Coupled Plasma Filtration Adsorption for Treatment of Capillary Leak Syndrome Superimposed to Acute Generalized Exanthematous Pustolosis: A Case Report

2020 ◽  
Vol 49 (3) ◽  
pp. 372-378 ◽  
Author(s):  
Vittorio Di Maso ◽  
Martina Cozzi ◽  
Ugo Gerini ◽  
Elisa Bedina ◽  
Elisa Olivo ◽  
...  
Keyword(s):  
Hypovolemic Shock ◽  
Supportive Therapy ◽  
Intravenous Immunoglobulins ◽  
Abrupt Onset ◽  
Capillary Leak Syndrome ◽  
Capillary Leak ◽  
Plasma Filtration ◽  
Threatening Condition ◽  
Life Threatening ◽  
Exanthematous Pustulosis

Coupled plasma filtration adsorption (CPFA) is an extracorporeal supportive therapy based on nonspecific adsorption of pro- and anti-inflammatory mediators combined with continuous renal replacement therapy. The main field of CPFA application is septic shock, and there are limited data about its efficacy in the treatment of other acute conditions characterized by a dysregulation in immune homeostasis. Capillary leak syndrome (CLS) defines a life-threatening condition sustained by hypercytokinemia and characterized by abrupt onset of increased capillary permeability leading to severe generalized edema and hypovolemic shock refractory to fluid administration. Therapy for CLS is not specific and, at present time, it consists in the use of steroids or intravenous immunoglobulins. We present the case of a 34-year-old woman who developed CLS superimposed to acute generalized exanthematous pustulosis after initiating therapy with hydroxychloroquine for undifferentiated connective tissue disease. CLS did not respond to steroids and intravenous immunoglobulins, while it was successfully treated with CPFA. This observation supports the possible role of CPFA in restoring a proper immunologic homeostasis not only in sepsis but also in other devastating conditions sustained by hypercytokinemia.

Idiopathic systemic capillary leak syndrome – An often missed diagnosis

2020 ◽  
pp. 004947552097929
Author(s):  
Remya Rajan ◽  
Nitin Kapoor ◽  
Hesarghatta S Asha ◽  
Thomas V Paul ◽  
Nihal Thomas
Keyword(s):  
Hypovolemic Shock ◽  
Capillary Leak Syndrome ◽  
Acute Episode ◽  
Capillary Leak ◽  
Systemic Capillary Leak Syndrome ◽  
Life Threatening ◽  
Missed Diagnosis

Idiopathic systemic capillary leak syndrome (ISCLS) is a potentially fatal disorder characterised by ‘attacks’ of varying intensity of hypovolemic shock in association with haemoconcentration and hypoalbuminaemia. It is a disease of exclusion, and the severity of attacks may mimic sepsis at presentation. We report a case of a lady with recurrent attacks of ISCLS with at least two life-threatening episodes, having been treated elsewhere as a case of steroid insufficiency. The diagnosis is often challenging, and treatment of an acute episode involves the judicious use of fluids and vasopressors, as required. Prophylaxis to prevent further attacks is of varied success.

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

2021 ◽  
Vol 20 (1) ◽  
pp. 74-77
Author(s):  
Kristen Davies ◽  
◽  
Kirsty Thomas ◽  
Lorna Barton ◽  
Chris Williams ◽  
...  
Keyword(s):  
Hypovolemic Shock ◽  
Intravenous Immunoglobulins ◽  
Capillary Leak Syndrome ◽  
High Dose ◽  
Capillary Leak ◽  
Systemic Capillary Leak Syndrome ◽  
Compartment Syndromes ◽  
Monthly Basis ◽  
History Of ◽  
Clarkson’S Disease

A 49-year old male with a past medical history of myocardial infarction and compartment syndromes requiring fasciotomies presented on five occasions with hypovolemic shock. We describe his admissions and presumptive diagnoses which required large volumes of intravenous fluids, admission to intensive care for vasopressors and renal replacement therapy. The presentations were always precipitated by a prodrome of fatigue and pre-syncopal episodes. On his last admission, a diagnosis of Idiopathic systemic capillary leak syndrome (ISCLS), also known as Clarkson’s Disease, was reached. He is currently receiving high dose intravenous immunoglobulins on a monthly basis.

