Background: Pseudoxanthoma elasticum (PXE) is a rare, genetic disorder characterised by progressive vision loss, skin changes, and early-onset arteriosclerosis. The latter makes PXE a risk factor not only for peripheral artery disease (PAD) but may also promote coronary artery disease. In this context, patients with PXE have repeatedly reported problems with the radial access during coronary angiography. Therefore, the aim of this study was to prospectively investigate the prevalence of upper extremity (UE) arterial occlusion in patients with PXE and to identify possible associated risk factors. Patients and methods: Between November 2022 and February 2024, 93 consecutive patients with PXE were examined. 65 eligible patients with PXE were retained for further analysis and compared with 57 in-hospital controls. All patients underwent extensive screening for UE arterial disease using colour-coded duplex sonography (CCDS). Results: UE artery occlusion was significantly more common in patients with PXE than in the control group (58.5% vs. 3.5%; p < .001). The radial arteries were most commonly affected, predominantly bilaterally (50.8% of all PXE patients). Age was significantly associated with the presence of UE artery occlusion with an odds ratio (OR) of 1.06 (confidence interval [CI]: 1.01-1.10; p = .013). Conclusions: Peripheral occlusion of the UE arteries is a common finding in patients with PXE. In clinical practice, these findings suggest that patients with PXE may require a screening for PAD and occlusion of the arteries of the UE using CCDS after the age of 40 years. This is particularly recommended prior to coronary angiography or percutaneous coronary intervention.

Sjögren's disease (SjD) is a chronic systemic autoimmune disorder primarily affecting the exocrine glands, while Takayasu arteritis (TAK) is a large-vessel vasculitis involving the aorta and its major branches. Although SjD frequently overlaps with other autoimmune diseases, its coexistence with TAK has been reported only rarely. A 47-year-old Turkish woman with seropositive SjD presented with constitutional symptoms, upper extremity claudication, and asymmetric peripheral pulses. Laboratory evaluation revealed elevated inflammatory markers while antiphospholipid antibodies were negative. Computed tomography angiography demonstrated stenotic lesions and vessel wall thickening involving the left subclavian, axillary, carotid, and brachiocephalic arteries. Additional positron emission tomography/computed tomography showed increased vascular uptake consistent with active large-vessel inflammation. Based on clinical, laboratory, and imaging findings, the patient fulfilled the criteria for TAK. Treatment resulted in marked clinical improvement and normalization of inflammatory markers, with sustained remission during a 2-year follow-up period. The coexistence of SjD and TAK is exceptionally rare, with only a limited number of cases reported to date. This case represents the first reported Turkish patient and adds to the growing evidence that large-vessel vasculitis should be considered in SjD patients who develop unexplained vascular or systemic inflammatory features.

Progressive claudication of the upper extremity is uncommon and can be misattributed to cervical radiculopathy or even shoulder pathology, such as tendonitis or bursitis. We report a patient presenting to the emergency department with several months of worsening left upper-extremity pain initially managed as suspected radiculopathy, but, in retrospect, was upper-extremity claudication. This was discovered through the presence of lesions beneath the fingernails of the left hand, splinter hemorrhages. A computed tomographic angiography (CTA) of the chest for aortic and vascular evaluation demonstrated a mural thrombus partially occluding the left subclavian artery. The patient was admitted for further management and observation. Following initiation of systemic anticoagulation, symptoms had significantly improved, and the patient was discharged home in stable condition. This case underscores the diagnostic importance of bedside examination and vascular imaging when evaluating atypical upper-extremity claudication. This also highlights arterial thrombus as a potentially reversible cause of upper-extremity pain when promptly recognized and treated.

Intraneural ganglion cysts are rare, benign mucinous cysts that arise within the epineurium of peripheral nerves. They most commonly involve the peroneal nerve, whereas involvement of the tibial nerve is exceptionally rare. Patients typically present with neurological deficits involving motor and sensory function of the affected nerve. We present a case of an intraneural ganglion cyst of the tibial nerve manifesting primarily with atypical, vascular compression-like symptoms of intermittent claudication. A 43-year-old patient was referred to our department with a 1-year history of exertional, cramp-like pain in the left calf. The pain occurred consistently within minutes of walking or running and subsided with rest, mimicking intermittent claudication. Duplex sonography and subsequent magnetic resonance imaging revealed an intraneural ganglion cyst of the tibial nerve originating from the superior tibiofibular joint, showing compression of the popliteal artery. On magnetic resonance imaging, compression of the popliteal artery to less than a third of its physiological diameter was demonstrated. Definitive surgical management involved decompression of the intraneural cyst and disconnection of the articular branch. Postoperatively, the patient's intermittent claudication symptoms ceased. Intraneural ganglion cysts of the lower extremity represent a rare cause of neurological symptoms. This case report showed that they can also compress adjacent vascular structures, thereby mimicking intermittent claudication. These atypical symptoms can lead to an initial suspicion of a vascular etiology and hence pose a risk for misdiagnosis. Decompression of the ganglion and addressing the articular branch are essential steps to prevent recurrence and provide definitive symptom relief.

