Clinically, inflammatory bowel disease (IBD) is a chronic inflammatory condition of the intestines that is marked by remission and relapses due to inappropriate mucosal immune response . TYPICAL IBD : (2 Major Types): Ulcerative Colitis (Colitis Ulcerosa) Crohn’s Disease (Regional Enteritis) ATYPICAL IBD: Lymphocytic Colitis Collagenous Colitis Ischaemic Colitis Diversion Colitis Indeterminate Colitis Bachet’s Disease Watery stools, blood or mucus in the stool Diarrhoea - persisting for more than 4 weeks Crampy abdominal pain, Nocturnal defecation Fever. Weight loss is significant. Anal fissures, anal fistulae, frank bleeding per rectum Abdominal masses can occur Symptoms are generally recurrent. The pathogenesis of EIM in IBD is not well understood. Diseased gastrointestinal mucosa may trigger immune responses at the extraintestinal site due to shared epitopes. E.g.: intestinal bacteria and the synovia : 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. Triggers of the autoimmune responses in certain organs seem to be influenced by genetic factors. EIM in patients with CD are more frequently observed in patients with HLA-A2, HLA-DR1, and HLA-DQw5 EIM in patients with UC are more likely to appear when the HLA-DR103 genotype is present. HLA-B8/DR3 is associated with an increased risk of PSC in UC. HLA-DRB1-0103, HLA-B-27, and HLA-B-58 are associated with EIM of joints, the skin, and eyes, respectively, in patients with IBD. HLA-B*27 itself does not seem to be associated with IBD, but HLA-B*27 shows a strong association with the development of ankylosing spondylitis, as 50% to 90% of patients with IBD are positive for this marker. 15% in CD & 10% in UC Skin lesions develop after the onset of bowel symptoms Concomitant active peripheral arthritis EN are hot, red, tender nodules measuring 1–5 cm in diameter and are found on the anterior surface of the lower legs, ankles, calves, thighs, and arms 1–12% of UC patients and less commonly in Crohn’s colitis May occur years before the onset of bowel symptoms Run a course independent of the bowel disease Respond poorly to colectomy Usually associated with severe disease Begins as a pustule and then spreads concentrically Lesions then ulcerate, with violaceous edges surrounded by a margin of erythema Centrally, they contain necrotic tissue with blood and exudates Lesions may be single or multiple and grow as large as 30 cm Pyoderma Vegetans Pyostomatitis Vegetans Sweet Syndrome Psoriasis Perianal Skin Tag Aphthous Stomatitis Arthritis Develops In 15–20% Of IBD Patients Common In CD > UC Worsens With Exacerbations Of Bowel Activity Asymmetric, Polyarticular, And Migratory And Most Often Affects Large Joints Of The Upper And Lower Extremities Colectomy frequently Cures The Arthritis ankylosing spondylitis: 10% Of

1. BY
Dr.AhmedYosry Mandour

2. ◾ INTRODUCTION
◾ CLINICAL FEATURESOF IBD
◾ RADIOLOGICALSIGNS
◾ EXTRAINTESTINAL MANIFESTATIONS
◾ CASESCENARIOS

3. Clinically, inflammatory bowel
disease (IBD) is a chronic inflammatory
condition of the intestines that is marked by
remission and relapses due to inappropriate
mucosal immune response .

4. ◾ TYPICAL IBD : (2 MajorTypes):
 UlcerativeColitis (Colitis Ulcerosa)
 Crohn’s Disease (Regional Enteritis)
◾ ATYPICAL IBD:
 LymphocyticColitis
 CollagenousColitis
 IschaemicColitis
 DiversionColitis
 IndeterminateColitis
 Bachet’s Disease

5. ◾ Watery stools, blood or mucus in the stool
◾ Diarrhoea - persisting for more than 4 weeks
◾ Crampy abdominal pain,
◾ Nocturnal defecation
◾ Fever.
◾ Weight loss is significant.
◾ Anal fissures, anal fistulae, frank bleeding per
rectum
◾ Abdominal masses can occur
◾ Symptoms are generally recurrent.

