Stroke is a rare and severe complication of giant cell arteritis (GCA). Although early diagnosis and treatment initiation are essential, the mechanism of stroke is often related to vasculitis complicated by arterial stenosis and occlusion. Its recurrence is often attributed to early steroid resistance or late GCA relapse, so immunosuppressive treatment is often reinforced. However, many questions concerning the mechanisms of stroke remain elusive, and no review to date has examined the whole data set concerning GCA-related stroke. We therefore undertook this scoping review. GCA-related stroke does not necessarily display general signs and inflammatory parameters are sometimes normal, so clinicians should observe caution. Ischemic lesions often show patterns predating watershed areas and are associated with stenosis or thrombosis of the respective arteries, which are often bilateral. Lesions predominate in the siphon in the internal carotid arteries, whereas all the vertebral arteries may be involved with a predominance in the V3-V4 segments. Ultrasonography of the cervical arteries may reveal edema of the intima (halo sign), which is highly sensitive and specific of GCA, and precedes stenosis. The brain arteries are spared although very proximal arteritis may rarely occur, if the patient has microstructural anatomical variants. Temporal artery biopsy reveals the combination of mechanisms leading to slit-like stenosis, which involves granulomatous inflammation and intimal hyperplasia. The lumen is sometimes occluded by thrombi (<15%), suggesting that embolic lesions may also occur, although imaging studies have not provided strong evidence for this. Moreover, persistence of intimal hyperplasia might explain persisting arterial stenosis, which may account for delayed stroke occurring in watershed areas. Other possible mechanisms of stroke are also discussed. Overall, GCA-related stroke mainly involves hemodynamic mechanisms. Besides early diagnosis and treatment initiation, future studies could seek to establish specific preventive or curative treatments using angioplasty or targeting intimal proliferation.

Thoracic aortic diseases contribute to a major part of cardiac surgeries. The severity of pathologies varies significantly from emergency and life-threatening to conservatively managed conditions. Life-threatening conditions include type A aortic dissection and rupture. Aortic aneurysm is an example of a conservatively managed condition. Pathologies that affect the arterial wall can have a profound impact on the presentation of such cases. Several risk factors have been identified that increase the risk of emergency presentations such as connective tissue disease, hypertension, and vasculitis. The understanding of aortic pathologies is essential to improve management and clinical outcomes.

In daily practice, the frequent appearance of limb and/or skin necrosis, which we term "acute peripheral and/or cutaneous ischemic syndrome" (APCIS), can be a manifestation of numerous underlying diseases, or it can sometimes be a clinical phenomenon whose etiology is undefined even after a wide investigation. The mechanisms for the development of APCIS include vessel wall abnormalities (atherosclerosis, vasculitis, and calciphylaxis), embolic processes (infectious endocarditis, atrial myxoma, and cholesterol emboli), local thrombotic injuries (genetic or acquired thrombophilias and heparin- and warfarin-induced ischemia), dysproteinemias (cryoglobulinemia and cryofibrinogenemia), or venous limb gangrene. Here, we report 5 illustrative cases of APCIS with different pathogenetic mechanisms, thereby highlighting some clinical conditions that cause APCIS that may be of special interest for rheumatologists, such as antiphospholipid syndrome, primary and secondary vasculitis, and cryoproteinemias. Furthermore, we describe a large spectrum of other causes of APCIS beyond the scope of rheumatology. Because there are no validated guidelines for APCIS, we tentatively propose an initial diagnostic workup and a therapeutic approach based on full-dose anticoagulation and immunosuppressive therapy.

To clarify the role of endovascular treatment in patients with Critical Hand Ischemia of the distal upper extremity.

From January 2012 to January 2017, 18 dialyzed patients presented chronic critical hand ischemia; 6 patients had a chronic occlusion of the ulnar artery and radial artery stenosis, 5 patients had a chronic occlusion of the radial artery and ulnar artery stenosis, 4 patients presented multiple stenosis of the ulnar, radial and interosseous arteries, 2 patients had only ulnar artery occlusion while one patient presented only radial artery occlusion. All patients underwent duplex ultrasound and a subsequent brachial angiography, in order to evaluate the distal run-off circulation. Revascularization was achieved via antegrade brachial puncture in all patients, with retrograde approach in 4 patients, with distal retrograde puncture in 3 patients and in one patient with loop technique.

