Prevalence of retinal changes in patients with systemic sclerosis: the association between retinal vascular changes and nailfold capillaroscopic findings

BackgroundSystemic sclerosis (SSc) is a connective tissue disease affecting the skin and multiple organs. Most of the evidence on ocular involvement comes from small studies. We evaluated the retinal vascular changes in patients with SSc and its associations with types, disease duration, skin score, and nailfold capillaroscopy changes.Material and methodsWe evaluated the demographic, clinical and nailfold capillaroscopy data of 52 patients referring to the clinic of scleroderma with SSc according to the 2013 ACR/EULAR SSc criteria. In addition, full ophthalmic examination in 52 patients and fundus photography in 40 patients were done in the ophthalmology clinic.ResultsThere were 52 (49 women and 3 men, 17 diffuse and 35 limited) patients with SSc with mean disease duration of 8 ±5 years and mean age of 40 ±9 years. Retinal changes in the ophthalmologic examination were seen in 30.7% as increased vascular tortuosity. None of the patients had cotton wool spot, hemorrhage or hard exudate. Forty patients underwent fundus photography and 22.5% of them had vascular tortuosity. Only the presence of hemorrhage in the nailfolds was correlated with retinal tortuosity, and the other characteristics of nailfold capillaroscopy did not have any association with retinal changes.ConclusionsRetinal vascular changes were seen in about one third of our patients. There was no correlation between nailfold capillaroscopy, disease duration, type and skin score of SSc patients and retinal vascular changes. These findings suggest that the mechanisms or the quality of changes in the retinal vessels and nailfold vasculature may be different.     

Cartilage Oligomeric Matrix Protein (COMP) in systemic sclerosis (SSc): role in disease severity and subclinical rheumatoid arthritis overlap

ObjectiveThe aim of the present study was to compare the serum level of COMP in both subsets of Systemic sclerosis (SSc) as a marker of arthritis and reveal an associated subclinical RA overlap and a relation to clinical, laboratory and radiological findings in SSc.MethodsForty adult SSc patients were included in the study and grouped into the two subsets diffuse (dSSc) and limited (lSSc) SSc. Their mean age was 40 ± 9 years. Thorough history taking and clinical examination were performed to all patients. Skin thickness was scored according to the modified Rodnan skin score method (MRSS). The disease activity was assessed by measuring the Medsger severity score. The joints were extensively examined and the tenderness counted according to the Ritchie articular index (RAI). Relevant laboratory and radiological investigations were carried out. The serum COMP level was determined by ELISA.ResultsThe serum COMP was significantly higher in the SSc patients compared to the control and obviously higher in the dSSc compared to the lSSc patients. The level of COMP was higher in the females and significantly higher in the SSc patients with arthritis (56.5 ± 6.8 ug/ml) compared to those without (34 ± 8.3 ug/ml) (P 0.000).ConclusionThe COMP level may become a nonspecific but useful marker for joint involvement in SSc patients to identify patients at risk of joint damage and developing SSc-RA overlap syndrome even with mild arthritis.     

Contrast enhanced colostography: New applications in preoperative evaluation of anorectal malformations

IntroductionUnderstanding details of anatomic relationships between the colon and surrounding structures is a critical piece of preoperative planning prior to surgical repair of anorectal malformations (ARMs).Traditional imaging techniques involve ionizing radiation, distention of the rectum with supraphysiologic intraluminal pressures, and sometimes require sedation. Recent developments in the field of contrast agents have allowed the emergence of an ultrasound-based technique that can avoid these requirements while continuing to provide high resolution structural information in three dimensions.MethodsFourteen children (13 male, 1 female, age 1–11 months) with ARMs underwent contrast enhanced colostography (ceCS) in addition to traditional preoperative imaging techniques to delineate anatomic relationships of pelvic structures.ResultsceCS and traditional imaging yielded concordant anatomic information, including structural relationships and fistulous connections, in 10/14 patients (71%). ceCS detected fistulous connection in 2/13 patients (15%) that were not seen by traditional imaging. Ultrasonography failed to detect the fistulous connection in one patient.ConclusionsceCS is a safe, effective and flexible method for defining important structural information in ARM patients. When compared with traditional methods, it provided equivalent or superior results 93% of the time and bears consideration as a standard tool in preoperative planning for this population.Type of StudyRetrospective Comparative Study.Level of EvidenceLevel III.     

