Ulnar artery involvement in systemic sclerosis (scleroderma)

OBJECTIVE: Microvascular disease is one of the hallmarks of systemic sclerosis (SSc, scleroderma), but macrovascular involvement also exists in some patients. Patients with SSc may have severe Raynaud's phenomenon (RP) characterized by refractory digital ulcerations. We investigated if large artery involvement, that is, ulnar artery occlusion, has a role in the development of refractory digital ulcerations, and if both screening for this involvement and revascularization of the ulnar artery occlusive disease may improve digital ulcer healing. METHODS: A retrospective chart review was performed of 15 patients with SSc, all of whom had severe RP and digital ulceration, together with a positive Allen test and ulnar artery occlusive disease documented by angiography. RESULTS: Women outnumbered men 2:1, with limited disease predominating (7), 5 patients having diffuse cutaneous disease and 3 overlap syndromes. All patients had positive antinuclear antibody and capillary microscopy findings consistent with SSc. Antiphospholipid antibodies were present in 4 of 6 patients tested. Tobacco use was seen in 5 patients, only 2 of whom were current smokers. All patients failed conventional medical therapy (nitrates, calcium channel blockers, antiplatelet agents) for RP and digital ulceration. Only 1/8 patients improved with stellate ganglion block, and one patient had no improvement following digital sympathectomy. Eight patients underwent ulnar artery revascularization combined with digital sympathectomy, and 8 experienced dramatic improvement in RP and healing of digital ulcers. CONCLUSION: An Allen test should be performed routinely on all SSc patients with severe RP and refractory digital ulceration to investigate the possibility of ulnar artery occlusive disease. If suspected ulnar artery occlusion is confirmed by angiography or ultrasonography, ulnar artery revascularization with or without digital sympathectomy should be considered in patients who fail conventional medical therapy.     

Successful percutaneous angioplasty and stenting of the radial artery in a patient with chronic upper extremity ischemia and digital gangrene.

PURPOSE: To report percutaneous treatment of a chronic radial artery occlusion in a multimorbid patient with ischemic tissue loss. CASE REPORT: A 62-year-old man with multiple comorbidities, including renal failure and severe coronary artery disease, presented with painful, ulcerated lesions of his right hand. He has severe peripheral vascular disease, with a history of 4 digital amputations of the left hand, a right above-knee amputation, and a left femoral to peroneal artery bypass. Arteriography demonstrated chronic occlusion of the radial and ulnar arteries, with a patent interosseous and collateral flow to the distal radial artery filling the palmar arch. Angioplasty and stenting of the radial artery was performed, relieving the patient's symptoms and allowing the lesions to heal. CONCLUSION: Percutaneous intervention can treat severe upper extremity ischemia with gangrene in patients with severe chronic ischemia and multiple comorbidities.     

Discrepancy between simultaneous digital skin microvascular and brachial artery macrovascular post-occlusive hyperemia in systemic sclerosis

OBJECTIVE: Vascular impairment, a main feature of the pathogenesis of systemic sclerosis (SSc), involves both the macro- and the microvasculature. We compared and correlated simultaneously measured skin microvascular and brachial artery macrovascular post-occlusive hyperemia in 3 groups: patients with SSc, patients with primary Raynaud's phenomenon (RP), and healthy volunteers. METHODS: Thirty-three healthy volunteers, 36 patients with primary RP, and 42 patients with SSc were enrolled. For each subject, brachial artery flow-mediated dilation (FMD) and cutaneous post-occlusive reactive hyperemia (PORH) were simultaneously recorded after 5-minute occlusion of the brachial artery. Local thermal hyperemia, nitroglycerin-mediated dilation (NMD), intima-media thickness (IMT), and pulse wave velocity (PWV) were also assessed. RESULTS: Digital cutaneous peak PORH was altered in patients with primary RP and SSc compared to healthy controls, whereas FMD was not significantly different among all groups. We observed a correlation between digital peak cutaneous vascular conductance and brachial FMD in healthy controls (r = 0.49; p = 0.004), but not in patients with primary RP or SSc. Thermal hyperemia was altered only in patients with SSc. Brachial NMD, IMT, and PWV were not different among all groups. CONCLUSION: We observed a loss of the correlation between brachial FMD and digital cutaneous PORH in patients with SSc and primary RP. Microvascular function is impaired in SSc, whereas brachial artery endothelial function is normal.     

