1. Extra GI Manifestations of IBD Dr. Matt W. Johnson BSc MBBS MRCP MD Consultant Gastroenterologist Luton & Dunstable FT Hospital

2. Luminology

4. To the ileum …and beyond

5. Extra GI Manifestations of IBD =40% OrganComplications Mouth Glossitis / Angular stomatitis / Orofacial granulomatosis Eyes Episcleritis / Iritis / Uveitis Skin Erythema nodosum / Pyoderma Gangrenosum Bones Sacroiliitis / Enteropathic Arthropathy / Ankylosing Spondylitis / Osteoporosis Lungs Fibrosing Alveolitis (UIP) Liver AICAH / Granulomatous Hepatitis / Amyloid Biliary Tract Gallstones / Bile acid malabsorption / Primary Sclerosing Cholangitis / AI pancreatitis / Cholangiocarcinoma Kidneys Stones (uric acid, oxalate) Blood Fe + B12 + Folate deficiency / A+V Thrombosis Constitutional Toxic megacolon / Weight loss / Growth retardation Post-Surgical Bile acid malabsorption / abscess / strictures / fistulae

6. EGIM of IBD CrDUCBothActivity IBD Rx OFG+++/- Gallstone + sb -- PSC+-- PBC+-- AIP+-- Epi/Scleritis+++ Iritis/Uveitis+++ EN+++ PG++/-+/- Serositis+++ Sacroilitis+++ T1 Arthro +++ T2 Arthro +-- AnkSpond+--

7. Mouth 1) Glossitis - 2) Angular Stomatitis 3) Orofacial granulomatosis

8. Glossitis B12 deficiency B12 deficiency –Red “beefy” tongue Fe deficiency Fe deficiency –Atrophic smooth tongue Rx = Supplements

9. Angular Stomatitis Fe deficiency Fe deficiency Rx = Supplements

10. Orofacial Granulomatosis Rare chronic inflammatory condition Rare chronic inflammatory condition Characterised by lip swelling Characterised by lip swelling 64% have histological granulomas similar to CrD 64% have histological granulomas similar to CrD Rx = Elemental or Cinnamon and benzoate free diet Rx = Elemental or Cinnamon and benzoate free diet

11. Eyes 1) Episcleritis 2) Iritis 3) Uvietis 4) Steroid Cataracts

12. Episcleritis  Incidence = 5%  Superficial redness of the episclera and conjuctiva  Burning + itching due to dilated vessels  Mx = Self resolves +/- NSAIDS

13. Scleritis  Deeper redness of sclera  Serious inflammatory condition  Ocular pain, photophobia, tearing, blindness  Rx = Treat the IBD + Systemic steroids, NSAIDS, antibiotics or immunosuppressant

14. Iritis / Uveitis  Inflammation of the iris (anterior uveitis)  0.5-3%  Acute self resolves within weeks  Chronic persists for months and needs Rx  Ocular pain, photophobia, blurry vision, synechia

15. Iritis  Complications include; synechia, cataracts, glaucoma, blindness  Rx = Steroids (PO + drops, subconjuctival injections)

16. Uveitis  Inflammation of middle/inner eye  10% of blindness in USA  Mx = Urgent referral to ophthalmologist  Treat the IBD  Rx = Steroids (PO + drops, subconjuctival injections), dilators + pressure reducing drops (brimonidine tartrate) +/- MTX, IFX

17. Skin 1) Erythema Nodosum 2) Pyoderma gangerenosum

18. Erythema Nodosum 8-15% of UC + CrD 8-15% of UC + CrD Usually reflects active disease Usually reflects active disease Can precede the IBD diagnosis Can precede the IBD diagnosis Red hot nodules on extensor surfaces Red hot nodules on extensor surfaces Assoc with pauciarticular arthropathy Assoc with pauciarticular arthropathy Rx the IBD and you Rx the EN Rx the IBD and you Rx the EN

19. Pyoderma Gangerenosum 5% UC 5% UC 2% of CrD patients 2% of CrD patients 50% assoc with IBD activity 50% assoc with IBD activity Starts with a red area + central pustules then develops into a painful necrotic ulcer Starts with a red area + central pustules then develops into a painful necrotic ulcer Steroids, IFX, Cyclosporin Steroids, IFX, Cyclosporin Colectomy does not always help Colectomy does not always help

20. Airway inflammation  UC > CrD  Chronic cough and mucopurulent sputum  Progressive airways narrowing leads to Chronic bronchitis + bronchiectasis + bronchiolitis obliterans  CXRs frequently normal, needs HRCT  Rx = Large airways - Inhaled steroids Small airways - Systemic steroids Small airways - Systemic steroids