Incomplete thymic involution in systemic sclerosis and rheumatoid arthritis

ObjectiveThe thymus plays a crucial role in immune system homeostasis. Thymic abnormalities have been reported in many autoimmune diseases, but data for systemic sclerosis (SSc) and rheumatoid arthritis (RA) are sparse. The aim of this study was to evaluate the prevalence and correlates of radiological incomplete involution of the thymus in SSc and RA patients, and in a non-autoimmune group of controls.MethodsAll patients were at least 40 years old: 96 SSc patients (median age 59 years, 80% women) and 65 RA patients (median age 57 years, 88% women) were compared with 32 control individuals (median age 63 years, 62% women). Pulmonary CT-scans performed for lung assessment were available for all individuals. For the purpose of our study, complete involution of the thymus was defined as the absence of a residual thymus or a gland thickness, corresponding to the short axis on the axial slice, of less than 7 mm. We defined incomplete involution of the thymus as a residual thymic tissue more than 7 mm thick.ResultsThe frequency of incomplete thymus involution was significantly higher in SSc and RA patients (respectively 15 and 14%) than in the control group (0%; P < 0.05). Incomplete thymus involution was associated with pulmonary restrictive syndrome in SSc patients, and with biotherapy and an absence of antinuclear antibodies in RA patients.ConclusionOur findings show that two autoimmune diseases, SSc and RA, are associated with incomplete thymus involution.     

Efficacy of Cyclophosphamide in the Treatment of Interstitial Lung Disease Associated with Systemic Sclerosis

BackgroundCyclophosphamide (CYC) stabilizes the parameters of lung function tests (LFT) of patients with systemic sclerosis (SSc) and interstitial lung disease (ILD) treated for 12 months. There is little information about long-term treatment (24 months). The aim of this study is to analyze the effect of intravenous CYC in LFT parameters in patients with SSc and ILD treated for 24 months.Patients and methodRetrospective study of 37 patients with ILD associated with scleroderma treated with intravenous CYC for 24 months and regularly assessed by LFT (at baseline, 6, 12 and 24 months) including forced vital capacity (FVC) and transfer capacity of carbon monoxide (DL_CO).ResultsThe differences between FVC and DL_CO values performed at baseline and those performed at 6, 12, and 24 months were less than 10%, which meant that CYC stabilized functional parameters. There were no differences in FVC or DL_CO when patients treated for 6 months were evaluated according to the type of SSc skin involvement of (diffuse or limited) or according to the evolution time of ILD before the start of treatment. Although patients with severe restriction (FVC<70%) showed more improvement, it was less than 10% in all casesConclusionIn this series of patients with ILD associated with SSc, intravenous CYC was effective in stabilizing lung function parameters in long-term treatment.     

