This document discusses extraintestinal manifestations (EIMs) of inflammatory bowel disease (IBD). Some key points: - EIMs occur in 25-40% of IBD patients and can involve many organ systems, including the musculoskeletal, dermatological, hepatopancreatobiliary, ocular, renal and pulmonary systems. - Common EIMs include arthritis, erythema nodosum, pyoderma gangrenosum, ankylosing spondylitis, episcleritis and uveitis. - EIMs can occur before or after an IBD diagnosis. Having one EIM increases the risk of developing another. - The pathogenesis of E

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Extraintestinal Manifestations of
Inflammatory Bowel Disease
DR. HIWA OMER AHMED
PROFESSOR IN GENERAL AND BARIATRIC SURGERY
UNIVERSITY OF SULAIMANI
COLLEGE OF MEDICINE – SULAIMANI CITY-
KURDISTAN

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Incidence
 Extraintestinal manifestations (EIMs) of inflammatory
bowel disease (IBD) are common in both ulcerative
colitis (UC) and Crohn's disease (CD). These
manifestations can involve nearly any organ system.
 EIMs are seen in 25–40% of IBD patients.
 Twenty-five percent of IBD patients have more than 1
EIM.
 The development of 1 EIM appears to increase the
risk of developing a second EIM.

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Domino principle
 31.4% of UC patients and 40.4% of CD patients had 1
of the 5 major manifestations; a smaller percentage of
patients had more than 1 major EIM.
 EIMs can occur before or after the diagnosis of IBD.

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Onset
 Up to 26% of cases have their first EIM before IBD
is diagnosed (median time 5 months before IBD
diagnosis) and in 74% of cases, the first EIM
manifested after IBD diagnosis (median, 92
months)1

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Musculoskeletal Manifestations
 Musculoskeletal pain occurs in 9–53% of IBD patients and is
considered the most common EIM.
1. Arthritis can affect the spine, sacroiliac joint, peripheral joints,
or a combination of these sites.
Classically, inflammatory arthritis is defined by
1. Pain
2. an increase in local temperature
3. joint swelling with or without effusion
4. leading to decreased joint mobility.

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2. peri-articular incvolvemens features include
1. Tendonitis
2. Clubbing
3. Periostitis
4. Granulomatous lesions of the joint and bone.

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3. osteoporosis in IBD patients.
1. Age
2. Corticosteroid therapy
3. reduced physical activity
4. inflammatory-mediated bone resorption (increased levels of interleukin [IL]-1, IL-6,
TNF-α)
5. calcium and magnesium dietary malabsorption
6. vitamin D deficiency,
7. decreased serum albumin levels
The overall risk of fracture in IBD patients is 1 per 100 patient-years—40% higher than
in the general population—and this risk increases with age.

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#
4. Unlike the risk in the general population, male and female patients with
IBD may have similar risks of fracture. Likewise, CD and UC are
associated with similar fracture risks.

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Dermatologic Manifestations
 Major dermatologic manifestations have been reported in 2–34% of
IBD patients.
 A broad spectrum of skin diseases may occur, and patients may
develop multiple dermatologic manifestations concurrently during
the natural course of their disease.
1. Erythema nodosum
2. pyoderma gangrenosum
3. Psoriasis
4. Oral aphthous stomatitis,
5. Sweet syndrome.

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Sweet syndrome
 Sweet syndrome, also
called acute febrile
neutrophilic
dermatosis, is an
uncommon skin
condition. It causes
fever and a painful
skin rash that appears
mostly on the arms,
face and neck.

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Hepatopancreatobiliary
Manifestations
 Hepatopancreatobiliary manifestations of IBD include
1. cholelithiasis
2. portal vein thrombosis
3. drug-induced hepatotoxicity
4. drug-induced pancreatitis.
5. One of the most serious complications of IBD is PSC, manifests
as inflammation, stricturing, and fibrosis of medium and large
intra- and extrahepatic bile ducts.

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primary sclerosing cholangitis:
PSC
 IBD, particularly UC.
 At least 75% of PSC patients have coexisting UC.
 Another 5–10% of PSC patients have CD.
 Patients with UC and extensive colonic involvement with or without
backwash ileitis are more likely to develop PSC than patients with only
left colon involvement. PSC is most common in
1. patients 30–59 years of age
2. there is a 2:1 male prevalence for the disorder.
PSC can develop either years before or after the development of bowel
symptoms.

