Scleroderma Renal Crisis - Steroids

3/02/2005

Question:  Why are steroids contraindicated for scleroderma renal crisis?

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12415594.ui or 12381259.ui or 10879669.ui or 9751093.ui or 8923600.ui or 8842768.ui or 2775321.ui

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12415594[PMID] OR 12381259[PMID] OR 10879669[PMID] OR 9751093[PMID] OR 8923600[PMID] OR 8842768[PMID] OR 2775321[PMID]

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Unique Identifier [PMID]: 12415594

Authors: Griffiths B. Miles S. Moss H. Robertson R. Veale D. Emery P.

Institution: Department of Rheumatology, Freeman Hospital, Newcastle Upon Tyne, England. bridget.griffiths@ncl.ac.uk

Title: Systemic sclerosis and interstitial lung disease: a pilot study using pulse intravenous methylprednisolone and cyclophosphamide to assess the effect on high resolution computed tomography scan and lung function.

Source: Journal of Rheumatology. 29(11):2371-8, 2002 Nov.

Abstract: OBJECTIVE: To document the effectiveness, including the longterm effect, of a course of intravenous (IV) pulses of methylprednisolone (MP) and cyclophosphamide (CYC) in patients with scleroderma (SSc) who had evidence of lung inflammation on high resolution computer tomographic (HRCT) scan of the chest. METHODS: Fourteen consecutive patients with SSc and lung involvement were treated with 6 pulses of IV MP (10 mg/kg) and IV CYC (15 mg/kg) given at 3-4 weekly intervals. HRCT scans and lung function tests were performed at baseline and after the 6th pulse. Further lung function tests were repeated at 12 months and annually thereafter. RESULTS: Modified Rodnan skin scores improved significantly by 35% from a median baseline score of 17 (IQR 14-26.5) to a posttreatment score of 13 (IQR 10.5-18.5; p = 0.0058). HRCT scan scores improved significantly (p = 0.04). Twelve of 13 patients experienced either improvement or stabilization of the HRCT score. Median DLCO and lung volumes remained stable during the first 12 months. After a median followup of 26 months (IQR 19-43), 67% of patients experienced deterioration in DLCO. Median deterioration was 23% (IQR 44-0.6), with the median rate of deterioration of the predicted value of the DLCO/month being 0.87% (IQR 1.24-0.02). The treatment was safe and well tolerated. CONCLUSION: This IV regimen stabilized lung disease in patients with SSc. When treatment was stopped, or reduced in intensity, a deterioration in lung function occurred in the majority of patients. Rate of deterioration of DLCO may be a useful marker for determining the intensity of treatment. These findings have implications for treating lung disease and designing clinical trials in patients with SSc.

Publication Type: Clinical Trial. Journal Article.

<2>

Unique Identifier [PMID]: 12381259

Authors: Lee AT. Burnet S.

Title: Corticosteroid-induced scleroderma renal crisis.

Source: Medical Journal of Australia. 177(8):459, 2002 Oct 21.

Publication Type: Case Reports. Letter.

<3>

Unique Identifier [PMID]: 10879669

Authors: Kohno K. Katayama T. Majima K. Fujisawa M. Iida S. Fukami K. Ueda S. Nishida H. Sata M. Kato S. Morimatsu M. Okuda S.

Institution: Department of Internal Medicine III, Kurume University School of Medicine, Fukuoka, Japan.

Title: A case of normotensive scleroderma renal crisis after high-dose methylprednisolone treatment.

Source: Clinical Nephrology. 53(6):479-82, 2000 Jun.

Abstract: A 68-year-old male was admitted for interstitial pneumonia associated with scleroderma. High-dose methylprednisolone was administered for treatment of the pneumonitis. Two weeks later, anemia, thrombocytopenia and progressive increase in BUN, creatinine and LDH were observed. Although the blood pressure remained normotensive, renal biopsy showed thrombosis of the polar arterioles and glomerular capillaries. The affected interlobular artery included concentric intimal thickening and thrombosis in the lumen. Our findings suggested that the antecedent use of high-dose corticosteroids is involved in precipitating normotensive renal crisis. Corticosteroids should be used in low doses and with great caution in scleroderma patients.

