Renal disease in systemic sclerosis (scleroderma), including scleroderma renal crisis
- John Varga, MD
John Varga, MD
- John and Nancy Hughes Professor of Medicine
- Northwestern University Feinberg School of Medicine
- Andrew Z Fenves, MD
Andrew Z Fenves, MD
- Associate Professor
- Harvard University, Department of Medicine
- Section Editors
- Richard J Glassock, MD, MACP
Richard J Glassock, MD, MACP
- Editor-in-Chief — Nephrology
- Section Editor — Glomerular Diseases
- Emeritus Professor
- The David Geffen School of Medicine at UCLA
- Gary C Curhan, MD, ScD
Gary C Curhan, MD, ScD
- Section Editor — Chronic Kidney Disease
- Professor of Medicine
- Harvard Medical School
The pathologic hallmarks of systemic sclerosis (SSc, scleroderma) are uncontrolled accumulation of collagen and widespread vascular lesions characterized by thickening of the vascular wall and narrowing of the vascular lumen. (See "Pathogenesis of systemic sclerosis (scleroderma)".)
Scleroderma may affect only the skin and subjacent tissues, or it may be associated with systemic involvement. The term systemic sclerosis is used when the characteristic skin lesions are associated with internal organ involvement. (See "Overview of the clinical manifestations of systemic sclerosis (scleroderma) in adults".)
Renal involvement is common in patients with SSc, with the most serious manifestation being scleroderma renal crisis (SRC), which occurs in a minority of patients. (See 'Prevalence and manifestations of renal disease' below.)
SRC is characterized by three major features (see 'Clinical features' below):
●Abrupt onset of moderate to severe hypertension that is typically associated with an increase in plasma renin activity
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- PREVALENCE AND MANIFESTATIONS OF RENAL DISEASE
- SCLERODERMA RENAL CRISIS
- Clinical features
- Risk factors
- - Diffuse skin involvement
- - Glucocorticoid use
- - Autoantibodies
- - Cyclosporine
- - Other risk factors
- Diagnosis and differential diagnosis
- - ACE inhibitors and other antihypertensive agents
- - Avoidance of glucocorticoids
- - Angiotensin-converting enzyme inhibitors
- Monitoring serum creatinine
- Unproven efficacy of angiotensin II receptor blockers
- - Non-angiotensin-converting enzyme inhibitor therapy
- - Initial approach
- - Long-term therapy
- Addition of other antihypertensive drugs
- End-stage renal disease
- - Efficacy of angiotensin-converting enzyme inhibitors
- - Dialysis
- - Renal transplantation
- Minimum wait time for transplantation
- Recurrent scleroderma renal crisis
- Patient survival
- SUMMARY AND RECOMMENDATIONS