Case Report

Percutaneous Treatment of a Patient with Sjogren’s Syndrome Presenting with Simultaneous STEMI and Right Brachial Artery Occlusion

Krzysztof Pawlowski, MD, Lukasz Lewicki, MD, PhD, Radoslaw Targonski, MD, PhD, Maciej Karwowski, MD, Pomorskie Centra Kardiologiczne, Gdansk, Poland

Krzysztof Pawlowski, MD, Lukasz Lewicki, MD, PhD, Radoslaw Targonski, MD, PhD, Maciej Karwowski, MD, Pomorskie Centra Kardiologiczne, Gdansk, Poland

Case Report 
 
A sixty-two-year-old female with a history of hypertension, hyperlipidemia, ischemic stroke, and Sjogren’s syndrome presented with sudden right upper limb pain lasting over 48 hours and new 6-hours typical chest pain. The presenting electrocardiogram showed ST-elevation myocardial infarction (STEMI). 
 
She was well oriented, and complained of both chest and right upper limb pain. On physical examination, signs of acute right upper limb ischemia were present. Blood pressure measured on the left arm was normal, and she showed no signs of heart failure or shock. The right upper limb was pale, cool, and pulse on both the arteries of the forearm and on the brachial level was absent. 
 
Angiography via right femoral access revealed significant narrowing of the mid-right coronary artery. The proximal left anterior descending coronary artery (LAD) had long, proximal narrowing to be assessed later.  
 
After stenting of the RCA, a right guiding catheter was placed in the right subclavian artery and acute occlusion of brachial artery was demonstrated. The occlusion was easily crossed with Balance Middleweight (BMW) II wire (Abbott Vascular) and several passes were made with the Export aspiration catheter (Medtronic). We performed balloon dilatation on the artery (3.5 mm x 38 mm compliant balloon) and massive occlusive thrombus with sluggish forward flow was restored. An attempt to further aspirate thrombus via STO1 catheter (Terumo) (mother and child technique) failed due to tortuosity of the brachiocephalic trunk. We contemplated stopping the procedure at that stage with intention to treat patient with low molecular weight heparin for a few days and do angiographic control, but after the retrieval of the BMW wire, the vessel collapsed. Bearing in mind the relatively long time of occlusion, we speculated it was old and well-formed thrombus, unlikely to disintegrate after stenting. Two stents, a Xience PRO 3.5 mm x 48 mm and a Xience 3.5 mm x 23 mm were deployed with high pressure post dilatation aiming for a 4.6 mm final diameter. The angiographic result was good and flow to the forearm arteries was restored. X-ray exposure was 526mGy and 300ml contrast was used.
 
After the procedure, a pulse was instantly palpable on both the radial and ulnar artery, and limb pain gradually disappeared. Right hand warmth and color soon returned to normal. The next day, Doppler scan showed good flow in the brachial, radial, and ulnar arteries. 
 
Two-month follow-up angiography showed a lasting result in the brachial artery, as well as normal radial and ulnar artery flow. The patient reported no symptoms apart from angina, which dissipated after LAD stenting.
 
Discussion
 
Acute limb ischemia is traditionally defined as a sudden drop in limb perfusion that may threaten its viability. There is usually sudden pain, and the extremity becomes pale and numb. Until recently, two therapies were known: surgical embolectomy and bypass grafting.1 Recently, other modalities for selected patients have become available: fibrinolytic therapy and percutaneous intervention. Three primary mechanisms are responsible for acute limb ischemia: embolism, thrombosis, and trauma. The majority of emboli will originate from the heart. Artery thrombosis is often associated with underlying atherosclerosis, but may also be present in arteritis or hypercoaguable states. In atherosclerosis, collaterals have time to develop and symptoms are much less dramatic. Acute limb ischemia may be secondary to trauma as well, especially in the era of widespread access to percutaneous intervention. Acute ischemia of the limb may be confirmed by Doppler ultrasound, angiography computed tomography (CT), or arteriography. The latter should be the method of choice, since it provides immediate and accurate information regarding etiology, and may be often followed by a therapeutic procedure. Angiography often allows us to distinguish between an embolic event and thrombosis. Embolus will be present in an unchanged artery, with either a reversed meniscus shape of the occluded vessel or rounded filling defect(s). In the event of thrombosis, a sharp or tapered vessel stamp will be present, along with clear atherosclerosis or developed collaterals. 
 
Anticoagulation remains the treatment of choice. Other treatment options include thrombolysis, percutaneous intervention, and surgery. Which to choose will depend on clinical presentation, the patient’s suitability for surgery and the availability of their own graft material. The time of ischemia, etiology (embolus vs thrombosis), and localization and extension of the lesion will determine treatment strategy. Proximal lesions are often successfully treated with embolectomy, while distal occlusions fare better with thrombolysis. Angioplasty is most frequently utilized in patients with residual artery stenosis after successful thrombolytic therapy. Based on results from the TOPAS and STILE trials, catheter-based thrombolytic therapy is generally recommended in patients with ischemia lasting less than 14 days.2-5 
 
There are new devices that allow for the reduction of clot burden in occluded vessels. These devices are designed to improve outcomes compared with standard catheter-based thrombolytic therapy. 
 
In the EkoSonic Endovascular System (EKOS), an ultrasound field accelerates lytic dispersion and allows reduction of drug dose by up to 68%. Compared to traditional catheter-directed thrombolysis (CDT) it is more effective in respect to completeness of clot dissolution, lowers both limb amputation rate and bleeding rate, and shortens hospitalization time.  
 