11. ◾ The pathogenesis of EIM in IBD is not well
understood.
◾ Diseased gastrointestinal mucosa may trigger
immune responses at the extraintestinal site due to
shared epitopes.
◾ E.g.: intestinal bacteria and the synovia : 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.

12. ◾ Triggers of the autoimmune responses in certain
organs seem to be influenced by genetic factors.
◾ EIM in patients with CD are more frequently
observed in patients with HLA-A2, HLA-DR1,
and HLA-DQw5
◾ EIM in patients withUC are more likely to appear
when the HLA-DR103 genotype is present.

13. ◾ HLA-B8/DR3 is associated with an increased risk
of PSC inUC.
◾ HLA-DRB1-0103, HLA-B-27, and HLA-B-58 are
associated with EIM of joints, the skin, and eyes,
respectively, in patients with IBD.
◾ HLA-B*27 itself does not seem to be associated
with IBD, but HLA-B*27 shows a strong
association with the development of ankylosing
spondylitis, as 50% to 90% of patients with IBD
are positive for this marker.

21. ◾ 15% inCD & 10% inUC
◾ Skin lesions develop after the onset of bowel
symptoms
◾ Concomitant active peripheral arthritis
◾ EN are hot, red, tender nodules measuring 1–5
cm in diameter and are found on the anterior
surface of the lower legs, ankles, calves, thighs,
and arms

25. ◾ 1–12% ofUC patients and less commonly in
Crohn’s colitis
◾ May occur years before the onset of bowel
symptoms
◾ Run a course independent of the bowel
disease
◾ Respond poorly to colectomy
◾ Usually associated with severe disease

26. ◾ Begins as a pustule and then spreads
concentrically
◾ Lesions then ulcerate, with violaceous edges
surrounded by a margin of erythema
◾ Centrally, they contain necrotic tissue with
blood and exudates
◾ Lesions may be single or multiple and grow
as large as 30 cm

30. ◾ PyodermaVegetans
◾ PyostomatitisVegetans
◾ SweetSyndrome
◾ Psoriasis
◾ PerianalSkinTag
◾ AphthousStomatitis

32. ◾ Arthritis Develops In 15–20%Of IBD Patients
◾ Common InCD >UC
◾ WorsensWith ExacerbationsOf Bowel
Activity
◾ Asymmetric, Polyarticular,And Migratory
And MostOftenAffects LargeJointsOfThe
UpperAnd Lower Extremities
◾ Colectomy frequentlyCuresTheArthritis

34. ◾ 10%Of IBD patients
◾ Common inCD >UC
◾ 2/3rd patients are HLA-B27 antigen positive
◾ Activity is not related to bowel activity

35. ◾ Symmetrical (both joints)
◾ Occurs equally inUC andCD
◾ Does not correlate with bowel activity

39. ◾ HypertrophicOsteoarthropathy
◾ Pelvic/FemoralOsteomyelitis
◾ Relapsing Polychondritis

41. ◾ Seen in 1-10% of cases
◾ Conjunctivitis
◾ Anterior uveitis
◾ Iritis
◾ Episcleritis (3-4%CD >UC)
◾ Uveitis : found during periods of remission and
develop in patients following bowel resection.

