Endocrine Abstracts

Antiphospholipid antibody (APLA) syndrome is a rare autoimmune systemic disorder which can occur as primary condition or secondary to connective tissue diseases, most frequently systemic lupus erythematosis. We are presenting a rare case of a lady who developed adrenal insufficiency secondary to adrenal vein thrombosis, as a late sequel to APLA Syndrome.

A 50-year-old lady admitted to the medical ICU with one day’s history of severe abdominal pain and vomiting. PMH includes APLA syndrome and recurrent intrauterine deaths. She was not on any regular medications except OCP which was started a month prior to her hospital admission. Investigations revealed serum sodium 134 (135–150) mEq/l, potassium 3.7 (3.5–5.0) mEq/l, cortisol 20.14 (0.49–58.60) μg/dl, APTT 30.6 (26.1–33.3) s, PT 11.7 (11.0–15.0) s, D-dimer 0.2 (0.0–0.3) mg/l. CT scan showed bilateral adrenal gland and retroperitoneal inflammation. In keeping with past history of APLA syndrome, bilateral adrenal vein thrombosis was considered and she was anticoagulated. Three weeks later, she visited the OPD with tiredness, dizziness and an episode of collapse. BP was 110/70 and 120/80 mmHg on sitting and standing respectively. Morning serum cortisol was 5.38 μg/dl. 250 μg ACTH stimulation test revealed baseline and 30 min cortisol 2.98 and 5.49 μg/dl respectively. She was diagnosed with adrenal insufficiency as a delayed complication of adrenal vein thrombosis. She was started on tablet hydrocortisone 10–5–5mg. Recent ACTH Stimulation test did not show improvement in adrenal response. She has been on maintenance dose of hydrocortisone.

Discussion: The characteristic feature of APLA Syndrome is recurrent arterial and venous thrombosis. However, adrenal vein thrombosis and adrenal insufficiency is rarely reported. OCP along with lack of anticoagulation therapy might have precipitated adrenal vein thrombosis. Even though patient did not have adrenal insufficiency acutely, she developed the condition as a delayed complication. Treating physicians should have high index of suspicion regarding rare complications and monitor them regularly so that life threatening consequences can be avoided.