Definitive Adrenal Insufficiency Due to Bilateral Adrenal Hemorrhage and Primary Antiphospholipid Syndrome

Caron, Philippe; Chabannier, Marie-Hélène; Cambus, Jean-Pierre; Fortenfant, Françoise; Otal, Philippe; Suc, J. M.

doi:10.1210/jcem.83.5.4833

Cited by 63 publications

(17 citation statements)

References 17 publications

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“…Recently, several reports have pointed out the relationship of primary adrenal failure with the presence of elevated aPL levels. 2,3 With the discovery of the aCL and the delineation of the antiphospholipid syndrome (APS) in the 1980s, however, it soon became clinically obvious that the association of these antibodies with hypoadrenalism was more than fortuitous, and indeed a strong 'cause and effect' relationship between the two conditions existed.…”

Section: Introductionmentioning

confidence: 99%

Adrenal involvement in the antiphospholipid syndrome

Espinosa¹,

Cervera²,

Font³

et al. 2003

Lupus

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Although Addison disease and hypoadrenalism are rare in patients with systemic lupus erythematosus (SLE), early reports of the association suggested the presence of antiphospholipid antibodies (aPL) in these patients. Data from literature reveal that adrenal failure was present in between 10 and 26% of patients with catastrophic APS and that one-third of patients presented with adrenal involvement during the course of catastrophic APS. Adrenal involvement may be the first clinical manifestation of this syndrome, whereas a few patients may have a history of Addison's disease in the past. The pathological mechanisms involved in the production of adrenal insufficiency in APS are still not clearly understood, but the hypercoagulable state in these patients supports the concept that adrenal haemorrhagic infarction may possibly be related to adrenal vein thrombosis. In the present article,we review the pathogenic mechanisms and main clinical, laboratory and treatment features of patients suffering adrenal involvement with aPL to support the idea that APS leads to the development of adrenal insufficiency.

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Section: Introductionmentioning

confidence: 99%

Adrenal involvement in the antiphospholipid syndrome

Espinosa¹,

Cervera²,

Font³

et al. 2003

Lupus

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“…The antiphospholipid syndrome is a recognized cause of adrenal failure which can, on rare occasions, be the heralding manifestation of PAPS (6,7). In such cases, CT or MRI usually reveals bilateral adrenal hemorrhage (6,8).…”

Section: Discussionmentioning

confidence: 99%

Acute Adrenal Failure Associated with Primary Antiphospholipid Syndrome and Homozygosity for MTHFR C677T

et al. 2011

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A 47-year-old woman with a recent clinical diagnosis of acute cholecystitis developed acute adrenal failure. Initial computed tomograms of the abdomen showed bilateral adrenal swelling which evolved into bilateral adrenal hemorrhage on repeat examination a few days later. Laboratory investigations revealed a previously undiagnosed primary antiphospholipid syndrome and homozygosity for the MTHFR C677T mutation with hyperhomocysteinemia. This case highlights the protean clinical manifestations of adrenal vascular accidents and the need for a thorough search for underlying prothrombotic states in this setting.

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“…Also patients suffering from the primary antiphospholipid antibody syndrome are prone to adrenal hemorrhage, which occurs usually when the patient has undergone surgery or requires anticoagulant treatment for recurrent arterial and venous thromboembolism. This syndrome is characterized by propensity to intravascular thrombosis and prolonged prothrombin time due to circulating antibodies to phospholipid; it may be primary or associated to a variety of conditions (connective tissue disorders, malignancies, after some prolonged treatments) (Caron et al 1998).…”

Section: Adrenal Hemorrhagementioning

confidence: 99%

Vascular Diseases of the Endocrine Organs: Special Considerations

Webb

2013

PanVascular Medicine

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The endocrine glands are highly vascular organs in order to allow the hormones they secrete to reach the circulation and their targets. Thus, vascular disorders, mainly hemorrhages, may occur in any gland. While a gonadal or thyroid hemorrhage will usually present with local signs and symptoms, specific enough to direct the patient to a urologist/andrologist, gynecologist, or internist/endocrinologist, and is not associated with acute hormonal deficiencies which are life threatening, a massive adrenal or pituitary vascular insult is clinically much more cryptic and may be fatal if the cortisol insufficiency which ensues is not promptly treated with high-dose IV glucocorticoids. This section will deal with these two entities.

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Definitive Adrenal Insufficiency Due to Bilateral Adrenal Hemorrhage and Primary Antiphospholipid Syndrome

Cited by 63 publications

References 17 publications

Adrenal involvement in the antiphospholipid syndrome

Adrenal involvement in the antiphospholipid syndrome

Acute Adrenal Failure Associated with Primary Antiphospholipid Syndrome and Homozygosity for MTHFR C677T

Vascular Diseases of the Endocrine Organs: Special Considerations

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