Comparison of the Minimum Bounding Rectangle and Minimum Circumscribed Ellipse of Rain Cells from TRMM

Based on the TRMM dataset, this paper compares the applicability of the improved MCE(minimum circumscribed ellipse), MBR(minimum bounding rectangle), and DIA(direct indexing area) methods for rain cell fitting. These three methods can reflect the geometric characteristics of clouds and apply geometric parameters to estimate the real dimensions of rain cells. The MCE method shows a major advantage in identifying the circumference of rain cells. The circumference of rain cells identified by MCE in most samples is smaller than that identified by DIA and MBR, and more similar to the observed rain cells. The area of rain cells identified by MBR is relatively robust. For rain cells composed of many pixels(N> 20), the overall performance is better than that of MCE, but the contribution of MBR to the best identification results,which have the shortest circumference and the smallest area, is less than that of MCE. The DIA method is best suited to small rain cells with a circumference of less than 100 km and an area of less than 120 km^(2), but the overall performance is mediocre. The MCE method tends to achieve the highest success at any angle, whereas there are fewer “best identification”results from DIA or MBR and more of the worst ones in the along-track direction and cross-track direction. Through this comprehensive comparison, we conclude that MCE can obtain the best fitting results with the shortest circumference and the smallest area on behalf of the high filling effect for all sizes of rain cells.

Eosinophilic fasciitis difficult to differentiate from scleroderma:A case report

BACKGROUND Eosinophilic fasciitis(EF)is a rare connective tissue disease that can cause swelling and sclerosis of the extremities,and special attention is needed to differentiate EF from systemic sclerosis.Misdiagnosis or omission markedly delays treatment of EF,and severe skin sclerosis in advanced stages can cause joint contracture and tendon retraction,worsening the patient's prognosis and quality of life.CASE SUMMARY We report a case of EF in a young woman diagnosed by tissue biopsy,confirming the difficulty of differential diagnosis with scleroderma.CONCLUSION Focusing on skin manifestations,completing tissue biopsy and radiography can help diagnose EF effectively.Clinicians should enhance their understanding of the differences between EF and scleroderma,and early diagnosis and standardized treatment can improve the prognosis of patients with EF.

Heart Disease Scleroderma Revelators: About a Clinical Case

Scleroderma (or systemic sclerosis) is a disease characterized by abnormalities in the functioning of small blood vessels and the immune system, ultimately leading to inflammation and excessive fibrosis of the skin and various organs, including the heart. Management must be multidisciplinary, to avoid complications that are often serious. We report the case of a 20-year-old patient with no known cardiovascular history who consults for dyspnea, and retrosternal pain associated with a dry cough. On physical examination, she had tachycardia, swelling of the lower limbs, jugular turgidity, and deafening heart sounds. Cardiac Doppler ultrasound shows dilation of the right cavities, paradoxical septum and significant pulmonary arterial hypertension, pericardial effusion of medium abundance. On oral examination, it presents an ulceration of the lips, dermatological examination finds scattered hypo chromic spots in the body, more accentuated in the face. Before the hypo chromic dermatosis, a dermatological consultation was carried out with an autoimmune assessment that came back positive for systemic scleroderma.

Application of integrated traditional Chinese and Western medicine in the treatment of juvenile localized scleroderma with skin ulcer: a case report

Background:Juvenile Localized Scleroderma(JLS)is a rare pediatric rheumatic disease characterized by inflammation and skin sclerosis.The side effect of consensus-recommended medications and the risk of disability posed challenges to the JLS treatment.We intend to demonstrate the potential of traditional Chinese medicine in treating JLS with skin ulcers and reducing the dose of glucocorticoid.Method:Here we report a case of a 13-year-old male with JLS who took oral methotrexate tablets of 10 mg/week and methylprednisolone of 6 mg/day for over six months without significant effect and suffered from skin ulcers on the dorsal feet one month after drug cessation.Subsequently,the patient was treated with integrated traditional Chinese and Western medicine of low-dose glucocorticosteroid,adjusted Shenqi Huoxue formula and Jinshe Xiaoyan formula,etc.Results:After integrated treatment,the patient’s dorsal feet ulcers healed and the skin sclerosis and hyperpigmentation improved significantly.Conclusions:This case report suggests that integrated traditional Chinese and Western medicine can be used as an effective treatment for JLS.

