7112 A Case of Bilateral Adrenal Insufficiency Associated With Borderline Lupus and Antiphospholipid Syndrome

Abstract Disclosure: K.M. Hernandez Mendiola: None. Bilateral adrenal hemorrhage (BAH) is a rare condition leading to acute adrenal insufficiency with a mortality rate of 15%. Known etiologies include abdominal trauma, adrenal vein thrombosis, sepsis, shock, and anticoagulation. Systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS) are uncommon conditions associated with BAH. We describe here a 79-year-old woman with a history of hypertension and a 23-year history of borderline SLE treated with hydroxychloroquine (HCQ) who presented at the ED with epigastric pain, diarrhea, and nausea. Six months before, HCQ was discontinued. The exam showed no abnormal findings, and an abdominal CT showed inflammation of the ascending colon. Augmentin was instituted, and the patient was sent home. After a week, the patient returned to the ED due to the persistence of symptoms, complaining of fatigue and right lower quadrant abdominal pain. Upon examination, the abdomen was soft but tender, with the CT suggesting pancolitis, while an MRI showed BAH. Laboratory findings included anemia, thrombocytopenia, elevated PT, low fibrinogen, and 11 mcg/dl cortisol. The patient was then admitted to the hospital, and hydrocortisone (HCT) therapy was initiated. After 12 hours of glucocorticoid initiation, cortisol level was 37 mcg/dl. On day 1, the patient remained hemodynamically stable. On day 2, a positive lupus anticoagulant was detected, with cortisol level dropping to 2 mcg/dl, warranting the continuation of HCT. From day 3 to 6, the patient suffered worsening coagulation and kidney functions. By day 7, an abdominal CT showed an extension of BAH. On day 8, the patient experienced a cerebral intraparenchymal hemorrhage and was transferred to the ICU, where, after two days passed away. The report presented here describes a catastrophic case of BAH associated with borderline SLE and APS in a patient who previously discontinued long-term treatment with HCQ, thereby enhancing the likelihood of a lupus flare-up. The adrenal glands are susceptible to thrombosis and hemorrhage due to their rich vascularity. In stress, the adrenals receive a significant blood volume, causing vascular congestion, potentially exceeding their capacity for venous drainage, and increasing the risk of hemorrhage. Autoimmune conditions, particularly SLE and APS, have been implicated in the etiology of adrenal hemorrhage. Antiphospholipid antibodies may bind to phospholipid-binding plasma proteins, promoting thrombosis in the adrenal vein by disrupting the coagulation fibrinolysis balance and the adrenal blood outflow, ultimately causing bleeding into the adrenal gland parenchyma. Although in this case, no cardiovascular collapse was observed, when unrecognized or undertreated, adrenal insufficiency complicating BAH can evolve into an adrenal crisis. Infections and medications that increase cortisol metabolism can also contribute to this complication. Presentation: 6/3/2024

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Journal
Journal of the Endocrine Society
Published
2024-10-01
DOI
https://doi.org/10.1210/jendso/bvae163.059
Primary Topic
Adrenal Hormones and Disorders
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article
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article

7112 A Case of Bilateral Adrenal Insufficiency Associated With Borderline Lupus and Antiphospholipid Syndrome

Karla Maria Hernandez Mendiola
Journal of the Endocrine Society
Adrenal Hormones and Disorders
article

7112 A Case of Bilateral Adrenal Insufficiency Associated With Borderline Lupus and Antiphospholipid Syndrome

Karla Maria Hernandez Mendiola
article en

Abstract

Abstract Disclosure: K.M. Hernandez Mendiola: None. Bilateral adrenal hemorrhage (BAH) is a rare condition leading to acute adrenal insufficiency with a mortality rate of 15%. Known etiologies include abdominal trauma, adrenal vein thrombosis, sepsis, shock, and anticoagulation. Systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS) are uncommon conditions associated with BAH. We describe here a 79-year-old woman with a history of hypertension and a 23-year history of borderline SLE treated with hydroxychloroquine (HCQ) who presented at the ED with epigastric pain, diarrhea, and nausea. Six months before, HCQ was discontinued. The exam showed no abnormal findings, and an abdominal CT showed inflammation of the ascending colon. Augmentin was instituted, and the patient was sent home. After a week, the patient returned to the ED due to the persistence of symptoms, complaining of fatigue and right lower quadrant abdominal pain. Upon examination, the abdomen was soft but tender, with the CT suggesting pancolitis, while an MRI showed BAH. Laboratory findings included anemia, thrombocytopenia, elevated PT, low fibrinogen, and 11 mcg/dl cortisol. The patient was then admitted to the hospital, and hydrocortisone (HCT) therapy was initiated. After 12 hours of glucocorticoid initiation, cortisol level was 37 mcg/dl. On day 1, the patient remained hemodynamically stable. On day 2, a positive lupus anticoagulant was detected, with cortisol level dropping to 2 mcg/dl, warranting the continuation of HCT. From day 3 to 6, the patient suffered worsening coagulation and kidney functions. By day 7, an abdominal CT showed an extension of BAH. On day 8, the patient experienced a cerebral intraparenchymal hemorrhage and was transferred to the ICU, where, after two days passed away. The report presented here describes a catastrophic case of BAH associated with borderline SLE and APS in a patient who previously discontinued long-term treatment with HCQ, thereby enhancing the likelihood of a lupus flare-up. The adrenal glands are susceptible to thrombosis and hemorrhage due to their rich vascularity. In stress, the adrenals receive a significant blood volume, causing vascular congestion, potentially exceeding their capacity for venous drainage, and increasing the risk of hemorrhage. Autoimmune conditions, particularly SLE and APS, have been implicated in the etiology of adrenal hemorrhage. Antiphospholipid antibodies may bind to phospholipid-binding plasma proteins, promoting thrombosis in the adrenal vein by disrupting the coagulation fibrinolysis balance and the adrenal blood outflow, ultimately causing bleeding into the adrenal gland parenchyma. Although in this case, no cardiovascular collapse was observed, when unrecognized or undertreated, adrenal insufficiency complicating BAH can evolve into an adrenal crisis. Infections and medications that increase cortisol metabolism can also contribute to this complication. Presentation: 6/3/2024

Journal of the Endocrine SocietyVol. 8(Supplement_1)
Berkshire Medical Center (US)
Good health and well-being
Openalex Percentile: Top 73%
Adrenal Hormones and Disorders
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