This document provides an overview of inflammatory bowel disease (IBD), including its typical and atypical forms, clinical features, radiological signs, and extraintestinal manifestations. IBD is characterized by chronic inflammation of the intestines that results in periods of remission and relapse. The two major types are ulcerative colitis and Crohn's disease. Extraintestinal manifestations can affect the skin, eyes, joints, liver, bones and other organs, and are thought to arise from an inappropriate immune response triggered by intestinal inflammation. The document reviews several case examples and references textbooks and studies on IBD and its extraintestinal manifestations.
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Extraintestinal Manifestations OF IBD Inflammatory Bowel Disease : A complete overview
1. Guide : Dr Chandrashekar K (Associate Professor)
Student : Dr Chetan K G
2. INTRODUCTION
CLINICAL FEATURES OF IBD
RADIOLOGICAL SIGNS
EXTRAINTESTINAL MANIFESTATIONS
CASE SCENARIOS
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 .
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.
6.
7.
8.
9.
10.
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 with UC are more likely to appear
when the HLA-DR103 genotype is present.
13. 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.
14.
15.
16.
17.
18.
19.
20.
21. 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
22.
23.
24.
25. 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
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
32. 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 OfThe
Upper And Lower Extremities
Colectomy frequently CuresTheArthritis
33.
34. 10% Of IBD patients
Common in CD > UC
2/3rd patients are HLA-B27 antigen positive
Activity is not related to bowel activity
35. Symmetrical (both joints)
Occurs equally in UC and CD
Does not correlate with bowel activity
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.
42.
43. Hepatic steatosis : 50% cases
Hepatomegaly is found on examination
Cholelithiasis is seen in 10-35% after ileal
resection or ileitis
Primary Sclerosing Cholangitis
Gall bladder polyps
44. Intrahepatic and extrahepatic bile duct inflammation
and fibrosis
Biliary cirrhosis and hepatic failure
~5% of patients with UC 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
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
54.
55.
56.
57.
58.
59. Increased risk of both venous and arterial
thrombosis even if the disease is not active
Abnormalities OfThe Platelet-endothelial
Interaction,
Hyperhomocysteinemia,
Alterations InThe Coagulation Cascade,
Impaired Fibrinolysis,
60. Involvement OfTissue Factor-bearing
Microvesicles,
Disruption OfThe Normal Coagulation System
By Autoantibodies
Genetic Predisposition
A spectrum of vasculitides involving small,
medium, and large vessels has also been
observed.
61.
62.
63.
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% in UC (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
Pain Control
Cord Decompression
Osteotomy
Joint Replacement.
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
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
Notes de l'éditeur
Inflammation of skin and subcutaneous fatty layer. It is usually secondary to ciculating antigens. Mcc = streptococcal infections. Sulfa drugs, sufonylureas, leprosy, OCP are also causatives. Bechets, sarcoidosis, lymphoma, hodgkins, rarely pregnancy. M:F=1:4
Unknown etiology. Mimicing lesions are infections, malignancy, vasculitis, CTD, diabetes and rarely trauma. Diagnosis of exclusion. IBD, RA, Collagen vasc disorders, HIV, carcinoids, intestinal cancers, leukemia, MDS, MPS, Propylthiouracil, gammopathies, Isotretinoin, Soratinib and Geftinib.
Pyoderma Vegetans : Rare, Large veruccous plaques, elevated borders and multiple pustules.Associated m.c with streptococcal and staphylococcal infections in pt with immune dysfunction/dysregulation. DLBL, HIV, UC, CD are associated.
Pyostomatitis vegetans is an inflammatory stomatitis and most often seen in association with IBD, namely UC and CD.
Sweet's syndrome (SS), or acute febrile neutrophilic dermatosis[1][2] is a skin disease characterized by the sudden onset of fever, an elevated white blood cell count, and tender, red, well-demarcated papules and plaques that show dense infiltrates by neutrophil granulocyteson histologic examination.
