Extra GI Manifestations Extra GI Manifestations of IBDof IBD

Dr. Matt W. Johnson Dr. Matt W. Johnson BSc MBBS MRCP MDBSc MBBS MRCP MDConsultant GastroenterologistConsultant GastroenterologistLuton & Dunstable FT HospitalLuton & Dunstable FT Hospital

LuminologyLuminology

To the ileum …and beyondTo the ileum …and beyond

Extra GI Manifestations of IBD Extra GI Manifestations of IBD =40%=40%

OrganOrgan ComplicationsComplicationsMouthMouth Glossitis / Angular stomatitis / Orofacial Glossitis / Angular stomatitis / Orofacial

granulomatosisgranulomatosis

EyesEyes Episcleritis / Iritis / UveitisEpiscleritis / Iritis / Uveitis

SkinSkin Erythema nodosum / Pyoderma GangrenosumErythema nodosum / Pyoderma Gangrenosum

BonesBones Sacroiliitis / Enteropathic Arthropathy / Ankylosing Sacroiliitis / Enteropathic Arthropathy / Ankylosing Spondylitis / OsteoporosisSpondylitis / Osteoporosis

LungsLungs Fibrosing Alveolitis (UIP)Fibrosing Alveolitis (UIP)

LiverLiver AICAH / Granulomatous Hepatitis / AmyloidAICAH / Granulomatous Hepatitis / Amyloid

Biliary TractBiliary Tract Gallstones / Bile acid malabsorption / Primary Gallstones / Bile acid malabsorption / Primary Sclerosing Cholangitis / AI pancreatitis / Sclerosing Cholangitis / AI pancreatitis / CholangiocarcinomaCholangiocarcinoma

KidneysKidneys Stones (uric acid, oxalate)Stones (uric acid, oxalate)

BloodBlood Fe + B12 + Folate deficiency / A+V ThrombosisFe + B12 + Folate deficiency / A+V Thrombosis

ConstitutionConstitutionalal

Toxic megacolon / Weight loss / Growth retardationToxic megacolon / Weight loss / Growth retardation

Post-Post-SurgicalSurgical

Bile acid malabsorption / abscess / strictures / Bile acid malabsorption / abscess / strictures / fistulaefistulae

EGIM of IBDEGIM of IBDCrDCrD UCUC BothBoth ActivityActivity IBD RxIBD Rx

OFGOFG ++ ++ +/-+/-GallstoneGallstone + sb+ sb -- --PSCPSC ++ -- --PBCPBC ++ -- --AIPAIP ++ -- --Epi/Epi/ScleritisScleritis

++ ++ ++

Iritis/Iritis/UveitisUveitis

++ ++ ++

ENEN ++ ++ ++PGPG ++ +/-+/- +/-+/-SerositisSerositis ++ ++ ++SacroilitisSacroilitis ++ ++ ++T1 ArthroT1 Arthro ++ ++ ++T2 ArthroT2 Arthro ++ -- --AnkSpondAnkSpond ++ -- --

MouthMouth1)1) Glossitis - Glossitis -

2)2) Angular StomatitisAngular Stomatitis

3)3) Orofacial granulomatosisOrofacial granulomatosis

GlossitisGlossitis• B12 deficiencyB12 deficiency

– Red “beefy” tongue Red “beefy” tongue • Fe deficiencyFe deficiency

– Atrophic smooth Atrophic smooth tongue tongue

Rx = SupplementsRx = Supplements

Angular StomatitisAngular Stomatitis• Fe deficiencyFe deficiency

Rx = SupplementsRx = Supplements

Orofacial GranulomatosisOrofacial Granulomatosis• Rare chronic Rare chronic

inflammatory conditioninflammatory condition• Characterised by lip Characterised by lip

swellingswelling• 64% have histological 64% have histological

granulomas similar to granulomas similar to CrDCrD

• Rx = Elemental or Rx = Elemental or Cinnamon and benzoate Cinnamon and benzoate free dietfree diet

EyesEyes1)1) EpiscleritisEpiscleritis

2)2) Iritis Iritis

3)3) UvietisUvietis

4)4) Steroid CataractsSteroid Cataracts

EpiscleritisEpiscleritis Incidence = 5% Incidence = 5%

Superficial redness of Superficial redness of the episclera and the episclera and conjuctivaconjuctiva

Burning + itching due Burning + itching due to dilated vesselsto dilated vessels

Mx = Self resolves +/- Mx = Self resolves +/- NSAIDSNSAIDS

ScleritisScleritis Deeper redness of scleraDeeper redness of sclera Serious inflammatory Serious inflammatory

condition condition Ocular pain, Ocular pain,

photophobia, tearing, photophobia, tearing, blindness blindness

Rx = Treat the IBD + Rx = Treat the IBD + Systemic steroids, Systemic steroids, NSAIDS, antibiotics or NSAIDS, antibiotics or immunosuppressantimmunosuppressant

Iritis / UveitisIritis / Uveitis Inflammation of the iris Inflammation of the iris

(anterior uveitis)(anterior uveitis)