21. Thrombo-embolic disorders TE events occur in 25% TE events occur in 25% 3 fold increase above general population 3 fold increase above general population Recurrence risk is 10-15% Recurrence risk is 10-15% UCCrD Incidence per 10,000 5040 Increase risk of DVT 2.82.9 Increase risk of PE 3.64.7

22. Liver + Pancreas 1) Abnormal LFTs = 30% eg. AZA 2) Gallstones = 13-34% of sb Crohn’s 3) PSC 4) PBC 5) AI Pancreatitis

23. Primary Sclerosing Cholangitis  5% of UC and 1-2% CrD  Can precede colitis by years  Symptoms = Pruritis, fatigue, RUQ pain, jaundice, cholangitis  Bedding and stricturing of IHDs  Associated with cholangiocarcinoma 6-20%  Increased risk of U+L GI cancer x6 and ampullary cancer  Colonoscopy every year, with OGD every 2 years  Survival if symptomatic = 15- 18y

24. Primary Biliary Cirrhosis  More commonly seen with UC  High cholesterol  Deficiencies in the fat soluble vitamins DEAK  Leads to cholestasis

25. Bones 1) Osteoporosis 2) Sacroileitis 3) Arthropathies (RhA, AnkSpond)

26. Osteopenia / Osteoporosis Peak bone mass reached in our 20-30s Then 0.5-1% per year thereafter 15% BMD lost in first 5y post menopause Osteopenia occurs in 40-50% Osteoporosis occurs in 2-30% Lifetime risk of fractures in IBD = 41% CrD women have 2.5 fold increase fracture risk

27. Osteoporosis Prevention 1) Weight bearing exercise 2) Stop smoking 3) Reduce weight 4) Moderate Xol intake 5) Ca intake (1000-1500mg/d) = 1 pint of semi skimmed is 700mg 6) Stop steroids ASAP 1)Bone loss starts rapidly 2)Occurs even with low doses 3)Fracture risk improves on cessation 7) Ca + Vit D = All patients on steroids 8) Bisphosphonates = steroids >3m, those >65y or low impact (fragility) fractures 9) HRT eg testosterone in steroid induced hypogonadism

28. BSG Mx of Osteoporosis  Calcium + Vit D  PO Bisphosphonates (eg alendronate, residronate)  IV Bisphosphonates (eg. pamidronate)  In those with difficult side effects eg. oesophagitis  Poor mucosal absorption  Avoids the problems  HRT (in PMP women) - risk of clots / breast+gynae cancer  Raloxifene - modulator of OR, without increased of breast Ca

29. Sacroilitis  Prevalence = 47%  Sacro-iliac pain  Hazziness of sacro- iliac joint  Can be one sided  Rx = COX II inhibitors  Try to avoid NSAIDS  Steroids / IFX  Mx = Treat the IBD

30. IBD Arthropathy  10-20% of IBD patients (esp in Colonic disease, EN, Eyes)  Not to be confused with arthralgia secondary to steroid withdrawal, AZA or steroid induced myopathy.  1) Type 1 (Large Joint) Arthropathy = 5%   6 joints, (typically 1 large joint eg. knee)  Attacks assoc with active inflammatory relapses, EN + Iritis  Usually self limiting, no role for NSAIDS  Treat the IBD = 5ASAs, Steroids, MTX, AZA, Colectomy  2) Type 2 (Small Joint) Arthropathy = 3-4%  Affects >5 joints, (typically small joints of hands and feet)  No direct assoc with IBD activity or Rx

31. Rx Algorithm for IBD Arthropathy 1st Line Physical exercises Simple analgesia Intra-articular injections Steroids + Lignocaine 2nd Line Sulfasalazine or Pentasa (sb) NSAIDS!!! / Codeine !!! MTX (esp. Crohns) (No evidence for AZA/Cyclo) Bonner G.F. AmJG. 2002 Thompson GT. JRheum 2000 3rd Line IFX (Type 1) Thalidomide (80% AnkSpon) Bisphosphonates

32. EGIM of IBD CrDUCBothActivity IBD Rx OFG+++/- Gallstone + sb -- PSC+-- PBC+-- AIP+-- Epi/Scleritis+++ Iritis/Uveitis+++ EN+++ PG++/-+/- Serositis+++ Sacroilitis+++ T1 Arthro +++ T2 Arthro +-- AnkSpond+--