Adult respiratory distress syndrome complicating a systemic capillary leak syndrome

Bouhaja, B

doi:10.1007/bf01708975

Cited by 13 publications

(8 citation statements)

References 4 publications

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“…During attacks, plasma leaves the intravascular space, resulting in generalized edema, hypotension and hemoconcentration, requiring aggressive resuscitation. Lung involvement is usually absent during the acute phase of SCLS but often seen in the recovery phase, when fluids massively enter the vascular space, resulting in pulmonary edema [3]. In 25 out of the 29 patients reported in the literature, a monoclonal IgG of either ~ of k isotype was present in the serum without further evidence of lymphoid or plasma cell proliferation.…”

Section: Discussionmentioning

confidence: 95%

Pulmonary infections associated with systemic capillary leak syndrome attacks in a patient with hypogammaglobulinemia

et al. 1998

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Systemic capillary leak syndrome (SCLS) is a rare disorder of unknown etiology, characterized by recurrent hypovolemic shock attacks associated in most cases with a serum monoclonal immunoglobulin. Prophylactic therapy is usually disappointing and the outcome is often fatal. We report on a patient with recurrent hypovolemic shocks consistent with the diagnosis of SCLS associated with severe serum panhypogammaglobulinemia but no detectable monoclonal immunoglobulin or B cell proliferation. Attacks were often preceded by severe respiratory infections. Both infections and attacks were successfully prevented by i.v. gammaglobulin replacement. Further evaluation is needed to assess the efficacy of i. v. gammaglobulins in patients with SCLS but without hypogammaglobulinemia.

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Section: Discussionmentioning

confidence: 95%

Pulmonary infections associated with systemic capillary leak syndrome attacks in a patient with hypogammaglobulinemia

et al. 1998

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“…During this recovery phase, life-threatening pulmonary edema can develop. 1,[10][11][12] The frequency of episodes can vary widely between patients, with intervals ranging from days to years. 13 The majority of patients have a detectable monoclonal protein in the serum, although the importance of the monoclonal protein in the disease pathogenesis is unclear.…”

Section: Epidemiologymentioning

confidence: 99%

“…fluids during resuscitation, life-threatening pulmonary edema once the blood pressure stabilizes can develop. 1,[10][11][12] Noncardiogenic pulmonary edema after i.v. fluid resuscitation is also associated with the hemorrhagic fevers.…”

Section: Pulmonary Manifestationsmentioning

confidence: 99%

“…Additional pulmonary manifestations include pulmonary hemorrhage in HLH 45,94 and differentiation syndrome, 45 as well as acute respiratory distress syndrome in SCLS, HLH, OHSS, engraftment syndrome, and drug-induced capillary leak syndrome. 12,42,44,94,95 Although pleural effusions and noncardiogenic pulmonary edema can be seen in OHSS, 88,96 the most common respiratory abnormalities observed are dyspnea and mild hypoxia due to the presence of large-volume ascites that restricts diaphragmatic descent. 96 This leads to an increase in the work of breathing, as well as low lung volumes.…”

Section: Pulmonary Manifestationsmentioning

confidence: 99%

“…For these reasons, a fluid-restrictive strategy is advocated during volume resuscitation. 1,8,9,11,12,62,63 This is best achieved by administering a fluid bolus, followed by reassessment of the intravascular volume and pulmonary status before further i.v. fluid administration.…”

Section: Managementmentioning

confidence: 99%

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Capillary leak syndrome: etiologies, pathophysiology, and management

Siddall

Khatri

Radhakrishnan

2017

Kidney International

254

240

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In various human diseases, an increase in capillary permeability to proteins leads to the loss of protein-rich fluid from the intravascular to the interstitial space. Although sepsis is the disease most commonly associated with this phenomenon, many other diseases can lead to a "sepsis-like" syndrome with manifestations of diffuse pitting edema, exudative serous cavity effusions, noncardiogenic pulmonary edema, hypotension, and, in some cases, hypovolemic shock with multiple-organ failure. The term capillary leak syndrome has been used to describe this constellation of disease manifestations associated with an increased capillary permeability to proteins. Diseases other than sepsis that can result in capillary leak syndrome include the idiopathic systemic capillary leak syndrome or Clarkson's disease, engraftment syndrome, differentiation syndrome, the ovarian hyperstimulation syndrome, hemophagocytic lymphohistiocytosis, viral hemorrhagic fevers, autoimmune diseases, snakebite envenomation, and ricin poisoning. Drugs including some interleukins, some monoclonal antibodies, and gemcitabine can also cause capillary leak syndrome. Acute kidney injury is commonly seen in all of these diseases. In addition to hypotension, cytokines are likely to be important in the pathophysiology of acute kidney injury in capillary leak syndrome. Fluid management is a critical part of the treatment of capillary leak syndrome; hypovolemia and hypotension can cause organ injury, whereas capillary leakage of administered fluid can worsen organ edema leading to progressive organ injury. The purpose of this article is to discuss the diseases other than sepsis that produce capillary leak and review their collective pathophysiology and treatment.

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Pustular and Erythrodermic Psoriasis Complicated by Acute Respiratory Distress Syndrome

Sadeh

Rudikoff²,

Gordon³

et al. 1997

Arch Dermatol

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Adult respiratory distress syndrome complicating a systemic capillary leak syndrome

Cited by 13 publications

References 4 publications

Pulmonary infections associated with systemic capillary leak syndrome attacks in a patient with hypogammaglobulinemia

Pulmonary infections associated with systemic capillary leak syndrome attacks in a patient with hypogammaglobulinemia

Capillary leak syndrome: etiologies, pathophysiology, and management

Pustular and Erythrodermic Psoriasis Complicated by Acute Respiratory Distress Syndrome

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