Thoracic lymphadenopathies in diffuse systemic sclerosis: an observational study on 48 patients using computed tomography.

BACKGROUND: Thoracic multidetector computed tomography (MDCT) is essential for the detection of interstitial lung disease (ILD) in patients with systemic sclerosis (SSc). Thoracic MDCT assessment can reveal the presence of thoracic lymphadenopathies (LAP) whose signification remains uncertain. The purpose of the study was to describe the characteristics and to assess the significance of thoracic LAP in patients with diffuse SSc. METHODS: We conducted a monocentric observational study on adult patients with diffuse SSc, and collected general patient and first thoracic MDCT characteristics, PET-CT and outcome data. Comparisons were made between patients with and without thoracic LAP. RESULTS: Forty-eight patients were included. There were 30 patients (62.5%) with an ILD and 23 (48%) with at least one thoracic LAP on the first MDCT assessment. Median number per patient of thoracic LAP was 3 [1-8], with a mean size of 11.7 ± 1.7 mm, mainly located in right para-tracheal area (22.8% of the total number of LAP), right hilar area (20.3%), left hilar area (6.5%), and sub-carinal area (15.2%). PET-CT showed lymph node hypermetabolism in 11/15 patients (73.3%) with mean SUVmax at 4 ± 1.3. There were significantly more males (p = 0.002) and more patients exposed to silica (p = 0.001) in patients with thoracic LAP. ILD was significantly more extended according to Goh score (p = 0.03), and using semi-quantitative score for mixed ground-glass reticulation (p = 0.01) and global abnormalities (p = 0.03) in patients with thoracic LAP and ILD. Thirteen patients (27.1%) died during follow-up without significant difference according to the presence or not of thoracic LAP (p = 0.15). There was also no significant difference concerning immunosuppressive treatment initiation (p = 0.17). CONCLUSIONS: Thoracic LAP are common in diffuse SSc and are generally multiple, not bulky, moderately hypermetabolic, and located at the base of the mediastinum lymph node chains. Their presence correlates with the extent of ILD. In absence of ILD, thoracic LAP presence seems to be often explained by silica exposure. TRIAL REGISTRATION: NA.

Cochleovestibular involvement in patients with Fabry disease: data from the multicenter cohort FFABRY.

PURPOSE: Fabry disease (FD) is a lysosomal storage disease responsible for cochleovestibular involvement. Exact prevalence and pathophysiological mechanisms behind ENT affections are still poorly known. Treating FD with enzyme replacement therapy (ERT) does not seem to significantly improve the ENT symptoms, while the impact of migalastat has yet to be determined. METHODS: We carried out a retrospective multi-centre study on 47 patients from the FFABRY cohort who had an ENT consultation in the context of their FD. The information collected were as follows: clinical examination, videonystagmoscopy, pure-tone speech audiometry, videonystagmography or VHIT (Video Head Impulse Test). Severe hearing loss was defined as greater than 70 dB. RESULTS: The median age of our cohort was 52 years with a non-negligible proportion of non-classic variants and female carriers. 72.3% of the patients complained of at least one of the following symptoms: hearing loss, tinnitus or vertigo. Pure-tone audiometry was abnormal in 61.7% of the patients (29/47), while speech audiometry was abnormal for 41.7% of the patients. The age of the patients and hypertrophic cardiomyopathy were significantly associated with the existence of an anomaly in pure-tone audiometry results. Severe hearing loss (> 70 dB) was significantly more common in male patients. DISCUSSION: Hearing loss is particularly frequent in FD and is not limited to classic phenotypes. Close ENT follow-up is essential for Fabry patients to detect those who might benefit from hearing aid. Further studies are needed to define the impact of migalastat on cochleovestibular symptoms.

Effect of cannabis consumption on characteristics and evolution of thromboangitis obliterans.

