCLS is critical for developing staging criteria, useful diagnostic markers, prevention, and treatment strategies for anti-cancer drug-induced SCLS to prevent early discontinuation of anti-cancer drugs.

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Section: Introductionmentioning

confidence: 99%

Systemic Capillary Leak Syndrome (SCLS) Presentation in Patients Receiving Anti-cancer Treatments

Anipindi¹,

Kacarow²,

Bitetto³

2023

Cureus

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Systemic capillary leak syndrome

Druey,

Arnaud,

Parikh

2024

Nat Rev Dis Primers

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The vascular endothelial barrier maintains intravascular volume and metabolic homeostasis. Although plasma fluids and proteins extravasate continuously from tissue microvasculature (capillaries, post-capillary venules), systemic vascular leakage increases in critical illness associated with sepsis, burns and trauma, among others, or in association with certain drugs or toxin exposures. Systemically dysregulated fluid homeostasis, which can lead to hypovolaemia, hypotensive shock and widespread tissue oedema, has been termed systemic capillary leak syndrome (SCLS) when overt secondary causes (for example, heart or liver failure) are excluded. In severe forms, SCLS is complicated by compartment syndrome in the extremities and multi-organ dysfunction syndrome due to shock and systemic hypoperfusion. The different forms of SCLS include idiopathic SCLS (ISCLS) and secondary SCLS (SSCLS), which can be triggered by several conditions, including certain infections and haematological malignancies. A subgroup of patients with ISCLS have monoclonal gammopathy-associated SCLS (also known as Clarkson disease), which is an ultra-rare and extreme form of ISCLS. ISCLS can be managed effectively with monthly prophylactic immunoglobulin therapy whereas SSCLS frequently does not recur once the underlying condition resolves or the offending agent is discontinued. Thus, differentiation between ISCLS, SSCLS and other causes of oedema is crucial for quick Nature Reviews Disease Primers | (2024) 10:86 2 0123456789();: Primerprophylactic therapy. A chronic form of SCLS consisting of persistent, non-paroxysmal peripheral oedema and hypoalbuminaemia has been described 6 ; however, because these patients do not experience acute flares meeting the clinical criteria for ISCLS or MG-CLS, we do not discuss this entity here.Although the precise molecular mechanisms underlying SCLS from all causes remain poorly understood, progress has been made in the past decade towards better disease recognition, prophylactic treatment and understanding of pathophysiology, particularly for MG-CLS, which include the identification of autonomous functional defects in the MG-CLS vascular endothelium 7-10 and a natural mouse model of disease 11 . In contrast to these vascular-centric mechanisms, many drugs or toxins implicated in SSCLS may exert their effects indirectly by modulating the immune system or eliciting release of pro-inflammatory cytokines 2 . Less frequently, drugs or infectious pathogens are directly toxic to endothelial cells, resulting in activation, injury, or cell death 12 and subsequent vascular leakage.Because there are no disease-specific biomarkers other than the monoclonal gammopathy present in some patients, ISCLS is diagnosed based on clinical characteristics: at least one episode in which hypotension (systolic blood pressure <100 mmHg), haemoconcentration (haemoglobin levels above the normal range for age and sex or ≥20% increase in haemoglobin or haematocrit (Hct) over the last reference value in each patient), and hypoalbuminaemia (seru...

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Ultrasound findings and specific intrinsic blood volume expansion therapy for neonatal capillary leak syndrome: report from a multicenter prospective self-control study

Liu,

Gao,

2024

Eur J Med Res

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Objective Capillary leak syndrome (CLS) is characterized by severe systemic edema without specific treatment, resulting in a high mortality rate. This study investigated whether there is organ edema in neonatal CLS patients and specific treatment strategies to improve patient prognosis. Methods Thirty-seven newborns diagnosed with CLS were included in this study. (1) Routine point-of-care ultrasound (POCUS) was used to identify whether the patients had visceral edema or fluid collection. (2) All patients were treated with 3% NaCl intravenously, and the clinical manifestations, laboratory indices and outcomes were compared before and after treatment. Results (1) Diffuse severe edema was found in 92.0% of the patients. (2) The POCUS examination revealed that CLS patients exhibited significant visceral edema in addition to diffuse severe edema, which included pulmonary edema in 67.6%, cerebral edema in 37.8%, severe intestinal edema in 24.3%, severe myocardial edema in 8.1%, pericardial effusion in 5.4%, pleural effusion in 29.7% and peritoneal effusion in 18.9%. Two patients (5.45%) had only myocardial edema without other manifestations. (3) Before and after the intravenous injection of 3% NaCl, there were no significant differences in the serum sodium or potassium levels of CLS patients, while the hemoglobin and hematocrit levels were significantly lower after treatment (p < 0.01). Her plasma ALB concentration and arterial pressure returned to normal levels after the treatment was completed. (4) All the patients survived, and no side effects or complications were observed during or after treatment with 3% NaCl. Conclusions (1) In addition to diffuse severe edema, visceral edema and effusion are common and important clinical manifestations of neonatal CLS and need to be detected by routine POCUS. (2) The intravenous injection of 3% NaCl is a safe, effective and specific treatment strategy for neonatal CLS, with a survival rate of 100% and no adverse effects.

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Cerebral Involvement in Systemic Capillary Leak Syndrome

Cited by 3 publications

References 2 publications

Systemic Capillary Leak Syndrome (SCLS) Presentation in Patients Receiving Anti-cancer Treatments

Systemic Capillary Leak Syndrome (SCLS) Presentation in Patients Receiving Anti-cancer Treatments

Systemic capillary leak syndrome

Ultrasound findings and specific intrinsic blood volume expansion therapy for neonatal capillary leak syndrome: report from a multicenter prospective self-control study

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