HomeHealth articlescolitis cystica profundaWhat Is Colitis Cystica Profunda?

Colitis Cystica Profunda - Causes, Symptoms, Diagnosis, and Treatment

Verified dataVerified data
0

4 min read

Share

It is a rare noncancerous lesion in the colon characterized by the presence of mucous cysts. Read the article to know the causes, symptoms, and treatment.

Medically reviewed by

Dr. Sugreev Singh

Published At October 11, 2022
Reviewed AtDecember 1, 2023

What Is CCP?

It is an inflammatory condition characterized by the presence of mucus-filled cysts in the muscular mucosa (outermost layer of the mucosa) of the colon; although it may diffusely involve the entire colon, this disease primarily affects the pelvic colon and the rectum.

Mucus-filled cyst forms due to cystic dilation of the mucus glands in the colon; based on the location, these cysts are of two types-

1) Colitis Cystic Superficialis (CCS)- In this type, the cysts are minute and are diffusely distributed throughout the entire colon.

2) Colitis Cystica Profunda (CCP)- Here, the cysts are submucosal in nature and are confined to the rectum and the pelvic colon. They are large in size compared to the lesions seen in the CCS; some of them are as large as two centimeters in diameter. There are three types of CCP-

  • Diffuse CCP- This form is frequently associated with inflammatory bowel disease (IBD); the lesions are observed throughout the colon and macroscopically appear as pedunculated polypoid lesions that are evenly ulcerated.
  • Segmental CCP- It is characterized by the presence of polypoid lesions, typically in the rectosigmoid tract.
  • Localized CCP- The lesion is situated along the anterior wall of the rectum, usually 5 cm to 12 cm from the anal orifice.

Common symptoms pertaining to CCP include rectal bleeding, mucus discharge, altered bowel habits, and long-standing constipation. The cause of CCP is unclear; however, it is associated with rectal prolapse (a condition where part of the large intestine slips out of the anus) in 50 % of the cases.

The endoscopic appearances of CCP are not unique, leading to a missed diagnosis of polyp or malignancy. Treatments of CCP include re-education of bowel habits to avoid straining, administration of fiber and laxatives, and biofeedback.

What Causes CCP?

The exact cause of CCP is unknown, but it’s histopathological (pathological changes in the tissue) features bear a close resemblance to those of solitary rectal ulcer syndrome (SRUS), leading to the belief that the causes responsible for the latter are also responsible for CCP. Some of the most accepted theories for CCP and SRUS are-

  • Straining: Lengthened straining during bowel movements in a patient who suffers from constipation may result in direct trauma to the mucosa.

  • Self-Induced Trauma: Self-instrumentation can occur when individuals try to remove impacted stools by rectal digitization and injure the mucosa in the process.

  • Paradoxical Contraction of Puborectalis Muscle: Uncoordinated muscle contraction in the puborectalis muscle has been indicated to be associated with increased intrarectal pressure resulting in ischemia, ulceration, and cyst formation.

  • Rectal Prolapse and Intussusception: Intussusception is where one part of the intestine slides into another. Rectal intussusception can lead to localized vascular trauma and consequently the onset of SRUS and CCP.

What Are the Symptoms of CCP?

The symptoms of CCP are nonspecific and often resemble those of IBD; some of them are-

  • Rectal pain.

  • Bleeding.

  • Pain.

  • Tenesmus (feeling like pooping even though the bowels are empty).

  • Mucus in the stool.

  • Chronic and severe constipation.

  • Lengthened straining on defecation.

  • Pelvic discomfort.

  • Sense of incomplete evacuation.

Nearly 25 % of the patients are asymptomatic, but when the symptoms do occur, the most common one is bleeding (hemorrhage); the amount of hemorrhage varies from patient to patient, and at times it can be a little too severe, creating a need for blood transfusion.

How Is CCP Diagnosed?

CCP is a well-known condition but is often misdiagnosed due to its rare occurrence. The clinician should keep in mind that not all CCP lesions are cystic in appearance; some of them show areas of atrophy with superficial ulceration.

The diagnosis of CCP can be performed by a combination of symptomatology, endoscopy, sigmoidoscopy, and histology. Some of these are described below-

  1. Flexible Sigmoidoscopy - It is a method in which a sigmoidoscope is inserted into the rectum to examine the rectum and part of the colon. It is used to determine the unknown cause of mucosal lesions, rectal ulcers, IBD, etc.

  2. Transrectal and Endoanal Ultrasonography - An imaging technique that can describe a series of changes like thickened muscularis propria, thickened internal and external anal sphincter, etc.

  3. Defecography - It is a type of radiological imaging in which different stages of defecation can be visualized by a fluoroscope.

  4. Magnetic Resonance (MR) Defecography - This method can show the cause of constipation and other problems, such as lower limb prolapse.

  5. Barium Enema - It is a type of X-ray imaging method that can be used for the examination of muscle function, coordination, and prolapse.

Although the above-mentioned investigations support the benign nature of CCP, only adequate biopsy offers a definitive diagnosis; the characteristic histological features of CCP are:

  • Thickening of the mucosal layer along with crypt distortion.