High prevalence of salivary gland ultrasound abnormalities in systemic sclerosis

ObjectiveTo evaluate salivary gland (SG) involvement in patients with systemic sclerosis (SSc) using SG ultrasound (SGUS).MethodsPatients with SSc (n = 62), primary Sjögren's syndrome (pSS) (n = 59), and idiopathic Sicca syndrome (n = 43) were evaluated using the outcome measures in rheumatology clinical trial (OMERACT) definitions of the SGUS scoring system. The hyperechogenic bands using the 0–3 scoring system, intraglandular power Doppler signal (PDS), and SG volumes were also assessed.ResultsThe proportion of patients with OMERACT grades (≥ 2) among the four SGs was significantly higher in SSc (51.6%) and pSS (62.7%) groups than those in the idiopathic Sicca syndrome group (4.7%). Patients with SSc and pSS had significantly higher total fibrosis grades than controls. No difference in fibrosis grades was observed between SSc and pSS groups. The PDS scores of SGs were higher in the SSc group than in the idiopathic Sicca syndrome group. SG volumes did not differ between the groups. SSc patients with SGUS grades ≥ 2 had more anti-centromere antibodies (ACA) (65.6% vs. 30.0%) than individuals with grades 0–1. SSc patients with fibrosis grades ≥ 2 reported more Sicca symptoms than those with grades 0–1. Inhomogeneity and hyperechogenic bands within the SGs were not associated with organ involvement in SSc.ConclusionsMore than half of patients with SSc, specifically with ACA, showed SG involvement. SG fibrosis was more prominent in SSc than in idiopathic Sicca syndrome and was associated with subjective Sicca symptoms. However, hyperechoic bands within the SGs are not features that can differentiate between SSc and pSS.     

The evaluation of a home-based program for hands in patients with systemic sclerosis

Study DesignThis study used a quasi-experimental design where patients were evaluated before and after participation in the self-management program.IntroductionHands are commonly affected in systemic sclerosis (SSc). Strategies to maintain or improve hand function are indicated upon diagnosis and throughout the course of the disease.Purpose of the StudyThe purpose of this study was to develop and evaluate a home-based program for hands in patients with SSc.MethodsA home-based self-management program that consisted of concise instructions about SSc and hand exercises was developed and evaluated in a group of patients with SSc during 8 weeks. Primary outcome measures were hand pain (Visual Analogue Scale) and hand function (Cochin Hand Function Scale). Secondary outcome measures were disability (Scleroderma Health Assessment Questionnaire), finger motion (delta finger-to-palm), grip strength, tip and key pinch strength, Raynaud phenomenon and digital ulcers impact, quality of life (Short Form Health Survey). For comparisons between different times analysis of variance for repeated measures was used. To calculate the effect size (ES), the Cohen's test was performed. To evaluate skin moisturizing and warming habits before and after intervention, the McNemar test was used. Statistical significance was set at P ≤ .05.ResultsTwenty-two SSc patients (19 women: 3 men; 16 limited scleroderma: 6 diffuse scleroderma) completed the program. Significant improvements were noted for hand pain (3.97 vs 2.21, ES: 0.69), Cochin Hand Function Scale (19.24 vs 12.48, ES: 0.48), Scleroderma Health Assessment Questionnaire (0.95 vs 0.48, ES: 1.01), delta finger-to-palm (92.86 vs 106.33, ES: 0.40), grip strength (14.43 vs 19, ES: 0.58), tip pinch strength (2.49 vs 4.18, ES: 1.15), key pinch strength (4.01 vs 5.22, ES: 0.76), Raynaud phenomenon impact (0.94 vs 0.47, ES: 0.75), Short Form Health Survey–role physical (47.38 vs 60.14, ES: 0.61), physical functioning (34.62 vs 61.9, ES: 0.18), social functioning (60.71 vs 75.6, ES: 0.64), bodily pain (50.55 vs 63.38, ES: 0.58), vitality (45.95 vs 62, ES: 2.22), mental health (56.62 vs 72.38, ES: 0.84) moisturizing, and cold avoidance habits. Patients considered the program easy to follow with no adverse effects related to exercises.DiscussionWe developed a home based hand care program to be offered to SSc patients. Improvements in hand function, strength, disability, motion, and overall quality of life were independent of age, income, education level, disease duration, and skin score. Our findings support those of other studies that reported the benefits of hand exercises in SSc. Some study limitations include the lack of a control group, the small number of subjects and the short-time follow up.ConclusionsThis home-based program for patients with SSc improved hand pain, function, mobility, and strength at the end of 8 weeks. Patient adherence and sustained efficacy is still to be determined.     

