In a related study we used an observational design to complete one of the largest prospective evaluations of immunosuppression in SSc skin disease, focusing on the more severe diffuse subset of the disease .
Scleroderma Case Study - Physiopedia
This UK observational study recruited nearly cases of dcSSc, more than half from our centre, and evaluated immunosuppressive therapies including mycophenolate mofetil, cyclophosphamide, methotrexate and anti-thymocyte globulin. This study was a landmark initiative as it demonstrated that protocolised approaches with standardised observation, analogous to oncology strategies, could be used to explore best therapies for SSc, a condition with outcome worse than many malignancies.
Transforming growth factor-beta and connective tissue growth factor: Relationship between change in skin score and disease outcome in diffuse cutaneous systemic sclerosis: Improved survival in systemic sclerosis is associated with better ascertainment of internal organ disease: A multicenter, prospective, randomized, double-blind, placebo-controlled trial of corticosteroids and intravenous cyclophosphamide followed by oral azathioprine for the treatment of pulmonary fibrosis in scleroderma.
Interstitial lung disease in systemic sclerosis: Recombinant human anti-transforming growth factor beta1 antibody therapy in systemic sclerosis: The last 20 years has seen a significant improvement in outcome for patients with scleroderma, and specifically an increase in survival, much of it attributable to research from our centre.
We have more than 2, cases, seen over the past 20 years, where we have explored the change in outcome over time and also used the uniquely well-characterised patient cohort to define timing and frequency of each of the major life-threatening complications of the disease.
Our SSc cohort saw approximately 20 fewer deaths in compared to The biomarkers we have described have enabled better prediction of complications such as scleroderma renal crisis and lung fibrosis, and identified subsets that benefit from more aggressive treatment. As a result of our definition of hallmark SSc antibodies associated with lung fibrosis and scleroderma renal disease, these tests are now routinely used in risk assessment of SSc worldwide.
This permits more effective patient education and earlier engagement with other specialised hospital services.
Scleroderma Case Study
We have defined the cases of lung fibrosis in scleroderma that are most likely to benefit from immunosuppression and developed a simple staging system for lung fibrosis that is now used in most centres in UK and abroad and has been validated in two independent studies in the USA [c] and Australia. This helps to avoid use of toxic immunosuppression in cases of SSc where this is unnecessary and enables us to target immunosuppressive therapy to more severe cases who gain the most benefit. This is a result of our definition of "good prognosis" cases of SSc.
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SSc-associated ILD commonly presents with the subacute onset of dyspnea on exertion, sometimes associated with a nonproductive cough. Radiographs obtained at the urgent care clinic appear to have some classic features of SSc-associated ILD: SSc-associated ILD usually seen at the base of the lungs. Assessment of the radiograph for hazy or reticular opacities in the lower lobes, to evaluate for ILD, or enlarged pulmonary arteries, to evaluate for pulmonary hypertension, is difficult.
In contrast, chest radiography has good specificity in identifying pulmonary hypertension PH , by detection of enlargement of right pulmonary artery, loss of peripheral vasculature and filling of the retrosternal space by the hypertrophic right ventricle. High-resolution computed tomography HRCT is ordered to evaluate the structural abnormalities associated with ILD and to further examine the nature and location of the ground-glass opacities detected on chest radiograph.