In systemic sclerosis macrovascular damage of hands digital arteries correlates with microvascular damage

ObjectiveTo assess morphology and blood flow of the proper palmar digital arteries (PPDA) by Color Doppler Ultrasonography (CDUS) and its relationship with nailfold videocapillaroscopy (NVC), skin blood perfusion and digital arteries pulsatility of hands in SSc patients and healthy controls.MethodsCDUS, NVC, Laser Doppler Perfusion Imaging (LDPI) and photoplethysmography (PPG) were performed in 36 systemic sclerosis (SSc) patients and 20 healthy controls.ResultsCDUS was pathologic in 69% of patients with SSc and in none of healthy controls (p < 0.0001). SSc patients with low vascular damage (early capillaroscopic pattern) have a normal morphology of PPDA, but the blood flow, evaluated by peak systolic velocity (PSV) and end diastolic velocity (EDV), is reduced and vascular resistance, measured by resistive index (RI) and pulsatility index (PI), increased. At this stage the LDPI mean perfusion and digital artery pulsatility, evaluated by PPG, were reduced. The US changes appear with microvasculare damage progression (active and late capillaroscopic patterns), while the PPDA blood flow progressively decreases (PSV and EDV decreased, RI and PI increased). The macrovascular damage correlates with disease duration. Anti-topoisomerase I represents an independent predictive factor for macrovascular damage. We not observed any association between digital ulcer history, pulmonary fibrosis and US findings.ConclusionPPDA blood flow dysfunction is already present in early disease. Structural macrovascular damage progresses with worsening of SSc microangiopathy.     

In systemic sclerosis macrovascular damage of hands digital arteries correlates with microvascular damage

Objective

To assess morphology and blood flow of the proper palmar digital arteries (PPDA) by Color Doppler Ultrasonography (CDUS) and its relationship with nailfold videocapillaroscopy (NVC), skin blood perfusion and digital arteries pulsatility of hands in SSc patients and healthy controls.

Methods

CDUS, NVC, Laser Doppler Perfusion Imaging (LDPI) and photoplethysmography (PPG) were performed in 36 systemic sclerosis (SSc) patients and 20 healthy controls.

Results

CDUS was pathologic in 69% of patients with SSc and in none of healthy controls (p < 0.0001). SSc patients with low vascular damage (early capillaroscopic pattern) have a normal morphology of PPDA, but the blood flow, evaluated by peak systolic velocity (PSV) and end diastolic velocity (EDV), is reduced and vascular resistance, measured by resistive index (RI) and pulsatility index (PI), increased. At this stage the LDPI mean perfusion and digital artery pulsatility, evaluated by PPG, were reduced. The US changes appear with microvasculare damage progression (active and late capillaroscopic patterns), while the PPDA blood flow progressively decreases (PSV and EDV decreased, RI and PI increased). The macrovascular damage correlates with disease duration. Anti-topoisomerase I represents an independent predictive factor for macrovascular damage. We not observed any association between digital ulcer history, pulmonary fibrosis and US findings.

Conclusion

PPDA blood flow dysfunction is already present in early disease. Structural macrovascular damage progresses with worsening of SSc microangiopathy.     

