There was no significant difference in efficacy between sildenafil and bosentan initial combination therapy and sildenafil monotherapy in patients with systemic sclerosis-related pulmonary arterial hypertension.
Smoking status in patients with systemic sclerosis was not significantly associated with systemic sclerosis-specific cutaneous or pulmonary changes.
Recent evidence suggests that systemic sclerosis and SLE in pulmonary arterial hypertension should be considered separate diseases.
The investigational drug SAR100842 was safe, moderately effective, and well tolerated in patients with early diffuse cutaneous systemic sclerosis.
Although pulmonary arterial hypertension occurs in a minority of patients with systemic sclerosis, clinicians must be attentive to it, as it can emerge at any point over the course of the disease.
Stratification by clinical features and autoantibody response identify scleroderma subgroups with distinct risks for cancer.
Researchers evaluated the prevalence, risk factors, and effect of venous thromboembolism on survival in systemic sclerosis.
Two predictive models for progressive skin thickening in patients with early diffuse cutaneous systemic sclerosis were developed to use in clinical practice and to improve recruitment in clinical trials.
Investigators assessed 3 versions of the Brief Fear of Negative Evaluation scale in patients with systemic sclerosis.
Researchers sought to determine the prevalence of radiation complications in patients with systemic scleroderma and breast cancer.
Patients who underwent autologous hematopoietic stem cell transplant for systemic sclerosis saw improvements in quality of life.
Use of the DETECT algorithm did not change incidence of PAH, but led to an increased frequency of cases of borderline pulmonary hypertension.
These results likely indicate the importance of skin thickening in contributing to impairment of hand function.
Patients with systemic sclerosis and early-diagnosed pulmonary arterial hypertension had disease progression over a relatively short period of time.
A blinded analysis sought to determine whether CT signs of pulmonary veno-occlusive disease are common and predictive of mortality in patients with systemic sclerosis and pulmonary arterial hypertension.
Patients with scleroderma-associated isolated pulmonary arterial hypertension (SSc-PAH) have a slightly higher mortality risk than patients SSc-PAH with concomitant interstitial lung disease.
Rheumatology specialists should be aware of the clinical features of SSc-PAH, as well as the tools available to facilitate a prompt, accurate diagnosis.
Patients with systemic sclerosis-associated pulmonary arterial hypertension display important differences in hemodynamic and pulmonary function parameters compared with patients with idiopathic PAH.
Microvascular damage evaluated using nailfold capillaroscopy and macrovascular features that include ulnar artery occlusion, assessed per hand power Doppler ultrasonography, have demonstrated associations with the main digital manifestations of systemic sclerosis.
Hyperbaric oxygen therapy completely healed 4 patient's systemic sclerosis ulcers.
Median levels of 24 elements were assessed in patients with systemic sclerosis and matched controls.
Prospective, observational study assessed 4 treatment protocols and their effects on skin thickening.
A thorough work-up can differentiate between primary and secondary forms, mitigating progression of a more severe underlying disease.
Dinesh Khanna, MD, MSc, discusses results of the open-label portion of a trial evaluating the safety and efficacy of placebo-treated patients with systemic sclerosis who were switched to received open-label tocilizumab.
Sarah Taber, MD, briefly reviews her ongoing research on genetic drivers of localized scleroderma in pediatric patients.
Researchers sought to clarify the rates and risk factors of gangrene formation in patients with SSc utilizing the Digital Ulcer Outcomes Registry.
As part of Scleroderma Awareness Month, physicians specializing in the management of scleroderma discuss mitigating effects of scleroderma-related pain and improving quality of life for their patients.
Treatment with the endothelin-1 blocker macitentan did not reduce the incidence of new digital ulcers over 16 weeks.
If conservative treatment with medications such as dihydropyridine, calcium channel blockers, alpha-adrenergic blockers, and phosphodiesterase-5 inhibitors is unsuccessful, clinicians can consider chemical sympathectomy, botulinum neurotoxin injection, and fat pad grafting.
Regression of skin fibrosis is a feature of the natural history of dcSSc. However, the pattern of regression can be quite heterogeneous despite improvements in predicting progression.
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