Background: Thromboangiitis obliterans (TAO) is a distal non atherosclerotic thrombotic vasculitis affecting tobacco smokers. The role of cannabis co-exposure remains controversial. The study aims to assess how cannabis consumption influences clinical presentation and outcome of TAO in tobacco smokers. Patients and methods: TAO patients, according to Papa's criteria, were included in a retrospective bicentric study between the 1st January 2003 and the 1st march 2020. Clinical characteristics, arterial involvement at TAO diagnosis, vascular event and amputations during follow-up were analyzed according to cannabis consumption. Results: Seventy-three patients with TAO patients were included. Forty-five patients were in Tobacco group (T) and 28 in Tobacco and cannabis group (T&C). Tobacco exposure was less important in T&C group than in T group (19.4±11.3 vs 31.6±16.6 pack-years) (p=0.005) and patients in T&C group were younger at TAO diagnosis than in T group (p=0.008). Patients in T&C group presented more claudication (33.3% vs 8.9%, p=0.01) and less upper limbs resting ischemia (25.9% vs 51.1%, p=0.04) than patients in the T group. No differences were found between groups with regard to arterial distribution. Amputation rate for patients who had at least one major or minor amputation did not differ between T and T&C group (25% vs 14.8%, p=0.38). Conclusions: Cannabis consumption was associated with a younger age of TAO onset. However, it does not affect amputation-free survival, Tobacco exposure is less important in T&C patients; data of this bicentric study suggest that cannabis could be a cofactor of tobacco which accelerates TAO onset.

An unexpected etiology of priapism: infection-related anti-protein s antibodies.

INTRODUCTION: In adolescents, the occurrence of priapism is commonly related to sickle cell disease and rarely to other causes. We hereby report a case of priapism due to an acquired protein S (PS) deficiency. AIM: The aim of this study was to describe a young man who developed a priapism with a thrombosis of the corpora cavernosa associated with an anti-PS antibody (anti-PS Ab). METHODS: One week after the onset of an influenza-like illness, a young male developed multiple extensive venous thromboses including a thrombosis of the corpora cavernosa causing painful partial priapism. These thromboses along with purpuric lesions with necrotic vesicles of the feet skin were linked to an acquired PS deficiency due to an anti-PS Ab. The optimal treatment of anti-PS Ab-associated thrombosis is debated but we chose to initiate (i) heparin; (ii) corticosteroids; and (iii) plasmapheresis. RESULTS: Even if priapism lasted more than 4 days, a full recovery of erectile function was observed within 3 months. As compared with priapism due to sickle cell disease, which is commonly associated with definitive erectile dysfunction, this favorable outcome is noteworthy. The skin healing was complete only 6 months later. CONCLUSION: Acquired PS deficiency complicating an infectious disease is a rare, life-threatening condition, associated with substantial morbidity related to amputations of limbs or digits. This is the first report of priapism due to acquired PS deficiency.

Cryoablation of Soft Tissues Low-Flow Vascular Malformations: Clinical Outcomes and Safety.

OBJECTIVE: To report the clinical effectiveness and the safety of cryoablation in first or second-line therapy in symptomatic soft tissues vascular malformation, a mini-invasive therapeutic alternative to sclerotherapy or surgery. MATERIALS AND METHODS: This retrospective and monocentric study included patients with symptomatic low-flow vascular malformation. The interventions were carried out under computed tomography (CT) scan, Cone-beam CT (CBCT) and/or USA guidance. Clinical response was evaluated by collecting the Numerical Rating Scale (NRS) for symptoms before and after the ablation. Safety was assessed based on criteria proposed by the Cardiovascular and Interventional Radiological Society of Europe. Imaging response was evaluated with post-ablation Magnetic Resonance Imaging. RESULTS: Twenty-one patients were included. Cryoablation was the first operative treatment for 12 patients (12/21, 57%). The remaining patients had already undergone surgery (2/21, 9%) or one or more sclerotherapy procedures (7/21, 33%).Symptoms assessed by Numerical Rating Scale dropped from a median of 7 [IQR 6-8] before the procedure to a median of 1 [IQR 0-2] after cryoablation (p < 0.001). Half of the patients declared a full effectiveness of cryoablation on their symptoms (11/21). No major complications and four minor adverse events (two skin lesions, two patients with neuropathic pain) were reported (19%). Lesional volume significantly decreased after cryoablation (median from 3.7 cm3 [1-10.4] to 0.25 cm3 [0-2.0], p < 0.001). CONCLUSION: Cryoablation seems to be a safe and effective first- or second-line therapy for soft tissue vascular malformations.

Case-Control Study on Exercise-Induced Vasculitis in Hikers.

