Related Experiment Videos
Systemic sclerosis sine scleroderma: an unusual presentation in scleroderma renal crisis
J F Molina1, J M Anaya, G E Cabrera
1Department of Medicine, Louisiana State University Medical Center, New Orleans 70112-2822, USA.
The Journal of Rheumatology
|March 1, 1995
Summary
Systemic sclerosis sine scleroderma can cause kidney failure before skin symptoms appear. This case highlights scleroderma renal crisis and anti-RNAP III antibodies in patients presenting without initial skin changes.
Area of Science:
- Rheumatology
- Nephrology
- Immunology
Background:
- Systemic sclerosis (SSc) significantly impacts prognosis, particularly with renal involvement.
- SSc sine scleroderma, characterized by organ damage without concurrent skin manifestations, presents diagnostic challenges.
- Early recognition is crucial for managing SSc-related complications.
Observation:
- A patient presented with symmetric polyarthritis followed by rapid progressive renal failure.
- Crucially, the patient exhibited no initial cutaneous symptoms of SSc.
- Anti-RNA Polymerase III (RNAP III) antibodies were detected, aiding in diagnosis.
Findings:
- Histological confirmation supported a diagnosis of scleroderma renal crisis.
- Typical SSc skin changes manifested only after the onset of renal failure.
- Despite therapeutic interventions, the patient progressed to end-stage renal disease.
Implications:
- This case underscores the importance of considering scleroderma renal crisis in patients with unexplained renal failure, even without skin findings.
- The presence of anti-RNAP III antibodies may indicate a higher risk for renal complications in early SSc.
- Highlights the potential for delayed cutaneous manifestations in SSc sine scleroderma, emphasizing the need for comprehensive patient monitoring.