Tireoidite de Hashimoto pode estar associada a um subgrupo de pacientes de esclerose sistêmica com hipertensão pulmonar

IntroductionRecent studies show an association between autoimmune thyroiditis and systemic sclerosis (SSc) and suggest that this condition may interfere with the ES phenotype. However these studies evaluate the autoimmune thyroiditis as a whole and none of them specifically addresses Hashimoto's thyroiditis (HT) in SSc.ObjectiveTo investigate the presence of HT in SSc patients and its possible association with disease manifestations.MethodsClinical manifestations of hypothyroidism, TSH and anti‐thyroid auto antibodies (anti‐TPO. anti TBG and TRAb) were studied in 56 patients with SSc. SSc patients with HT were compared with SSc patients without thyroiditis.ResultsHT was observed in 19.64% of patients with SSc. No association was observed between HT and the different forms of disease or profile of autoantibodies. Likewise, there was no difference between the mean modified Rodnan score and presence of Raynaud's phenomenon, scars, digital necrosis, myositis, arthritis, sicca symptoms, esophageal dysmotility and scleroderma renal crisis when the groups were compared. On the other hand, patients with HT had higher frequency of pulmonary hypertension in relation to patients without HT (66.6% vs 22.5%, p = 0.016).ConclusionsIn the studied sample patients with ES and HT had higher prevalence of pulmonary hypertension. Long‐term follow‐up studies with a larger number of TH and SSc patients are needed to confirm these data.     

Vitamin D supply in patients with rheumatic diseases in Poland – a pilot study

ObjectivesIn rheumatic diseases, vitamin D supply is recommended as part of the prophylaxis and treatment of osteoporosis, especially in patients undergoing glucocorticoid therapy, but also due to its immunoregulatory and anti-inflammatory properties. We aimed to evaluate serum 25-hydroxyvitamin D [25(OH)D₃] levels in Polish patients with systemic sclerosis (SSc), systemic lupus erythematosus (SLE) and granulomatosis with polyangiitis (GPA), in relation to various clinical parameters, and to assess the initial range of doses for the purpose of further research.Material and methods112 patients (39 with SLE, 44 with SSc and 29 with GPA), referred to the Department of Rheumatology and Internal Medicine in Poznan, Poland, were enrolled in this retrospective study. Demographic and clinical data were collected, including 25(OH)D₃ serum levels, vitamin D supplementation doses and season of blood sampling.ResultsMean (SD) serum 25(OH)D₃ concentrations were 31 (19.4) ng/ml for SLE, 28.8 (12.5) ng/ml for SSc and 28 (15.2) ng/ml for GPA, and they did not significantly differ between the groups. Vitamin D levels below the optimal range were found in 43.8% of SLE, 65.9% of SSc and 72.4% of GPA patients. 80% of patients reported vitamin D intake, with a mean daily dose of 1398 IU for SLE, 1345 IU for SSc and 1689 IU for GPA. Vitamin D insufficiency and deficiency were frequent among patients with rheumatic diseases, independently of the diagnosis and season.ConclusionsPatients with rheumatic diseases seem to require higher doses of vitamin D than recommended for the general population. The present results indicate the necessity to use higher initial doses of vitamin D in this group of patients (2000 to 4000 UI) and to maintain the dose of vitamin D regardless of the change of seasons.     

