Cortisone-Treated Scleroderma

Sharnoff, J. G.

doi:10.1001/jama.1951.02920340008003

Cited by 72 publications

(7 citation statements)

References 2 publications

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“…Multiple case reports [6][7][8][9][10][11] and retrospective studies subsequently supported a possible association [12][13][14]. In a case-control study of 110 SSc patients from the Pittsburg cohort who developed SRC between 1981 and 1993, new use of prednisone in dosages C15 mg/ day was associated with a fourfold increase in the onset of SRC (odds ratio 4.37, 95% confidence interval 2.03-9.43) [12].…”

Section: Introductionmentioning

confidence: 99%

Corticosteroids and the risk of scleroderma renal crisis: a systematic review

et al. 2010

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Scleroderma renal crisis (SRC) has been associated with the use of corticosteroids (CS) in retrospective studies. Using an evidence-based approach, we undertook a systematic review of the literature to identify prospective studies in which scleroderma patients were administered CS to ascertain the risk of SRC in those patients. A comprehensive search was conducted using Medline, EMBASE, the Cochrane Library, and Web of Science. All original prospective clinical studies were eligible if they enrolled SSc patients newly treated with CS. Selected studies were reviewed, and data extraction was systematically performed for the dose and duration of the CS intervention as well as the occurrence of SRC. Twenty-six studies with a total of 500 SSc patients commencing new CS therapy were included in the systematic review. Ten definite cases of SRC, equivalent to a rate of 2%, were identified. In the subset of early diffuse patients, the rate of SRC was 4%. All 10 definite cases of SRC occurred in patients who received medium- to high-dose CS therapy. Seven cases occurred in the setting of stem cell transplant. CS are associated with SRC, although this may be due to confounding by disease severity and/or co-intervention. Great caution must continue to be exerted when initiating such therapy, especially in high doses and in the early diffuse subset of SSc patients.

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Section: Introductionmentioning

confidence: 99%

Corticosteroids and the risk of scleroderma renal crisis: a systematic review

et al. 2010

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“…Predictors of renal crisis include early disease, diffuse cutaneous involvement, rapid progression of skin thickening (2), and possibly, anti-RNA polymerase I11 antibody (3). Anecdotal comments and a few case reports (4,5) have suggested that corticosteroids are associated with the development of renal crisis. However, patients who have early scleroderma with prorninent inflammatory features are precisely those who are most likely to receive steroids.…”

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confidence: 99%

Case-control study of corticosteroids and other drugs that either precipitate or protect from the development of scleroderma renal crisis

Steen

Medsger

1998

Arthritis & Rheumatism

434

170

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Objective. To determine whether the initiation of corticosteroids or other types of therapy affects the development of scleroderma renal crisis (SRC).Methods. Using a case-control study, 110 patients with systemic sclerosis who developed SRC between 1981 and 1993 were closely matched with controls on sex, race, age, disease duration, skin score, levels of creatine phosphokinase, and presence of tendon friction rubs. Corticosteroid use was determined prior to the onset of SRC in cases or prior to the first visit in controls. Cases were compared with matched controls using McNemar's matched-pair analysis and conditional logistic regression analysis. The effects of other drugs, including D-penicillamine, nonsteroidal antiinflammatory drugs (NSAIDs), calcium channel blockers, and angiotensin-converting enzyme (ACE) inhibitors, were also evaluated.Results. In the 6 months prior to SRC onset or to the first visit, high-dose corticosteroids (215 mg/day prednisone or equivalent) were administered significantly more frequently in SRC patients (36%) than in the controls (12%) (McNemar's odds ratio 4.37, 95% confidence interval 2.03-9.43, P < 0.0001). New use of low-dose steroids, continuous use of any steroid dose, NSAIDs, calcium channel blockers, and ACE inhibitors were not associated with an increased risk of SRC. Antecedent D-penicillamine therapy may have been protective against the development of SRC in controls.Conclusion. This retrospective case-control study has shown a significant association between antecedent high-dose corticosteroid therapy and the development of SRC. These results should discourage the use of highdose corticosteroids in patients with early diffuse scleroderma who are at increased risk of developing SRC.Scleroderma renal crisis (SRC) has been considered one of the most severe complications of systemic sclerosis. Although early treatment with angiotensinconverting enzyme (ACE) inhibitors has dramatically improved the outcome in patients with this complication, not all patients escape death or permanent dialysis (1). Predictors of renal crisis include early disease, diffuse cutaneous involvement, rapid progression of skin thickening (2), and possibly, anti-RNA polymerase I11 antibody (3). Anecdotal comments and a few case reports (4,5) have suggested that corticosteroids are associated with the development of renal crisis. However, patients who have early scleroderma with prorninent inflammatory features are precisely those who are most likely to receive steroids. The purpose of the present study was to determine whether corticosteroids are independently associated with renal crisis by controlling for the above-mentioned factors. It has been reported that D-penicillamine may prevent renal crisis (6) and ACE inhibitors may also be potentially prophylactic. Therefore, the effects of these drugs were also studied.

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“…Previous autopsy series of cardiac involvement in SSc describe hypertrophy and/or fibrosis primarily of the myocardium; however, fibrosis of the endocardium, pericardium, and epicardium has been reported [13,14]. These autopsy studies suggest that cardiac fibrosis represents a late feature of disease; however, cardiac remodeling, characterized by myocardial fibrosis, particularly within the interstitium in patients with SSc, can be identified on endomyocardial biopsy before any signs or symptoms of heart failure appear [15].…”

Section: Discussionmentioning

confidence: 99%

Cardiac metabolomics and autopsy in a patient with early diffuse systemic sclerosis presenting with dyspnea: a case report

et al. 2015

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IntroductionDiffuse systemic sclerosis is associated with high mortality; however, the pathogenesis of cardiac death in these patients is not clear.Case presentationA 56-year-old Caucasian female patient presented with dyspnea and requested to donate her body to science in order to improve understanding of diffuse systemic sclerosis pathogenesis. She had extensive testing for dyspnea including pulmonary function tests, an echocardiogram, cardiac magnetic resonance imaging, and right heart catheterization to characterize her condition. Her case highlights the morbidity seen in this disease, including the presence of extensive skin thickening, digital ulcerations, and scleroderma renal crisis.ConclusionIn this case report, we present the finding of cardiac tissue metabolomics, which may indicate a problem with vasodilation as a contributor to cardiac death in diffuse systemic sclerosis. The use of autopsy and tissue metabolomics in rare disease may help clarify disease pathogenesis.

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Cortisone-Treated Scleroderma

Cited by 72 publications

References 2 publications

Corticosteroids and the risk of scleroderma renal crisis: a systematic review

Corticosteroids and the risk of scleroderma renal crisis: a systematic review

Case-control study of corticosteroids and other drugs that either precipitate or protect from the development of scleroderma renal crisis

Cardiac metabolomics and autopsy in a patient with early diffuse systemic sclerosis presenting with dyspnea: a case report

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