Pathophysiology and clinical consequences of Raynaud's phenomenon related to systemic sclerosis

According to the so-called vascular hypothesis, Raynaud's phenomenon (RP) is one initial event in the pathophysiological cascade leading to sclerosis in systemic sclerosis (SSc). It is characterized by recurrent, reversible spasms of small arterioles and digital arteries, usually triggered by cold and emotional stress. Clinical signs of RP are a sudden pallor of single digits of fingers followed by reactive hyperaemia and in severe cases also by cyanosis. Besides imbalances between vasoconstrictive and vasodilatory processes, structural alterations of the involved vessels are fundamental to secondary RP in SSc. The latter is the reason why secondary RP in SSc, in contrast to primary RP, often leads to ischaemia and re-perfusion injuries. New insights into the pathophysiology of RP feature a special role for alpha2c-adrenoreceptors, Rho-kinase signalling pathways and soluble mediators. They have resulted in promising therapeutic options, including antagonism of endothelin receptors, inhibition of phosphodiesterases or selective blockade of alpha2c-adrenoreceptors. They should also have a positive impact on the course of SSc in general.     

Hypothenar hammer syndrome. Form of reversible Raynaud's phenomenon

Hypothenar hammer syndrome is a reversible yet uncommonly encountered cause of Raynaud's phenomenon. Characteristic findings include coldness in the dominant hand of a male, absence of triphasic color change and thumb involvement, and occupational or recreational use of the hand as a hammer. Angiography demonstrates the specific findings of irregularity or occlusion of the ulnar artery, downstream occluded proper digital arteries, and intraluminal emboli at the sites of distal obstruction. A vulnerable area of traumatic occlusion is provided by the anatomic relationship of the superficial branch of the ulnar artery to the hamate bone and the palmar aponeurosis. Pathologic studies separate the hypothenar hammer syndrome from clinically similar vasculitis. It is important to recognize the hypothenar hammer syndrome as a distinct entity because treatment is curative and consists of resection of the affected vascular segment and avoidance of the aggravating conditions.     

Increased circulating platelet-leucocyte complexes in patients with primary Raynaud's phenomenon and Raynaud's phenomenon secondary to systemic sclerosis: a comparative study.

Platelet activation and circulating platelet-leucocyte complexes increase in vascular ischemic events and autoimmune inflammatory diseases. Platelet activation markers and platelet-leucocyte complexes were evaluated in primary Raynaud's phenomenon (RP) and in RP secondary to systemic sclerosis (SSc). Whole-blood flow cytometry was utilized to quantify CD62P, platelet microparticles (PMP), platelet-monocyte complexes (PMC) and platelet-neutrophil complexes (PNC) in primary RP and in SSc patients with secondary RP. SSc patients with secondary RP had significantly higher platelet CD62P expression than primary RP patients and controls (P = 0.017 and 0.004, respectively). Primary and secondary RP patients had higher mean PMC and PNC levels than controls (all P < or = 0.001). PMP level in SSc patients with pulmonary hypertension was significantly higher than in others (P = 0.048). All parameters were similar in SSc patients with and without digital ulcers, aspirin-users and nonusers (P > 0.05). CD62P level decreased significantly after iloprost administration in four patients with digital ulcers (16.1 +/- 17.4 vs 7.4 +/- 3.8%, P = 0.03). Our results suggest there is platelet-leucocyte complex formation in RP, and, despite antithrombotic therapy, platelet activation and platelet-leucocyte interaction are ongoing in SSc. This is important as it might have potential therapeutic implications with respect to using antiplatelet drugs in SSc.     

Digital arteriopathies of the arms and their treatment by posterior spinal cord neurostimulation]

Digital arteriopathies of the upper limbs are distinguished from those of the lower limbs by their rarity and their particular etiologies. Digital arteriopathies are defined as the presence of organic lesions causing stenosis and obstruction of the distal upper limb arteries: radial arteries, ulnar arteries, palmar arches, digital arteries.     

Relative roles of endothelial cell damage and platelet activation in primary Raynaud's phenomenon (RP) and RP secondary to systemic sclerosis.