43. ◾ Hepatic steatosis : 50% cases
◾ Hepatomegaly is found on examination
◾ Cholelithiasis is seen in 10-35% after ileal
resection or ileitis
◾ PrimarySclerosingCholangitis
◾ Gall bladder polyps

44. ◾ Intrahepatic and extrahepatic bile duct inflammation
and fibrosis
◾ Biliary cirrhosis and hepatic failure
◾ ~5% of patients withUC have PSC ,UC >CD
◾ IBD and PSC are commonly p-ANCA positive
◾ Both ERCP and MRCP demonstrate multiple bile duct
strictures alternating with relatively normal segments

45. ◾ Gallbladder polyps in patients with PSC have a
high incidence of malignancy and
cholecystectomy is recommended, even if a
mass lesion is less than 1 cm in diameter
◾ Patients with symptomatic disease develop
cirrhosis and liver failure over 5–10 years
◾ IBD and PSC are at increased risk of colon cancer
and should be surveyed yearly by colonoscopy
and biopsy

50. ◾ Biliary
Lithiasis

52. ◾ Calculi
◾ Ureteral obstruction
◾ Ileal-bladder fistulas
◾ Nephrolithiasis (10–20%) occurs in patients with
CD following small bowel resection
◾ Calcium oxalate stones develop secondary to
hyperoxaluria, which results from increased
absorption of dietary oxalate

53. ◾ In patients with ileal dysfunction,
nonabsorbed fatty acids bind calcium and
leave oxalate unbound.
◾ The unbound oxalate is then delivered to the
colon, where it is readily absorbed, especially
in the presence of inflammation

59. ◾ Increased risk of both venous and arterial
thrombosis even if the disease is not active
◾ AbnormalitiesOfThe Platelet-endothelial
Interaction,
◾ Hyperhomocysteinemia,
◾ Alterations InTheCoagulationCascade,
◾ Impaired Fibrinolysis,

60. ◾ InvolvementOfTissue Factor-bearing
Microvesicles,
◾ DisruptionOfThe NormalCoagulationSystem
ByAutoantibodies
◾ Genetic Predisposition
◾ A spectrum of vasculitides involving small,
medium, and large vessels has also been
observed.

64. ◾ Low bone mass occurs in 14–42% of IBD
patients
◾ An increased incidence of hip, spine, wrist,
and rib fractures has been noted: 36% in CD
and 45% inUC (spine and hip are highest with
age >60years)

65. ◾ Up to 20% of bone mass can be lost per year
with chronic glucocorticoid use
◾ Glucocorticoids, methotrexate (MTX), and
total parenteral nutrition (TPN) further
increases the risk

66. ◾ Osteonecrosis is characterized by death of
osteocytes and adipocytes and eventual bone
collapse
◾ The pain is aggravated by motion and
swelling of the joints.
◾ It affects the hips more often than knees and
shoulders

67. ◾ Osteonecrosis diagnosis is made by bone
scan or MRI
Treatment consists of
◾ PainControl
◾ Cord Decompression
◾ Osteotomy
◾ Joint Replacement.

71. ◾ Endocarditis
◾ Myocarditis
◾ Pleuropericarditis
◾ Interstitial Lung Disease

72. ◾ Secondary or reactive amyloidosis causing
diarrhea, constipation, and renal failure.
◾ The renal disease can be successfully treated
with colchicine.

73. ◾ Pancreatitis is a rare extraintestinal
manifestation of IBD
◾ It results from duodenal fistulas, ampullary CD,
gallstones, PSC
◾ Drugs such as 6-mercaptopurine, azathioprine,,
5-ASA agents can also lead to the pancreatitis
◾ Autoimmune pancreatitis

75. CASE SERIESON EXTRA INTESTINAL
MANIFESTATIONOF IBD

92. ◾ Harrisons principles of internal medicine, 20th Edition
◾ Bailey and Love’s short practice of surgery 27th edition
◾ API text book of medicine, 9th edition
◾ Sherlock’s disease of the liver and biliary system
◾ Beyond the Bowel: Extraintestinal Manifestations of Inflammatory
Bowel Disease, Jeffery D et al., Multisystem radiology, May 26
2017,Volume 26, no4
◾ Extraintestinal Manifestations of Inflammatory Bowel Disease,
Stephan R.Vavricka et al., Inflamm Bowel Dis Volume 21, Number
8,August 2015