No complications were observed during the periprocedural follow-up. One patient was not successfully revascularized (technical success rate: 94.4%). The patient had no direct flow after ulnar artery angioplasty. The procedure was clinical effective in 15 patients (clinical success rate: 83.3%). Clinical evaluation and Duplex-US were performed in the follow up period. TcpO2 evaluated in the perilesional skin surface increased from 20.2 ± 6.5 mmHg to 53.8 ± 13.1 mmHg in the follow up period (P < .01). We observed an improvement of pain, ulcers and infection healing in all treated remaining patients during the postoperative period.

Percutaneous intervention prevents hand loss and functional impairment in patients with Critical Hand Ischemia and multiple comorbidities.

Glucocorticoids remain the cornerstone of medical therapy in giant cell arteritis (GCA) and should be started immediately to prevent severe consequences of the disease, such as blindness. However, glucocorticoid therapy leads to significant toxicity in over 80% of the patients. Various steroid-sparing agents have been tried, but robust scientific evidence of their efficacy and safety is still lacking. Tocilizumab, a monoclonal IL-6 receptor blocker, has shown promising results in a number of case series and is now being tested in a multi-centre randomized controlled trial. Other targeted treatments, such as the use of abatacept, are also now under investigation in GCA. The need for surgical treatment is rare and should ideally be performed in a quiescent phase of the disease. Not all patients follow the same course, but there are no valid biomarkers to assess therapy response. Monitoring of disease progress still relies on assessing clinical features and measuring inflammatory markers (C-reactive protein and erythrocyte sedimentation rate). Imaging techniques (e.g., ultrasound) are clearly important screening tools for aortic aneurysms and assessing patients with large-vessel involvement, but may also have an important role as biomarkers of disease activity over time or in response to therapy. Although GCA is the most common form of primary vasculitis, the optimal strategies for treatment and monitoring remain uncertain.

Large vessel vasculitis (LVV) covers a spectrum of primary vasculitides predominantly affecting the aorta and its major branches. The two main subtypes are giant cell arteritis (GCA) and Takayasu arteritis (TA). Less commonly LVV occurs in various other diseases. Clinical manifestations result from vascular stenosis, occlusion, and dilation, sometimes complicated by aneurysm rupture or dissection. Occasionally LVV is discovered unexpectedly on pathological examination of a resected aortic aneurysm. Clinical evaluation is often unreliable in determining disease activity. Moreover, the diagnostic tools are imperfect. Acute phase reactants can be normal at presentation and available imaging modalities are more reliable in delineating vascular anatomy than in providing reliable information on degree of vascular inflammation. Glucocorticoids are the mainstay of therapy of LVV. Patients may develop predictable adverse effects from long-term glucocorticoid use. Several steroid-sparing agents have also shown some promise and are currently in use. Endovascular revascularization procedures and open surgical treatment for aneurysms and dissections are sometimes necessary, but results are not always favorable and relapses are common. This article, the first in a series of two, will be devoted to GCA and isolated (idiopathic) aortitis, while TA will be covered in detail in the next article.

Upper/lower limb vasculitis has been considered an uncommon manifestation of giant cell arteritis, occurring in 3 to 16% of patients. Upper/lower limb vasculitis is still associated with significant morbidity, leading to limb/toe amputation in 5.6 to 15.8% of patients. Yearly clinical vascular examination should be performed systematically to screen upper/lower limb vasculitis at an early stage in patients with giant cell arteritis. Duplex ultrasound has proved to be a reliable non-invasive imaging method for detecting arterial stenoses of the upper/lower limbs in patients with giant cell arteritis. Patients with giant cell arteritis-related upper/lower limb vasculitis should undergo routine investigations to detect underlying aortic complications, concomitant aortic localizations being encountered in more than 50% of cases. Prednisone is the first-line therapy at an initial dose of 0.7-1 mg/kg daily. Prevention of platelet aggregation with low-dose aspirin is potentially effective in preventing ischemic complications of GCA. The indication of surgical therapy should be based on the severity of giant cell arteritis-related upper/lower limb clinical symptoms to avoid unnecessary morbidity in the course of interventional therapy.