Juvenile systemic sclerosis – observations of one clinical centre

ObjectivesThe systemic form of scleroderma (SSc) in children is a very rare disease; therefore, it is recognized relatively late, which increases the risk of complications. The aim of the study was to assess the clinical symptoms of juvenile systemic sclerosis (JSSc) in our cohort patients.Material and methodsA group of (N = 22) scleroderma patients aged between 2 and 16 years were observed. Demographic data and all clinical results obtained during 16 years of observation in the clinic of rheumatic diseases of developmental age were collected and analysed.ResultsIn all observed children the major JSSc criterion was found, i.e. skin thickening proximal to the metacarpal phalangeal and/or metatarsophalangeal joints. Other symptoms are presented as follows: nailfold capillary abnormalities – 100%, Raynaud’s phenomenon – 90.9%, sclerodactyly – 27.3%, digital tip ulcers – 27.3%, dysphagia – 18.2%, gastroesophageal reflux – 27.3% (assessed in only 10 children), arrhythmias – 22.7%, heart failure – 9.1%, new-onset arterial hypertension – 9.1%, pulmonary fibrosis – 72.7%, pulmonary arterial hypertension – 9.1%, neuropathy – 13.6%, carpal tunnel syndrome – 4.5%, tendon friction rubs – 4.5%, arthritis – 22.7%, and myositis – 13.6%. There were no cases of renal crisis. Decreased diffusing capacity of oxygen was confirmed in 12 patients (58.3%). The presence of antinuclear antibodies was noticed in 86.7% of patients, and among SSc selective autoantibodies: anticentromere – 31.8%, anti-topoisomerase I – 18.2%, anti-PM-Scl 100 or 75 – 45.5%, anti-RP11, Th/To, PCNA in total in 27.3% were presented. In 4.5% of cases, apart from the presence of anti-PM-Scl autoantibodies, positive lupus band test, reduced concentration of complement, and antiphospholipid antibodies were also found. In 59% of studied children, the body mass index was below the 25th percentile.ConclusionsThe presented retrospective analysis shows that the occurrence of Raynaud’s phenomenon with changes in nailfold capillaroscopy is the best screening toll for the assessment of risk of JSSc. All patients of developmental age with Raynaud’s phenomenon, especially in the case of the appearance of antinuclear antibodies, should be monitored with capillaroscopy regardless of other laboratory or imaging tests.     

Serotonin and systemic sclerosis. An emerging player in pathogenesis

ObjectivesSystemic sclerosis (SSc) is a complex, autoimmune disease characterized by multiple organ fibrosis and vasculopathy. Experimental and clinical evidence indicates that serotonin is crucially involved in the fibrotic process and mediates vascular manifestations such as Raynaud's phenomenon (RP) or pulmonary arterial hypertension (PAH), all key features of SSc. In this review, we summarize the current knowledge on the potential contribution of serotonin in SSc pathogenesis and provide a rationale for further investigation of this molecule as a therapeutic target.MethodsMedline and Cochrane databases were searched from inception to April 2021 using the search terms (systemic sclerosis OR scleroderma OR Raynaud OR Pulmonary arterial hypertension) AND serotonin.ResultsSerotonin, a key molecule in an array of central and peripheral functions, has a multifaceted role in regulating fibrosis and vasculopathy. Experimental data suggest that serotonin drives fibrosis in the skin and visceral organs, promotes platelet aggregation, induces vasoconstriction and increases pulmonary vascular resistance. Earlier human trials regarding drugs that inhibit serotonin signaling produced mixed results. However, recent advances in the understanding of the underlying molecular mechanisms could help identify novel therapeutics targeting the serotonin pathway and inform future clinical trials.ConclusionsSerotonin may be a mediator in both fibrosis and vasculopathy. Further exploration of the potential role of serotonin in SSc is justified.     

Diffusion tensor imaging predicts functional impairment in mild-to-moderate cervical spondylotic myelopathy

Background contextMagnetic resonance imaging (MRI) is the standard imaging modality for the assessment of cervical spinal cord; however, MRI assessment of the spinal cord in cervical spondylotic myelopathy patients has not demonstrated a consistent association with neurologic function or outcome after surgical or medical intervention. Thus, there is a need for sensitive imaging biomarkers that can predict functional impairment in patients with advanced cervical spondylosis.PurposeTo implement diffusion tensor imaging (DTI) as an imaging biomarker for microstructural integrity and functional impairment in patients with cervical spondylosis.Study designNonrandomized, single institution study.Patient sampleForty-eight cervical spondylosis patients with or without spinal cord signal change underwent DTI of the spinal cord along with functional assessment.Outcome measuresFunctional measures of neurologic function via modified Japanese Orthopedic Association (mJOA) score.MethodsA zoomed-echoplanar imaging technique and two-dimensional spatially selective radiofrequency excitation pulse were used for DTI measurement. Fractional anisotropy (FA), mean diffusivity (MD), radial and axial diffusion (AD) coefficient, AD anisotropy, ψ, defined as AD-MD, and the standard deviation (SD) of primary eigenvector orientation were evaluated at the site of compression.ResultsResults suggest average FA, transverse apparent diffusion coefficient, ψ, and SD of primary eigenvector orientation at the spinal level of highest compression were linearly correlated with mJOA score. Receiver-operator characteristic analysis suggested FA and ψ could identify stenosis patients with mild-to-moderate symptoms with a relatively high sensitivity and specificity.ConclusionsThe results of this study support the potential use of DTI as a biomarker for predicting functional impairment in patients with cervical spondylosis.     

