Extraintestinal Manifestations OF IBD Inflammatory Bowel Disease : A complete overview
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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 .
- 4. TYPICAL IBD : (2 MajorTypes): Ulcerative Colitis (Colitis Ulcerosa) Crohn’s Disease (Regional Enteritis) ATYPICAL IBD: Lymphocytic Colitis Collagenous Colitis Ischaemic Colitis Diversion Colitis Indeterminate Colitis 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 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.
- 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
- 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
- 30. PyodermaVegetans PyostomatitisVegetans Sweet Syndrome Psoriasis Perianal SkinTag Aphthous Stomatitis
- 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
- 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
- 39. Hypertrophic Osteoarthropathy Pelvic/Femoral Osteomyelitis 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 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
- 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.
- 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.
- 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 SERIES ON 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
Editor's Notes
- 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