A Rare Presentation of Probable Catastrophic Antiphospholipid Syndrome with Bilateral Adrenal Haemorrhage Secondary to Systemic Lupus Erythematosus

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Published: June, 2026

Keywords: Anti Phospholipid Syndrome (APS); Bilatera Adrenal Haemorrhage (BAH); Systemic Lupus Erythematosus (SLE)

Author
  • Ajitesh Roy
  • Jyotirmay Maji
Abstract

Background :
Catastrophic antiphospholipid syndrome (CAPS), a rare thrombotic manifestation of antiphospholipid syndrome (APS), carries high mortality. Bilateral adrenal haemorrhage (BAH), an unusual complication of APS, is seldom reported. This case highlights a rare presentation of bilateral adrenal haemor- rhage due to probable CAPS secondary to systemic lupus erythematosus SLE).

Case Presentation :
A 57-year-old diabetic female from India, presented with hypovolemic shock, abdominal pain, and vomiting. Clinical evaluation, imaging(CECT abdomen), and laboratory investigations(anti-42GP1 IgG, lupus anticoagulant, anticardiolipin antibodies) were conducted.Secondary causes like sepsis, trauma, and coagulopathies were excluded. SLE workup included ANA and anti-dsDNA testing.
CECT revealed bilateral adrenal haemorrhage. Persistent positivity for antiphospholipid antibodies confirmed APS. Elevated ANA (1:100) and anti-dsDNA suggested underlying SLE, despite absent typical symptoms. Serum cortisol.
(9.2 ug/ dL) and ACTH (180 pg/mL) indicated evolving adrenal insufficiency. The patient received methylprednisolone pulses, IVIG, anticoagulants, and immunosuppres-sants (MMF), achieving rapid stabilisation without mineralocorticoid replace- ment.

Conclusion :
This case underscores Bilateral Adrenal haemorrhage as a rare and atypical manifestation of CAPS and any case of APS should always be evaluated for background SLE. Early diagnosis via antibody testing and imaging, combined with aggressive immunosuppression and anticoagulation, improves outcomes.This report enriches the limited literature on CAPS-related BAH multidisciplinary management in thrombotic-autoimmune syndromes.

Reference
  1. Gémez-Puerta, J. A., Cervera, R. (2014). Diagnosis and classification of the antiphospholipid syn- drome. J Autoimmun, 48-49, 20-25. https://doi.org/ 10.1016/j.jaut.2014.01.006.
  2. Kazzaz, N. M., McCune, W. J., Knight, J. $. (2016). Treatment of catastrophic antiphospholipid syndrome. Curr Opin Rheumatol, 28(3), 218-227. doi
    10.1097/BOR,0000000000000269.
  3. Rodriguez-Pinté, L., Moitinho, M., Santacreu, I., Shoenfeld, Y., Erkan, D., Espinosa, G., Cervera, R., CAPS Registry Project Group (European Forum on Antiphospholipid Antibodies). (2016). Cat- astrophic antiphospholipid syndrome (CAPS): Descriptive analysis of 500 patients from the In- ternational CAPS Registry. Autoimmun Rev, 15(12), 1120-1124. doi 10.1016/j.autrev.2016.09.010.
  4. Aldaajani, H., Albahrani, S., Saleh, K., Alghanim, K. (2018). Bilateral adrenal hemorrhage in an-tiphospholipid syndrome. Anticoagulation for the treatment of hemorrhage. Saudi Med J, 39(8), 829-833. doi: 10.15537/smj.2018.8.22437.
  5. Bansal, R., Nath, P. V., Hoang, T. D., Shakir, M. K. M. (2020). Adrenal Insufficiency Secondary to Bilateral Adrenal Hemorthage Associated with Antiphospholipid Syndrome. AACE Clinical Case Reports, 6(2), e65-e69. https://doi.org/10.4158/ACCR-2019-0376.
  6.  Aguila, L. A., Lopes, M. R., Pretti, F. Z., et al. (2023).Clinical and laboratory features of overlap syndromes of idiopathic inflammatory myopathies associated with systemic lupus erythematosus, systemic sclerosis, or rheumatoid arthritis. Clin Rheumatol, 33(8), 1093-1098
  7. Meroni, P. L., Borghi, M. O., Raschi, E., et al. (2023). Pathogenesis of antiphospholipid syndrome: Understanding the antibodies. Nat Rev Rheumatol, 7(6), 330-339.
  8. Giannakopoulos, B., Krilis, S. A. (2024). The pathogenesis of the antiphospholipid syndrome. N Engl J Med, 368(11), 1033-1044.
  9. Presotto, F., Fornasini, F., Betterle, C., et al. (2023). Acute adrenal failure as the heralding symptom of primary antiphospholipid syndrome: Report of a case
    and review of the literature. Eur J Endocrinol, 153(4), 507-514.
  10. Ramon, I. Mathian, A., Bachelot, A., et al. (2024). Primary adrenal insufficiency due to bilateral adrenal hemorrhage-adrenal infarction in the antiphospholipid syndrome: Long-term outcome of 16 patients, J Clin Endocrinol Metab, 98(8), 3179-3189.
  11. Cervera, R., Rodriguez-Pint6, I., Espinosa, G. (2023). The diagnosis and clinical management of the catastrophic antiphospholipid syndrome: A  comprehensive review. J Autoimmun, 92, 1-11.
  12. Espinosa, G., Bucciarelli, S., Cervera, R., et al. (2024). Laboratory studies in the diagnosis of catastrophic antiphospholipid syndrome: A review. Clin Rev Allergy
    Immunol, 36(2-3), 86-91.