Bilateral adrenal hemorrhage revealed antiphospholipid syndrome in a male patient: benefit from comprehensive treatment

Abstract Introduction: Primary Adrenal insufficiency is an uncommon complication of antiphospholipid syndrome, with an incidence of 0.4% [2]. It is often secondary to bilateral adrenal hemorrhage. We present a case of bilateral adrenal hemorrhage in a patient with APLS on anticoagulation with rivaroxaban. Case: 46-year-old m with a history of antiphospholipid syndrome, recently transitioned from warfarin to rivaroxaban for anticoagulation, who presented to the ED after a syncopal episode following a prior episode of abdominal pain with an unremarkable work-up. He subsequently developed severe fatigue, dizziness, headaches, nausea and 15 lbs weight loss. On presentation, the patient was hypotensive(72/45 mmHg) and tachycardic. Intravenous hydration was started with minimal response. Initial laboratory testing showed serum sodium of 121mmol/L, potassium of 5.5mmol/L and random cortisol of 0.8mcg/dL. The patient was admitted to the intensive care unit where he was started on vasopressors and hydrocortisone 50 mg IV every 8 hours. A non-contrast CT of the abdomen and pelvis showed thickening of the adrenal glands with decreased attenuation. MRI of the abdomen showed hyper-intensity of the adrenal glands bilaterally (T1 images), without post-contrast enhancement suggestive of bilateral adrenal hemorrhage. His electrolytes normalized, and he was successfully discharged home on hydrocortisone and fludrocortisone replacement with outpatient follow-up. Discussion: Atraumatic bilateral adrenal hemorrhage is rare, but remains one of the most common endocrine-related complications of antiphospholipid syndrome (APLS). The venous anatomical configuration of the adrenal gland increases risk of thrombotic hemorrhagic infarction[1]. Patients with APLS are commonly anticoagulated to prevent thrombosis. The ideal anticoagulation regimen remains controversial. Only three other cases of spontaneous bilateral adrenal hemorrhage on patients with APLS using new oral anticoagulants (NOACs) were reported. The use of NOACs seem to increase the already-elevated risk of adrenal hemorrhage seen in patients with APLS. References: 1. Aldaajani, H. et al. Bilateral adrenal hemorrhage in antiphospholipid syndrome: Anticoagulation for the treatment of hemorrhage. Saudi Med J. 2018; 39(8): 829-833. 2. Cervera R, Piette JC, Font J, Khamashta MA, Shoenfeld Y, Camps MT, et al. Antiphospholipid syndrome: Clinical and immunologic manifestations and patterns of disease expression in a cohort of 1,000 patients. Arthritis Rheum. 2002;46:1019-27.

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

The Journal of Clinical Endocrinology & Metabolism ◽

10.1210/jcem.83.5.4833 ◽

1998 ◽

Vol 83 (5) ◽

pp. 1437-1439 ◽

Cited By ~ 35

Author(s):

Philippe Caron ◽

Marie-Hélène Chabannier ◽

Jean-Pierre Cambus ◽

Françoise Fortenfant ◽

Philippe Otal ◽

...

Keyword(s):

Adrenal Insufficiency ◽

Antiphospholipid Syndrome ◽

Adrenal Hemorrhage ◽

Primary Antiphospholipid Syndrome ◽

Bilateral Adrenal Hemorrhage

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Primary Adrenal Insufficiency Due to Bilateral Adrenal Hemorrhage-Adrenal Infarction in the Antiphospholipid Syndrome: Long-Term Outcome of 16 Patients

The Journal of Clinical Endocrinology & Metabolism ◽

10.1210/jc.2012-4300 ◽

2013 ◽

Vol 98 (8) ◽

pp. 3179-3189 ◽

Cited By ~ 31

Author(s):

Isolde Ramon ◽

Alexis Mathian ◽

Anne Bachelot ◽

Baptiste Hervier ◽

Julien Haroche ◽

...

Keyword(s):