Combined Application of Monopolar Radiofrequency Technology and Injective Carboxytherapy in the Correction of Involutive Skin Changes

BackgroundIn aesthetic medicine, high-power instrumental technologies that exert a significant positive effect with a minimal rehabilitation period are becoming more popular in the correction of involutive changes of the skin. Such methods include monopolar radiofrequency therapy. However, high temperature can lead to the development of unfavorable events and complications.ObjectiveThe study aimed to design and evaluate a combined method of monopolar radiofrequency therapy and injective carboxytherapy in patients with local adverse events (edema and neuropathy) in the facial area.MethodsThe study included 72 female patients aged 38 to 52 years old that earlier received monopolar radiofrequency therapy in the facial region for the correction of involutive changes in the skin and who developed edema and neuropathy in the area of radiofrequency impact. Four groups received injectable carboxytherapy locally, in the projection of lymph nodes, or in combination.ResultsCombined application of monopolar radiofrequency therapy in the facial region and an extended protocol of carboxytherapy as local application or application in the projection of regional lymph nodes exerts a significant positive effect in the short-term and long-term study period reducing the period of rehabilitation. This was demonstrated by a statistically significant reduction of the intradermal edema and quick regression of pain syndrome in the studied group with the extended protocol of carboxytherapy in comparison with other studied groups.ConclusionIt is feasible to apply an extended protocol of carboxytherapy (locally, in the area of expressed manifestation, and in the projection of regional lymph nodes) in patients with local edema and neuropathy that developed after received monopolar radiofrequency therapy in the facial region, which significantly reduces the rehabilitation period and contributes to faster reduction of local edema and neuropathy and provides a stable clinical result.     

Prospective observational study comparing burn surgeons’ estimations of wound healing after skin grafting to photo-assisted methods

IntroductionAppropriate graft healing after split-thickness skin graft and early recognition of complications (graft loss) are critical to burn patient management. Larger mesh ratio expansions and Meek micrografting may pose a greater challenge in estimating the percentage of wound healing. This study looks at the reliability of photograph assessments and the concordance of bedside evaluation to photograph assessments of wound healing after skin grafting.MethodsThree assessment methods for percentage of wound healing after skin Grafting were assessed: (1) clinicians’ bedside rating, (2) clinician assessment of high-definition photographs, and (3) digital image analysis through color subtraction using Adobe Photoshop. We compared each method using a mixed-effects model on absolute agreement using intra-class correlation (ICC) and Bland Altman (BA) plots.ResultsFourteen burn patients were enrolled with 38 grafted wounds (100 sites). Bedside assessments had a mean ICC of 0.64 (compared to digital image analysis) and 0.69 (compared to photo assessment), with a wide range on BA-plots. Inter-rater reliability of photo assessment was excellent (0.96) among six clinicians. Repeated photo-assisted assessments had good intra-rater reliability (ICC: photo assessment: 0.88; digital analysis: 0.97).ConclusionsBedside wound healing assessments show variability; photograph documentation of sequential wound progression could supplement active clinical management or studies for more reliable assessments.     