OBJECTIVE: To evaluate the relative roles of endothelium and platelets in the pathogenesis of primary RP and RP secondary to SSc. METHODS: Endothelial derived ET-1, t-PA, PAI-1, and platelet derived beta-TG, PDGF, TGF-beta were measured in 36 patients with primary RP, 14 patients with RP secondary to SSc and 30 age and sex matched controls. RESULTS: A significative increase of ET-1, t-PA, PAI-1, TGF-beta, and beta-TG were the most relevant changes in patients with RP secondary to SSc with respect to the controls. Less relevant increases of t-PA, PAI-1, PDGF, and beta-TG levels were observed in patients with primary RP vs controls. CONCLUSIONS: These data seem to confirm the involvement of endothelial cells and platelets in the pathogenesis of RP, with mild changes in primary RP and more relevant changes in RP secondary to SSc.     

Calcium-channel blockers for Raynaud's phenomenon in systemic sclerosis

OBJECTIVE: Most patients with systemic sclerosis (SSc) have Raynaud's phenomenon (RP), which is often more severe than idiopathic RP. This study was a meta-analysis to determine the efficacy of calcium-channel blockers for the treatment of RP in SSc. The primary outcome measures were frequency and severity of ischemic attacks, digital skin temperature, patient and physician global assessments, and digital ulcers. METHODS: The Cochrane search strategy was used to ascertain all trials in all languages. Primary data sources included Medline, Current Contents, and the Cochrane Controlled Trials Register. Studies that met the inclusion criteria were randomized controlled trials of >2 days' duration with a dropout rate of <35%. Twenty-nine studies were found, of which 8 randomized controlled trials were eligible for inclusion. The total number of patients included was small (n = 109). Most trials included primary and secondary RP, and the main reasons for trial exclusion were inability to extract subset data on SSc patients (18 trials), data published previously (2 trials), and lack of a control group (1 trial). Data were abstracted independently by 2 reviewers, and either a weighted mean difference (WMD) or a standardized mean difference (SMD) was calculated for all continuous outcomes; however, information was not available for all outcomes within trials. RESULTS: The WMD of all calcium-channel blockers versus placebo (6 trials) and of nifedipine alone versus placebo (5 trials) for the reduction in the frequency of ischemic attacks over a 2-week period was -8.31 (95% confidence interval [95% CI] -15.71, -0.91) and -10.21 (95% CI -20.09, -0.34), respectively. The SMD of all calcium-channel blockers versus placebo (3 trials) and of nifedipine alone versus placebo (2 trials) for the reduction in the severity of ischemic attacks was -0.69 (95% CI -1.21, -0.17) and -0.99 (95% CI -1.74, -0.24), respectively. CONCLUSION: Calcium-channel blockers for RP in SSc have been tested in several small clinical trials and appear to lead to significant clinical improvement in both the frequency and the severity of ischemic attacks. Most trials were crossover trials in which order effect was not studied. This could have introduced bias. The results of this study suggest that the efficacy of calcium-channel blockers in reducing the severity and frequency of ischemic attacks in RP secondary to SSc is moderate at best (mean reduction of 8.3 attacks in 2 weeks and 35% less severity), and a further large, randomized controlled trial needs to be conducted.     

