HTN: Scleroderma Flashcards
Scleroderma Renal Crises
Scleroderma renal crisis
occurs in 2% with limited disease (skin involvement below elbows and knees only)
SRC occurs in ~5% to 10% of diffuse scleroderma (skin involvement below and above elbows and knees)
Scleroderma renal crisis
Clincal Manifestations
Typically presents at diagnosis of scleroderma or within 3 to 4 years of disease onset.
Acute onset of moderate to severe “accelerated” HTN and oliguric kidney failure
Accompanying features: hyperrenin, thrombotic microangiopathy, anemia, congestive heart failure, and/or hypertensive encephalopathy and retinopathy
10% of SRC occurs with relative normotension which may reflect low baseline BP or concurrent acute illness with associated fall in BP.
Scleroderma renal crisis
Risks
Early diffuse scleroderma, rapidly progressive skin disease and tendon friction rubs
Scleroderma renal crisis
Risks
Corticosteroid exposure
Scleroderma renal crisis
Risks
HLA DRB10407, HLA-DRB11304, endothelin B receptor polymorphisms, soluble CD147
Scleroderma renal crisis
Factors not associated with SRC
Positive anticentromere antibody
Baseline BP, creatinine, proteinuria, hematuria
Gender
Scleroderma renal crisis
Management
ACEI are first-line therapy. ARB are not sufficient as first-line to control BP
Scleroderma renal crisis
Management
Goal: reduce SBP/DBP by 20/10 mm Hg per 24 hours.
Scleroderma renal crisis
Management
Prophylaxis therapy: None proven effective
Prophylactic ACEI may lead to worse outcome and higher likelihood or dialysis dependency for unclear reasons.
Scleroderma renal crisis
Management
Effective and improve survival
Scleroderma renal crisis
Management
Patients should be on ≥2 years on dialysis before consideration for transplant due to high recovery rates.
Scleroderma renal crisis
Management
Recurrence of SRC < 5% and more common in those with early native kidney loss due to SRC.