Anti-citrullinated peptides antibodies in systemic sclerosis: Meta-analysis of frequency and meaning

Objectives

Diagnosis of systemic sclerosis (SSc) is partially determined by the presence of specific autoantibodies often associated with specific clinical features. Recent studies report the presence of ACPA in SSc. We aimed to evaluate the prevalence of ACPA in SSc and to assess their influence on clinical presentation of SSc.

Non-invasive measurement of liver and pancreas fibrosis in patients with cystic fibrosis

BackgroundPatients with cystic fibrosis (CF) have a relevant morbidity and mortality caused by CF-related liver-disease. While transient elastography (TE) is an established elastography method in hepatology centers, Acoustic-Radiation-Force-Impulse (ARFI)-Imaging is a novel ultrasound-based elastography method which is integrated in a conventional ultrasound-system. The aim of the present study was to evaluate the prevalence of liver-fibrosis in patients with CF using TE, ARFI-imaging and fibrosis blood tests.Methods106 patients with CF were prospectively included in the present study and received ARFI-imaging of the left and right liver-lobe, ARFI of the pancreas TE of the liver and laboratory evaluation.ResultsThe prevalence of liver-fibrosis according to recently published best practice guidelines for CFLD was 22.6%. Prevalence of significant liver-fibrosis assessed by TE, ARFI-right-liver-lobe, ARFI-left-liver-lobe, Fibrotest, Fibrotest-corrected-by-haptoglobin was 17%, 24%, 40%, 7%, and 16%, respectively. The best agreement was found for TE, ARFI-right-liver-lobe and Fibrotest-corrected-by-haptoglobin. Patients with pancreatic-insufficiency had significantly lower pancreas-ARFI-values as compared to patients without.ConclusionsARFI-imaging and TE seem to be promising non-invasive methods for detection of liver-fibrosis in patients with CF.     

The "sclerodermic hand": a radiological and clinical study

ObjectiveTo assess the clinical and radiographic features of hand involvement in patients with systemic sclerosis (SSc).MethodsForty-one unselected Sardinian SSc patients (32 women, 9 men; mean age 58.9, range 31–81 years; mean disease duration 11.8 years, range 1–36 years) were evaluated in this observational cross-sectional study. Twenty-six patients had diffuse scleroderma (dSSc) and 15 limited scleroderma (lSSc). Radiological examination of the hands was performed and the films were read by two independent rheumatologists blinded to the diagnosis using a classification system of four predefined radiological patterns (normal/minimal changes, articular degenerative, articular inflammatory and periarticular pattern). Correlations between radiological pattern, clinical and serological features were assessed.ResultsThe skeletal and articular involvement of the hand was frequent in SSc, being clinically evident in 30/41 (73%) and radiologically in 33/41 (80%) of patients. The periarticular pattern (defined as the occurrence of bone resorption of ungueal tufts, soft tissue calcifications and/or flexion deformities) was the most frequent pattern detected (14/41, 34.1%) and finger flexion contractures and bone resorptions were significantly associated with interstitial lung disease, reduced FVC, oesophagus involvement and prostacycline therapy. Calcinosis (29.2%) was found to be associated with erosions, suggesting a pathogenic link. An inflammatory pattern was also radiologically frequent (8/41, 19.5%), but erosions, with the exception of those localized at distal interphalangeal joints, were demonstrated mainly in patients with clinical picture of rheumatoid arthritis overlapped with SSc. We found no significant differences in terms of radiographic findings between lSSc and dSSc with the exception of calcinosis, which was more frequent in patients with lSSc.ConclusionThis cross-sectional study confirms that the skeletal and articular involvement of the hand is frequent in SSc.     

