Pickering syndrome: a rare presentation in emergency department

Abstract

Sympathetic crash acute pulmonary edema (SCAPE) is a life-threatening injury, which requires early recognition and intervention to prevent mortality. We present a case of 18-year-old woman with no previous comorbidity, presenting with SCAPE who was successfully resuscitated and eventually diagnosed with renal artery stenosis. Pickering syndrome is a rare cause of hypertensive emergency and should be considered in a young patient presenting with SCAPE in emergency department.

Background

Acute pulmonary edema (APE) is a life-threatening emergency frequently seen in clinical practice. There can be cardiogenic as well as non-cardiogenic causes of APE, which is difficult to differentiate in emergency department (ED).¹ Sympathetic crashing APE (SCAPE) is the extreme end of the spectrum of APE which has a dramatic presentation.² In SCAPE there is abrupt increase in sympathetic tone and release of catecholamine with precipitates APE.³ The increased sympathetic tone causes stress failure of the pulmonary capillaries leading to increases the permeability of the pulmonary circulation.⁴ Early recognition and prompt intervention helps in early resolution of the symptoms. SCAPE commonly presents in patients with renal disease, which includes both renal vascular diseases and chronic kidney disease. We present a case of 18-year-old woman with no previous comorbidity, presenting with SCAPE and eventually being diagnosed with renal artery stenosis (RAS). It is important to understand this disease as it is relatively common in the ED and has better outcomes when managed appropriately.

Case presentation

An 18-year-old woman with no previous comorbidities presented to the ED at around 02:00 with the sudden onset of breathlessness for 2 hours. She did not complain of any other symptoms and was apparently well prior to this current presentation. She had no previous comorbidity. She did not smoke, drink alcohol or use illicit drugs and was not on any medications. There was no other relevant family history.

On examination, she was agitated and severely tachypneic. Airway was patent. Respiratory rate was 40 breaths/min, the oxygen saturation was 86% under room air, the blood pressure was 190/130 mm Hg and pulse rate was 118 beats/min. On auscultation of the chest, coarse inspiratory crackles were heard in the bilateral lung fields. On cardiac auscultation, there were no murmurs.

Meanwhile, the patient was immediately started on bilevel positive airway pressure (BiPAP) as per our protocol of SCAPE management. She was severely agitated and not tolerating the BiPAP. Unfortunately the patient became unresponsive and had a cardiac arrest. The time interval between arrival of the patient and cardiac arrest was 10 min. High quality cardiopulmonary resuscitation (CPR) was started according to standard advanced cardiac life support(ACLS) protocol. The patient was intubated and started on mechanical ventilation. We were successful in reviving the patient after 4 min of CPR. Point of care ultrasonography revealed inferior vena cava(IVC) distensibility >50%, Bilateral B profile on lung ultrasound and normal point of care echocardiography (video 1). Post return of spontaneous circulation, her blood pressure was 200/130 mm Hg followed which she was given an intravenous 800 mcg nitroglycerin bolus followed by high dose infusion at 100 mcg/min.

Video 1.

Download video file ^{(906.9KB, mp4)}

DOI: 10.1136/bcr-2020-239421.video01

Investigations

The complete blood count, differential count, electrolytes, glucose, calcium, phosphorus, magnesium and troponin T were normal. Pro-Brain natriuretic peptide was elevated with a value of 20 400 pg/mL. Urea was 65 mg/dL and creatinine was 1.6 mg/dL. Fundus examination was normal. ECG was unremarkable except for sinus tachycardia. Clinical resolution and treatment were successful with early extubation in the ED within 6 hours of presentation.

Differential diagnosis

At the time of presentation, the diagnosis considered in a young woman with acute onset breathlessness were acute exacerbation of asthma, APE (cardiogenic/non-cardiogenic), pulmonary embolism, pneumothorax and psychogenic. With no previous history or risk factors of allergies, hypercoagulable state or trauma and examination findings of high blood pressure with crepitation in the chest, a provisional diagnosis of APE was made. We also did a bedside point of care echocardiogram, which showed no dilation of right atria and right ventricle making the diagnosis of pulmonary embolism less likely.