scholarly journals Multiglandular Hormone Deficiency in a Patient with Systemic Capillary Leak Syndrome

2015 ◽  
Vol 2015 ◽  
pp. 1-6 ◽  
Author(s):  
Cornelia Then ◽  
Katrin Ritzel ◽  
Christa Seibold ◽  
Johannes F. E. Mann ◽  
Martin Reincke
Keyword(s):  
Hypovolemic Shock ◽  
Intravenous Immunoglobulins ◽  
Primary Hypothyroidism ◽  
Interstitial Space ◽  
Capillary Leak Syndrome ◽  
Hormone Deficiency ◽  
Capillary Leak ◽  
Hormone Binding ◽  
Systemic Capillary Leak Syndrome ◽  
Peripheral Edema

Systemic capillary leak syndrome (SCLS) is a rare but potentially fatal disorder characterized by a loss of fluid and proteins into the interstitial space leading to intravascular hypovolemia up to the point of hypovolemic shock. We report the case of a 64-year-old man with SCLS and multiple hormone abnormalities (primary hypothyroidism, hypoadrenalism, and hypogonadism), deficiency of hormone binding globulins, and hypogammaglobulinemia. The patient was successfully treated with intravenous immunoglobulins, theophylline, and terbutaline. Strikingly, with the dissolution of peripheral edema, hormone levels improved. To our knowledge, this is the first reported case of SCLS associated with polyglandular abnormalities.

scholarly journals Nivolumab induced systemic capillary leak syndrome as an ultra rare life-threatening phenomenon of late toxicity and intravenous immunoglobulin efficacy

Immunotherapy ◽  
2021 ◽  
Author(s):  
Ilya Polishchuk ◽  
Alexander Yakobson ◽  
Melanie Zemel ◽  
Adam A Sharb ◽  
Walid Shalata ◽  
...  
Keyword(s):  
Intravenous Immunoglobulin ◽  
Hypovolemic Shock ◽  
Late Toxicity ◽  
Immunoglobulin Therapy ◽  
Capillary Leak Syndrome ◽  
High Dose ◽  
Capillary Leak ◽  
Systemic Capillary Leak Syndrome ◽  
Intravenous Immunoglobulin Therapy ◽  
Life Threatening

Systemic capillary leak syndrome (SCLS) is a life-threatening disease. It is characterized by severe capillary hyperpermeability to proteins resulting in hemoconcentration, hypoalbuminemia and hypovolemic shock. Its treatment remains supportive, and the prognosis is generally poor. We report on a 51-year old male with melanoma treated with nivolumab for 1 year. 1 month following the completion of the treatment, the patient presented with signs of hypovolemic shock, anasarca, hemoconcentration and hypoalbuminemia. After excluding other diseases, a diagnosis of nivolumab-induced systemic capillary leak syndrome was made. A high dose of intravenous steroid therapy was promptly initiated without any significant clinical improvement. Intravenous immunoglobulin therapy was then administered with normalization of blood pressure, hemoconcentration and complete resolution of anasarca. Intravenous immunoglobulin should be considered a first-line treatment option for this rare phenomenon.

scholarly journals Continuous renal replacement therapy rescued life-threatening capillary leak syndrome in an extremely-low-birth-weight premature: a case report

2021 ◽  
Vol 47 (1) ◽  
Author(s):  
Li-Fen Yang ◽  
Jia-Chang Ding ◽  
Ling-Ping Zhu ◽  
Li-Xia Li ◽  
Meng-Qi Duan ◽  
...  
Keyword(s):  
Birth Weight ◽  
Renal Replacement Therapy ◽  
Low Birth Weight ◽  
Continuous Renal Replacement Therapy ◽  
Replacement Therapy ◽  
Therapeutic Option ◽  
Capillary Leak Syndrome ◽  
Extremely Low Birth Weight ◽  
Capillary Leak ◽  
Life Threatening