The aim of this study was to identify clinical factors associated with exercise-induced vasculitis (EIV). This study included EIV cases and controls matched for age. Cases included were all members of a hiking club and participated in extended hiking trips. Exercise-induced vasculitis was diagnosed based on clinical signs occurring only after prolonged walks. Chronic venous disease was defined using the Clinical Etiological Anatomical Pathophysiologic classification. This study included 162 hikers: 32 EIV cases and 130 matched controls. Mean age at EIV diagnosis was 47.1 years and 24 (75.0%) of EIV cases were women. Chronic venous disease was present in 19 (57.6%) of EIV cases vs 39 (30.0%) in controls (P = .001); those with EIV had significantly more saphenous vein insufficiency and C3 venous insufficiency than controls, 85.0 vs 52.6% and 8 (25.0%) vs 13 (10.0%) (P = .02), respectively. For EIV cases, mean walking distance per hike was significantly higher than for controls (P = .002). Exercise-induced vasculitis symptoms were typical with rash and/or purpura on the leg in warm conditions. Lesions spontaneously disappear in <10 days. In this study, EIV cases had more chronic venous disease and longer mean walking distances than controls.

The Ottawa Score Performs Poorly to Identify Cancer Patients at High Risk of Recurrent Venous Thromboembolism: Insights from the TROPIQUE Study and Updated Meta-Analysis.

The Ottawa score (OS) for predicting the risk of recurrent venous thromboembolism (VTE) in cancer patients with VTE may help to guide anticoagulant treatment decisions that will optimize benefit-risk ratios. However, data on its reliability are conflicting. We applied the OS to all cancer patients with VTE enrolled in the prospective multicenter TROPIQUE study who received low-molecular-weight heparin over a 6-month period. Of 409 patients, 171 (41.8%) had a high-risk OS. The 6-month cumulative incidence of recurrent VTE was 7.8% (95%CI 4.2-14.8) in the high-risk OS group versus 4.8% (95%CI 2.6-8.9) in the low-risk OS group (SHR 1.47; 95%CI 0.24-8.55). The Area Under the Receiver Operating Characteristic curve (AUROC) of the OS in identifying patients who developed recurrent VTE was 0.53 (95%CI 0.38-0.65), and its accuracy was 57.9%. Among individual variables included in the OS, only prior VTE was significantly associated with the 6-month risk of recurrent VTE (SHR 4.39; 95% CI 1.13-17.04). When pooling data from all studies evaluating this score for predicting VTE recurrence in cancer patients (7 studies, 3413 patients), the OS estimated pooled AUROC was 0.59 (95%CI 0.56-0.62), and its accuracy was 55.7%. The present findings do not support the use of the OS to assess the risk of recurrent VTE in cancer patients.

Prevalence of post-thrombotic syndrome in a cohort of upper extremity vein thrombosis.

OBJECTIVE: Post-thrombotic syndrome (PTS) is one of the main complications that occurs after venous thrombosis. There are few data on the proportion of patients that will develop upper extremity PTS (UE-PTS) after upper extremity venous thrombosis (UEVT). The main objective of the study was to assess the prevalence of PTS in a UEVT cohort and to identify predictive factors of UE-PTS. METHODS: This study included patients with a history of proximal or arm UEVT, diagnosed on duplex ultrasound examination, between January 1, 2015, and December 31, 2017, in a university hospital. After UEVT, each patient was evaluated by a prospective standardized recording of clinical manifestations and duplex ultrasound examination in case of upper limb symptoms. UE-PTS was defined as a modified Villalta score of 4 or higher. RESULTS: Ninety-two patients were included; 68 (73.9%) had deep vein thrombosis (DVT) and 24 (19.2%) arm superficial vein thrombosis. Thirteen patients had PTS (14.1%), 12 (17.6%) in the DVT group and 1 (4.2%) in the superficial vein thrombosis group. There was a history of DVT in 92.3% of the cases of PTS. PTS was more frequent in patients with strokes with limb movement reduction (P = .01). On multivariate Cox analysis, a history of stroke (hazard ratio, 5.4; 95% confidence interval, 1.46-20.22; P = .01) was predictive of UE-PTS. CONCLUSIONS: UE-PTS occurred in 14.1% of cases after UEVT. Stroke with a decrease in limb movement was a predictor of developing PTS. Diagnostic criteria should be established for UE-PTS and prospective studies are needed to improve the description and management of UE-PTS.

