Endocrine Abstracts

Introduction: Waterhouse Friedrichsen syndrome-Severe adrenal insufficiency is a rare but life-threatening condition secondary to bilateral adrenal haemorrhages. In many cases, it is caused by fulminant meningococcaemia, but there are numerous other aetiologies, it can also be caused by use of medications that promote blood clotting, reduce platelet count, primary antiphospholipid syndrome, renal vein thrombosis or steroid use and has been associated with splenectomy as well. It is characterised by fever, rash and septic shock.

Case History: We present a convoluted case of 32 years old lady who presented with pleuritic chest pain and shortness of breath after long flight. Her past medical history included ocular surface disorder and Sjogren”s syndrome. A CT Pulmonary Angiogram confirmed a pulmonary embolism and consolidation. She was discharged on Apixaban and antibiotic. She presented a week later with worsening shortness of breath, headache, nausea and vomiting. Meningitis ruled out with normal lumbar puncture. With persistent and worsening inflammatory markers, CT thorax abdomen and pelvis was performed which suggested oedematous adrenal glands bilaterally consistent with adrenal haemorrhage and wedge-shaped, pleural-based regions of consolidation in lower lobes of both lungs. Random Cortisol levels were profoundly low at 14 nmol/l and Short Synacthen test confirmed adrenal insufficiency. She was replaced with hydrocortisone. Her Pituitary profile was normal apart from slightly raised prolactin at 1158 mU/l. In view of her ongoing headache and low cortisol, she underwent an MRI head that ruled out Pituitary Apoplexy but showed incidental finding of occluded superior sinus likely secondary to thrombus. Further CT Venogram confirmed multifocal cerebral Dural venous sinus thrombosis within the superior sagittal sinus and right sigmoid sinus/proximal right Internal Jugular Vein. Biochemical testing for thrombosis revealed isolated rise in lupus anticoagulant, which was deemed to be of minimal significance in absence of raised CLIP and Beta2 glycoproteins. Antiphospholipid was ruled out by Rheumatologist on clinical assessment.

Conclusion: Waterhouse-Friedrichsen syndrome is associated with high mortality. Management comprises of treating underlying condition and supportive care. It is important to suspect and investigate adrenal insufficiency as steroid replacement is crucial for good outcome. A good education regarding regular steroid replacement including sick day rules when commencing steroid replacement is paramount. A meticulous follow up plan in an Endocrine clinic is essential to monitor steroid replacement and progression of adrenal haemorrhages.