Outcome and follow-up

The patient was admitted for a detailed workup. Echocardiography at rest was normal with normal left ventricular (LV) systolic and diastolic function. Erythrocyte sedimentation rate (ESR) was 36 mm/hour and C-reactive protein (CRP) was 27.78 mg/L. Urine spot protein/creatinine ratio was 325 mg/g. Ultrasound abdomen showed left kidney 7.2 cm and right kidney 7.8 cm (relatively small bilateral kidneys) with preserved echo texture. A CT angiography done showed bilateral severe RAS along with coeliac and mesenteric artery stenosis likely suggestive of vasculitis (figure 1). A provisional diagnosis of Takayasu arteritis was made and the patient was started on tablet prednisolone 60 mg OD (1 mg/kg). She was discharged in a stable condition and planned for intervention for RAS.

Figure 1.

(A) Coronal CT angiographic image showing thickening of wall of abdominal aorta (asterisk) with short segment narrowing of proximal right and left renal arteries (red arrows). (B) Sagittal CT angiographic image showing luminal narrowing at the origin of coeliac artery and superior mesenteric artery (red arrows). (C) Volume rendered image from three dimensional CT data showing short segment narrowing of proximal bilateral renal arteries.

Discussion

Our patient presented with SCAPE, also known as flash pulmonary oedema, which presents with rapid onset and dramatic progression of symptoms, giving the emergency physicians a very narrow time window to intervene. Our patient was agitated at presentation and went into arrest due to the severe hypoxia. The distinguishing factor for SCAPE from other forms of decompensated heart failure is the acute increase of LV end diastolic pressure which floods of the alveolar space within minutes resulting in an acute life-threatening emergency.⁵ The risk factors associated with heart failure such as hypertension, coronary ischaemia, valvular heart disease and diastolic dysfunction are associated with development of SCAPE. In our case, the patient had no such previous history and further evaluation in the hospital revealed a bilateral RAS.

Bilateral RAS has been identified has a common cause of SCAPE. In 1988, Pickering et al (4) reported a series of 11 hypertensive patients with bilateral atheromatous reno-vascular disease who presented to the emergency with multiple episodes of pulmonary oedema. This clinical entity was later named as pickering syndrome, in honour to the author.⁵ Bilateral RAS can lead to SCAPE by three main pathophysiological mechanisms: (1) defective natriuresis; (2) increased haemodynamic burden leading to worsening diastolic dysfunction and (3) pulmonary capillary blood–gas barrier breakdown.⁶ The renal ischaemia due to RAS causes activation of the renin–angiotensin system, which lead to sympathetic nervous system stimulation causing LV wall stress and a mismatch between increased oxygen demand and delivery to the myocardium. This aggravates the LV diastolic dysfunction resulting in a further increase in LV end diastolic pressure.^7–9 Our patient was discharged with the provisional diagnosis of Takayasu arteritis. Takayasu arteritis can cause SCAPE by two mechanisms depending on the region of involvement—one by causing RAS and second due to aortitis.¹ These patient will need renal revascularisation by renal stenting to prevent future recurrence of the episodes.¹⁰

High dose nitroglycerine (NTG) has been successfully used in managing cases of SCAPE, though the evidence has been limited.^{2 11} The dosing of intravenous bolus as well as continuous infusion of NTG differs considerably among various studies.^{11 12} Physicians have used bolus doses ranging from 5 to 400 mcg/min given over 1–2 min and infusion rates of 5–200 mcg/min.^{13 14} As per our institutional protocol, we use a bolus of 800–1000 mcg over 2 min and start infusion rates at 100 mcg/min titrating according to the blood pressures and response of the patient. In our patient, post-RSC, we started the patient on high dose NTG which helped in early resolution of her symptoms leading to her early extubation. The use of furosemide and morphine in treatment of such patients have shown no benefit, rather could be potentially deleterious.¹⁵

The presentation of SCAPE is dramatic as the patient is extremely agitated, restless and usually diaphoretic finding it hard to breathe. Early identification of this condition with prompt intervention leads to good outcome in such patients.

In conclusion, Pickering syndrome is a rare cause of hypertensive emergency and should be considered in a young patient presenting with SCAPE in ED.

Learning points.

• Pickering syndrome is a rare differential diagnosis in young patients presenting with sympathetic crash acute pulmonary edema (SCAPE).

• SCAPE is a life-threatening emergency which requires early intervention to prevent mortality.

• Acute onset breathlessness with a background of sympathetic surge (high blood pressures, tachycardia, diaphoresis, marked agitation) think SCAPE.

• The treatment of this disorder involves the use of nitroglycerin and non-invasive ventilation in most cases.