Adrenal Insufficiency ◽

Acute Phase ◽

Antiphospholipid Syndrome ◽

Adrenal Hemorrhage ◽

Primary Adrenal Insufficiency ◽

Term Outcome ◽

Long Term Outcome ◽

Bilateral Adrenal Hemorrhage ◽

Adrenal Infarction

Context: Primary adrenal insufficiency due to bilateral adrenal hemorrhage-adrenal infarction is a rare and life-threatening manifestation of the antiphospholipid syndrome (APLS). Data on the long-term outcome are scarce. Objective: The aims of the present study were to analyze the long-term outcome related to APLS per se and to characterize the course of adrenal involvement. Design: We conducted a retrospective study of patients with bilateral adrenal hemorrhage-adrenal infarction secondary to APLS seen in the Department of Internal Medicine of Pitié-Salpêtrière Hospital in Paris (France) between January 1990 and July 2010. Results: Three patients died during the acute phase related to APLS manifestations. Sixteen patients (7 males; 9 females) were followed up during a median period of 3.5 years (range 0.3–28.1 years). Three episodes of recurrent thrombosis were noted. One patient died from cerebral hemorrhage 3 months after the onset of adrenal insufficiency. Repeated Synacthen tests showed complete absence of response in 8 of the 10 patients assessed; cortisol and aldosterone increased appropriately in one patient and to some extent in another one. Dehydroepiandrosterone levels and 24-hour urinary epinephrine levels remained abnormally low in all evaluated patients. Adrenal imaging performed more than 1 year after the initial event revealed completely atrophic glands in 9 of 11 patients. Conclusions: This particular subset of APLS patients who survive the acute phase has a rather favorable long-term outcome. Although adrenal dysfunction is generally irreversible, adrenocortical function may, at least partially, recover in rare cases. In this view, measurement of early morning cortisol during follow-up is indicated to detect these patients.

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Adrenal insufficiency revealing a bilateral adrenal hemorrhage-adrenal infarction related to antiphospholipid syndrome

The American Journal of Medicine ◽

10.1016/j.amjmed.2021.08.011 ◽

2021 ◽

Author(s):

Anne-Claire Billet ◽

Olivier Chevallier ◽

Maxime Samsom ◽

Jean-Michel Petit ◽

Bernard Bonnotte ◽

...

Keyword(s):

Adrenal Insufficiency ◽

Antiphospholipid Syndrome ◽

Adrenal Hemorrhage ◽

Bilateral Adrenal Hemorrhage ◽

Adrenal Infarction

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SAT-182 Bilateral Adrenal Hemorrhage with Clinically Preserved Adrenal Function Leading to the Diagnosis of Antiphospholipid Syndrome

Journal of the Endocrine Society ◽

10.1210/jendso/bvaa046.338 ◽

2020 ◽

Vol 4 (Supplement_1) ◽

Author(s):

Carolina Hurtado ◽

Sarah Sangnim Rhee Kim ◽

Shira Grock

Keyword(s):

Abdominal Pain ◽

Adrenal Insufficiency ◽

Antiphospholipid Syndrome ◽

Adrenal Hemorrhage ◽

Adrenal Function ◽

Stimulation Test ◽

Close Monitoring ◽

Acth Stimulation Test ◽

Acth Stimulation ◽

Bilateral Adrenal Hemorrhage

Abstract Background: Spontaneous bilateral adrenal hemorrhage (BAH) is a rare complication of antiphospholipid syndrome (APS), which is the most common identifiable risk factor for BAH. Although adrenal dysfunction is generally irreversible, adrenal function might be preserved or even recover in rare cases1. Clinical case: A 48 year-old man with history of hypertension and gout presented with right upper quadrant abdominal pain following trauma to his left leg. He was found to have a left lower extremity deep vein thrombosis and bilateral pulmonary emboli (PE) and was started on anticoagulation therapy. He continued to have abdominal pain and a CT abdomen revealed BAH. Three am cortisol level was 21 mcg/dL (8–25 mcg/dL), ACTH 37 pg/mL (6–59 pg/mL), aldosterone <3 ng/dL (4–31 ng/dL), renin 2.6 ng/mL/hr (0.2–1.6 ng/mL/hr), sodium 130 mmol/L (135–146 mmol/L) and potassium 4.3 mmol/L (3.6–5.3 mmol/L). Patient was hemodynamically stable and did not report symptoms of adrenal insufficiency. Hypercoagulable work-up was consistent with APS and Lupus. Despite normal cortisol levels, he was started on hydrocortisone in the setting of anticoagulation and recent hemorrhage. Given low aldosterone with slightly high renin he was also started on fludrocortisone. Six weeks after discharge, his morning cortisol was 6 mcg/dL and ACTH was elevated at 76 pg/mL which was concerning for adrenal insufficiency. However, 250 mcg IM ACTH stimulation test showed peak cortisol of 17 mcg/dL which is considered adequate. Aldosterone and renin levels normalized so fludrocortisone was discontinued. Patient subsequently self-discontinued all medications for 1 month with no symptoms of adrenal insufficiency, and later restarted hydrocortisone on his own. Repeat ACTH stimulation test showed baseline ACTH 57 pg/mL with peak cortisol of 17 mcg/dL. Patient was tapered off hydrocortisone and displayed no subsequent symptoms of adrenal insufficiency. Conclusion: This case highlights the need to consider APS in patients with spontaneous BAH. Additionally, patients with BAH may have relatively preserved adrenal function. There is limited data to guide when steroid replacement is necessary for patients without clear adrenal insufficiency. It may be reasonable to monitor these patients off hydrocortisone replacement with close monitoring. 1. Ramon I, Mathian A, Bachelot A, et al. Primary adrenal insufficiency due to bilateral adrenal hemorrhage-adrenal infarction in the antiphospholipid syndrome: long-term outcome of 16 patients. J Clin Endocrinol Metab. 2013;98(8):3179–3189.

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