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Case presentation: 

We present a very interesting case of a patient who presented with the initial symptom of refractory hypertension requiring multiple admissions into the cardiovascular unit. Hypertensive crises were episodic and were associated with tachycardia and sweating. His chronic history of refractory hypertension led to end stage renal failure requiring renal transplant. Work up for secondary causes for hypertension including screening for pheochromocytoma was negative in our patient.  Eventual presentation of the disease with orthostatic hypotension came into attention late in the disease course when the patient had two consecutive episodes of syncope. Due to development of orthostasis, tilt test and quantitative sudomotor axon reflex test (QSART) was done, which was remarkable. Acetylcholine receptor ganglionic neuronal antibody was sent which came back positive. He was treated with IVIG which resolved his hypertensive crisis and autonomic dysautonomia.

We highlight the importance of the possibility of autoimmune etiology in patients with refractory hypertensive crisis.  Awareness of an underlying autoimmune process as a potential primary cause of hypertensive crisis or other manifestations of autonomic dysfunction promotes timely diagnosis and appropriate management.