The AngioJet peripheral thrombectomy system (Boston Scientific) is another viable option where the lysis effect is enhanced by catheter-based mechanical removal of the thrombus. In the limb ischemia subgroup of the PEARL registry, pharmacomechanical thrombolysis compared to standard CDT achieved a higher percentage of substantial lysis.  
 
The Indigo percutaneous mechanical thrombectomy catheter (Penumbra) represents a different approach, where suction-based clot removal isn’t accompanied by lysis. High level, continuous suction, as well as flexible catheters, allow effective large thrombus removal from both arterial and venous beds.
 
Upper limb ischemia is most often associated with atherosclerosis, but many other conditions may be responsible. Larger arteries may be affected by injury, dissection, embolism, thrombosis, and arteritis, while small arteries may be affected by vasculitis (e.g., in rheumatoid arthritis, systemic lupus erythematosus, scleroderma, and Sjogren’s syndrome), vasospasm, and repetitive injury.6,7
 
Antiphospholipid syndrome may be diagnosed when arterial thrombosis is accompanied by lupus anticoagulant test (LA), anticardiolipin antibody (aCL), or antibodies to B2-glycoprotein-I (Beta2-GP-I). 
 
Had the patient developed upper extremity ischemia in different circumstances, she certainly would have been referred to vascular surgeons. Yet, in the setting of acute myocardial infarction referred to invasive cardiology, percutaneous treatment of the acutely occluded brachial artery seemed most straightforward. The decision was also affected by late presentation, and available vascular access and tools alongside vascular expertise. Successful peripheral percutaneous interventions in patients with coronary artery disease and relative contraindications to surgery have been previously reported.8
 
This is an intriguing case of potential simultaneous plaque destabilization in two vascular beds. There are studies that suggest some patients have more than one unstable plaque in different vascular beds.9 An embolic event should be considered in differential diagnosis. The angiographic appearance suggested thrombosis rather than embolism — the occlusion site was tapered and the amount of active collateralization suggested a long-lasting process. On echocardiography, no thrombus, aneurysm, spontaneous contrast in the left ventricular cavity, or valve involvement was obvious. We did not observe any atrial arrhythmias. 
 
The patient’s medical history suggested the possibility of arterial vascular disease. Arteriosclerosis risk factors as well as ischemic stroke were present in the history. Atherosclerotic plaques in the aorta and iliac arteries were observed on abdominal CT.
 
In the patient with Sjogren’s syndrome, vasculitis must be considered. This cutaneous form of vasculitis often involves small vessels and leads to manifestations like palpable purpura and urticarial skin lesions.10,11 Diagnosis is made based on the clinical picture, presence of specific antibodies, and if possible, vessel biopsy. Patients with Sjogren’s may experience leukocytoclastic vasculitis11,12, but only around 5% will involve medium-sized vessels13. In our patient, we did not observe any constitutional symptoms or elevated CRP. Autoimmune tests were taken: LA, serum complement, anticardiolipin antibodies, ANCA, PR3-ANCA, and MPO-ANCA were normal. Only IgG class Beta2-GP-I was elevated. According to Sapporo criteria, antiphospholipid syndrome is diagnosed with two positive separate tests (at least 12 weeks apart). In our patient, should confirmatory levels of B2-GP-I be positive or complex atrial arrhythmias found on Holter monitoring, oral anticoagulants will be added to dual antiplatelet therapy. 
 
Summary
 
Various mechanisms may be responsible for simultaneous occlusion of vessels in two vascular beds. Thorough investigation is warranted to elucidate mechanism of the event in order to prevent further episodes. Acute plaque destabilization in different vascular beds is uncommon. While treatment of STEMI is well established, management of concomitant acute limb ischemia leaves room for discussion. Acute limb ischemia may be treated by embolectomy, catheter-directed thrombolysis, or anticoagulation. There are new systems for acute limb ischemia that improve outcomes, allow significant reduction of thrombolytic dose (AngioJet, EkoSonic) or eliminate lytic entirely by removing clot in purely mechanical fashion (Indigo). Effective reduction of clot burden may be followed by surgery or stenting, depending on the anatomy of the affected peripheral vessel. In unusual situations where two vascular beds are simultaneously involved, percutaneous treatment is a viable strategy. Patients with connective tissue disease may be at a higher risk of vascular events and should be screened for vasculitis and antiphospholipid syndrome, as well as atherosclerosis. When the mechanism of a suspected embolism is unclear, screening for silent atrial arrhythmias may be reasonable.
 
References
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  12. Alexander EL, Arnett FC, Provost TT, S tevens MB. Sjögren’s syndrome: association of anti-Ro(SS-A) antibodies with vasculitis, hematologic abnormalities, and serologic hyperreactivity. Ann Intern Med. 1983; 98:155. 
  13. Ramos-Casals M, Font J, Garcia-Carrasco M, et al. Primary Sjögren syndrome: hematologic patterns of disease expression. Medicine (Baltimore). 2002; 81:281. 
 
The authors can be contacted via Krzysztof Pawlowski MD, tel. +48502949280, e-mail: krzysztof.pawlowski@wp.pl, address:  Pomorskie 
Centra Kardiologiczne ul. Piekarnicza 12, 80-126 Gdansk, Poland.