Iatrogenic scleroderma renal crisis:A case report and mini literature review for renal crisis

Background:Systemic sclerosis is a connective tissue disease characterized by fibrosis of the skin and organs,marked changes in microvascular structure,cellular and humoral immune disorders.Renal involvement is more frequent and mainly characterized by moderate proteinuria,elevated serum creatinine levels,and hypertension.The most common kidney involvement in SSc is scleroderma renal crisis(SRC)that is fatal without prompt intervention.Case report:A 52-year-old Caucasian male with known diffuse cutaneous systemic sclerosis was hospitalized with communityacquired pneumonia.On the fifth day after appropriate antibiotic therapy and 60 mg/day methylprednisolone,decreased urine output,arterial hypertension,decreased renal function and pulmonary edema developed.The patient was diagnosed with a scleroderma renal crisis.Emergency hemodialysis was applied to the patient,and captopril 6×25 mg/day and nifedipine 2*60 mg/day treatment were given.He received a routine hemodialysis program for about three months.The hemodialysis program was terminated when the patient’s urine quality and quantity increased.Conclusions:SRC,characterized by malignant hypertension,azotemia,microangiopathic hemolytic anemia,and kidney failure,is one of the most important complications of systemic sclerosis with a poor prognosis without prompt intervention.Steroid use is one of the important risk factors that precipitate SRC development.With angiotensin-converting enzyme inhibitors,survival increased after SRC,the need for dialysis decreased,and usually allowed the discontinuation of dialysis treatment within about 6-18 months.Suspicion of SRC in the presence of the above-mentioned findings in patients with a diagnosis or suspected systemic sclerosis can be considered the most important treatment step.

Adipose tissue-derived stem cells ameliorates dermal fibrosis in mouse models of scleroderma

Objective: To investigate the therapeutic potential of adipose-derived stem cells(ADSCs) for limited cutaneous scleroderma(LS) in mouse models,Methods: ADSCs were isolated from pathogen-free female C57BL/6 mice and LS was induced in wild type(WT) C57BL/6 mice via daily injection of bleomycin(0.1 m L x 300 μg/m L) for 4 weeks; then the ADSCs were subcutaneously injected into the dorsal area in the model treatment group,and 100 μL of phosphate buffered saline(PBS) solution was injected into the same site in the model control group,Green fluorescent protein(GFP) was used to track the cells using an in vivo imaging system on days 7,14,21 and 28 after transplantation,All mice were sacrificed and histologic analyses were performed after 4 weeks,and the skin thickness,collagen deposition and the total content of hydroxyproline were evaluated,Additionally,immunohistochemistry were performed to compare the tissue expression and distribution of TGF-β1 and VEGF between the ADSCs treatment group and the treatment control group,Results: WT C57BL/6 LS mouse model were successfully established and GFP in vivo fluorescence imaging showed that the translated ADSCs survived at the local for at least 4 weeks,Compared with the control group,the ADSCs treatment group significantly attenuated bleomycin-induced dermal fibrosis,reduced the skin thickness and the total content of hydroxyproline(P<0.05),The ADSCs treatment group displayed significantly lower levels of TGF-β1 and higher levels of VEGF than the control group(P<0.05),Conclusions: ADSCs may provide a feasible and practical treatment for autoimmune diseases such as LS and ameliorate dermal fibrosis.