Long-term follow-up of hypothenar hammer syndrome: a series of 47 patients

Hypothenar hammer syndrome (HHS) is an uncommon form of secondary Raynaud phenomenon, occurring mainly in subjects who use the hypothenar part of the hand as a hammer; the hook of the hamate strikes the superficial palmar branch of the ulnar artery in the Guyon space, leading to occlusion and/or aneurysm of the ulnar artery. In patients with HHS, such injuries of the palmar ulnar artery may lead to severe vascular insufficiency in the hand with occlusion of digital artery. To date, only a few series have analyzed the long-term outcome of patients with HHS. This prompted us to conduct the current retrospective study to 1) evaluate the prevalence of HHS in patients with Raynaud phenomenon and 2) assess the short-term and long-term outcome in patients with HHS.From 1990 to 2006, 4148 consecutive patients were referred to the Department of Internal Medicine at the University of Rouen medical center for evaluation of Raynaud phenomenon using nailfold capillaroscopy. HHS was diagnosed in 47 of these 4148 patients (1.13% of cases).Forty-three patients (91.5%) had occupational exposure to repetitive palmar trauma. The more common occupations were factory worker (21.3%), mason (12.8%), carpenter (10.6%), and metal worker (10.6%); the mean duration of occupational exposure to repetitive palmar trauma at HHS diagnosis was 21 years. One patient (2.1%) had recreational exposure (aikido training) to repetitive trauma of the palmar ulnar artery, and 3 other patients (6.4%) developed HHS related to a single direct injury to the hypothenar area. Clinical manifestations were more often unilateral (87.2%) involving the dominant hand (93%). HHS complications included digital ischemic symptoms (ischemia: n = 21, necrosis: n = 20) and irritation of the sensory branch of the ulnar nerve (n = 11). In HHS patients, angiography demonstrated occlusion of the ulnar artery in the area of the Guyon space (59.6%), aneurysm of the ulnar artery in the area of the Guyon space (40.4%), and embolic multiple occlusions of the digital arteries (57.4%).All patients were advised to change their occupational exposure. They were given vasodilators, including calcium channel blocker (n = 37) and buflomedil (n = 12); 36 patients (76.6%) also received oral platelet aggregation inhibitors. Twenty-one patients with digital ischemia/necrosis were further given hemodilution therapy to reduce the hematocrit level to 35%. In 3 patients with HHS-related digital necrosis who exhibited partial improvement with vasodilators, prostacyclin analog therapy (a 5-day regimen of intravenous prostacyclin analog) was instituted, resulting in complete healing of digital ulcer in these 3 patients. Other conservative treatment options included controlling risk factors (smoking cessation, low-lipid diet, therapy for arterial hypertension) and careful local wound care of fingers in the 20 patients with digital necrosis. Only 2 patients, exhibiting digital necrosis and multiple digital artery occlusions, with nonthrombotic ulnar artery aneurysm underwent reconstructive surgery, that is, resection of the aneurysm with end-to-end anastomosis of the ulnar artery. The median length of follow-up in patients with HHS was 15.9 months. Thirteen patients (27.7%) exhibited clinical recurrences of HHS; the median time of HHS recurrence onset was 11 months. Outcome of HHS relapse was favorable with conservative measures in all cases.Awareness of HHS is required to increase suspicion of the disorder so that further exposure to risk factors like repetitive hypothenar trauma can be avoided for these patients; this is of great importance for their overall prognosis. We found favorable outcomes in most patients after conservative measures were initiated; therefore we suggest that surgery may be undertaken in the subgroup of patients who exhibit partial improvement while receiving conservative therapy. Finally, because we observed recurrence of HHS in 27.7% of patients, we note that HHS patients require close follow-up, including both regular and systematic physical vascular examination.     

White fingers after excessive motorcycle driving: a case report

An 18-year-old male presented with Raynaud's phenomenon which was found to be caused by occlusion of the proper palmar digital arteries on the right hand and obstruction of the superficial palmar arterial arch on the left hand. These lesions in the arteries of both hands resemble those found in patients with vibration-induced white fingers such as in mine or foundry workers. The only likely cause for the pathological vascular findings in our patient was an exposure to vibration due to excessive off-street motorcycle driving. Therapy with intraarterial prostaglandins resolved the ischemic syndrome but it promptly recurred when the patient resumed motor cycle driving. Therefore, we suggest that excessive cross country motor cycle driving may cause vibration-induced white fingers.     

Prévalence de l’atteinte artérielle macrovasculaire des 4 membres dans la sclérodermie systémique : à propos de 14 patients

Introduction

Trophic disorders of the extremities are a common complication of systemic sclerosis (SSc), mainly related to microvascular damage. However, SSc seems to be a risk factor for premature athero-thrombotic disease that can affect the peripheral arteries, participate in the occurrence of trophic disorders and promote the occurrence of infectious complications. The objective of this study was to assess the prevalence of arterial disease of the limbs in SSc patients.