We conducted this retrospective study to determine the prevalence of giant cell arteritis (GCA) in patients exhibiting nonatherosclerotic upper and/or lower extremity arterial involvement and to evaluate the clinical features and long-term outcome of those patients.From January 1997 to March 2008, 36 consecutive patients in the Department of Internal Medicine at the University of Rouen medical center received a diagnosis of symptomatic upper/lower extremity vasculitis related to GCA. In the 36 patients, upper/lower extremity vasculitis preceded the initial GCA diagnosis in 7 patients (19.4%), it was identified in association with GCA in 13 patients (36.1%), and it developed after the onset of GCA in the remaining 16 patients (44.4%). GCA clinical manifestations were severe resulting in ischemic complications of the extremities in 10 patients (27.8%). GCA-related large-vessel involvement was located in the upper extremity alone in 21 patients (58.3%), the lower extremity alone in 7 patients (19.4%), and both the upper and lower extremities in 8 patients (22.2%).Arterial involvement in GCA patients with upper extremity vasculitis was distributed in the subclavian (55.6%), axillary (47.2%), and brachial (22.2%) arteries. In patients with lower extremity vasculitis, involvement included the internal iliac artery (11.1%), common femoral artery (13.9%), superficial femoral artery (33.3%), deep femoral artery (5.6%), and popliteal and anterior tibial arteries (5.6%). Aortic localizations were common in GCA patients with upper/lower extremity vasculitis (68.9% of cases).All patients were given steroid therapy at a median daily dose of 1 mg/kg initially. Reconstructive study was performed in 10 patients (27.8%): venous bypass graft (n = 6), angioplasty (n = 1), thromboendarteriectomy (n = 2), or thrombectomy (n = 1); 2 other patients with extremity ischemia underwent amputation. The median observation time was 32 months; the outcome of upper/lower extremity vasculitis was disappearance of clinical symptoms (44.4%), improvement of clinical manifestations (44.4%), and deterioration of clinical manifestations (11.1%). At last follow-up, the median daily dose of prednisone was 6 mg. Steroid therapy could be discontinued in 12 patients (33.3%).We found that upper/lower extremity vasculitis is not uncommon in patients with GCA, and may be present in the early acute phase of GCA. Nevertheless, because upper/lower extremity vasculitis occurs during the course of GCA, yearly clinical vascular examinations may be adequate to screen for upper/lower extremity vasculitis at an early stage in GCA patients. Early diagnosis of GCA-related upper/lower extremity vasculitis is crucial, and can result in decreased severe ischemic complications. Because aortic localizations were common, GCA patients with upper/lower extremity vasculitis should undergo routine investigations for underlying life-threatening aortic complications (aortic ectasia/aneurysm). We also suggest that patients exhibiting aortic complications should undergo routine clinical vascular examination to detect upper/lower extremity vasculitis.

Although balloon angioplasty in heart and lower extremity vessels has been extensively studied and reported, little information exists regarding its use for digital ischemia in the hand. We report a case of successful balloon angioplasty of the distal radial artery to reverse present and prevent further digital tip cyanosis and necrosis.

To evaluate the outcome of balloon angioplasty in the arteries of the upper extremities in patients with giant-cell arteritis (GCA) and stenosing extracranial involvement.

Percutaneous transluminal angioplasty (PTA) for symptomatic upper limb artery stenoses (n = 29) and occlusions (n = 1) resistant to medical treatment was carried out in 10 patients (all women, mean age 65 years) with GCA. Vascular lesions were located in the subclavian (n = 4), axillary (n = 10) and brachial (n = 16) arteries. Interventional treatment was accompanied by immunosuppressive drugs in all patients. Follow-up included clinical and serological examination, magnetic resonance angiography and colour duplex ultrasound.

