Kidney crisis, hypertensive crisis in systemic sclerosis 4.8/5 (5)

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Acute hypertensive renal crisis ("renal crisis") / scleroderma renal crisis is a serious complication that occurs in 5-10% with the diffuse form of Systemic sclerosis  and less frequently (2%) in the limited form. Kidney crisis most often occurs during the first two to three years after the onset of the disease. The kidneys lose function quickly and blood pressure rises.

  • Occurrence of Anti-RNA polymerase III antibody (10-60%) in blood sample is a determining factor
    • RNA polymerase III antibody occurs in 10-60% who experience scleroderma renal crisis
    • Renal crisis usually develops within the first few years of the disease course
  • Corticosteroids (Prednisolone, SoluMedrol) are unfavorable and may trigger a new crisis in systemic sclerosis
  • Fast onset treatment with ACE inhibitors or AT inhibitors is important, but preventive ACE inhibitor therapy does not help.


  • New headache
  • Nausea
  • Visual disturbances
  • Epileptic cramps
  • Renal function falls rapidly
    • Renal function is measured by creatinine or GFR in serum (blood sample)
  • Blood pressure increases sharply (more than 150 / 90mmHg, often much higher)
  • Normotensive scleroderma kidney crisis occurs (10%)
    • Creatinine rises above 50% from baseline values
    • New proteinuria
    • New Hematuria
    • Platelets under 100.000
  • Kidney crisis can quickly cause lasting kidney damage, and high blood pressure damages the brain (encephalopathy), eyes (retinopathy) and the heart (failure)

    Renal crisis

    Kidney crisis, tissue sample: Micronephro-angiopathy in scleroderma renal crisis Affiliation: Batal I, 2010. Department of Pathology, University of Pittsburgh Medical Center, Pittsburgh, PA, USA. Open


  • Blood pressure measurements every hour until normalization (monitoring department)
  • Blood tests (renal function)
    • Creatinine (rising) and GFR (falling), LD (rising), Haptoglobin (falling: exclude) Hemolytic anemia), Platelets (falls), reticulocytes (rises), blood smears (Schistocytter over 1%)
    • RNA polymerase III is examined in a blood sample (if unknown status) by agreement with the Immunological Lab, Oslo University Hospital
    • ADAMTS13 (Willebrand factor-cleaving protease activity in plasma) is normal, unlike low values ​​at Thrombotic thrombocytopenic purpura, TTP/ Thrombotic microangiopathy which is a differential diagnosis
      • Sending sample to the Department of Biochemistry, St Olavs Hospital
  • Urine (proteinuria and hematuria may be present). Urine production (falling) must be measured
  • Collaboration with nephrologist
  • Kidney biopsy can be done when blood pressure is normalized
  • Eye doctor examination / ophthalmoscopy to assess retinopathy
  • ECG, troponins and echocardiography if cardiac symptoms
  • Telemetry


  • Known systemic sclerosis, most often diffuse form with short disease duration
  • Declining renal function
    • 50% increase in creatinine in the blood or 20% over the upper reference range
  • Proteins in the urine (> 2+ on stick)
  • Blood in the urine (> 2+ or 10 reds per field of view)
  • Platelets in the blood are below 100.000

Incorrect diagnosis (Similar conditions, Differential diagnoses)


Treatment is extremely important for the prognosis and early treatment is essential.

  • Treatment is often started at an intensive care unit that monitors the course of the disease


  • ACE inhibits captopril (Capoten®) first choice
    • The starting dose is 6,25 mg - 12,5 mg which is gradually increased to 12,5 -25mg every 4 to 8 hours if blood pressure dictates
    • Maximum doses are 300-450mg / day
    • The systolic blood pressure should decrease by approx. 20 mmHg per day, diastolic blood pressure reduction by 10 mmHg per day until normalization
    • One strives to reach the patient's baseline blood pressure within 3 days
    • Also, normotensive patients (with other symptoms of kidney crisis) should be treated, but then with lower doses, such as captopril 6,25-12,5mg / day
    • Increasing creatinine in blood samples is common due to the disease, but also as a side effect to ACE inhibitor
    • If cough or other side effects occur from ACE inhibitors, may Angiotensin blockers (AT-II inhibitors) be alternatives. Similarly, due to lack of effect of ACE inhibits during 2 day

If a lack of treatment response can be attempted:


The prognosis is best when treatment begins early, but differs from person to person. Normalization of renal function in 10-40% of cases. Transient dialysis is required in approx. 50%. Approximately 20% need sustained dialysis / kidney transplantation.

  • Relapse of scleroderma renal crisis is very uncommon


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