UVA1 irradiation inhibits fibroblast proliferation and alleviates pathological changes of scleroderma in a mouse model

The purpose of the present study was to compare the effects of different doses of ultraviolet radiation A1 （UVA1） on human fibroblast proliferation and collagen level in a mouse model of scleroderma, so as to identify appropriate irradiation doses for clinical treatment of scleroderma. Monolayer from human fibroblasts was cultured in vitro, and a mouse model of scleroderma was established by subcutaneous injection of 100 μL of 400 μg/mL bleomycin into the back of BALB/c mice for 4 weeks. The mouse models and human fibroblasts were divided into UVA1- exposed （100, 60 and 20 J/cm2） and UVA-unexposed groups. At 0, 24 and 48 h after exposure, cell proliferation and levels of hydroxyproline and collagen were detected. UVA1 irradiation was performed 3 times weekly for 10 weeks, and the pathological changes of skin tissues, skin thickness and collagen level were observed after phototherapy. Cell proliferation and the levels of hydroxyproline and collagen were inhibited after phototherapy, and there was a significant difference between the UVAl-exposed cells and UVAl-unexposed cells （P 〈 0.001）. In addition, UVA1 phototherapy improved dermal sclerosis and softened the skin, and there were significant differences between the high-dose UVA1 group and the model group, and the negative group （P 〈 0.05）. It is concluded that UVA1 radiation can reduce cell proliferation, and decrease hydroxyproline and collagen levels in a dose-dependent manner in vitro. High-dose UVA1 phototherapy has marked therapeutic effect on scleroderma in the mouse model. Decreased collagen level may be related to the reduced number and activity of cells, as well as inhibition of collagen synthesis.

Follow-up Efficacy of Integrative Chinese and Western Drugs on Localized Scleroderma with Vitamine B 6 and Xuefu Zhuyu Decoction (血府逐瘀汤)

Objective: To investigate the therapeutic effects of vitamine B 6 (Vit B 6) and Xuefu Zhuyu Decoction (血府逐瘀汤,XFZY, for activating blood circulation to remove stasis) in patients with localized scleroderma(LSD). Methods: Thirty-three patients were treated with XFZY and Vit B 6, with 15 cases taking orally prednisone acetate and 20 healthy volunteers as the control. Their level of soluble interleukin-2 receptor (sIL-2R) and tumor necrosis factor-α (TNF-α) in the patients with LSD before and after treatment were observed. Results: The level of sIL-2R and TNF-α in the serum from the patients with LSD were higher than those of healthy volunteers ( P <0.01). After treatment with Vit B 6 and XFZY, the level of sIL-2R and TNF-α from the patients with LSD decreased significantly ( P <0.01), but there were no difference between the group taking Vit B 6 plus XFZY and the group given prednisone. Conclusion: The activating blood circulation to remove stasis approach in treating LSD with integrative Chinese and Western drugs got better results, and metabolic disorder of tryptophan might be correlated with the etiology of LSD.

Comparison of outcomes between overlapping-spot and single-spot photodynamic therapy for circumscribed choroidal hemangioma

AIM: To compare the efficacy and safety of photodynamic therapy(PDT) with overlapping multiple spots and single spot for treating circumscribed choroidal hemangioma. · METHODS: Twenty-two patients(22 eyes) with symptomatic circumscribed choroidal hemangioma received PDT treatment. Fourteen patients received overlapping spots(two to three spots) PDT,whereas eight patients received single-spot PDT. Laser was used at 50J/cm2for 83s in the overlapping-spot group and 50J/cm2for 166s in the single-spot group. Clinical examination,funduscopy,fluorescein angiography,and ultrasonography were performed at baseline and after treatment. ·RESULTS: The mean follow-up time was 28.5 ±8.0 months in the overlapping-spot group and 27.0 ±5.0 months in the single-spot group. Nine patients(64.2%) had their vision improved over two lines on the Snellen chart,and five patients showed stable visual acuity in the overlapping-spot group. The mean thickness of tumor decreased from 2.7 ±0.8mm to 1.2±0.9mm,and the mean greatest tumor linear dimension decreased from 7.4 ± 1.5mm to 4.5 ±3.5mm after treatment. In the single-spot group,two patients(25%) had their vision improved over two lines on the Snellen chart,and six patients had unchanged stable vision. The mean tumor thickness in this group decreased from 2.5±0.7mm to 1.4±1.0mm,and the mean greatest tumor linear dimension decreased from 7.2±1.3mm to 4.7±3.6mm. No significant differences in visual improvement and tumor regression were found between the two groups. ·CONCLUSION:Overlapping-spotPDTunderappropriate treatment parameters and strategies is as effective andsafe as single-spot PDT for treating symptomatic circumscribed choroidal hemangioma. Improved or stabilized visual acuity was achieved as a result of tumor regression.