Transulnar cardiac catheterization and percutaneous coronary intervention: techniques,transradial comparisons,anatomical considerations,and comprehensive literature review

Ulnar arterial access for cardiac catheterization and intervention is an alternative approach compared with radial or femoral access. Ulnar access is infrequently performed since the radial artery is readily palpable and is commonly used worldwide to minimize vascular complications from femoral access. Nevertheless, ulnar access provides a suitable access site in patients who are poor candidates for femoral access, have pre‐existing radial occlusion, radial artery hypoplasia or hyperplasia from prior radial artery procedures, radial stenosis, radial loops, radial tortuosity, small radial arteries, and/or have future need for radial graft for dialysis or coronary artery bypass graft. Furthermore, femoral access is the standard default option if radial access fails. Consequently, learning ulnar access provides a suitable forearm alternative to avoid femoral access when deemed high risk or undesirable. This review discusses the techniques of ulnar access, advantages and disadvantages of ulnar versus radial access, the clinical trials on ulnar cardiac catheterization and its associated complications.     

Digital flow exploration by color Doppler ultrasound in patients with Raynaud's disease or systemic sclerosis

BACKGROUND: Raynaud's phenomenon (RP) is a frequent vascular paroxysmal syndrome of the extremities. Generally benign, the condition is called Raynaud's disease (RD), which may reveal a connective tIssue disease, particularly systemic sclerosis (SS). We evaluated digital blood flow in patients with RD and SS using color Doppler ultrasound. PATIENTS AND METHODS: Ultrasound examination was performed with a newly developed multi-D linear array transducer (VFX 13-5 Siemens), which allows better resolution. We first measured the diameters of the digital arteries (appendix 1) then epidermal, dermal, and hypodermal thickness for each patient (appendix 3) and performed a qualitative and quantitative analysis of pulpar microcirculation (appendix 4 & 5). All measures were made at 25 degrees C, 45 degrees C, 11 degrees C and after recovery. Thirty-three patients were included: 14 with primary RD and 19 with SS as assessed by American College of Rheumatology criteria. RESULTS: The diameters of the digital arteries showed significant discrepancies allowed to distinguish primary RP from SS. At 11 degrees C, diameters were 0.6 +/- 0.2 mm for primary RP versus 0.2 +/- 0.3 mm for SS on lateral digital arteries (LDA) [p=0.005]; 0.7 +/- 0.2 mm for primary RP versus 0.4 +/- 0.3 mm for SS on medial digital arteries (MDA) [p=0.004]. After recovery, these diameters were respectively 1 +/- 0.2 mm versus 0.5 +/- 0.4 mm for LDA [p=0.000] and 1 +/- 0.2 mm versus 0.6 +/- 0.3 mm for MDA [p=0.000] (tables 1, 2, 3, 4). Similarly, pulpar vascularization was significantly higher in primary RP than in SS (tables 6, 7, 8). No difference was found in skin thickness nor in the epidermal aspect between the two groups (table 5). CONCLUSION: Color Doppler ultrasound shows morphological and dynamic differences between RD and SS.     

Significant attenuation of macrovascular involvement by bosentan in a patient with diffuse cutaneous systemic sclerosis with multiple digital ulcers and gangrene

Abstract

Systemic sclerosis (SSc) is characterized by vascular injuries, and bosentan has recently been proved to be efficacious for the prevention of new digital ulcers in SSc. We herein report a case of SSc in a patient with refractory digital ulcers and gangrene treated with bosentan. Stenosis of the ulnar artery, evaluated by magnetic resonance angiography, was attenuated by the bosentan treatment, suggesting that bosentan exerts a reverse remodeling effect against the pathological organic changes of arteries in SSc.     

