Pickering syndrome in a patient with Takayasu’s arteritis

ABSTRACT

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.

KEYWORDS:

• Pickering syndrome
• Takayasu arteritis
• pulmonary edema
• arterial hypertension
• Renal artery stenosis

Abbreviations

ACR	=	American College of Rheumatology
CT	=	Computed tomography
HF	=	Heart failure
NT-proBNP	=	N-terminal pro-brain natriuretic peptide
RAS	=	Renal artery stenosis
TA	=	Takayasu arteritis

Declaration of financial/other relationships

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties. Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Author contributions

Umberto di vita and Pier Paolo Bocchino collected the patient’s data, wrote the first draft of the manuscript and revised it; Tullio Usmiani and Gaetano Maria de Ferrari revised and edited the manuscript.

Ethics statement

Our case report follows the principles of the Declaration of Helsinki. Ethical approval was not needed. The patient provided written informed consent for the involvement in this case report and the publishing of her anonymous data.

Additional information

Funding

This paper was not funded.