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Objective. Nephrogenic fibrosing dermopathy (NFD) is a newly recognized cutaneous fibrotic disorder occurring in individuals with end-stage renal disease (ESRD).The aim of the present study was to describe the clinical and histopathologic features of 9 new cases and to characterize the inflammatory cells and expression of transforming growth factor ␤1 (TGF␤1) in affected skin.Methods. Clinical and laboratory assessments, including serology and pulmonary function studies, were performed in 9 patients undergoing long-term dialysis (8 hemodialysis; 1 peritoneal dialysis) for ESRD of diverse etiologies. Skin, fascia, striated muscles, lungs, and heart were examined by histopathology. Inflammatory cells were characterized by immunophenotyping using specific monoclonal antibodies. TGF␤1 expression was determined by in situ hybridization.Results. All patients displayed cutaneous features resembling both systemic sclerosis and diffuse fasciitis, with severe loss of motion and flexion contractures in multiple joints. Six patients displayed woody induration of the muscles of the legs, thighs, and forearms. Five of the 6 patients with lung involvement had a reduced diffusion capacity for carbon monoxide on pulmonary function testing. Marked elevations of the erythrocyte sedimentation rate and/or C-reactive protein level were found in 6 patients. Antinuclear antibodies were present at low titers in 4 patients. Histopathologic studies indicated that in addition to the dermis, the fibrotic process affected the subcutaneous tissue, fascia, striated muscles, lungs, and myocardium. Large numbers of CD68؉/factor XIIIa؉ dendritic cells and increased expression of TGF␤1 were found in affected skin and muscle.Conclusion. Our findings indicate that the fibrotic process of NFD affects not only the dermis, but also the subcutaneous tissues, fascia, and other organs, including striated muscles, heart, and lungs. We therefore believe this is a systemic fibrosing process, and we suggest that dialysis-associated systemic fibrosis would be a better term for the condition.Nephrogenic fibrosing dermopathy (NFD) is a newly recognized cutaneous fibrotic disorder occurring in individuals with end-stage renal disease (ESRD) (1,2). With a few exceptions, the nearly 100 patients described to date were undergoing long-term hemodialysis or peritoneal dialysis and many had received kidney transplants. Although the etiologic factor(s) responsible for NFD is not known, renal insufficiency and dialysis appear to be intimately involved (for review, see ref.3).Initially, this fibrotic process was considered a form of scleromyxedema (1); however, subsequent studies indicated that the disease was unique (2). The histopathologic changes in affected skin included Dr. Jiménez's work was supported by NIH grant AR-19161.

Background Eosinophilic fasciitis is a rare scleroderma-like illness. The clinical spectrum of the disease has evolved since its initial description. MethodsWe identified all patients diagnosed with eosinophilic fasciitis over the past 10 years at our scleroderma clinic. Demographics, disease pattern, serologies, tissue pathology and reponse to treatment were all recorded.Results Twelve patients with eosinophilic fasciitis were identified in our clinic over the past 10 years. The mean age at diagnosis was 49.8 ± 9.8 years, with nine female and three male patients. The first symptoms were noticed at an average of 8.8 ± 6.1 months before diagnosis.The mean initial absolute peripheral blood eosinophil count was 1188 ± 1059 cells/L. Two patients had a monoclonal gammopathy, and two had positive ANA titers. All patients received corticosteroids, 10 of whom received the equivalent dose of > 20 mg/day of prednisone for more than a month. Five patients received hydroxychloroquine, two received methotrexate, one received cyclosporine, one received topical tacrolimus, and one received sulfasalazine.At a mean follow up of 17.6 months (range 2-94 months), 8 patients had a good response to treatment, 2 patients had no effect, and 2 patients had a poor response to treatment. ConclusionHigh dose corticosteroid treatment lasting longer than a month with or without an immunosuppressive agent helped most patients with eosinophilic fasciitis, best results seen in those patients who were initiated treatment early on after their first symptoms.

