Contents
Definition
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.
Symptoms
- 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)
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
Examinations
- 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
Diagnosis
- 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)
- Anti - GBM is taken for differential diagnostic reasons (Goodpastures disease)
- Catastrophic Antiphospholipid Syndrome (Lupus Anticoagulant, Cardiolipin, Beta-2 GP)
- TTP-HUS, aHUS
Treatment
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
Medication
- 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:
Prognosis
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