It can be classical(idiopathic), drug induced or malignancy associated. AML, Streptococcal infections, IBD, Solid tumors, Non lymphocytic leukemias, G-CSF, GM-CSF, RA, Bechets
AS is a type of seronegative spondyloarthropathy, known as axial spondyloarthritis, Males are more often affected than females, tween 20 and 30 years of age
The Bath Ankylosing Spondylitis Disease Activity Index (BASDAI), developed in Bath (UK), is an index designed to detect the inflammatory burden of active disease
The Schober's test is a useful clinical measure of flexion of the lumbar spine performed during the physical examination
Sacroiliitis in a 50-year-old man with ankylosing spondylitis: Anteroposterior pelvic radiograph shows fusion of the sacroiliac joints and severe secondary peripheral osteoarthritis at the hip joints (white arrow), as well as chronic spine findings, including the “dagger spine sign” (black arrow), which represents ossification of the supraspinous and interspinous ligaments
Sacroiliitis in a 65-year-old woman with Crohn disease who had undergone proctocolectomy
White arrow : Anteroposterior pelvic radiograph shows evidence of the prior bowel surgery (white arrow), with fusion of the sacroiliac joints (black arrow)
Axial contrast-enhanced CT image of the same patient shown in b obtained 3 years later shows dilatation of the small bowel, with an enterocutaneous fistula (white arrow). The bilateral sacroiliac joints (black arrow) are fused.
medical condition combining clubbing and periostitis of the small hand joints, especially the distal interphalangeal joints and the metacarpophalangeal joints.
Lung cancer(mc = small cell CA) being the most common cause but also occurring with ovarian or adrenal malignancies
bone scans showing parallel lines of activity along the cortex of the shafts and ends of tibiae, femurs and radii; especially around the knees, ankles and wrists
often painful disease can cause joint deformity and be life-threatening if the respiratory tract, heart valves, or blood vesselsare affected.
Wegeners, Churgstrauus, UC, PAN, Bechets, GCA, SLE, Sclerodderma, APLA, MCTD, RA, MDS, MPS, Psoriasis, Atopy, Vitiligo, UC, CD, PSC, Biliary cirrhosis, IPF etc
Ear, Nose, Trachea, Ribs, Joints, Eye –episcleritis/scleritis), CN palsy, Hemiplegia, meningitis, Gnitis, Slomerulosclerosis, Aphthous ulcers
Prompt intervention, sometimes with systemic glucocorticoids, is required to prevent scarring and visual impairment. Episcleritis is a benign disorder that presents with symptoms of mild ocular burning.
Episcleritis is usually idiopathic, acute and associated with mild pain, redness and irritation. Vessels are mobile, blach with phenylephrine and reddish huw. Self limited condition.
Scleritis is autoimmune and subacute in onset with severe pain and pain with ocular movements. Blurred vision and photophobia may be seen. Visual loss can occur. Vessels are adherent, don’t blanch and slit lamp may show nodules, scleral thinning, corneal opacity.
long-term progressive disease of the liver and gallbladder characterized by inflammation and scarring of the bile ducts.
SC increases the risk of various cancers including liver cancer, gallbladder carcinoma, colorectal cancer, and cholangiocarcinoma.
most definitive treatment for PSC is a liver transplant but it can recur after transplantation
50% cases : no symptoms
an important association between the intestinal microbiota and PSC
a process referred to as cellular senescence and the senescence-associated secretory phenotype (SASP) in the pathogenesis
T2-weighted fat-saturated MR image shows diffuse contour irregularity throughout the liver. Diffusely irregular calibers of the bile ducts are noted throughout the liver, with predominant involvement of the lateral segment of the left hepatic lobe
MR cholangiopancreatographic image shows diffuse irregularity of the bile ducts, with areas of stenosis (arrows) and dilatation throughout the liver.
oronal T2-weighted MR image shows diffuse dilatation of a bile duct within the right hepatic lobe. Multiple filling defects (arrow) are noted throughout the duct, findings consistent with biliary lithiasis.
MR image shows a diffusely irregular macrolobular liver contour with central or caudate hypertrophy (arrows) and peripheral atrophy (*). Scattered areas of mildly increased signal intensity are noted throughout the peripheral liver, findings consistent with diffuse fibrosis.
after administration of oral contrast material shows a large communication (black arrow) between the colon (white arrow) and the contrast material–filled vagina
Coronal contrast-enhanced CT image shows a fistula (white arrow) between a segment of diffusely thickened sigmoid colon (*) and the urinary bladder wall.
dome of the bladder is markedly thickened and contains an intramural abscess (black arrow)
abscessogram obtained after percutaneous drainage of an intramural bladder dome abscess shows opacification of the intramural abscess cavity (arrow), with persistent fistulous communication (*) with the adjacent sigmoid colon.
obstructive uropathy : moderate hydronephrosis of the right kidney .
diffuse thickening and enhancement of the terminal ileum and cecum (black arrow)
Bilateral nephrolithiasis
diffuse edematous enlargement of the entire pancreas (*), with peripancreatic fat stranding (arrows) s/o Acute pancreatitis in a patient with CD
Trauma, Steroid use, Chr. Alcoholism, cushings, hypercortisol, Hemoglobinopathies like Sickle cell, pancreatitis, SLE, CTD, HIV, MDS, MPS