Pharmaco-mechanical catheter-directed thrombolysis versus recanalization and stenting for post thrombotic syndrome after lower limb deep vein thrombosis: a comparative study.

Background: Endovenous interventional procedures can be used in addition to therapeutic anticoagulation to treat deep vein thrombosis in selected patients with proximal vein involvement (vena cava, iliac and/or common femoral). The aim of this study was to compare venous patency and the post-thrombotic syndrome (PTS) in patients treated with pharmaco-mechanical catheter-directed thrombolysis (PMT) versus recanalization-stenting for PTS after a proximal lower limb deep vein thrombosis. Methods: Between January 2014 and December 2020, this retrospective and monocentric study included patients with very symptomatic acute iliofemoral deep vein thrombosis treated with PMT within 21 days after diagnosis (PMT group) and patients with PTS caused by chronic venous obstruction treated with recanalization and stenting (CRS group). Results: A total of 116 patients were included (26 PMT, 90 CRS). The rate of primary patency was 81.8% (18/22 patients) in the PMT group and 78.4% (69/88) in the CRS group (P>0.99). The rate of venous patency at the last follow-up was 76.9% (20/26) in the PMT group and 82.2% (74/90) in the CRS group (P=0.57). The median number of stents was 2 (range, 0-5) in the PMT group and 3 (range, 0-7) in the CRS group (P<0.001). The median stent length was 150 mm (range, 60-390 mm) and 280 mm (range, 120-820 mm), respectively (P<0.001). The median last Villalta score was 2 (range, 0-10) in the PMT group and 2 (range, 0-21) in the CRS group (P=0.55). The rate of venous claudication at the last follow-up was 19.0% (4/21) in the PMT group and 12.0% (10/83) in the CRS group (P=0.47). Conclusions: In this study, there was no difference in venous patency and in the rate and severity of PTS between the PMT and CRS groups. The number of stent and their length were significantly lower in the PMT group compared with the CRS group.

Juvenile temporal vasculitis: a rare case in a middle-aged woman.

BACKGROUND: Classic giant cell arteritis affects older adults who are aged >50 years. Temporal arteritis is uncommon in young adults but juvenile temporal vasculitis (JTV) is the most frequent form found in young people. Clinical presentation is usually poor, with localized temporal inflammatory changes without consistent systemic manifestations. Generally, the patients have a benign clinical course, without ophthalmic or ischemic manifestations. In these rare JTVs, excision of the involved section of temporal artery is often curative and corticosteroid therapy is not required. METHOD: The present study reports a case of JTV in a middle-aged woman. RESULTS: A 44-year-old woman complained of violent temporal headache, with a slight inflammatory syndrome. She had no vascular systemic manifestation and no cause of secondary vasculitis. Doppler ultrasonography suggested a localized inflammatory arteritis. Temporal biopsy was performed. Histologic findings were compatible with JTV (nongranulomatous panarteritis with mononuclear cells and eosinophils). All the symptoms disappeared after excision. One year later, she remains well and reports neither systemic manifestation nor recurrence. CONCLUSION: Vasculitis of the temporal arteries in young people is uncommon and JTV is rare in middle-aged people. It is necessary to search for systemic or secondary vasculitis. In contrast to giant cell arteritis, steroids are not required.

Takayasu arteritis associated with hepatic sinusoidal dilatation.

Hepatic sinusoidal dilatation (HSD) is pathological entity that is characterized by peliosis hepatis (PH) like lesions, with vascular lesions that consist of multiple cyst-like, blood-filled cavities within the liver. To the best of our knowledge, neither PH nor HSD have been associated with systemic vasculitis. We describe herein two cases of idiopathic HSD associated with Takayasu arteritis (TA), diagnosed at an early stage of vasculitis. The same endothelial target in HSD and TA, and the favorable outcome of HSD with treatment of TA, suggest a pathogenic link between the two diseases.

Symptomatic aortitis at giant cell arteritis diagnosis: a prognostic factor of aortic event.