Initial technical success of angioplasty was achieved in the case of all vascular lesions. In five patients, marked recurrent stenoses (vascular territories; n = 10/30) were found during follow-up (mean 24 months). The cumulative primary patency rate was 65.2%. All recurrent lesions developed in the territories of the initial long-segment stenoses. Repeated PTA (vascular territories, n = 8; patients, n = 5) provided a cumulative secondary patency rate of 82.6% and a cumulative tertiary patency rate of 89.7%.

Despite a tendency to restenoses, balloon angioplasty of the upper-extremity artery, in combination with immunosuppressive treatment, is an efficient method for the treatment of extracranial GCA.

Because the prognosis of giant cell arteritis (GCA) is related to the development of ischemic complications, we sought to assess the possible influence of traditional risk factors of atherosclerosis in the development of severe ischemic complications of GCA. We conducted a retrospective study of patients with biopsy-proven GCA diagnosed from 1981 to 2001 at the single hospital for a well-defined population of almost 250,000 people. Patients were considered to have severe ischemic manifestations if they suffered visual manifestations, cerebrovascular accidents, jaw claudication, or signs of occlusive changes in large arteries of the extremities. Patients were assessed for the presence of hypercholesterolemia, hypertension, diabetes mellitus, and heavy smoking at the time of GCA diagnosis. The presence of traditional risk factors of atherosclerosis at the time of GCA diagnosis in this series of 210 patients increased significantly the risk of developing at least 1 of the severe ischemic complications (odds ratio [OR], 1.79; 95% confidence intervals [CI], 1.03-3.11; p = 0.04). Patients with traditional atherosclerosis risk factors had fever less commonly than the rest of GCA patients (5.2% vs. 16.0%; p = 0.01). GCA patients with hypertension exhibited a significantly increased risk of developing severe ischemic complications (OR, 1.80; 95% CI, 1.00-3.25; p = 0.05). The current study suggests that the presence of atherosclerosis risk factors at the time of diagnosis of GCA may influence the development of severe ischemic manifestations of the disease.

Giant cell arteritis or temporal arteritis occurs almost exclusively in people over 50 years of age. It classically presents with new onset temporal headache, scalp tenderness and jaw claudication. Proximal muscle pain and stiffness is often present because of frequent association with polymyalgia rheumatica. In most cases, the erythrocyte sedimentation rate is markedly elevated. Uncommon presentations include systemic symptoms and symptoms related to large artery involvement. We report a case of giant cell arteritis without symptoms related to the temporal artery, diagnosed angiographically following upper limb claudication and confirmed by temporal artery biopsy.

The vasculitides constitute a heterogeneous group of diseases characterized by blood vessel inflammation and necrosis with different but frequently overlapping clinical and pathologic manifestations. The incidence of these conditions is frequently controversial. To further investigate the incidence and clinical manifestations of vasculitides, we reviewed the spectrum of these diseases in an unselected population of adults (age > 20 years) from northwestern Spain during a 10-year period. From January 1988 through December 1997, 267 adults were diagnosed as having vasculitis. The overall average annual incidence rate of vasculitis in the region of Lugo, Spain, between 1988 and 1997 for the population older than 20 years was 141.54/million. Primary vasculitis (115.04/million for the population older than 20 years; 81.3%), especially giant cell arteritis (GCA) was the most common group. Small vessel primary vasculitis (hypersensitivity vasculitis and Henoch-Schönlein purpura) was the second most common group. Both GCA and small vessel primary vasculitis had a good outcome. However, although less common, patients with medium and small vessel primary vasculitis, in particular those with polyarteritis nodosa, had a high mortality related to the systemic manifestations of the disease or to the immunosuppressive therapy. Among the group of adults with secondary vasculitis (26.51/million; 18.7%), rheumatic diseases and specifically those occurring in the context of rheumatoid arthritis were the most common group. Patients with secondary vasculitis had clinical or laboratory data that may suggest the presence of an underlying disease. In summary, systemic vasculitides are somewhat more common than previously considered. As in other western countries, GCA constitutes the most common type of vasculitis in northwestern Spain. Better physician awareness may contribute to the progressive increase in the recognition of these conditions.