Adrenal Insufficiency Secondary to Bilateral Adrenal Hemorrhage Associated with Antiphospholipid Syndrome

Abstract

Objective: Antiphospholipid syndrome (APS) can involve multiple organ systems but endocrine manifestations are rare. In most cases adrenal insufficiency (AI) is the first endocrine manifestation of APS. The prompt diagnosis of AI is critical as this disorder is a life-threatening disease that may lead to fatal outcomes if left untreated. We present a case of AI associated with APS the patient was diagnosed promptly and managed successfully. Methods: The diagnosis of APS was based on a combination of clinical features (deep venous thrombosis and pulmonary embolism) and laboratory findings (lupus anticoagulant, anticardiolipin antibody, anti-beta-2 glycoprotein-I antibody), without alternative diagnosis to explain the clinical findings. AI was diagnosed by low morning serum cortisol with elevated adrenocorticotropic hormone (ACTH) level as well as an ACTH stimulation test. Results: A 50-year-old male presented with deep venous thrombosis of the left extremity diagnosed by compressive ultrasound, and was subsequently diagnosed with a pulmonary embolism by computed tomography angiography and treated with heparin. Two days later, he developed hypotension and bilateral flank pain, and an abdominal computed tomography scan revealed bilateral adrenal hemorrhage. Laboratory results showed a serum cortisol of 3.3 mcg/dL (stress normal, 25 to 35 mcg/dL) and ACTH of 319 pg/mL (stress normal, 128 to 218 pg/mL), consistent with primary AI. Symptoms improved quickly with hydrocortisone therapy. The patient still required glucocorticoid therapy for at least 4 years thereafter. Conclusion: In all cases of adrenal hemorrhage and infarction with unknown etiology, screening with lupus anticoagulant and anticardiolipin antibodies is imperative. Recognition of this high-mortality condition allows for appropriate screening and confirmatory tests leading to a prompt diagnosis and timely management.

Adrenal Hemorrhage in Patients with Systemic Lupus Erythematosus and Antiphospholipid Syndrome: A Case Report and Literature Review

“…However, bilateral adrenal hemorrhage or thrombosis may lead to acute adrenal insufficiency. During our literature review, we encountered a case of bilateral adrenal hemorrhage associated with APS that was reported by Bansal R et al [ 9 ]. After carefully comparing the first adrenal CT image with the second, the evolvement of adrenal images was similar to the development of a hematoma.…”

“…We used the keywords "systemic lupus erythematosus," "antiphospholipid syndrome," "adrenal insufciency," and "adrenal hemorrhage" and searched for articles published between 1980 and 2023. In total, 22 other cases of adrenal insufciency owing to APS or SLE were identifed [3,[6][7][8][9][10][11][12][13][14][15][16][17][18][19]. However, adrenal insufciency has rarely been reported in patients with APS, and no common treatment guidelines are available.…”

Primary antiphospholipid syndrome with adrenal insufficiency

“…Antiphospholipid syndrome (APS) can be defined as a disease characterized by hypercoagulability and pregnancy complications, and linked with a group of antibodies called 'antiphospholipid antibodies that are associated with it. (1)anticardiolipin antibody, antibeta-2 glycoprotein-I antibody APS can be primary or linked with other autoimmune disorders like systemic lupus erythematosus (SLE).…”

Primary adrenal insufficiency due to bilateral adrenal hemorrhage-adrenal infarction in a patient with systemic lupus erythematosus and antiphospholipid syndrome: case presentation and review of the literature

“…APS, first described in 1983, is an autoimmune disorder characterized by hypercoagulability, potentially resulting in thrombosis of all segments of the vascular system. It is diagnosed in less than 0.5% of all patients presenting with PAI [ 19 , 20 ]. Conversely, Addison’s disease is a rare APS complication.…”