N‐acetilcisteína oral no tratamento do fenômeno de Raynaud secundário à esclerose sistêmica: ensaio clínico randomizado,placebo‐controlado e duplo‐cego

ObjectiveTo evaluate the safety and efficacy of N‐acetylcysteine (NAC) orally on digital microcirculation blood flow in patients with Raynaud's phenomenon (RP) secondary to systemic sclerosis (SSc).MethodsThis was a randomized, double‐blind, placebo‐controlled trial in which 42 patients with SSc received oral NAC at a dose of 600 mg tid (21 patients, mean age 45.6 ± 9.5 years) or placebo (21 patients, mean age 45.0 ± 12.7 years) for four weeks. The primary endpoint was the change in cutaneous microcirculation blood flow before and after cold stimulation measured by laser Doppler imaging (LDI) at weeks 0 and 4. The frequency and severity of RP and the number of digital ulcers were also measured at weeks 0 and 4. The adverse events were recorded in the fourth week.ResultsThere was no significant change in digital blood flow assessed by LDI before or after cold stimulus after four weeks of NAC or placebo. Both groups showed significant improvement in the frequency and severity of RP attacks, with no difference between the two groups. At the end of the study, the placebo group had three digital ulcers, while the NAC group showed no ulcers. NAC was well tolerated and no patient discontinued the treatment.ConclusionsNAC orally at a dose of 1800 mg/day showed no vasodilator effect on hands’ microcirculation after four weeks of treatment in patients with RP secondary to SSc.     

Use of imaging for active surveillance in testicular cancer: Is real-world practice concordant with guidelines?

IntroductionImaging is an integral component of active surveillance (AS) following orchiectomy for stage 1 non-seminoma (NSGCT) and seminoma germ cell tumors. In this population-based study, we describe use of imaging among patients with early-stage testicular cancer and evaluate whether they are concordant with guideline recommendations.MethodsThis is a population-based, retrospective cohort study to describe use of imaging among all patients with early-stage testicular cancer treated with AS in the Canadian province of Ontario. The Ontario Cancer Registry was linked to electronic records of treatment to identify use of chest and abdomen/pelvis imaging. Data from 2000–2010 were included, with followup for up to five years for patients with non-seminoma and 10 years for patients with seminoma. The key outcome of interest was the frequency of imaging at temporal milestones following orchiectomy. Compared to the most contemporaneous guidelines in Ontario, any discordant frequency of imaging was defined as underuse or overuse. Substantial under- or overuse was defined as >1 imaging test less/ more than what was recommended during a 12-month period.ResultsThe study population included 569 patients with NSGCT (median age 28) and 1107 with seminoma (median age 37). Among patients with NSGCT, adherence with body imaging was low in years 1–3 of surveillance (range 26–37%, predominantly underuse) and higher in years 4–5 (63–67%, predominantly overuse). Adherence with chest imaging was even lower (range 11–34% during years 1–5). Among patients with seminoma, adherence with abdominal and chest imaging was relatively stable and comparable throughout the 10-year followup period (range 23–47% abdomen and 28–47% chest). Multivariable analysis confirmed that underuse of imaging was more common in recent years. NSGCT histology was associated with underuse in years 1–2 but overuse in years 3–5.ConclusionsIn routine clinical practice, patients with testicular cancer commonly receive imaging discordant to the protocol for AS, with a substantial proportion receiving both under- and overuse at various times during surveillance followup.     

高频超声诊断乳腺佩吉特病的价值

目的观察高频超声对乳腺佩吉特病(PDB)的诊断价值。方法回顾性分析经病理证实的30例PDB患者的乳腺高频超声资料,其中13例合并浸润性导管癌(IDC),比较PDB合并与未合并IDC患者乳腺超声表现的差异。结果 PDB超声表现包括乳头内低回声、乳晕皮肤增厚、乳腺内结节、钙化、导管扩张及腋窝淋巴结肿大。PDB合并IDC患者超声显示结节率(P=0.001)及腋窝淋巴结肿大率(P=0.002)均高于未合并IDC者,超声显示二者乳腺影像报告和数据系统(BI-RADS)分类、乳头内低回声、乳晕皮肤增厚、钙化及乳腺导管扩张比例差异均无统计学意义(P均0.05)。结论 PDB超声表现具有一定特征性,高频超声有助于诊断及评估病情。