  • Fibromuscular distortion in the lamina propria has been known to be the cornerstone for the diagnosis of CCP.

  • The extension of the muscle fibers in an upward direction between the cryptans (deep pits in the small intestinal mucosa).

  • Glandular crypt abnormalities.

  • Surface ulceration.

  • Mucus cell proliferation, hyperplastic, and serrated mucosa.

  • Mild inflammation.

How Is the CCP Treated?

Treatment of CCP is based on its symptoms; this includes-

  • Conservative Treatment- This is preferred in patients who are mildly symptomatic. Treatment comprises a fiber diet, bulk laxatives, training for straining prevention, and anal digitation.

  • Biofeedback- It includes a variety of behavioral changes that are effective in reducing excessive straining with defecation through correction of the abdominal pelvic floor and stopping the use of suppositories and laxatives.

This is the choice of treatment for patients in whom the conservative treatment is no longer effective and in cases where there is a high degree of intussusception in the rectum and fibrosis or external prolapse.

  • Topical Therapy- This includes Sucralfate enema, corticosteroids, and Sulfasalazine; however, the long-term effectiveness of these medications is yet to be determined.

  • Surgery- It is preferred in patients who suffer from rectal mucosal prolapse and are resistant to conservative management and biofeedback treatment. Some preferred surgical approaches are excision, diversion, perineal proctectomy (removal of part of the rectum), etc.

Conclusion:

CCP is a misleading condition that mimics clinical features of IBS, lipoma, sarcoma, colorectal adenocarcinoma, etc. Diagnosis should be made by analyzing the outcome of symptoms, sigmoidoscopy, and histology. Treatment should involve implementing a conservative approach and biofeedback as the first strategy, failure of which surgery should be considered.

Frequently Asked Questions

1.

How Are CCP Tests Done?

The CCP (cyclic citrullinated peptide) test quantifies the blood's level of CCP antibodies. These proteins are a component of the immune system's attack against normal tissues and cells, including the joints. This test may be prescribed by a doctor to assist in identifying rheumatoid arthritis (RA). The test involves drawing blood samples to detect the presence of CCP.

2.

How Reliable Is the CCP Exam?

CCP tests are considered to be more than 90 % specific. A negative anti-CCP result does not entirely rule out RA, but a positive result indicates that RA is likely. Hence, it is regarded as a reliable diagnosis for RA.

3.

What Does a Positive Anti-CCP Antibody Mean?

Patients with rheumatoid arthritis (an inflammatory condition that destroys the joints all over the body) are generally presented with anti-cyclic citrullinated peptide (anti-CCP) antibodies. These antibodies are created by the body, directly attacking particular proteins that are present naturally. Hence, positive anti-CCP indicates the presence of the condition.

4.

What Is CCP Level Considered High?

CCP levels can be raised in various rheumatologic disorders connected to autoimmune disease and other inflammatory conditions. But they are typically elevated in rheumatoid arthritis. A level over 20 units indicates the potential for RA

5.

How Long Does the CCP Test Take?

Since CCP is a blood test, the duration of the test involves only the time taken to draw the blood sample by the healthcare professional, which usually takes less than five minutes. The patient may be given a piece of gauze or a cotton ball to be placed on top of the needle's entrance site. To stop the bleeding, you might need to apply pressure to this portion while holding it and then cover it with a bandage.

6.

Is Fasting Required for a CCP Test?

No, CCP test specifications and preparations do not enlist a fasting state to appear for the test. Hence, CCP can be done at any time of the day, irrespective of the fasting state

7.

Can Prednisone Reduce Anti-CCP?

Prednisone is used to reduce the pain and inflammation in rheumatoid arthritis. The effect of the drug on reducing anti-CCP is to be discussed with the physician. However, it should be noted that during the treatment course, the reduction in anti-CCP levels is probable.

8.

Do CCP Levels Change?

Studies have shown that CCP levels change during the therapy period regardless of the drug and the clinical response of the patients.
Dr. Sugreev Singh
Dr. Sugreev Singh

Internal Medicine

Tags:

colitis cystica profunda
Community Banner Mobile

iCliniq's FREE Newsletters

Expert-backed health and wellness information, delivered to your email.

Subscribe iCliniq
By clicking Subscribe, I agree to the iCliniq Terms & Conditions and Privacy Policy and understand that I may opt out of iCliniq subscriptions at any time.

Source Article ArrowMost popular articles

Do you have a question on

colitis cystica profunda

Ask a doctor online

*guaranteed answer within 4 hours

Disclaimer: No content published on this website is intended to be a substitute for professional medical diagnosis, advice or treatment by a trained physician. Seek advice from your physician or other qualified healthcare providers with questions you may have regarding your symptoms and medical condition for a complete medical diagnosis. Do not delay or disregard seeking professional medical advice because of something you have read on this website. Read our Editorial Process to know how we create content for health articles and queries.

This website uses cookies to ensure you get the best experience on our website. iCliniq privacy policy