Predictors and outcomes of scleroderma renal crisis: Data from the high‐dose versus low‐dose D‐penicillamine in early diffuse systemic sclerosis trial

Strand, Vibeke

doi:10.1002/art.10602

Cited by 4 publications

(2 citation statements)

References 14 publications

(13 reference statements)

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“…AZA, which has been shown to improve survival in IPF and is also associated with a favorable side effect profile, was used as maintenance therapy, in order to avoid the need for ongoing parenteral treatment. The concurrent use of corticosteroids reflects current consensus management in both IPF and SSc‐associated pulmonary fibrosis, although RCT data are lacking; the steroids were administered in low doses to avoid the potentially increased risk of scleroderma renal crisis (32). Therapy was well tolerated, with transient nausea, rashes, hepatic dysfunction, and diarrhea in a very small proportion of patients.…”

Section: Discussionmentioning

confidence: 99%

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

Hoyles¹,

Ellis²,

Wellsbury³

et al. 2006

Arthritis & Rheumatism

652

384

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Objective. The lack of randomized controlled trials (RCTs) in pulmonary fibrosis in systemic sclerosis (SSc) has hampered an evidence-based approach to treatment. This RCT was undertaken to investigate the effects of intravenous (IV) cyclophosphamide (CYC) followed by azathioprine (AZA) treatment in pulmonary fibrosis in SSc.Methods. Forty-five patients were randomized to receive low-dose prednisolone and 6 infusions (monthly) of CYC followed by oral AZA, or placebo. Primary outcome measures were change in percent predicted forced vital capacity (FVC) and change in single-breath diffusing capacity for carbon monoxide (DLCO). Secondary outcome measures included changes in appearance on high-resolution computed tomography and dyspnea scores. An intent-to-treat statistical analysis was performed.Results. At baseline, there were no significant group differences in factors linked to outcome, including severity of pulmonary fibrosis and autoantibody status. Sixty-two percent of the patients completed the first year of treatment. Withdrawals included 9 patients (6 from the placebo group) with significant decline in lung function, 2 with treatment side effects (both from the active treatment group), and 6 with non-trialrelated comorbidity. No hemorrhagic cystitis or bone marrow suppression was observed. Estimation of the relative treatment effect (active treatment versus placebo) adjusted for baseline FVC and treatment center revealed a favorable outcome for FVC of 4.19%; this between-group difference showed a trend toward statistical significance (P ‫؍‬ 0.08). No improvements in DLCO or secondary outcome measures were identified.Conclusion. This trial did not demonstrate significant improvement in the primary or secondary end points in the active treatment group versus the group receiving placebo. However, for FVC there was a trend toward statistical significance between the 2 groups. This suggests that treatment of pulmonary fibrosis in SSc with low-dose prednisolone and IV CYC followed by AZA stabilizes lung function in a subset of patients with the disease. Therapy was well tolerated with no increase in serious adverse events.

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Section: Discussionmentioning

confidence: 99%

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

Hoyles¹,

Ellis²,

Wellsbury³

et al. 2006

Arthritis & Rheumatism

652

384

View full text Add to dashboard Cite

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“…Multivariate regression analysis demonstrated that clinical characteristics rather than prednisone use was more important in predicting the development of SRC. [29,30] Therefore, if systemic steroids are used in patients with SSc, the renal function and blood pressure must be monitored carefully. The use of prophylactic ACE-I is controversial but should be considered in this particular clinical situation.…”

Section: Discussionmentioning

confidence: 99%

Acute Kidney Injury in Patients with Systemic Sclerosis Participating in Hematopoietic Cell Transplantation Trials in the United States

Hosing

Nash

McSweeney

et al. 2011

Biology of Blood and Marrow Transplantation

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Recipients of hematopoietic cell transplantation may be at risk for developing acute kidney injury and this risk may increase in patients who undergo transplantation for severe systemic sclerosis due to underlying scleroderma renal disease. Acute kidney injury after transplantation can increase transplant related mortality. To better define these risks, we analyzed 91 patients with systemic sclerosis who were enrolled in three clinical trials in the United States of autologous or allogeneic hematopoietic cell transplantation. Eleven (12%) of the 91 scleroderma patients in these studies (8 autologous, 1 allogeneic, 1 pre-transplant, 1 given intravenous cyclophosphamide on transplant trial) experienced acute kidney injury of whom eight required dialysis and/or total plasma exchange. Acute kidney injury in the 9 transplant recipients developed a median of 35 (range, 0–90) days after transplantation. Ten of 11 patients with acute kidney injury received angiotensin converting enzyme-inhibitors (ACE-I) treatment. The etiology of acute kidney injury was attributed to scleroderma renal crisis in 6 patients (including two with normotensive renal crisis), acute kidney injury of uncertain etiology in 2 patients and acute kidney injury superimposed on scleroderma kidney disease in 3 patients. Eight of the 11 patients died: causes of death included progression of SSc (1), multiorgan failure (1), gastrointestinal and pulmonary bleeding (1), pericardial tamponade and pulmonary complications (1), diffuse alveolar hemorrhage (1), pulmonary embolism (1), graft-versus-host disease (1) and malignancy (1). Limiting nephrotoxins, cautious use of corticosteroids, renal shielding during total body irradiation, strict control of blood pressure and aggressive use of ACE-I may be of importance in preventing renal complications after hematopoietic cell transplantation for systemic sclerosis.

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High-dose versus low-dose ?-penicillamine in early diffuse systemic sclerosis trial: lessons learned

Clements

Seibold

Fürst

et al. 2004

Seminars in Arthritis and Rheumatism

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Predictors and outcomes of scleroderma renal crisis: Data from the high‐dose versus low‐dose D‐penicillamine in early diffuse systemic sclerosis trial

Cited by 4 publications

References 14 publications

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

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

Acute Kidney Injury in Patients with Systemic Sclerosis Participating in Hematopoietic Cell Transplantation Trials in the United States

High-dose versus low-dose ?-penicillamine in early diffuse systemic sclerosis trial: lessons learned

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