What is Scleroderma Renal Crisis (SRC)?

In systemic scleroderma, the kidneys can be damaged due to a sudden and severe increase in blood pressure. This is called scleroderma renal crisis (SRC).

Scleroderma Renal Crisis is very serious. There are risks that can help identify it early. Scleroderma renal crisis risks include:

• Rapidly progressing diffuse systemic sclerosis, often within the first year.
• Having tendon friction rubs
• Prior use of high dose corticosteroids
• Presence of anti-RNA polymerase III (RNAP III) antibodies, specific autoantibodies strongly linked to Systemic Sclerosis (SSc)
• New anemia, a possible early clue to the onset of SRC typically presenting as microangiopathic hemolytic anemia (MAHA)
• Cardiac events, like cardiac insufficiency or pericardial effusion, may precede SRC

Symptoms of Scleroderma Renal Crisis

Sudden onset of high blood pressure (hypertension) of more than 150/85mmHg is the key symptom of renal crisis. It causes an almost immediate decrease in kidney function.

Scleroderma Renal Crisis symptoms to watch for are:

• Headache
• Visual disturbances
• Seizures
• Fever
• General malaise
• Changes in blood or urine chemistry, including suddenly rising creatinine and blood urea nitrogen (BUN)
• Pulmonary edema (fluid in the lungs)

Scleroderma Renal Crisis Treatments

One class of antihypertensive medications known as angiotensin converting enzyme inhibitors (ACE inhibitors) are effective, although many patients require more than one drug for treatment of renal crisis. If the blood pressure can be promptly and completely controlled, kidney injury can be prevented and over time can reverse.

What can you do to minimize Scleroderma Renal Crisis symptoms?

• Learn how to measure your blood pressure and do so at least every other day.
• Avoid or minimize the use of Corticosteroids
• Avoid or minimize the use of nonsteroidal anti-inflammatory drugs (NSAIDs), like Ibuprofen or Naproxen, which can reduce blood flow to the kidneys.