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Extra-Intestinal Manifestations of IBD

Extra-Intestinal Manifestations of IBD

Gastrointestinal System

At least one extraintestinal disease is manifested in up to one-third of patients with Inflammatory Bowel Disease (IBD). The extra-intestinal manifestations include:

  • Dermatologic: Erythema nodosum, Pyoderma gangrenosum, Pyoderma vegetans, Pyostomatitis vegetans, Sweet syndrome (neutrophilic dermatosis), Metastatic CD (Cutaneous granuloma formation), Psoriasis, Perianal skin tags, Oral mucosal lesions.
  • Rheumatologic: Peripheral arthritis, Ankylosing spondylitis, Sacroiliitis, Hypertrophic osteoarthropathy, Pelvic/femoral osteomyelitis, Relapsing polychondritis.
  • Ocular: Uveitis, Episcleritis, Conjunctivitis.
  • Hepatobiliary: Hepatic steatosis (Fatty liver), Cholelithiasis (Gallstones), Primary sclerosing cholangitis (PSC).
  • Urologic: Nephrolithiasis.
  • Metabolic Bone Disorders: Low Bone Mass, Increased incidence of hip, sppine, wrist and rib fractures, Osteonecrosis.
  • Venous and arterial thrombosis.
  • Cardiopulmonary Manifestations: Endocarditis, Pleuropericarditis, Interstitial lung disease.

Dermatologic Manifestations

  • Erythema nodosum (CD - 15%, UC - 10%)

    • Hot, red, tender nodules, 1-5 cm in diameter.
    • Found on the anterior surface of the lower legs, ankles, calves, thighs, and arms.
    • Develop after the onset of bowel disease and concomitant active peripheral arthritis is frequently seen.
    • Treatment is directed toward the underlying bowel disease.

  • Pyoderma gangrenosum ( UC - 1-2%)

    • Begins as a pustule and then spreads concentrically to rapidly undermine healthy skin. This is followed by ulceration, with violaceous edges surrounded by a margin of erythema. At the center, the lesions contain necrotic tissue with blood and exudates.
    • Lesions may be single or multiple and grow as large as 30 cm.
    • Usually presents after the diagnosis of IBD.
    • Lesions are commonly found on the dorsal surface of the feet and legs (may occur on the arms, chest, stoma and even face).
    • Sometimes very difficult to treat and often require IV antibiotics, IV glucocorticoids, dapsone, azathioprine, thalidomide, IV cyclosporine, infliximab or adalimumab.

  • Oral mucosal lesions : Aphthous stomatitis and cobblestone lesions of the buccal mucosa, seen often in CD (rarely in UC).

Rheumatologic Manifestations

  • Peripheral arthritis (15-20%)

    • Asymmetric, polyarticular and migratory.
    • Most often affects large joints of the upper and lower extremities.
    • Worsens with exacerbations of bowel activity.
    • Treatment: Reduce bowel inflammation. Severe UC: Colectomy frequently cures arthritis.

  • Ankylosing spondylitis (10%)

    • Most often affects the spine and pelvis, producing symptoms of diffuse low-back pain, buttock pain and morning stiffness.
    • Continuous and progressive in nature leading to permanent skeletal damage and deformity.
    • 2/3rd IBD patients with ankylosing spondylitis express the HLA-B27 antigen.
    • Not related to bowel activity, does not remit with glucocorticoids or colectomy.
    • Anti-TNF therapy reduces spinal inflammation and improves functional status and quality of life.

  • Sacroiliitis

    • Occurs equally in UC and CD.
    • Often asymptomatic and does not correlate with bowel activity, does not always progress to ankylosing spondylitis.

Ocular Manifestations

  • Uveitis

    • Symptoms: Ocular pain, photophobia, blurred vision and headache.
    • May be found during periods of remission and may develop following bowel resection.
    • Prompt intervention, sometimes with systemic glucocorticoids, is required to prevent scarring and visual impairment.

  • Episcleritis (3-4%)

    • Benign disorder, presents with symptoms of mild ocular burning.
    • Treated with topical glucocorticoids.

Hepatobiliary Manifestations

  • Hepatic steatosis (Fatty liver)

    • Usually present with hepatomegaly.
    • Results from a combination of chronic debilitating illness, malnutrition, and glucocorticoid therapy.

  • Cholelithiasis (Gallstones)

    • Occurs in 10-35% of CD with ileitis or ileal resection.
    • Caused by malabsorption of bile acids, resulting in depletion of the bile salt pool and secretion of lithogenic bile.

  • Primary sclerosing cholangitis (PSC)

    • Characterised by both intrahepatic and extrahepatic bile duct inflammation and fibrosis, frequently leading to biliary cirrhosis and hepatic failure.
    • 50-75% of patients with PSC have IBD.
    • Most patients have no symptoms at the time of diagnosis, symptoms include fatigue, jaundice, abdominal pain, fever, anorexia and malaise.
    • Traditional gold standard diagnostic test is Endoscopic Retrograde Cholangiopancreatography (ERCP), however, Magnetic Resonance Cholangiopancreatography (MRCP) is sensitive, specific and safer.
    • Patients with gallbladder polyps have a high incidence of malignancy and cholecystectomy is recommended (even if the lesion is < 1 cm in diameter).
    • Patients with IBD and PSC are at increased risk of colon cancer and should be surveyed yearly by colonoscopy and biopsy.

Urologic Manifestations

  • Nephrolithiasis (10-20%)

    • Presents with Calculi, Ureteral obstruction and ileal bladder fistulas.
    • Due to hyperoxaluria which results from increased absorption of unbound oxalate in the colon. (In ileal dysfunction, non-absorbed fatty acids bind calcium and leave oxalate unbound.)

Metabolic Bone Disorders

  • Low Bone Mass (14-42% cases)

    • Risk is increased by glucocorticoids, cyclosporine, methotrexate and total parenteral nutrition.
    • The effect of glucocorticoids id dose-dependent. Up to 20% of bone mass can be lost per year with chronic glucocorticoid use.
    • Low bone density also occurs due to malabsorption and inflammation mediated by IL-1, IL-6, TNF and other inflammatory mediators.

  • Increased incidence of hip, sppine, wrist and rib fractures (CD- 36%, UC - 45%).

  • Osteonecrosis: Characterised by death of osteocytes and adipocytes and eventual bone collapse, seen in 4.3% patients within 6 months of starting glucocorticoids. Treatment consists of pain control, cord compression, osteotomy and joint replacement.

Thromboembolic disorders

  • Both venous and arterial thrombosis

  • Etiologic factors include:

    • Abnormalities of platelet-endothelial interaction.
    • Hyperhomocysteinemia.
    • Alterations in coagulation cascade.
    • Impaired fibrinolysis.
    • Involvement of tissue factor-bearing microvesicles.
    • Disruption of the normal coagulation system by autoantibodies.
    • Genetic predisposition.

References

  • Harrison's Principles of Internal Medicine (21st edition), Loscalzo, Fauci, Kasper, Hauser, Longo, Jameson, The McGraw-Hill Companies.
  • The image used in the cover is licensed under the Creative Commons Attribution-Share Alike 4.0 International license. (Author : WolfpackBME, Source : Wikimedia commons).

*This article is an excerpt from the above mentioned book and Medical Sutras does not make any ownership or affiliation claims.