Pickering syndrome in a patient with Takayasu's arteritis

Takayasu arteritis (TA) is a chronic granulomatous large vessel arteritis. The renal arteries are affected in up to 60% of patients with TA, with renal artery stenosis (RAS) potentially leading to ischemic nephropathy, severe arterial hypertension, and heart failure. Bilateral RAS may rarely present with recurrent flash pulmonary edema, a life-threatening association which has been termed Pickering syndrome. In this report, we describe a 55-year-old woman with severe refractory arterial hypertension admitted for acute pulmonary edema, initially treated unsuccessfully with medical therapy with vasodilators and diuretics. Given the instrumental findings of bilateral RAS and suggestive signs and symptoms, the diagnosis of TA was made, resulting as the first described case of Pickering syndrome being the clinical presentation of TA. Interventional therapy with renal artery angioplasty procedure was performed with stenting of both right and left renal arteries, leading to the resolution of the clinical scenario and the successful discharge of the patient. At the 1 year follow-up visit the patient was asymptomatic and in good clinical conditions; a significant reduction in antihypertensive therapy was achieved while immunosuppressive therapy was continued. This case highlights that secondary causes of TA should always be sought in patients with refractory hypertension who do not respond to standard treatment; also, TA should be suspected in young patients with bilateral RAS, especially when other typical signs of TA are present; lastly, a thorough investigation is essential in complicated cases, as rare diseases like TA may manifest in unusual ways.