BACKGROUND: Giant cell arteritis (GCA) is frequently associated with aortic involvement that is likely to cause life-threatening structural complications (aneurysm, dissection). Few studies have investigated the occurrence of these complications, and no predictive factor has been identified so far. The aim of this study was to investigate factors associated with the risk of aortic complications in a cohort of GCA aortitis. METHODS: Data of all patients managed with aortitis (CT or 18 FDG PET) at the diagnosis of GCA in five hospitals from May 1998 and April 2019 were retrospectively collected. Clinical features were compared according to the presence of aortitis symptoms. The predictive factors of occurrence or aggravation of aortic structural abnormalities were investigated. RESULTS: One hundred and seventy-one patients with GCA aortitis were included; 55 patients (32%) had symptoms of aortitis (dorsal/lumbar/abdominal pain, aortic insufficiency) at diagnosis. The median follow-up was 38 months. Aortic complications occurred after a median time of 32 months. There were 19 new aortic aneurysms or complications of aneurysm and 5 dissections. Survival without aortic complication was significantly different between the symptomatic and non-symptomatic groups (Log rank, p = 0.0003). In multivariate analysis the presence of aortitis symptoms at diagnosis (HR 6.64 [1.95, 22.6] p = 0.002) and GCA relapse (HR 3.62 [1.2, 10.9] p = 0.02) were factors associated with the occurrence of aortic complications. CONCLUSION: In this study, the presence of aortitis symptoms at the diagnosis of GCA aortitis and GCA relapse were independent predictive factors of occurrence of aortic complications during follow-up.

Risk factors for symptomatic vascular events in giant cell arteritis: a study of 254 patients with large-vessel imaging at diagnosis.

AIMS: To identify factors associated with vascular events in patients with giant cell arteritis (GCA). METHODS: We performed a retrospective study of GCA patients diagnosed over a 20-year-period, who all underwent vascular imaging evaluation at diagnosis. Symptomatic vascular events were defined as the occurrence of any aortic event (aortic dissection or symptomatic aortic aneurysm), stroke, myocardial infarction, limb or mesenteric ischemia and de novo lower limbs arteritis stage 3 or 4. Patients with symptomatic vascular event (VE+) and without were compared, and risk factors were identified in a multivariable analysis. RESULTS: Thirty-nine (15.4%) of the 254 included patients experienced at least one symptomatic vascular event during follow-up, with a median time of 21.5 months. Arterial hypertension, diabetes, lower limbs arteritis or vascular complication at diagnosis were more frequent in VE+ patients (p < 0.05), as an abnormal computed tomography (CT)-scan at diagnosis (p = 0.04), aortitis (p = 0.01), particularly of the descending thoracic aorta (p = 0.03) and atheroma (p = 0.03). Deaths were more frequent in the VE+ group (37.1 versus 10.3%, p = 0.0003). In multivariable analysis, aortic surgery [hazard ratio (HR): 10.46 (1.41-77.80), p = 0.02], stroke [HR: 22.32 (3.69-135.05), p < 0.001], upper limb ischemia [HR: 20.27 (2.05-200.12), p = 0.01], lower limb ischemia [HR: 76.57 (2.89-2027.69), p = 0.009], aortic atheroma [HR: 3.06 (1.06-8.82), p = 0.04] and aortitis of the descending thoracic aorta on CT-scan at diagnosis [HR: 4.64 (1.56-13.75), p = 0.006] were independent predictive factors of a vascular event. CONCLUSION: In this study on GCA cases with large vessels imaging at diagnosis, aortic surgery, stroke, upper or lower limb ischemia, aortic atheroma and aortitis of the descending thoracic aorta on CT-scan, at GCA diagnosis, were independent predictive factors of a vascular event. PLAIN LANGUAGE SUMMARY: Risk factors for symptomatic vascular events in giant cell arteritisThis study was performed to identify the risk factors for developing symptomatic vascular event during giant cell arteritis (GCA) because these are poorly known.We performed a retrospective study of GCA patients diagnosed over a 20-year-period, who all underwent vascular imaging evaluation at diagnosis.Patients with symptomatic vascular event (VE+) and without (VE-) were compared, and risk factors were identified in a multivariable analysis.Thirty-nine patients experienced at least one symptomatic vascular event during follow-up, with a median time of 21.5 months.Arterial hypertension, diabetes, lower limbs arteritis or vascular complication at diagnosis were significantly more frequent in VE+ patients, as an abnormal CT-scan at diagnosis, aortitis, particularly of the descending thoracic aorta and atheroma. Deaths were more frequent in the VE+ group.Among 254 GCA patients, 39 experienced at least one vascular event during follow-up.Aortic surgery, stroke, upper and lower limb ischemia were vascular event risk factors.Aortic atheroma and descending thoracic aorta aortitis on CT-scan were vascular event risk factors.This study on GCA cases with large vessels imaging at diagnosis, showed that aortic surgery, stroke, upper or lower limb ischemia, aortic atheroma and aortitis of the descending thoracic aorta on CT-scan, at GCA diagnosis, were independent predictive factors of a vascular event.

