Scleroderma renal crisis: Difference between revisions
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== Clinical |
== Clinical Manifestations == |
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* Acute onset renal failure without another cause |
* Acute onset renal failure without another cause |
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Latest revision as of 10:58, 2 August 2020
Clinical Manifestations
- Acute onset renal failure without another cause
- Bland urine sediment, though can have proteinuria and hematoria
- Acute onset moderate tosevere hypertension, often with hypertensive emergency
- Papilledema
- Hypertensive retinopathy
- Hypertensive encephalopathy
- Additional findings
- Bicytopenia with MAHA and thrombocytopenia
- Flash pulmonary edema
- New-onset proteinuria or hematuria without other cause
Management
- Blood pressure control, primarily with ACE inhibitors, is the primary treatment
- First-line: captopril has the most evidence
- Initial dose 6.25 to 12.5 mg, increase by 12.5 to 25 mg every 4 to 8 hours to achieve target
- Alternatives: enalapril or ramipril
- Target return to baseline BP within 72 hours, with about 20 mmHg reduction daily
- First-line: captopril has the most evidence
- For CNS disease, can add nitroprusside
- Monitor creatinine
Prevention
- No clear role for ACE inhibitors for primary prevention
- ACE inhibitors are continued indefinitely for secondary prevention
- Avoid beta blockers, which can worsen Raynaud phenomenon
- Avoid glucocorticoids if possible, as they can precipitate crises
Prognosis
- Untreated, SRC progresses to ESRD within month
- Treated, 20 to 50% still end up on dialysis