Treatment review of sight threatening circumscribed choroidal haemangioma

AIMTo describe our clinical experience in treating circumscribed choroidal haemangioma (CCH) in a tertiary referral centre over a fifteen year period prior to photodynamic therapy.

Associations with Organ Involvement and Autoantibodies in Systemic Sclerosis: Results from the Canadian Scleroderma Research Group (CSRG)

Objective: Serum from SSc patients was analyzed centrally to determine ANA patterns and extractable nuclear antigens (ENAs) between lcSSc and dcSSc and associations with organ involvement. Methods: 1145 SSc patients had ANA and ENA analyzed by indirect immunofluorescence on HEp-2 substrate at a screening serum dilution of 1/160. Most ENA antibodies [Sm. U1-RNP, Ro52, SS-A/Ro60, topoisomeraseI (Topo1), SS-B/La, chromatin, ribosomal P and Jo1] were measured by laser bead immunoassay;and RNA polymerase III (RNAP) by ELISA. Results: ANA was positive in 95% (same in lcSSc, and dcSSc). Centromere pattern was present in 34%, speckled 22%, nucleolar 18%, homogeneous and speckled (H&S) 16%, multiple nuclear dots 6%. Anti-centromere Ab (ACA) occurred in 46% of lcSSc and 11% of dcSSc (P = 0.0001). ENAs that differed between lcSSc and dcSSc subsets were Topo1 (OR 2.4, P = 0.0001) and RNAP (OR 5.6, P 0.0001) more common in dcSSc. Overall, 15% had positive Topo1;usually with a H&S pattern (67%);Topo1 was associated with ILD on CXR (OR 2.3;95% CI 1.5 - 3.5) and HRCT (OR 3.8;95% CI 1.8 - 8.2). RNAP occurred in 18.5% (35.4% in dcSSc vs. 8.9% in lcSSc). Scleroderma renal crisis (SRC) was 13 times more likely if RNAP positive;P = 0.0001. ACA was only weakly associated with sPAP > 50 mmHg (OR 1.8;95%CI 1.1 - 3.0). Conclusion: ANA homogeneous pattern alone is rare in SSc;ACA was significantly more common in lcSSc. Many ENAs are equal in lcSSc and dcSSc except RNAP and Topo1. RNAP has the highest OR of SRC. Topo1 is less strongly associated with ILD. Abstract word count: 249, Body word count 1246, Figures 2, Tables 2. Key Messages: 1) 95% of SSc has a positive ANA and ANA patterns in SSc include centromere, nucleolar, and homogeneous and speckled together;2) Most ENAs are equal in both dcSSc and lcSSc except anti RNA polymerase III and topoisomerase I;3) RNA polymerase III has the highest association (odds ratio) with scleroderma renal crisis, topoisomerase I is associated with interstitial lung disease;whereas anticentromere was not associated with elevated pulmonary arterial pressures on echocardiogram.

