Bilateral adrenal haemorrhage causing primary adrenal insufficiency in a patient with primary anti-phospholipid syndrome
M Modi, S Zaman, N Hawkins, H Al-Daour, E Benson, M Hameed, M Sohal, V Lecamwasam, Ealing Hospital
Case report: A 36 year old lady, with a background history of primary anti-phospholipid syndrome and Factor V Leiden, presented to Ealing Hospital 6 days post partum with pleuritic chest pain. On clinical suspicion of a pulmonary embolism, a CT pulmonary angiogram was performed. This excluded a pulmonary embolism, but revealed an incidental finding of left adrenal enlargement, suspicious of adrenal infarction. A short Synacthen test was performed on advice of the Endocrine team, which demonstrated an appropriate cortisol response.
During the admission, the patient became clinically septic, with pyrexia, hypotension and increasing inflammatory markers. She was treated by the Gynaecology team for suspected endometritis. However, she did not demonstrate clinical improvement on intravenous antibiotics. In light of this, a CT scan of the chest, abdomen and pelvis was performed, which revealed cholecystitis and mild pancreatitis. Furthermore, the scan revealed an increase in the size of the right adrenal gland.
The patientís care was transferred to the Endocrine team for further assessment and management, with ongoing input from the General Surgical team. The findings of the two previous CT scans raised the suspicion of adrenal haemorrhage. A repeat short Synacthen test confirmed a diagnosis of hypoadrenalism. Upon discussion with Radiology, an adrenal dedicated MRI was conducted, which confirmed bilateral adrenal haemorrhage with little normal adrenal tissue visualised. The patient was commenced on oral hydrocortisone and fludrocortisone, and showed a marked clinical response in blood pressure and symptoms.
The patient had also been on prophylactic dose clexane since delivery due to her underlying haematological conditions. On advice of the Haematology team, this was continued as the patient was deemed to be at increased risk of clot formation particularly as she was post-partum, septic and less mobile than her baseline.
Discussion: This case illustrated the importance of the multidisciplinary team in the management of a complex patient with multiple pathologies. Professionals from Endocrinology, Gynaecology, General Surgery, Radiology and Haematology worked in tandem to identify the underlying diagnosis and initiate appropriate management.
The phenomenon of adrenal haemorrhage and consequent adrenal insufficiency is a rare but recognised complication in patients with primary anti-phospholipid syndrome in response to sepsis. This case highlights the importance of a high index of suspicion for this phenomenon as it can be life threatening. Furthermore, the case reinforces the utility of serial investigations in light of ongoing pathology.