Endocrinologic Manifestations of the Antiphospholipid Syndrome

Uthman, Imad; Salti, Ibrahim; Khamashta, Munther A.

doi:10.1191/0961203306lu2318rr

Cited by 40 publications

(33 citation statements)

References 43 publications

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“…Addison's disease has been reported in only 0.4% of patients with confirmed APLS; conversely, APLS is diagnosed in !0.5% of all patients with Addison's disease. (102). Furthermore, 10-26% of APLS patients can have adrenal failure in catastrophic form.…”

Section: Primary Aimentioning

confidence: 99%

MECHANISMS IN ENDOCRINOLOGY: The spectrum of haemostatic abnormalities in glucocorticoid excess and defect

Isidori

Minnetti

Sbardella

et al. 2015

European Journal of Endocrinology

101

107

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Glucocorticoids (GCs) target several components of the integrated system that preserves vascular integrity and free blood flow. Cohort studies on Cushing's syndrome (CS) have revealed increased thromboembolism, but the pathogenesis remains unclear. Lessons from epidemiological data and post-treatment normalisation time suggest a bimodal action with a rapid and reversible effect on coagulation factors and an indirect sustained effect on the vessel wall. The redundancy of the steps that are potentially involved requires a systematic comparison of data from patients with endogenous or exogenous hypercortisolism in the context of either inflammatory or non-inflammatory disorders. A predominant alteration in the intrinsic pathway that includes a remarkable rise in factor VIII and von Willebrand factor (vWF) levels and a reduction in activated partial thromboplastin time appears in the majority of studies on endogenous CS. There may also be a rise in platelets, thromboxane B2, thrombin-antithrombin complexes and fibrinogen (FBG) levels and, above all, impaired fibrinolytic capacity. The increased activation of coagulation inhibitors seems to be compensatory in order to counteract disseminated coagulation, but there remains a net change towards an increased risk of venous thromboembolism (VTE). Conversely, GC administered in the presence of inflammation lowers vWF and FBG, but fibrinolytic activity is also reduced. As a result, the overall risk of VTE is increased in long-term users. Finally, no studies have assessed haemostatic abnormalities in patients with Addison's disease, although these may present as a consequence of bilateral adrenal haemorrhage, especially in the presence of antiphospholipid antibodies or anticoagulant treatments. The present review aimed to provide a comprehensive overview of the complex alterations produced by GCs in order to develop better screening and prevention strategies against bleeding and thrombosis.

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Section: Primary Aimentioning

confidence: 99%

MECHANISMS IN ENDOCRINOLOGY: The spectrum of haemostatic abnormalities in glucocorticoid excess and defect

Isidori

Minnetti

Sbardella

et al. 2015

European Journal of Endocrinology

101

107

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“…One of the reviews highlighted studies which described increases in the frequency of aPL antibodies in the serum of diabetic patients implying macroangiopathic complications, besides the finding of aCL IgG antibodies in the serum of first-degree relatives of diabetic patients. On the other hand, another literature review highlighted the need for further studies to clarify the relationship between aPL and diabetes mellitus, one time found no consistent data on the prevalence and significance of aPL in diabetes mellitus [33, 34]. …”

Section: Traditional Risk Factorsmentioning

confidence: 99%

Cardiovascular Risk Factors in the Antiphospholipid Syndrome

Silva

Levy

Carvalho

2014

Journal of Immunology Research

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A major cause of morbidity and mortality in the context of the antiphospholipid syndrome (APS) is the occurrence of thrombotic events. Besides the pathogenic roles of antiphospholipid antibodies (aPL), other risk factors and medical conditions, which are conditions for traditional risk of an individual without the APS, can coexist in this patient, raising their risk of developing thrombosis. Therefore, the clinical and laboratory investigation of comorbidities known to increase cardiovascular risk in patients with antiphospholipid antibody syndrome is crucial for the adoption of a more complete and effective treatment. Experimental models and clinical studies show evidence of association between APS and premature formation of atherosclerotic plaques. Atherosclerosis has major traditional risk factors: hypertension, diabetes mellitus, obesity, dyslipidemia, smoking, and sedentary lifestyle that may be implicated in vascular involvement in patients with APS. The influence of nontraditional risk factors as hyperhomocysteinemia, increased lipoprotein a, and anti-oxLDL in the development of thromboembolic events in APS patients has been studied in scientific literature. Metabolic syndrome with all its components also has been recently studied in antiphospholipid syndrome and is associated with arterial events.

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“…It carries significant hospital mortality and can have devastating consequences, particularly in patients at high thrombotic risk, such as those with antiphospholipid syndrome (APS). Adrenal hemorrhages are rare, occurring in 0.4% of patients with APS, and little literature details precipitating factors [2]. I report one such case and the role of sepsis in inducing it.…”

Section: Introductionmentioning

confidence: 99%

Bilateral adrenal hemorrhage in the background of Escherichia coli sepsis: a case report

Khwaja

2017

J Med Case Reports

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BackgroundSepsis is a syndrome of life-threatening organ dysfunction caused by a dysregulated host response to infection. It can have devastating consequences, including bilateral adrenal hemorrhage, particularly in patients at high thrombotic risk, such as those with antiphospholipid syndrome and those on long-term anticoagulation.Case presentationA 49-year-old white woman re-presented to hospital with a history suggestive of sepsis. She had a medical background of primary antiphospholipid syndrome on lifelong warfarin. Ten days prior to this presentation, she had been hospitalized following Escherichia coli bacteremia, commenced on intravenous antibiotics, and discharged 2 days later with a prescribed 5-day course of oral amoxicillin. On readmission, she had ongoing fever, myalgia, malaise, and hypotension. Investigations revealed anemia with thrombocytopenia, hyponatremia, and acute-on-chronic kidney injury. Despite treatment for urosepsis, she became tachypneic, clammy, light-headed, drowsy, and hypothermic. Computed tomography revealed bilateral adrenal hemorrhage, and biochemical examination confirmed hypoadrenalism. Following discharge, she had persistent renal and hepatic injury lasting 3 months.ConclusionsEarly identification, intensive monitoring, and aggressive support may reduce the acquired thrombotic risk and avoid potentially life-threatening outcomes of sepsis.

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Endocrinologic Manifestations of the Antiphospholipid Syndrome

Cited by 40 publications

References 43 publications

MECHANISMS IN ENDOCRINOLOGY: The spectrum of haemostatic abnormalities in glucocorticoid excess and defect

MECHANISMS IN ENDOCRINOLOGY: The spectrum of haemostatic abnormalities in glucocorticoid excess and defect

Cardiovascular Risk Factors in the Antiphospholipid Syndrome

Bilateral adrenal hemorrhage in the background of Escherichia coli sepsis: a case report

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