Predictive factors of venous recanalization in upper-extremity vein thrombosis.

BACKGROUND: Upper extremity venous thrombosis (UEVT) represents about 10% of venous thrombo-embolic disease. This is mainly explained by the increasing use of central venous line, for oncologic or nutritional care. The factors associated with venous recanalization are not known. OBJECTIVE: The aim of this study was to investigate prognosis factor associated with venous recanalization after UEVT. METHODS: This study included patients with UEVT diagnosed with duplex ultra-sonography (DUS) from January 2015 to December 2017 with DUS evaluations during follow-up. A multivariate Cox proportional-hazards-model analysis was performed to identify predictive factors of UEVT complete recanalization. RESULTS: This study included 494 UEVT, 304 proximal UEVT and 190 distal UEVT. The median age was 58 years, 39.5% were women. Clinical context was: hematological malignancy (40.7%), solid cancer (14.2%), infectious or inflammatory context (49.9%) and presence of venous catheters or pacemaker leads in 86.4%. The rate of recanalization without sequelae of UEVT was 38%. For all UEVT, in multivariate analysis, factors associated with complete vein recanalization were: thrombosis associated with central venous catheter (CVC) (HR:2.40, [1.45;3.95], p<0.001), UEVT limited to a venous segment (HR:1.94, [1.26;3.00], p = 0.003), occlusive thrombosis (HR:0.48 [0.34;0.67], p<0.0001), the presence of a PICC Line (HR:2.29, [1.48;3.52], p<0.001), a thrombosis of deep and distal topography (HR:1.70, [1.10;2.63], p = 0.02) or superficial thrombosis of the forearm (HR:2.79, [1.52;5.12], p<0.001). For deep and proximal UEVT, non-occlusive UEVT (HR:2.23, [1.49;3.33], p<0.0001), thrombosis associated with CVC (HR:1.58, [1.01;2.47], p = 0.04) and infectious or inflammatory context (HR:1.63, [1.10;2.41], p = 0.01) were factors associated with complete vein recanalization. CONCLUSION: In this study, factors associated with UEVT recanalization were UEVT limited to a venous segment, thrombosis associated with CVC, a thrombosis of deep and distal thrombosis topography and superficial thrombosis of the forearm. Occlusive thrombosis was associated with the absence of UEVT recanalization.

Large-vessel involvement is predictive of multiple relapses in giant cell arteritis.