Clinical presentation of subclavian and innominate artery peripheral arterial disease (PAD) varies from hand claudication to cerebral hypoperfusion to distal embolization and digital ischemia. Upper extremity PAD could manifest as coronary steal or lower extremity claudication in patients who have had CABG or extra-anatomic axillary to femoral bypass. Physical examination is significant for weak pulses on an ipsilateral extremity, a systolic blood pressure difference of more than 10mmHg compared to contralateral extremity. Sometimes the affected hand may feel cool to touch, and in severe cases, digital ischemia has also been described. Often patients present during their sixth or seventh decades of life, and they have associated peripheral arterial disease risk factors such as smoking, diabetes mellitus, hyperlipidemia, hypertension, lower-extremity peripheral arterial artery disease, and less common inherited genetic disorders such as inflammatory or Takayasu arteritis.[1]

Thoracic outlet syndrome (TOS) results from the compression of neurovascular structures at the thoracic outlet. The clinical presentation of arterial compression ranges from chronic ischemic symptoms, such as upper limb pallor, coldness, and exercise-induced claudication, to acute vascular complications including arterial thrombosis, which may progress to limb-threatening ischemia. We report the case of a 33-year-old patient with no significant medical history who presented with acute ischemia of the left upper limb. Radiological investigations revealed TOS with a cervical rib as the underlying cause of acute limb ischemia. This case underscores the pivotal role of imaging in establishing the etiological diagnosis and identifying vascular complications, thereby enabling rapid therapeutic management.

Endovascular stenting has emerged as the preferred treatment modality for coarctation of the aorta (CoA). However, CoA can sometimes extend beyond the aortic arch, involving adjacent vessels such as the left subclavian artery (LSA), which complicates conventional interventions. We present a case of CoA associated with proximal LSA stenosis which was successfully treated using a double-wire stent technique. The technique does not compromise the LSA flow and offers a promising alternative in complex CoA cases. A 19-year-old female presented with palpitations with dyspnea (New York Heart Association grade III) for 15 days. She also gave a history of intermittent claudication in the left upper limb for 3 years. Clinical examination revealed pallor and weak pulses in the left upper extremity and both lower extremities, with radio-radial and radio-femoral delays. Blood pressure measurements indicated significant gradients between the limbs, with readings of 244/112 mmHg in the right upper limb, 162/104 mmHg in the left upper limb, and 114/74 mmHg and 116/78 mmHg in the right and left lower limbs, respectively. Auscultation revealed normal S1 and S2 and a systolic murmur in the right interscapular area. Electrocardiogram revealed sinus arrhythmia with T-wave inversions in leads II, aVF, and V1-V6. Echocardiogram revealed severe postductal coarctation with a gradient of 84 mmHg. Computed tomography aortography confirmed a severe coarctation-preductal diameter of 12 mm and postductal diameter of 14 mm, with a concomitant LSA stenosis of 7 mm. The critical challenge in this case was stenting the coarctation without compromising the already symptomatic LSA stenosis. A novel endovascular approach was employed, utilizing two preplaced wires in both the aorta and LSA, followed by deployment of an uncovered stent and final kissing balloon angioplasty. This is the first instance in literature of such an approach being taken. Patients with CoA with associated bifurcation stenosis of the LSA are extremely rare and pose significant challenges for endovascular management. This case highlights a novel and effective interventional strategy, offering a tailored approach to preserve LSA patency while addressing the complex CoA anatomy.

Thoracic outlet syndrome (TOS) is a group of disorders characterized by the compression of neurovascular structures at the thoracic outlet. Arterial TOS, the least common but most severe form, carries significant thromboembolic risks and has a known association with cervical ribs. Synostosis of a complete first and second rib, termed the Srb anomaly, is rare and occurs in approximately 0.2% of the population. Here, we present a unique case of a 17-year-old boy with right upper extremity claudication owing to arterial TOS from an Srb anomaly. This case emphasizes the successful management of an uncommon condition, the importance of accurate diagnosis and timely intervention.

Takayasu arteritis (TA), a rare large-vessel vasculitis, primarily affects women of childbearing age, causing granulomatous inflammation in the aorta and its major branches. This inflammation can lead to stenosis, aneurysms, or occlusion, with the abdominal aorta, subclavian, and brachial arteries commonly involved. We present the case of a 26-year-old female with TA with a rare involvement of the superior mesenteric artery (SMA). The patient presented with progressive shortness of breath and exertional pain in the left upper limb, suggesting claudication. Echocardiography showed an ejection fraction of 45% and right ventricular systolic pressure of 65 mmHg. CT angiography (CTA) revealed diffuse involvement of the abdominal aorta with non-opacification of SMA, and other findings were consistent with TA type 5. The patient was successfully treated with methylprednisolone to induce remission, along with guideline-directed medical therapy for heart failure.

Adherence to the Mediterranean diet (MeD) has been associated with a reduced incidence of peripheral arterial disease (PAD)/lower extremity arterial disease (LEAD) in observational trials and in a randomized trial. In secondary prevention, a lower hazard ratio for composite major adverse cardiovascular events has been associated with better adherence to MeD in a relatively small, randomized trial. This has not been confirmed in a sub-analysis of a large interventional trial of dual antithrombotic treatment. The effects of vegetarian, vegan or low carbohydrate/ketogenic diets on the incidence and outcomes of PAD/LEAD are not known. While abdominal obesity is associated with diabetes mellitus and PAD/LEAD, the lowest incidence of PAD/LEAD has been found in subjects with a body mass index 25-29.9 kg/m[2]. Malnutrition is a negative prognostic factor for survival of patients with chronic limb threatening ischemia. Physical activity (PA) is an acute stressor, but habitual recreational PA results in beneficial adaptations and improved health. In observational studies, lower levels of exercise and lower physical fitness have been associated with more prevalent PAD/LEAD. In contrast to coronary artery disease, that shows a reverse J-shaped relationship between long-term endurance exercise and coronary atherosclerosis, such a relationship is not known for PAD/LEAD. A general recommendation for maintaining cardiovascular health is performing regular moderate-intensity exercise with some vigorous-intensity aerobic PA, and resistance exercise at least twice a week. Combinations of healthy behaviors are more effective in preventing PAD/LEAD than a single behavioral component. In treatment of PAD/LEAD causing intermittent claudication, supervised walking training is recommended among measures of first-line treatment, while unsupervised walking training should be considered as an alternative.