Management of cutaneous vascular complications in systemic scleroderma: experience from the German network

Major cutaneous vascular complications of systemic sclerosis (SSc) are secondary Raynaud's phenomenon (RP) and digital ulcers. Even though SSc is a rare disease, timely and appropriate management of its vascular complications is mandatory for reducing the rate of major impairment. It should therefore be aware to physicians from different specialties. We evaluated the diagnostic and therapeutic approach toward secondary RP and ulceration in SSc patients at 28 German clinical centers at the time of initiation of the German network for Systemic Scleroderma (DNSS). We retrieved data via questionnaires and from the DNSS patient registry. Management of RP and ulcerations in SSc were heterogeneous at initiation of the network, reflecting a sometimes insufficient use of the diagnostic and therapeutic possibilities. As such, (1) calcium channel blockers were the first line therapy in most centers; but often in insufficient dosages, and (2) only 21.2% of patients with acral ulceration had received prostacyclins when recruited into the network. The sometimes insufficient care of vascular complications of SSc in Germany revealed the need for their standardized management, e.g. within a network for SSc and for consensus on a diagnostic or therapeutic algorithm.     

Hand vascular involvement assessed by magnetic resonance angiography in systemic sclerosis

OBJECTIVE: Impairment of the microcirculation is a cardinal feature of systemic sclerosis (SSc). Magnetic resonance angiography (MRA) has improved the assessment of vascular lesions of the hand. The aim of this study was to evaluate vascular abnormalities in the hands of patients with SSc, using MRA. METHODS: Thirty-eight patients with SSc were compared with 7 healthy subjects and 7 patients with rheumatoid arthritis. Among patients with SSc, the mean +/- SD age was 52 +/- 14 years, the mean +/- SD Health Assessment Questionnaire (HAQ) score was 0.9 +/- 0.8, and the mean +/- SD systolic pulmonary artery pressure (PAP) was 32.2 +/- 8.4 mm Hg. Ten patients had a history of digital ulcers. The MRA protocol consisted of 4 successive acquisitions, each lasting 52 seconds, of 3-dimensional coronal cross-sectional images after gadolinium injection. The primary criteria were distality and quality of arterial opacification, avascular areas, and venous return. RESULTS: Thirty-five of the patients with SSc (92%) had at least 1 true digital artery that did not reach the first phalanx at the initial arterial analysis, and 23 patients (61%) had > or =4 damaged arteries. Twenty-eight patients (74%) had thin arteries, and 20 patients (53%) had >1 avascular area. Nearly all patients (35 of 38 [92%]) had abnormal venous return, and a lack of visible venous return was observed in 16 patients (42%). Results for all of the control subjects were considered normal. Digital ulcers were more frequently observed in patients with SSc who had > or =4 damaged proper digital arteries compared with other patients (P = 0.003), the HAQ score was associated with thin-caliber arteries (P = 0.04), and systolic PAP was associated with tissue enhancement secondary to ischemia (P = 0.04). CONCLUSION: These results show the substantial vascular involvement in SSc. Lesions were diffuse and involved both arterial and venous vessels of small caliber as well as the microcirculation.     

Successful treatment of patients with severe secondary Raynaud's phenomenon with the endothelin receptor antagonist bosentan

Secondary Raynaud's phenomenon (RP) in patients with systemic sclerosis (SSc) and other collagen vascular diseases is a serious manifestation of microvascular damage that may precede the onset of visceral and/or cutaneous sclerosis for years. Recent studies have demonstrated that the endothelin receptor antagonist bosentan prevents the development of new digital ulcers in SSc. We investigated the potential benefits of bosentan in patients with secondary RP associated with pre-scleroderma and with SSc, independent of digital ulcers. Three patients with secondary RP received bosentan 62.5 mg twice daily for 4 weeks followed by 125 mg twice daily for 12 weeks during the winter season. Pain (visual analogue scale), Raynaud's disease activity (Scleroderma Health Assessment Questionnaire), number and severity of daily Raynaud's attacks (diary) and peripheral thermoregulation (thermography) were assessed during treatment periods. Pain, Raynaud's disease activity, number and severity of Raynaud's attacks significantly decreased during treatment periods. Thermography after 16-week treatment demonstrated improved peripheral thermoregulation. Although this is a small observational study, treatment with bosentan appears to measurably reduce the daily impact of Raynaud's disease and improve peripheral thermoregulation in patients with secondary RP, independent of digital ulcers.