BACKGROUND: Giant cell arteritis (GCA) is the most common systemic vasculitis. Relapses are frequent. The aim of this study was to identify relapse risk factors in patients with GCA with complete large-vessel imaging at diagnosis. METHODS: Patients with GCA followed in our institution between April 1998 and April 2018 were included retrospectively. We included only patients who had undergone large vascular imaging investigations at diagnosis by computed tomography (CT)-scan and/or positron emission tomography (PET)-scan and/or angio-magnetic resonance imaging (MRI). Clinical, biological, and radiological data were collected. Relapse was defined as the reappearance of GCA symptoms, with concomitant increase in inflammatory markers, requiring treatment adjustment. Relapsing patients (R) and non-relapsing patients (NR) were compared. Relapse and multiple relapses (>2) risk factors were identified in multivariable Cox analyses. RESULTS: This study included 254 patients (73.2% women), with a median age of 72 years at diagnosis and a median follow up of 32.5 months. At diagnosis, 160 patients (63%) had an inflammatory large-vessel involvement on imaging, 46.1% (117 patients) relapsed at least once, and 21.3% (54 patients) had multiple relapses. The median delay of first relapse after diagnosis was 9 months. The second relapse delay was 21.5 months. NR patients had more stroke at diagnosis than R (p = 0.03) and the brachiocephalic trunk was involved more frequently on CT-scan (p = 0.046), as carotids (p = 0.02) in R patients. Multivariate Cox model identified male gender [hazard ratio (HR): 0.51, confidence interval (CI) (0.27-0.96), p = 0.04] as a relapse protective factor, and peripheral musculoskeletal manifestations [HR: 1.74 (1.03-2.94), p = 0.004] as a relapse risk factor. Peripheral musculoskeletal manifestations [HR: 2.78 (1.23-6.28), p = 0.014], negative temporal artery biopsy [HR: 2.29 (1.18-4.45), p = 0.015], large-vessel involvement like upper limb ischemia [HR: 8.84 (2.48-31.56), p = 0.001] and inflammation of arm arteries on CT-scan [HR: 2.39 (1.02-5.58), p = 0.04] at diagnosis were risk factors of multiple relapses. CONCLUSION: Male gender was a protective factor for GCA relapse and peripheral musculoskeletal manifestations appeared as a relapsing risk factor. Moreover, this study identified a particular clinical phenotype of multi-relapsing patients with GCA, characterized by peripheral musculoskeletal manifestations, negative temporal artery biopsy, and large-vessel involvement with upper limb ischemia or inflammation of arm arteries. PLAIN LANGUAGE SUMMARY: At giant cell arteritis diagnosis, large-vessel inflammatory involvement is predictive of multiple relapses 46.1% of patients with GCA relapse, and 21.3% undergo multiple relapses;Male gender appears as a protective factor for relapsing in GCA;Peripheral musculoskeletal manifestations are a relapse and multiple relapses risk factor;A negative temporal artery biopsy is predictive of multiple relapses;Large-vessel involvement is predictive of multiple relapses.

Digital ischaemia aetiologies and mid-term follow-up: A cohort study of 323 patients.

ABSTRACT: Upper extremity digital ischaemia (UEDI) is a rare heterogeneous condition whose frequency is 40 times less than that of toe ischaemia. Using a large cohort, the aim of this study was to evaluate aetiologies, prognosis and midterm clinical outcomes of UEDI.All patients with UEDI with or without cutaneous necrosis in a university hospital setting between January 2000 to December 2016 were included. Aetiologies, recurrence of UEDI, digital amputation and survival were analyzed retrospectively.Three hundred twenty three patients were included. UEDI due to cardio-embolic disease (DICE) was the highest occurring aetiology with 59 patients (18.3%), followed by DI due to Systemic Sclerosis (SSc) (16.1%), idiopathic causes (11.7%), Thromboangiitis obliterans (TAO) (9.3%), iatrogenic causes (9.3%), and cancer (6.2%). DICE patients tended to be older and featured more cases with arterial hypertension whereas TAO patients smoked more tobacco and cannabis. During follow-up, recurrences were significantly more frequent in SSc than in all other tested groups (P < .0001 vs idiopathic and DICE, P = .003 vs TAO) and among TAO patients when compared to DICE patients (P = .005). The cumulated rate of digital amputation was higher in the SSc group (n = 18) (P = .02) and the TAO group (n = 7) (P = .03) than in DICE (n = 2).This retrospective study suggests that main aetiologies of UEDI are DICE, SSc and idiopathic. This study highlights higher frequency of iatrogenic UEDI than previous studies. UEDI associated with SSc has a poor local prognosis (amputations and recurrences) and DICE a poor survival. UEDI with SSc and TAO are frequently recurrent.

Exploring how students think: a new method combining think-aloud and concept mapping protocols.

OBJECTIVES: A key element of medical competence is problem solving. Previous work has shown that doctors use inductive reasoning to progress from facts to hypotheses and deductive reasoning to move from hypotheses to the gathering of confirmatory information. No individual assessment method has been designed to quantify the use of inductive and deductive procedures within clinical reasoning. The aim of this study was to explore the feasibility and reliability of a new method which allows for the rapid identification of the style (inductive or deductive) of clinical reasoning in medical students and experts. METHODS: The study included four groups of four participants. These comprised groups of medical students in Years 3, 4 and 5 and a group of specialists in internal medicine, all at a medical school with a 6-year curriculum in France. Participants were asked to solve four clinical problems by thinking aloud. The thinking expressed aloud was immediately transcribed into concept maps by one or two 'writers' trained to distinguish inductive and deductive links. Reliability was assessed by estimating the inter-writer correlation. The calculated rate of inductive reasoning, the richness score and the rate of exhaustiveness of reasoning were compared according to the level of expertise of the individual and the type of clinical problem. RESULTS: The total number of maps drawn amounted to 32 for students in Year 4, 32 for students in Year 5, 16 for students in Year 3 and 16 for experts. A positive correlation was found between writers (R = 0.66-0.93). Richness scores and rates of exhaustiveness of reasoning did not differ according to expertise level. The rate of inductive reasoning varied as expected according to the nature of the clinical problem and was lower in experts (41% versus 67%). CONCLUSIONS: This new method showed good reliability and may be a promising tool for the assessment of medical problem-solving skills, giving teachers a means of diagnosing how their students think when they are confronted with clinical problems.