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Ocular Manifestations
Ocular manifestations occur in 0.3–5% of all IBD
patients. Patients with colitis or ileocolitis are affected
more frequently than patients with isolated small-
bowel disease.
 An immune complex hypersensitivity reaction to a
colonic antigen
 Ocular complications often present concurrently with
other EIMs, particularly peripheral arthritis and EN.
Less commonly, ocular complications can be seen with
axial involvement.

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Episcleritis
1. The most common ocular manifestation is episcleritis.
Flares characteristically parallel intestinal activity and
resolve with treatment of the intestinal disease.
 acute redness in one or both eyes
 Irritation
 burning
 Tenderness to palpation is common
 No change in vision

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Scleritis
2. Scleritis is a more severe disorder, as it can impair vision.
VS

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Uveitis
3. Uveitis is less common and is often associated with joint and
dermatologic manifestations.
Uveitis is 4 times more common in women and is often chronic in nature.
Patients present with
 eye pain
 visual blurring
 Photophobia
 headaches.
 Ciliary flush, in which the redness is most intense in the center and
radiates outward with diminishing intensity.

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Others
 4. others
1. conjunctivitis
2. Several IBD treatments may cause ocular pathology.
 Steroids can lead to cataracts and open-angle
glaucoma.
 Anticholinergics can cause pupil dilatation.
 Cyclosporine has been linked to optic neuropathy,
ophthalmoplegia, and nystagmus.

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Aphthus ulcer

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Sweet's syndrome

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Erythema nodosum

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Ppyoderma gangrenosum

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Episcleritis

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uveitis with hypopyon and dilated
iris vessels

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Peristomal pyoderma gangrenosum

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Syndesmophytes (bamboo spine)

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Bilateral sacroiliitis

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Sacrum with bilateral ankylosis

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MRI active left side and chronic inflammatory
changes on both sacroiliac .

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Renal Manifestations
1. Nephrolithiasis
2. obstructive uropathy
3. Fistulization of the urinary tract are relatively common
EIMs, occurring in 6–23% of patients with IBD.
4. Secondary amyloidosis is a rare systemic
complication that involves the kidneys
 3-fold increased risk in males
 10-fold increased risk in CD patients (over UC
patients).

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Pulmonary
 Subclinical disturbances in lung function are common in IBD patients.
 Clinically significant disease is extremely rare.
1. Chronic bronchitis
2. subglottic stenosis
3. Bronchiectasis
4. Bronchiolitis
5. Sulfasalazine and mesalamine can induce interstitial lung disease on
rare occasions
 These conditions can occur in nonsmokers
 Do not parallel bowel disease.

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Hematological
 Anemia
 Approximately one in three patients with IBD has
anemia (low red blood cell count). Causes of anemia
include:
 Low iron caused by:
 Inflammation in your intestines interfering with the body's ability to use
or absorb iron
 Blood loss from intestinal bleeding
 Poor absorption of vitamins and minerals, like vitamin B12 or
folic acid)

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The pathogenesis of EIM in IBD
1. shared epitopes
• It is believed that the diseased gastrointestinal
mucosa may trigger immune responses at the
extraintestinal site due to shared epitopes, e.g., of
intestinal bacteria and the synovia.
 This would mean that bacteria that are
translocated across the leaky intestinal barrier
trigger an adaptive immune response that finally
is unable to discriminate between bacterial
epitopes and epitopes of joints or the skin.

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2. Genetic Risk Factors
concordance for EIMs in 70% of parent–child pairs and
84% of sibling pairs.
In addition, there is a considerable overlap between
genetic risk loci for EIMs and IBD.
 The first risk variant identified in patients with CD,
NOD2/CARD15, has also been associated with
sacroiliitis and uveitis

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3. Environmental Factors
Patients with CD who smoke are more likely to present with EIMs
compared with nonsmokers.
 It is important to note that smoking has also been identified as an
important environmental factor in the pathogenesis and severity of
luminal and perianal CD.
 Interestingly, smokers are protected from the onset of
UC and former smoking is a known risk factor for
the development of UC.

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Erythematous plaque, Crohn’s
disease.

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Aphthous stomatitis in a patient
with Crohn’s disease

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Irregular violaceous, ulcerative colitis-
associated pyoderma gangrenosum

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Erythematous papulonodular Sweet’s
syndrome (CD)

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pyodermatitis vegetans in UC