Tags

Type your tag names separated by a space and hit enter

Scleroderma renal crisis and renal involvement in systemic sclerosis.
Nat Rev Nephrol. 2016 11; 12(11):678-691.NR

Abstract

Scleroderma renal crisis (SRC) is a rare, potentially life-threatening complication that affects 2-15% of patients with systemic sclerosis (SSc, also known as scleroderma). SRC typically presents in patients with early, rapidly progressive, diffuse cutaneous SSc within the first 3-5 years after the onset of a non-Raynaud sign or symptom. SRC is characterized by an acute, usually symptomatic increase in blood pressure, a rise in serum creatinine levels, oliguria and thrombotic microangiopathy in about 50% of patients. The prognosis of SRC substantially improved in the 1980s with the introduction of angiotensin-converting-enzyme inhibitors for rapid blood pressure control, with additional antihypertensive agents as required. However, the survival of patients with SRC can still be improved. Current patient survival is 70-82% at 1 year, but decreases to 50-60% at 5 years despite dialysis support. Patients with SRC who show no signs of renal functional recovery despite timely blood pressure control are candidates for transplantation. In this Review, we discuss progress made in the identification and proactive management of patients at risk of SRC and make recommendations aimed at optimizing management for those who progress to chronic kidney failure.

Authors+Show Affiliations

Division of Rheumatology, David Geffen School of Medicine, University of California, 100 Veterans Avenue, Los Angeles, California 90025, USA.Division of Rheumatology, David Geffen School of Medicine, University of California, 100 Veterans Avenue, Los Angeles, California 90025, USA. Rheumatology and Rehabilitation Department, Assiut University Hospital, Assiut, Egypt.Division of Rheumatology, David Geffen School of Medicine, University of California, 100 Veterans Avenue, Los Angeles, California 90025, USA.Division of Rheumatology, David Geffen School of Medicine, University of California, 100 Veterans Avenue, Los Angeles, California 90025, USA.Division of Rheumatology, David Geffen School of Medicine, University of California, 100 Veterans Avenue, Los Angeles, California 90025, USA.

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

27641135

Citation

Woodworth, Thasia G., et al. "Scleroderma Renal Crisis and Renal Involvement in Systemic Sclerosis." Nature Reviews. Nephrology, vol. 12, no. 11, 2016, pp. 678-691.
Woodworth TG, Suliman YA, Li W, et al. Scleroderma renal crisis and renal involvement in systemic sclerosis. Nat Rev Nephrol. 2016;12(11):678-691.
Woodworth, T. G., Suliman, Y. A., Li, W., Furst, D. E., & Clements, P. (2016). Scleroderma renal crisis and renal involvement in systemic sclerosis. Nature Reviews. Nephrology, 12(11), 678-691. https://doi.org/10.1038/nrneph.2016.124
Woodworth TG, et al. Scleroderma Renal Crisis and Renal Involvement in Systemic Sclerosis. Nat Rev Nephrol. 2016;12(11):678-691. PubMed PMID: 27641135.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Scleroderma renal crisis and renal involvement in systemic sclerosis. AU - Woodworth,Thasia G, AU - Suliman,Yossra A, AU - Li,Wendi, AU - Furst,Daniel E, AU - Clements,Philip, Y1 - 2016/09/19/ PY - 2016/9/20/pubmed PY - 2017/5/26/medline PY - 2016/9/20/entrez SP - 678 EP - 691 JF - Nature reviews. Nephrology JO - Nat Rev Nephrol VL - 12 IS - 11 N2 - Scleroderma renal crisis (SRC) is a rare, potentially life-threatening complication that affects 2-15% of patients with systemic sclerosis (SSc, also known as scleroderma). SRC typically presents in patients with early, rapidly progressive, diffuse cutaneous SSc within the first 3-5 years after the onset of a non-Raynaud sign or symptom. SRC is characterized by an acute, usually symptomatic increase in blood pressure, a rise in serum creatinine levels, oliguria and thrombotic microangiopathy in about 50% of patients. The prognosis of SRC substantially improved in the 1980s with the introduction of angiotensin-converting-enzyme inhibitors for rapid blood pressure control, with additional antihypertensive agents as required. However, the survival of patients with SRC can still be improved. Current patient survival is 70-82% at 1 year, but decreases to 50-60% at 5 years despite dialysis support. Patients with SRC who show no signs of renal functional recovery despite timely blood pressure control are candidates for transplantation. In this Review, we discuss progress made in the identification and proactive management of patients at risk of SRC and make recommendations aimed at optimizing management for those who progress to chronic kidney failure. SN - 1759-507X UR - https://www.unboundmedicine.com/medline/citation/27641135/Scleroderma_renal_crisis_and_renal_involvement_in_systemic_sclerosis_ L2 - http://dx.doi.org/10.1038/nrneph.2016.124 DB - PRIME DP - Unbound Medicine ER -