What Is Perianal Crohn's Disease? - Health Central

Got Crohn's? While Crohn's disease is most likely to impact your intestines, the condition can affect any part of a person's gastrointestinal tract. When it occurs in the rectum or the anus, experts call it perianal or fistulizing Crohn's disease.

If those were the cards you were dealt, the news isn't great: A less common form of Crohn's that's more aggressive and harder to treat, perianal Crohn's disease can be particularly challenging to live with. "It can have a dramatic impact on someone," because inflammation and infections around the perianal area can be particularly uncomfortable and embarrassing, says Brooks Cash, M.D., a gastroenterologist at the Ertman Digestive Disease Center at Memorial Hermann-Texas Medical Center in Houston.

Still, it's possible to get perianal Crohn's disease under control with the right care team and treatment options. Learn more about what causes this condition, how it differs from traditional Crohn's, and how best to manage it.

What Is Perianal Crohn's Disease?

Perianal Crohn's disease is a more severe form of Crohn's disease that occurs in the rectum and anal canal. People with perianal Crohn's often develop anal fistulas—tunnels that form between the anal area and other nearby structures like the bowel, bladder, or genitals. While fistulas can happen at any point along the GI tract with Crohn's, they are a defining characteristic of perianal disease—and can really affect quality of life in people with this form of Crohn's.

In some cases, the tunnels can extend through the thickness of the bowel wall and cause the tissue to deteriorate, which can lead to bowel leakage. If the tunnels don't make it out to the skin, feces and discharge can build up (rather than drain) and cause an abscess, a closed pocket of pus caused by an infection, explains Adam Erlich, M.D., the co-medical director of the Inflammatory Bowel Disease Program at Temple University Hospital in Philadelphia.

Perianal Crohn's can also increase the chances for developing anal fissures or tears, hemorrhoids, or skin tags, which can also become infected. "The challenge with anything in the anal area is that there's stool continuously running past it. So these injured areas are continuously irrigated with bacteria every time a person moves their bowels, which poses a high infection risk," Dr. Erlich explains.

Roughly a quarter of people with Crohn's have perianal disease, according to the Crohn's and Colitis Foundation. Fistulas and other hallmarks of perianal disease usually develop after a person has already been diagnosed with Crohn's. But in around 10% of cases, perianal symptoms will come along before a person goes on to experience other symptoms of Crohn's disease, according to a paper in the journal Gastroenterology & Hepatology.

Symptoms of Perianal Crohn's

People with perianal Crohn's disease experience the same general symptoms that all people with Crohn's can have, like diarrhea, urgency, abdominal pain or cramping, and in some cases, rectal bleeding, Dr. Erlich explains.

However, anal fistulas and other growths can cause additional symptoms, says Dr. Cash. Early on, these can include:

  • Scabs or pimple-like bumps around the anus

  • Irritation or pain around the bumps

  • Anal discharge or drainage that's milky white, yellow, green, or brown and has a foul odor, either in your underwear or when you wipe

Symptoms become more serious if the fistula becomes infected or forms an abscess, Dr. Cash notes. At that point, a person may start to experience:

  • Chills

  • Fever

  • Increased abdominal pain

In cases of a severe infection, you might notice fast or shallow breathing, sweating, lightheadedness, or a change in mental status. These may be signs of sepsis (the body's extreme reaction to infection, which can lead to tissue damage and even death) and warrant immediate medical attention.

Causes and Risk Factors for Perianal Crohn's Disease

Just like Crohn's disease that affects other areas of the GI tract, active disease around the anus is thought to be fueled by inflammation, says Dr. Erlich. But why some people with Crohn's develop perianal disease while others don't isn't well understood. "We haven't identified any useful clinical markers that will predict whether someone gets one form of Crohn's or another," Dr. Cash says.

That said, certain factors might make perianal Crohn's more likely. According to a paper published in Clinics in Colon and Rectal Surgery, having anal strictures, or narrowing in areas of the anal canal that make it more difficult for stool to pass, can increase the risk.

Race also seems to play a role. African Americans with Crohn's disease are around twice as likely to develop perianal disease compared to people of other races, the paper reports.

Additionally, "we do know there are certain immune markers and immune profiles that tend to predict fistulizing disease," explains Dr. Cash.

Diagnosing Perianal Crohn's

Perianal Crohn's disease can literally be tough to spot. "It can be hard for a patient to see what's going on back there, and many people don't want to have a significant other or family member take a look," Dr. Erlich says. So if you think you might be experiencing symptoms of perianal disease, it's important to let your gastroenterologist know.