Subtotal gastrectomy combined with chemotherapy: An effective therapy for patients with circumscribed Borrmann type Ⅳ gastric cancer

BACKGROUND Although Borrmann type Ⅳ(B-4) gastric cancer has a higher mortality rate and presents distant metastasis easily, especially peritoneal metastasis, when diagnosed, some B-4 patients were found to have no distant metastasis by preoperative detection and underwent curative surgery, which was defined as circumscribed B-4 in our study. In this study, we focused on the circumscribed B-4 patients without distant metastasis during surgery to identify factors related to prognosis and postoperative peritoneal cavity metastasis(PPCM), which is important for selecting an appropriate therapeutic strategy.AIM To identify factors related to the prognosis and PPCM of B-4 patients.METHODS A total of 117 B-4 patients who underwent gastrectomy between January 2005 and December 2012 were included in this study. Survival analysis was performed using Kaplan–Meier analysis and Cox multivariate models. Pearson correlation analyses were performed to identify the factors related to PPCM. All statistical analyses were performed using SPSS 20.0.RESULTS Lymph node status, gastrectomy type, and postoperative chemotherapy were independent prognostic factors in 117 circumscribed B-4 patients. Subtotal gastrectomy combined with chemotherapy could significantly improve the longterm survival time. Six patients who were diagnosed with pN0 and received the combination therapy had a 3-year survival rate of 100% and a median survival of 77.7 mo. Even for patients with metastatic lymph nodes(n = 13), the combination therapy also increased the 3-year overall survival rate to 57.1%. In addition, positive lymph node status was the only factor(P = 0.005) correlated with PPCM in certain B-4 patients, and chemotherapy was useful for suppressing PPCM in patients with subtotal gastrectomy but not in those with total gastrectomy.CONCLUSION Lymph node status is an independent prognostic factor for circumscribed B-4 patients. In addition, subtotal gastrectomy and postoperative chemotherapy could effectively improve prognosis and even suppress PPCM.

High-dose ruthenium-106 plaque therapy for circumscribed choroidal hemangioma: a retrospective study of 25 Chinese patients

AIM:To evaluate the efficacy and safety of ruthenium-106(106Ru)plaque radiotherapy at a dose(>50 Gy)higher than recommended(29-50 Gy)for treatment of circumscribed choroidal hemangioma(CCH)in Chinese patients.METHODS:This retrospective study included 25symptomatic CCH patients undergoing 106Ru plaque brachytherapy involving 25 eyes between January 2005and August 2016.Ophthalmic examination was performed at the baseline and at each post-treatment follow-up visit,using best-corrected visual acuity(BCVA),dilated fundus examination,and B-scan ultrasonography.The primary efficacy outcome measures included the changes in BCVA and hemangioma dimensions at the last followup visit from the baseline.RESULTS:The mean follow-up duration was 28.0±26.6(range,12-110)mo.All the hemangiomas were located in the posterior pole except for two involving the fovea.The mean apex dose of 106Ru plaque radiotherapy was84.4±19.7 Gy.The mean BCVA improved from 41.4±29.3(0-97)at the baseline to 53.0±33.8(0-97)ETDRS letters at the last visit(P=0.01).The mean hemangioma height declined from 3.98±0.88(2.40-5.50)mm to 0.84±1.63(0-6.47)mm(P≤0.001),and the greatest linear diameter(GLD)reduced from 9.36±2.23(6.80-15.00)to 7.40±2.45(0-13.00)mm(P≤0.001).Hemangioma size increased in one(4%)eye with a worsened vision,and subretinal fluid completely resolved in all but one patient(4%).Radiationrelated retinopathy was observed in two patients at posttreatment 9 and 11mo,respectively.CONCLUSION:106Ru plaque brachytherapy at a dose(>50 Gy)higher than recommended(29-50 Gy)is an effective treatment regimen for symptomatic CCH associated with significantly improved visual acuity and a favorable safety profile in Chinese patients.

