What are the other Names for this Condition? (Also known as/Synonyms)

• Gadolinium-Associated Nephrogenic Systemic Fibrosis
• NSF (Nephrogenic Systemic Fibrosis)

What is Nephrogenic Systemic Fibrosis? (Definition/Background Information)

• Nephrogenic Systemic Fibrosis (NSF) is a rare condition observed with the involvement of several internal organs including the skin and eye. In this condition, there can be fibrosis of major organs of the body such as the heart, lungs, or liver, which can result in disruption of their function
• The cause of the condition is unknown, but it is strongly linked to gadolinium exposure during magnetic resonance angiography (MRA) studies. Also, a majority of cases are known to develop in individuals undergoing dialysis for kidney failure
• Nephrogenic Systemic Fibrosis mostly affects young, middle-aged, and old adults. The symptoms are systemic and may also include skin thickening, limited range of motion due to joint pain, and weak muscles
• It is difficult to treat Nephrogenic Systemic Fibrosis; though in some patients, the use of monoclonal antibodies has been found to be beneficial
• The prognosis of this chronic disorder is typically poor in spite of appropriate treatment, since it is associated with systemic fibrosis and many vital organs are typically affected

Who gets Nephrogenic Systemic Fibrosis? (Age and Sex Distribution)

• Nephrogenic Systemic Fibrosis is a very infrequently occurring condition that can be observed in young, middle-aged, and older adults; in the 30-80 years age category
• Both male and female genders are affected
• Individuals of all racial and ethnic groups may be affected

What are the Risk Factors for Nephrogenic Systemic Fibrosis? (Predisposing Factors)

Following are the risk factors for Nephrogenic Systemic Fibrosis:

• There is a strong association with exposure to gadolinium, which is used as a contrast material for magnetic resonance angiography (MRA) studies (in individuals with renal insufficiency)
• Kidney failure necessitating dialysis
• Individuals with hepatitis C virus (HCV) infection
• Those undergoing systemic immunosuppressive therapy for various causes

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Nephrogenic Systemic Fibrosis? (Etiology)

The cause of development of Nephrogenic Systemic Fibrosis is unknown.

• Most of the cases occur in patients having kidney failure and receiving dialysis therapy
• NSF is also associated with the contrast material gadolinium, which is used for imaging studies in individuals with kidney conditions
• It can also occur in cases, where there is acute kidney failure but the patient has not undergone a dialysis yet

What are the Signs and Symptoms of Nephrogenic Systemic Fibrosis?

The signs and symptoms of Nephrogenic Systemic Fibrosis may include the following:

• The signs and symptoms are dependent upon the organs affected; the function of the organs can be severely compromised
• Internal organs, such as the heart, lung, liver, and brain, may be affected with the formation of fibrosis
  • Fibrosis of the lungs can cause chest pain, cough, and shortness of breath
  • Liver fibrosis may result in scarring and inflammation of the liver
  • If the brain is affected, then there may be neurological symptoms
• Additionally, the skin, joints, eye, and muscles are also involved. When the skin is affected, the condition is known as Nephrogenic Fibrosing Dermopathy (NFD)
  • If the joints are affected, then it can result in joint pain. If the muscles are affected, then poor functioning of the muscles can occur
  • Itchy and painful skin lesions, usually on the lower extremities, are noted. It may appear like nodules or plaques
  • Since the kidney is affected due to kidney disease, it may give rise to peripheral edema, malnutrition, cognitive function decline, and several other symptoms

How is Nephrogenic Systemic Fibrosis Diagnosed?

Diagnostic tests that are performed for Nephrogenic Systemic Fibrosis may include:

• A thorough physical examination and evaluation of a complete medical history, including history of kidney disease
• Blood tests, to rule out other causes such as infections
• Tests to rule out skin conditions such as dermoscopy, KOH test, and allergy skin test
• Tissue biopsy of the affected region (skin): A pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Nephrogenic Systemic Fibrosis?

The complications of Nephrogenic Systemic Fibrosis may include:

• It can severely affect the function of the involved organ(s); important organs such as the heart, lung, liver, and brain may be severely affected with fibrosis
• Secondary skin infections
• Emotional and psychological stress
• The existing renal condition may worsen on exposure to gadolinium (during the imaging study)
• NSF is a chronic condition that can be fatal

How is Nephrogenic Systemic Fibrosis Treated?

• Nephrogenic Systemic Fibrosis is generally difficult to treat
• Newer monoclonal antibody treatments have been found to be helpful in some individuals
• Symptomatic treatment may be considered based on the organ affected
• Some treatment measures to improve comfort for the skin signs and symptoms may include:
  • Maintain cleanliness and body hygiene
  • Using moisturizers or other lotions to prevent the skin from drying
  • Use of topical steroidal creams and lotions
  • Antibiotics may be used to treat infections
• Undertaking treatment of any underlying disorders or conditions

How can Nephrogenic Systemic Fibrosis be Prevented?

Currently, it is not possible to prevent Nephrogenic Systemic Fibrosis. However, the following factors may be considered to avoid the condition:

• Take steps to ensure prevention of hepatitis C virus infection, which includes:
  • Practicing safe sex; avoid exposure to infected blood and other bodily fluids
  • Individuals with risk factors for hepatitis C, such as intravenous drug users, kidney dialysis patients, and individuals who do not practice safe sex and have multiple partners, should be tested for HCV
• Adequately treat kidney disease and kidney failure
• Closely monitor individuals receiving immunosuppressants for various reasons
• Minimize the use of MRA studies (using gadolinium) in ‘at-risk’ patients

What is the Prognosis of Nephrogenic Systemic Fibrosis? (Outcomes/Resolutions)

• The prognosis of Nephrogenic Systemic Fibrosis is generally poor despite suitable treatment
• The prognosis also depends on the severity of the underlying condition and the involvement of various internal organs

Additional and Relevant Useful Information for Nephrogenic Systemic Fibrosis:

End-stage kidney disease (ESKD) is a condition defined by near-complete or complete failure of the kidneys to excrete waste products and excess water from the body.