Addison’s disease in antiphospholipid syndrome: a rare complication

Abstract

SummaryAddison’s disease (AD) is the most common endocrine manifestation of antiphospholipid syndrome (APS), but it remains a very rare complication of the syndrome. It is caused by adrenal venous thrombosis and consequent hemorrhagic infarction or by spontaneous (without thrombosis) adrenal hemorrhage, usually occurring after surgery or anticoagulant therapy. We present a clinical case of a 36-year-old female patient with a previous diagnosis of APS. She presented with multiple thrombotic events, including spontaneous abortions. During evaluation by the third episode of abortion, a CT imaging revealed an adrenal hematoma, but the patient was discharged without further investigation. A few weeks later, she presented in the emergency department with manifestations suggestive of adrenal insufficiency. Based on that assumption, she started therapy with glucocorticoids, with significant clinical improvement. After stabilization, additional investigation confirmed AD and excluded other etiologies; she also started mineralocorticoid replacement. This case illustrates a rare complication of APS that, if misdiagnosed, may be life threatening. A high index of suspicion is necessary for its diagnosis, and prompt treatment is crucial to reduce the morbidity and mortality potentially associated.Learning points:AD is a rare but life-threatening complication of APS.It is important to look for AD in patients with APS and a suggestive clinical scenario.APS must be excluded in patients with primary adrenal insufficiency and adrenal imaging revealing thrombosis/hemorrhage.Glucocorticoid therapy should be promptly initiated when AD is suspected.Mineralocorticoid replacement must be started when there is confirmed aldosterone deficiency.Hypertension is a common feature of APS; in patients with APS and AD, replacement therapy with glucocorticoids and mineralocorticoids may jeopardize hypertension management.

Adrenal failure and antiphospholipid syndrome

“…However, AI had probably developed gradually during the last few years because mild hyponatraemia had been recorded at previous outpatient visits. Our cases and others published in recent years 27,28 indicate that AI must be considered in all patients with APS-both those with well-known APS and those with newly diagnosed APS.…”

Addison's disease in antiphospholipid syndrome: A rare complication

“…The most common etiologies of this disease are autoimmune (80%-90% of cases) and other etiologies are drugs, congenital adrenal hyperplasia, infectious or infiltrative disorders, adrenal metastases, Adrenoleukodystrophy, and in rare cases hemorrhage or infarction caused by APS. 6,7 Numerous organs, including the adrenal glands, may be affected by APS. Antiphospholipid syndrome is an autoimmune systemic disorder that leads to arterial and venous thrombosis, pregnancy morbidity, and loss.…”

“…7 Antiphospholipid syndrome can be seen primarily or in association with other autoimmune diseases, including lupus. 7 According to the positive clinical and serological findings, the cause of APS in our patient is probably lupus, which can also explain the patient's proteinuria. To evaluate the patient for lupus nephritis as a cause of proteinuria, a kidney biopsy was considered for the patient, which was not performed due to the impossibility of discontinuing anticoagulants in the patient.…”

“…7 And if a patient with APS presenting with symptoms such as abdominal complaints, weakness, or asthenia, the patient's blood pressure, and serum sodium and potassium levels should be carefully evaluated, and additional diagnostic tests and treatment should be performed at the appropriate time for the patient. 7 On the other hand, in patients whose etiology of adrenal insufficiency is unknown, APS tests should be performed even in the absence of thrombolytic disorders. 7 Anticoagulation is still the standard treatment for APS patients.…”

“…7 On the other hand, in patients whose etiology of adrenal insufficiency is unknown, APS tests should be performed even in the absence of thrombolytic disorders. 7 Anticoagulation is still the standard treatment for APS patients. The purpose of international normalized ratio (INR) in these patients is yet debated.…”