Arterial Imaging in Digital Gangrenes Associated with Scleroderma-Spectrum Disorders

Objectives: Digital refractory gangrene is rarely found in collagen diseases, including systemic sclerosis and is possibly caused by similar underlying vascular damage in peripheral arterial disease (PAD) such as arteriosclerosis obliterans (ASO) and/or thromboangiitis obliterans (TAO) by unclarified mechanisms other than vasculitis and thrombosis. This study evaluated the radiological imaging in patients with digital gangrene associated with collagen disease and compared the images with those of PAD based on the results of laboratory and histopathological examinations. Methods: Angiography, MR angiography and/or CT angiography were performed on 6 patients with refractory gangrene or extensive ulcers accompanied by scleroderma-spectrum disorders;3 with diffuse systemic sclerosis, 1 with limited systemic sclerosis, 1 with overlap syndrome and 1 with Sj?gren’s syndrome. Results: Although the vascular alterations in collagen diseases were similar to those in PAD, the abnormal image findings (occlusion or stenosis of the arteries with smooth vessel walls) found in collagen diseases did not include atheromatous plaque, which are worm-like vessels that are characteristic of those observed in PAD. Conclusions: Some cases of digital gangrene seen in collagen diseases show similar vascular imaging patterns to those of PAD and comprehensive examinations including arterial imaging can be useful for the diagnosis of these unrecognized vascular changes other than vasculitis or digital thrombosis.

B cell depletion in scleroderma lung disease: A promising new treatment?

Evidence suggests that B cells may participate in the fibrotic process Based on this experimental evidence, treatment with rituximab(RTX) has been tried in systemic sclerosis(SSc) with promising results. In a randomized controlled study from our research group it was shown that treatment with 2 courses of RTX leads to a significant improvement of lung function at 1 year compared to baseline. All relevant studies have so far reported clinical and/or histologic improvement of skin fibrosis something that adds further evidence in favor of a disease modifying role of RTX in SSc. It is more than obvious that novel therapies for SSc-associated lung disease are needed. A large scale, randomized, controlled study assessing the efficacy of RTX in SSc associated interstitial lung disease is warranted. If RTX turns out to be effective it would be a major therapeutic advance in SSc since it can be administered on a long term basis due to its acceptable safety profile.

Anaemia in a Patient with Diffuse Systemic Scleroderma

We hereby present a case of anaemia in a 73 years old patient with known past medical history of diffuse systemic scleroderma, who presented with acute onset of dizziness and haemetemesis. Blood tests revealed sudden drop of haemoglobin and an urgent gastroscopy revealed gastric antral vascular ectasia (GAVE) or “watermelon stomach”. GAVE is a rare but well recognised cause of acute bleeding in systemic scleroderma patients and should be kept as a differential diagnosis in the work up of anaemia in these patients.

Systemic Scleroderma at University Teaching Hospital (UTH) of Cocody (Abidjan—Cote d’Ivoire): A 19 Cases Report

Objective: The aim of this study was to describe the epidemiological, clinical and therapeutic features of systemic scleroderma at Cocody UTH. Methodology: We conducted a retrospective and descriptive study over a period of 10 years (September 15, 2008 to April 15, 2019) on the files of patients hospitalized for systemic scleroderma in the rheumatology unit of the UTH of Cocody. We used the classification criteria of the American Society of Rheumatology (1980) to retain the diagnosis. Results: Nineteen patients’ files had been collected, representing a hospital frequency of 0.32%. The average age was 37.25 ± 13.82 years old. There were 15 women and 4 men. The average consultation time was 26.44 months. The mode of revelation of the disease was mostly cutaneous and articular. All patients had cutaneous sclerosis (average Rodnan score = 27.63/11.61 (min = 4, max = 49).) Scleroderma was diffuse in 70.59% of cases;a Raynaud’s phenomenon was seen in 47.37%. The main clinical manifestations were: cutaneous (100%), articular (89.47%), pulmonary (57.89%) and digestive (63.16%). No renal damage was found. Pulmonary fibrosis (5 cases), pulmonary arterial hypertension (3 cases) and pericardial effusion (2 cases) were sometimes founded in explorations. The positivity of antinuclear antibodies (ANA) was seen in 72% of patients and anti scl70 antibodies in 42.85%. The treatment included corticosteroids and immunosuppressants, respectively used in 84.2% and 63.16% of cases. The outcome was marked by 5 cases of death attributed to respiratory distress. Conclusion: Systemic scleroderma seems to be a very rare condition in Ivorian rheumatology milieu. The main systemic manifestations were digestive and pulmonary. Treatment was very often symptomatic sometimes associated with D-penicillamine.