OBJECTIVES: To measure the prevalence of, and factors associated with, left ventricular (LV) dysfunction in systemic sclerosis (SSc). METHODS: The EUSTAR database was first searched. A casecontrol study of a patient subset was then performed to further identify independent factors associated with LV dysfunction by simple and multiple regression. RESULTS: Of 7073 patients, 383 (5.4%) had an LV ejection fraction (EF) of <55%. By multiple regression analysis, age, sex, diffuse cutaneous disease, disease duration, digital ulcerations, renal and muscle involvement, disease activity score, pulmonary fibrosis and pulmonary arterial hypertension were associated with LV dysfunction. In the second phase, 129 patients with SSc with LVEF <55% were compared with 256 patients with SSc with normal LVEF. Male sex (OR 3.48; 95% CI 1.74 to 6.98), age (OR 1.03; 95% CI 1.01 to 1.06), digital ulcerations (OR 1.91; 95% CI 1.05 to 3.50), myositis (OR 2.88; 95% CI 1.15 to 7.19) and use of calcium channel blockers (OR 0.41; 95% CI 0.22 to 0.74) were independent factors associated with LV dysfunction. CONCLUSION: The prevalence of LV dysfunction in SSc is 5.4%. Age, male gender, digital ulcerations, myositis and lung involvement are independently associated with an increased prevalence of LV dysfunction. Conversely, the use of calcium channel blockers may be protective. Prevalence and factors associated with left ventricular dysfunction in the EULAR Scleroderma Trial and Research group (EUSTAR) database of systemic sclerosis patientsAllanore Y (1), Meune C (2), Vonk MC (3), Airo P (4), Hachulla E (5), Caramaschi P (6), Riemekasten G (7), Cozzi F(8), Beretta L (9), Derk CT (10) AbstractStudy objectives and methods To measure the prevalence of, and factors associated with, left ventricular (LV) dysfunction in SSc, we first queried the EUSTAR database. In a second phase, we performed a case-control study of a patient subset, to further identify independent factors associated with LV dysfunction by simple and multiple regression. Results Among 7,073 patients, 383 (5.4%) had a LV ejection fraction (EF) <55%. By multiple regression analysis, age, sex, diffuse cutaneous disease, disease duration, digital ulcerations, renal and muscle involvement, disease activity score, pulmonary fibrosis and pulmonary arterial hypertension (PAH) were associated with LV dysfunction. In a second phase, 129 SSc patients with LVEF <55% were compared with 256 SSc patients with normal LVEF. Male sex (OR 3.48; 95% CI1.74-6.98), age (OR 1.03; 95% CI 1.01-1.06), digital ulcerations (OR 1.91; 95% CI 1.05-3.50), myositis (OR 2.88; 95% CI 1.15-7.19), and calcium channel blockers (CCB) use (OR 0.41; 95% CI 0.22-0.74) were independent factors associated with LV dysfunction.

Objective. To assess cumulative survival rates and identify independent predictors of mortality in patients with incident systemic sclerosis (SSc)-associated pulmonary arterial hypertension (PAH) who had undergone routine screening for PAH at SSc centers in the US. Methods. The Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma registry is a prospective registry of SSc patients at high risk for PAH or with definite pulmonary hypertension diagnosed by right-sided heart catheterization within 6 months of enrollment. Only patients with World Health Organization group I PAH (mean pulmonary artery pressure >25 mm Hg and pulmonary capillary wedge pressure <15 mm Hg without significant interstitial lung disease) were included in these analyses. Results. In total, 131 SSc patients with incident PAH were followed for a mean ؎ SD of 2.0 ؎ 1.4 years. The 1-, 2-, and 3-year cumulative survival rates were 93%, 88%, and 75%, respectively. On multivariate analysis, age >60 years (hazard ratio [HR] 3.0, 95% confidence interval [95% CI] 1.1-8.4), male sex (HR 3.9, 95% CI 1.1-13.9), functional class (FC) IV status (HR 6.5, 95% CI 1.8 -22.8), and diffusing capacity for carbon monoxide (DLCO) <39% predicted (HR 4.2, 95% CI 1.3-13.8) were significant predictors of mortality. Conclusion. This is the largest study describing survival in patients with incident SSc-associated PAH followed up at multiple SSc centers in the US who had undergone routine screening for PAH. The survival rates were better than those reported in other recently described SSc-associated PAH cohorts. Severely reduced DLCO and FC IV status at the time of PAH diagnosis portended a poor prognosis in these patients.

dcSSc is associated with earlier development of GAVE, as well as more severe anemia requiring more therapeutic interventions. Rapid progression of cutaneous disease may suggest earlier development of GAVE. Absence of antitopoisomerase I antibodies and presence of antibodies to RNAP III/speckled antinuclear antibody pattern may be useful to identify the subset of patients with SSc with increased risk for GAVE.

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Background Patients with normal (mean pulmonary arterial pressure ≤20 mmHg) and borderline mean pulmonary pressures (mPAP) (boPAP; 21–24 mmHg) are “at risk” of developing pulmonary hypertension(PH). The objectives of this analysis were 1)to examine the baseline characteristics in systemic sclerosis(SSc) with Normal and boPAP, and 2) to explore long term outcomes in SSc patients with boPAP vs. Normal hemodynamics. Methods PHAROS is a multicenter prospective longitudinal cohort of patients with SSc “at risk” or recently diagnosed with resting PH on right heart catheterization (RHC). Baseline clinical characteristics, pulmonary function tests, high resolution computed tomography(HRCT), 2-D echocardiogram, and RHC results were analyzed in Normal and boPAP groups. Results A total of 206 patients underwent RHC (35 Normal, 28 boPAP, 143 had resting PH). There were no differences in the baseline demographics. Patients in the boPAP group were more likely to have restrictive lung disease (67% vs. 30%), fibrosis on HRCT and a higher estimated right ventricular systolic pressure on echocardiogram (46.3 vs. 36.2mmHg; p<0.05) than patients with Normal hemodynamics. RHC revealed higher pulmonary vascular resistance (PVR) and more elevated mPAP on exercise(mPAP ≥30; 88% vs. 56%) in the boPAP group(p<0.05 for both). Patients were followed for a mean of 25.7 months and 24 patients had a repeat RHC during this period. During follow up, 55% of the boPAP group and 32% of the Normal group developed resting PH (p=NS). Conclusions Patients with boPAP have a greater prevalence of abnormal lung physiology, pulmonary fibrosis and presence of exercise mPAP ≥30mmHg.