Your doctor will usually start by conducting a physical exam to look for signs of fistulas. "Sometimes that's all we'll need to make the diagnosis," Dr. Cash says. If your doctor suspects that a fistula is internal (like a fistula that forms from the bowel to the bladder) or if they think you have an abscess, they'll recommend imaging scans like an MRI or CT scan.

In some cases, your doctor might also recommend an imaging test called a fistulagram, in which dye is injected into the outside of the fistula. "It can track where the fistula goes and how complex it is," says Dr. Cash.

Treatment for Perianal Crohn's

Ultimately, the treatment for any type of Crohn's will likely require the same systemic medications since it is caused by an inflammatory reaction of the body's immune system. In addition, the goal of perianal Crohn's treatment specifically is to prevent fistulas or other lesions in the anal area from becoming infected or to encourage fistulas to close. It's also important to quickly manage infections that do develop, to keep them from becoming serious. But it isn't always easy. "The perianal area is inherently prone to moisture and warmth, so there's a higher likelihood of chronic infection and irritation," Dr. Cash explains.

You and your care team might rely on a combination approach depending on your symptoms and their severity. Options include:

Antibiotics

Oral antibiotics as well as ointments or creams are a first-line option that can be used to prevent infections or treat existing ones, according to the National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK). Combination therapies, like pairing the immunomodulator azathioprine with metronidazole or ciprofloxacin, may be more effective than using a single antibiotic on its own, concluded the authors of the Gastroenterology & Hepatology paper referenced above.

Seton Placement

Setons are thin pieces of tubing that can be placed inside a fistula to keep it open, encouraging it to continue draining. "It helps keep the fistula open so you don't get an abscess. Some patients have them short-term while they heal or get better, or long-term if they have recurrent issues," Dr. Erlich says. The procedure is typically performed under general anesthesia, according to the National Library of Medicine.

Incision and Drainage

Abscesses that have become infected need to be cut open and drained to clear out the pus. This procedure can be very painful, so it's typically done with general anesthesia, according to the Cleveland Clinic. After the abscess is drained, you'll typically be given antibiotics or anti-inflammatory medications to further fight off the infection and keep it from coming back, Dr. Cash explains.

Medications

While the options above treat symptoms of perianal Crohn's, any type of advanced Crohn's will likely require system medication to treat the underlying cause, including perianal Crohn's, says Dr. Erlich. "Many patients are already on these medications [when they develop perianal Crohn's], but if not, you'll start," he says. "Higher doses are often needed to get healing."

According to the Gastroenterology & Hepatology paper, effective medications include:

  • Biologics such as infliximab, adalimumab, and vedolizumab. These medicines reduce Crohn's inflammation by targeting proteins in the immune system that "turn off" certain inflammatory pathways in the body. Research involving injecting biologic agents like inflixumab directly into a fistula is currently being explored.

  • Immunomodulators such as azathioprine, 6-mercaptopurine, tacrolimus, thalidomide, and mycophenolate mofetil. These medicines reduce Crohn's inflammation by suppressing immune system activity.

Surgery

When other options haven't done enough, surgery may be a last-resort option for managing fistulas, Dr. Erlich says. A surgeon may try to close the fistula by using a flap of tissue from elsewhere in the rectum or with synthetic glue or plugs, or by using minimally invasive techniques such as laser surgery, according to a review on surgical strategies for perianal Crohn's published in the World Journal of Gastroenterology. Emerging evidence has shown that injecting stem cells into the fistula can also reduce inflammation and encourage the tunnel to heal, per a report in the journal Frontiers in Surgery.

In rare instances when someone's perianal disease is very severe, they may need to have their rectum and anus removed and receive a permanent ostomy, which allows waste to pass into a pouch attached to the abdomen. "This is a last-ditch effort," says Dr. Erlich. Removal of the anus does not cure you of Crohn's: Even with an ostomy, you may need to continue your medication to keep inflammation under control.

Outlook

Perianal Crohn's is harder to treat and poses a higher risk for complications than Crohn's without perianal involvement. "It's considered to be a more aggressive form of Crohn's," Dr. Erlich says. Even after a fistula closes, it's possible that it could reopen or that new ones could form.

That said, "we have effective medications that can get the disease under control," says Dr. Cash. "And we know that by treating someone with medications aggressively, we can enhance that healing rate."

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