Predictors of a Cerebrovascular Accident in a Population of Systemic Sclerosis Patients Followed at a Large Academic Center with a Dedicated Scleroderma Center

Aim: The aim of this study is to describe the clinical characteristics of patients with a diagnosis of systemic sclerosis who later suffer a stroke and to identify associations for this relationship. Background: Prior studies have showed an increased risk of cardiovascular disease among patients with chronic inflammatory disorders, with chronic inflammation leading to atherosclerosis believed to be the culprit. Systemic sclerosis (SSc) is a chronic inflammatory disease characterized by diffuse fibrosis of the skin and internal organs. Previous studies have suggested a possible link between systemic sclerosis and macrovascular complications such as stroke. Methods: This is a retrospective chart review of patients treated within the University of Pennsylvania Health System from October 2015 to April 2019 with a diagnosis of SSc. Using ICD10 codes, we identified a cohort of SSc patients who suffered a stroke. Information regarding demographics and stroke risk factors were gathered from the charts of patients with a diagnosis of both SSc plus stroke and compared to a control group of randomly selected patients with SSc who never suffered a stroke. Continuous variables were conveyed using a mean plus a standard deviation. A two-sample t-test was used to compare the two groups of patients. Qualitative variables were compared using a two-tailed Fisher’s exact test. Results: Based on a large cohort of SSc patients (n = 2080) followed between October 2015 and April 2019, we identified 36 SSc patients who developed a subsequent stroke (1.7% of cohort). When looking at risk factors for stroke in SSc patients, we identified hypertension and atrial fibrillation to be associated with the diagnosis of stroke in such patients. Specifically, 28 of the 36 patients with both SSc and stroke also had a diagnosis of hypertension while in the control group, only 17 of 36 patients had hypertension. Atrial fibrillation was seen in 9 of 36 patients with both SSc and CVA while it was seen in only 2 of 36 patients in the control group. Conclusions: This case control study demonstrated that the presence of hypertension and atrial fibrillation had a statistically significant association with the diagnosis of CVA in patients with SSc.

Scleroderma: Not an orphan disease any more

Scleroderma(or systemic sclerosis) is a rare disease associated with significant morbidity and mortality.Although previously thought to have a uniformly poor prognosis,the outlook has changed in recent years.We review recent insights into the pathogenesis,clinical features,assessment and management of scleroderma.

Primary Cardiac Involvement in Scleroderma and Role of Cardiac MRI

Systemic sclerosis (scleroderma) is a connective tissue disease characterized by vascular dysfunction and fibrosis that can affect multiple organ systems. We present case of primary cardiac involvement and the diagnostic role of cardiac MRI. Cardiovascular magnetic resonance imaging (MRI) is an accurate, quantitative method for the non-invasive assessment of myocardial perfusion. The presence of clinically apparent myocardial involvement in scleroderma portends a very poor prognosis. One study of US veterans found that clinical cardiac disease in scleroderma was associated with a 70% mortality rate at five years. Management of heart failure and conduction system abnormalities in scleroderma is similar to other cardiac disease. It includes afterload reduction, beta-blockade, defibrillator placement, etc. Patients with reduced cardiac function and normal coronary arteries may benefit from increased immune suppresion.