Publication Type: Case Reports. Journal Article.

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Unique Identifier [PMID]: 9751093

Authors: Steen VD. Medsger TA Jr.

Institution: Georgetown University, Washington, DC, USA.

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

Source: Arthritis & Rheumatism. 41(9):1613-9, 1998 Sep.

Abstract: 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 (> or =15 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 high-dose corticosteroids in patients with early diffuse scleroderma who are at increased risk of developing SRC.

Publication Type: Journal Article.

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Unique Identifier [PMID]: 8923600

Authors: Steen VD.

Institution: Division of Rheumatology, Immunology, and Allergy, Georgetown University Medical Center, Washington, DC 20007-2197, USA.

Title: Scleroderma renal crisis. [Review] [72 refs]

Source: Rheumatic Diseases Clinics of North America. 22(4):861-78, 1996 Nov.

Abstract: Renal crisis occurs in systemic sclerosis patients with rapidly progressive diffuse cutaneous thickening early in their disease. SRC is characterized by malignant hypertension, hyperreninemia, azotemia, microangiopathic hemolytic anemia, and renal failure. This complication, which in the past has been almost uniformly fatal, is now successfully treated in most cases with ACE inhibitors. This therapy has improved survival, reduced requirement for dialysis, and in those on dialysis has often allowed discontinuation of this procedure 6 to 18 months later. Prompt diagnosis and early, aggressive initiation of therapy with ACE inhibitors will result in the most optimal outcome. Chronic nonrenal crisis renal insufficiency is unusual and rarely progresses to significant renal dysfunction. [References: 72]

Publication Type: Journal Article. Review.

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Unique Identifier [PMID]: 8842768

Authors: Yamanishi Y. Yamana S. Ishioka S. Yamakido M.

Institution: Second Department of Internal Medicine, Hiroshima University School of Medicine.

Title: Development of ischemic colitis and scleroderma renal crisis following methylprednisolone pulse therapy for progressive systemic sclerosis.

Source: Internal Medicine. 35(7):583-6, 1996 Jul.

Abstract: We describe a patient with progressive systemic sclerosis who developed ischemic colitis and scleroderma renal crisis following steroid pulse therapy. The possible pathogenic mechanisms of ischemic colitis and scleroderma renal crisis development are discussed. We conclude that the administration of steroids in high doses, especially via steroid pulse therapy, should be undertaken with caution for progressive systemic sclerosis patients.

Publication Type: Case Reports. Journal Article.

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Unique Identifier [PMID]: 2775321

Authors: Helfrich DJ. Banner B. Steen VD. Medsger TA Jr.

Institution: Department of Medicine, University of Pittsburgh School of Medicine, Pennsylvania 15261.

Title: Normotensive renal failure in systemic sclerosis.

Source: Arthritis & Rheumatism. 32(9):1128-34, 1989 Sep.

Abstract: Of 140 patients with "scleroderma renal crisis" encountered during a 33-year period, 15 of 131 (11%) whose blood pressures were recorded were normotensive during this complication. In comparison with 116 patients with hypertension, the normotensive patients significantly more often had microangiopathic hemolytic anemia (90% versus 38%) and thrombocytopenia (83% versus 21%). Pulmonary hemorrhage occurred in 6 normotensive patients. More normotensive patients had received high doses of corticosteroids (prednisone greater than or equal to 30 mg/day) during the 2 months immediately preceding renal crisis (64% versus 16%). A role for corticosteroids in precipitating renal crisis is suggested. The 12-month survival was significantly reduced in the normotensive patients (13% versus 35%).

Publication Type: Journal Article.