Retroperitoneal Fibrosis: Treatment, Cost and Side Effects

Retroperitoneal Fibrosis is defined as the formation of excess fibrous tissues that develops into a mass and leads inflammation of the kidney. What is retroperitoneal? It is basically the area behind the abdomen or stomach which is lined by a tissue and covers most of the organs of the abdomen (kidney, aorta, renal tract and other structures). The abdominal cavity is termed as peritoneum which is divided into three parts. The lower section of abdominal cavity is known as retroperitoneum. What is fibrosis? It refers to the growth of excess connective tissue (known as fascia that binds every organ together), leading to the formation of a mass. This overgrown mass leads to compression and blockage of the ureters which carry the urine from your kidneys to your bladder.

The formation of excess fibrous tissue can lead to the blockage of one or both the ureters that in turn causes urine to build up in your blood and kidney causing more harm to your body. It is progressive condition that typically starts with inflammation and fibrosis in the abdominal aorta (abdominal aorta is the large artery that brings blood from your heart to the areas below the kidneys). As the condition worsens, it starts to affect other organs leading to pain, leg swelling and decreased kidney functions.

The exact cause of the disease is not known but it is generally seen in autoimmune disorders where the body’s immune system starts to attack the healthy cells of your body. It can also occur when are having any one of these diseases or treatments like asbestos exposure, smoking, neoplasms (tumour), infections (like actinomysis, histoplasmosis), trauma, radiotherapy, surgery and use of certain drugs.

Retroperitoneal fibrosis is a rare condition where the kidney becomes inflamed and the ureters become blocked due to excess fiber tissue formation. Therefore, a proper diagnosis will involve CT and MRI scan of the abdomen to known the extent of the condition. Additional tests may include blood tests to measure kidney function, anemia and inflammation, an X-ray of the kidneys and ureters (intravenous pyelogram), an ultrasound of the kidneys and a biopsy to check for cancer cells. A diagnostic finding will reveal the presence of hypertension, abdominal mass and obstruction of the ureters.

Treatment for retroperitoneal fibrosis will depend on the severity and location of the fibrosis. If you are in the early stage, then your doctor may prescribe corticosteroids which are given on case by case basis but will usually range between 30 and 60 mg per day. It will be decreased after a few months but in few cases your doctor can give low doses of corticosteroids for a longer period of time. Other medications like anti-inflammatory and immunosuppressants may also be prescribed along with corticosteroids. However, if medications do not work, your doctor might suggest doing a stent (drainage tube) or surgery to clear the obstruction. Stents are either inserted through the back or through the ureter from the bladder into the kidney.

In severe cases where the kidneys have been affected badly, surgery is done to either free the ureter from the fibrosis or wrap the ureter in fat tissue from the intestines to protect it from regrowth or reposition the affected ureter away from the inflammation. The goal of a surgery is to remove the blockage, repair the affected ureter and prevent it from occurring again. Therefore, your doctor may suggest a combination of medication and surgery to treat the condition.

The condition is generally seen in the age group of 40 and 60 and affects more men than women. Symptoms can vary depending on the severity of the condition. Early stage symptoms consist of dull pain in the abdomen or back, pain on one side between your upper abdomen and back, leg pain, swelling of the leg and intense abdominal pain with bleeding and swelling of the scrotum in men. As the condition worsens over time, the symptoms include appetite loss, weight loss, fever, nausea or vomiting, decreased urine output, inability to urinate (anuria), and impaired limb movement, anemia and kidney failure. Anybody experiencing these symptoms is eligible for the treatment.

If a person is having difficulty in urination due to some other conditions like dysuria and vaginal infection, he or she is not eligible for the treatment.

The medications given for treating retroperitoneal fibrosis includes corticosteroids, anti-inflammatory and immunosuppressant drugs. Side effects are varied for the different medications. Side effects of corticosteroids include osteoporosis (fragile bones), hypertension (high blood pressure), diabetes, weight gain, increased vulnerability to infection, cataracts and glaucoma, thinning of the skin and others.

Side effects of anti-inflammatory drugs include stomach pain and heartburn, stomach ulcers, headaches and dizziness, ringing in the ears, allergic reactions such as rashes, wheezing and throat swelling, high blood pressure and others.The most significant side effect of immunosuppressant drugs is an increased risk of infection. Other side effects include loss of appetite, nausea, vomiting, increased hair growth and hand trembling. However, surgical side effects include infection and bleeding.

There are no specific post-treatment guidelines for retroperitoneal fibrosis. However, you should try to follow a well-balanced diet with increased leg movement to prevent it from swelling up.

Since the condition does not have any definite cause, preventing it is also not possible. However, there is some debate about the appearance of the condition with the consumption of certain high blood pressure medications like ergotamines. Ask your doctor about the possible side effects of the drug if you have high blood pressure.

Recovery is pretty good for patients diagnosed at early stage of the condition with renal recovery occuring after 2 weeks of the treatment. It will usually take longer ranging from one to two months if you are undergoing surgery.

The cost of retroperitoneal fibrosiscan be pretty expensive as the medication charges starts from Rs. 10,000 while surgical cost usually starts from Rs.2,00,000.

Post-treatment results of the condition are pretty good for early stage retroperitoneal fibrosis. However, obstruction of the ureter can reoccur in half of the patients undergoing surgery and medications of the disease.Reoccurence may lead to total kidney failure that might require kidney transplant.

Alternative treatments for retroperitoneal fibrosis includes robotic laproscopy on which research is ongoing and still in the initial phase.