A STROBE cohort study of 755 deep and superficial upper-extremity vein thrombosis.

Upper extremity vein thrombosis (UE-VT) are more and more frequent pathologies and yet little studied. The aim is to describe the clinical and ultrasound features, UE-VT-related diseases, and the prevalence of pulmonary embolism (PE) and associated deaths.All UE-VT patients diagnosed by Doppler-ultrasound in Nantes University Hospital, from January 2015 to December 2017, were included retrospectively. UE-VT suspicion patterns, clinical features, UE-VT topography, and prevalence of PE and death were analyzed.Seven hundred and fifty-five UE-VT were analyzed, including 427 deep thrombosis (UE-DVT) and 328 superficial thrombosis (UE-SVT). In 86.2% (n = 651) UE-VT were related to endovascular devices. Among these thrombosis, one third is in connection with a PICC LINE and one quarter with a peripheral venous line. Forty nine percent (n = 370) of the patients had solid neoplasia or hematological malignancies. An inflammatory or systemic infectious context was found in 40.8% (n = 308) of the cases. The most frequently observed clinical sign at the UE-VT diagnosis was edema (28.6%). Among the UE-SVT it was the presence of an indurated cord (33.2%) and among the UE-DVT the indication of the Doppler-ultrasound was mainly a suspicion of infection on endovascular device (35.1%). In 10.6% (n = 80) of the cases the UE-VT were asymptomatic. The most frequently thrombosed veins were brachial basilic veins (16.7% of all thrombosed segments) followed by jugular (13%) and subclavian (12.3%) veins; 61.3% (n = 463) of UE-VT were in the right upper extremity; 63.3% (n = 478) UE-VT were occlusive. The occurrence of PE is 4% and the death rate is 10.2%, mainly related to the severe comorbidities of patients with UE-VT.UE-VT occurs in particular clinical contexts (hematological malignancies, solid cancers, systemic infections) and in the majority of endovascular devices (86.2%). The occurrence of PE is low.

Juvenile temporal arteritis: A clinicopathological multicentric experience.

INTRODUCTION: Juvenile temporal arteritis (JTA) is a recently-described and little-known inflammatory disease and its etiology is undetermined. Less than forty cases have been published. This paper is aimed at reporting the largest JTA series and to compare it to literature data to better evaluate its characteristics at diagnosis, its evolution and treatment options. MATERIAL AND METHODS: We conducted a retrospective and descriptive multicentric study in France by identifying adult patients under the age of 50 which had a pathological temporal artery biopsy owing to the presence of a temporal arteritis. Patients with temporal arteritis as a manifestation of systemic vasculitis were excluded. RESULTS: We included 12 patients and the literature review identified 32 cases described in 27 articles, thus a total of 44 patients - 34 men and 10 women - with a median age of 30 and a maximum of 44. All patients presented either a lump in the temporal region or prominent temporal arteries, and 47.7% of patients suffered from headaches. Only 11.4% of patients presented general symptoms and 6.8% a biological inflammatory syndrome; 34.1% had peripheral blood eosinophilia; 83.7% presented a single episode and complete excision without further treatment was documented for 72.7%. Pathology analysis revealed infiltrate of inflammatory cells in the arterial wall in 97.6% of patients but also sparse giant cells for 25% and granuloma for 22.9%, perivascular extension of the inflammation for 82.6%, and presence of lymphoid follicles or germinal centres for 60%. Clinical relapses were present in 16.3% of cases. CONCLUSION: JTA is a rare, localized and benign disease. The majority of cases have only one episode which is cured by local surgery.