Abstract Background Capillary leak syndrome (CLS) is a rare disease characterized by profound vascular leakage and presents as a classic triad of hypotension, hypoalbuminemia and hemoconcentration. Severe CLS is mostly induced by sepsis and generally life-threatening in newborns, especially in premature infants. Continuous renal replacement therapy (CRRT) plays an important role of supportive treatment for severe CLS. Unfortunately, CRRT in preterm infants has rarely been well defined. Case presentation We report the case of a 11-day-old girl with CLS caused by sepsis, who was delivered by spontaneous vaginal delivery (SVD) at gestational age of 25 weeks and 4 days, and a birth weight of 0.89 Kilograms(kg). The infant received powerful management consisting of united antibiotics, mechanical ventilation, intravenous albumin and hydroxyethyl starch infusion, vasoactive agents, small doses of glucocorticoids and other supportive treatments. However, the condition rapidly worsened with systemic edema, hypotension, pulmonary exudation, hypoxemia and anuria in about 40 h. Finally, we made great efforts to perform CRRT for her. Fortunately, the condition improved after 82 h’ CRRT, and the newborn was rescued and gradually recovered. Conclusion CRRT is an effective rescue therapeutic option for severe CLS and can be successfully applied even in extremely-low-birth-weight premature.

scholarly journals A case report of capillary leak syndrome with recurrent pericardial and pleural effusions

2020 ◽  
Vol 4 (2) ◽  
pp. 1-5
Author(s):  
Habib R Khan ◽  
Saima Khan ◽  
Asha Srikanth ◽  
William H T Smith
Keyword(s):  
Treatment Options ◽  
Correct Diagnosis ◽  
Disease Process ◽  
Capillary Leak Syndrome ◽  
Limiting Factor ◽  
Capillary Leak ◽  
Pleural Effusions ◽  
Pericardial Effusions ◽  
Life Threatening ◽  
High Degree

Abstract Background Capillary leak syndrome (CLS) is a rare connective tissue disease, triggered by the leak of serous fluid into the interstitial spaces, characterized by a hallmark of oedema and effusions in confined spaces. The limiting factor in CLS management appears to be its diagnosis rather than treatment, which is usually to contain the disease progression rather than a cure. Case summary We report a case of a 51-year-old woman with recurrent life-threatening presentations of pericardial effusions, pleural effusions, and generalized swelling of face and extremities. The only notable past medical history was of Type 1 diabetes. Numerous investigations did not lead to specific disease accounting for pericardial effusions and pleural effusions. Eventually, the diagnosis of CLS was made based on hypovolaemic shock, hypoalbuminaemia, and haemoconcentration without the presence of albuminuria. She was managed with steroids to reduce system inflammation and later with immunoglobulins and tumour necrosis factor to contain the disease process. Since her diagnosis and subsequent appropriate management, she has not had further admissions with cardiac tamponade 16 months of follow-up. Discussion The diagnosis of CLS is difficult to make unless there is a high degree of suspicion and until other causes have been ruled out. It remains a challenging condition to manage as the treatment options are limited and patients recurrently present with emergencies until the correct diagnosis is made and the optimal treatment is provided.

scholarly journals It’s never too late- spontaneous rupture of spleen and life-threatening hypovolemic shock in a patient recuperating from legionnaire’s disease

2021 ◽  
Vol 7 (2) ◽  
Keyword(s):  
Splenic Rupture ◽  
Hypovolemic Shock ◽  
Traumatic Rupture ◽  
Quadrant Pain ◽  
Legionella Pneumonia ◽  
Threatening Condition ◽  
Life Threatening ◽  
Legionnaires Disease ◽  
Low Threshold ◽  
Rupture Of The Spleen

Spontaneous non-traumatic rupture of the spleen in the setting of Legionnaires’ disease is very uncommon but a life-threatening condition. The splenic rupture can present within a few days after symptom onset with significant hypotension with drop in haemoglobin along with left side upper quadrant pain. Most of the cases described in the previous literature have presented within 0-11 (mean 4) days of the pneumonia but this case we are reporting presented after 3 weeks after being treated with Legionella pneumonia. The case also highlights an atypical presentation and emphasises the need to maintain a low threshold for diagnosis especially in resource constrained setting so that patient can be transferred at the earliest to a centre where appropriate corrective measures including surgery can be safely undertaken. Keywords: splenic rupture, pneumonia, hypovolemic shock

Sign in / Sign up

Export Citation Format

Share Document