Systemic Sclerosis and Life-Threatening Renal Crisis

By Lauren Thayer from In The Cloud Copy

Systemic sclerosis is a rare autoimmune disease that is characterized by vascular abnormalities and fibrosis of various parts of the body, including the joints, skin, and many internal organs such as the lungs, heart, kidneys, and parts of the gastrointestinal tract. Systemic sclerosis, or SS, is caused by an increased in collagen production in the body that accumulates in the tissue. The exact cause of SS is unknown at this point and there is no cure for the disease. Healthcare providers can only manage symptoms with various medications and alternative therapies. There are many serious complications of SS, but one of the most serious is renal involvement from vasospasm and arterial damage, called renal scleroderma.

What is Renal Scleroderma?

Renal scleroderma typically starts with rapid onset of high blood pressure, which is not usually recognized immediately. The longer the blood pressure is high, the more serious the condition becomes, with the kidneys ultimately being damaged. Renal scleroderma can ultimately result in a scleroderma renal crisis or SRC. An SRC is characterized by severe hypertension, with BPs consistently over 150/90 mm Hg, as well as acute onset of renal failure. Blood vessel spasms usually occur in SRC, resulting in even higher blood pressure and further kidney damage. Kidney tissue deprived of blood flow will not function normally. It is believed that both genetic and environmental factors play a factor in the development and pathogenesis of SRC.

Symptoms of Scleroderma Renal Crisis

Patients may experience a range of symptoms, but it’s important to recognize what could be causing them for prompt treatment. Symptoms may include headaches, encephalopathy, seizures, malaise, fevers, pulmonary edema, and hypertensive retinopathy. Lab tests on patients experiencing SRC may present with increased creatinine, increased renin concentration, proteinuria and hematuria. It is important for patients with known systemic sclerosis to be aware of these symptoms and seek prompt treatment if any develop.

Risk Factors for Development of SRC

The main risk factor that has been identified appears to be a rapidly progressive skin thickening as most SRC patients develop symptoms within 7.5 months to 4 years of initial disease onset. Other risk factors that have been identified include anemia, myopathy, large joint contractures, cardiac events, and myalgia. Other identified risk factors include high doses of corticosteroids as well as cyclosporin use.

Treatment and Prevention of SRC

ACE Inhibitors are a first line treatment if SRC is caught early enough. If diagnosis is not prompt and ACE inhibitors are not started and used aggressively, then kidney damage and kidney death are more likely to occur. If SRC is not detected at an early stage then other serious complications are likely to occur, such as hypertension and pulmonary edema. Controlling blood pressure is at the forefront of treatment. If ACE inhibitors are not sufficient in controlling blood pressure, then addition of other anti-hypertensive medications would be warranted.

Preventative measures include things such as blood pressure monitoring and renal function monitoring through lab testing are imperative.

Even with aggressive treatments, unfortunately around half of SRC patients will require permanent dialysis. Some patients ultimately end up needing renal transplantation, but this is not always possible for patients as multi-organ involvement is often seen.

Overall, patients with systemic sclerosis need regular blood pressure monitoring and possibly other non-invasive testing to monitor for renal involvement. Controlling blood pressure at first signs of hypertension is essential to better outcomes.

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