0.5-3%0.5-3%

Acute self resolves within Acute self resolves within weeksweeks

Chronic persists for Chronic persists for months and needs Rxmonths and needs Rx

Ocular pain, photophobia, Ocular pain, photophobia, blurry vision, synechia blurry vision, synechia

IritisIritis Complications Complications

include; include; synechia, synechia, cataracts, cataracts, glaucoma, glaucoma, blindness blindness

Rx = Steroids Rx = Steroids (PO + drops, (PO + drops, subconjuctival subconjuctival injections)injections)

UveitisUveitis Inflammation of Inflammation of

middle/inner eyemiddle/inner eye

10% of blindness in USA 10% of blindness in USA

Mx = Urgent referral to Mx = Urgent referral to ophthalmologistophthalmologist

Treat the IBDTreat the IBD

Rx = Steroids (PO + Rx = Steroids (PO + drops, subconjuctival drops, subconjuctival injections), dilators + injections), dilators + pressure reducing drops pressure reducing drops (brimonidine tartrate) +/- (brimonidine tartrate) +/- MTX, IFXMTX, IFX

SkinSkin1)1) Erythema NodosumErythema Nodosum

2)2) Pyoderma gangerenosumPyoderma gangerenosum

Erythema NodosumErythema Nodosum• 8-15% of UC + CrD8-15% of UC + CrD• Usually reflects active Usually reflects active

diseasedisease• Can precede the IBD Can precede the IBD

diagnosisdiagnosis

• Red hot nodules on extensor Red hot nodules on extensor surfacessurfaces

• Assoc with pauciarticular Assoc with pauciarticular arthropathyarthropathy

• Rx the IBD and you Rx the ENRx the IBD and you Rx the EN

Pyoderma GangerenosumPyoderma Gangerenosum• 5% UC 5% UC • 2% of CrD patients2% of CrD patients• 50% assoc with IBD 50% assoc with IBD

activityactivity

• Starts with a red area + Starts with a red area + central pustules then central pustules then develops into a painful develops into a painful necrotic ulcernecrotic ulcer

• Steroids, IFX, CyclosporinSteroids, IFX, Cyclosporin• Colectomy does not Colectomy does not

always helpalways help

Airway inflammationAirway inflammation UC > CrD UC > CrD Chronic cough and mucopurulent Chronic cough and mucopurulent

sputumsputum Progressive airways narrowing leads Progressive airways narrowing leads

to Chronic bronchitis + bronchiectasis to Chronic bronchitis + bronchiectasis + bronchiolitis obliterans+ bronchiolitis obliterans

CXRs frequently normal, needs HRCTCXRs frequently normal, needs HRCT Rx = Large airways - Inhaled steroids Rx = Large airways - Inhaled steroids Small airways - Systemic steroids Small airways - Systemic steroids

Thrombo-embolic disordersThrombo-embolic disorders

• TE events occur in 25%TE events occur in 25%• 3 fold increase above general population3 fold increase above general population• Recurrence risk is 10-15%Recurrence risk is 10-15%

UCUC CrDCrDIncidence per 10,000Incidence per 10,000 5050 4040Increase risk of DVTIncrease risk of DVT 2.82.8 2.92.9Increase risk of PEIncrease risk of PE 3.63.6 4.74.7

Liver + PancreasLiver + Pancreas1)1) Abnormal LFTs = 30% eg. AZAAbnormal LFTs = 30% eg. AZA

2)2) Gallstones = 13-34% of sb Gallstones = 13-34% of sb Crohn’sCrohn’s

3)3) PSCPSC

4)4) PBCPBC

5)5) AI PancreatitisAI Pancreatitis

Primary Sclerosing Primary Sclerosing CholangitisCholangitis

5% of UC and 1-2% CrD5% of UC and 1-2% CrD Can precede colitis by yearsCan precede colitis by years Symptoms = Pruritis, Symptoms = Pruritis,

fatigue, RUQ pain, jaundice, fatigue, RUQ pain, jaundice, cholangitischolangitis

Bedding and stricturing of Bedding and stricturing of IHDsIHDs

Associated with Associated with cholangiocarcinoma 6-20%cholangiocarcinoma 6-20%

Increased risk of U+L GI Increased risk of U+L GI cancer x6 and ampullary cancer x6 and ampullary cancercancer

Colonoscopy every year, Colonoscopy every year, with OGD every 2 years with OGD every 2 years

Survival if symptomatic = Survival if symptomatic = 15-18y15-18y

Primary Biliary CirrhosisPrimary Biliary Cirrhosis More commonly More commonly

seen with UCseen with UC

High cholesterolHigh cholesterol

Deficiencies in the Deficiencies in the fat soluble vitamins fat soluble vitamins DEAKDEAK

Leads to Leads to cholestasischolestasis

BonesBones1)1) OsteoporosisOsteoporosis

2)2) SacroileitisSacroileitis

3)3) Arthropathies (RhA, AnkSpond)Arthropathies (RhA, AnkSpond)

Osteopenia / OsteoporosisOsteopenia / OsteoporosisPeak bone mass reached in our 20-Peak bone mass reached in our 20-

30s30sThen 0.5-1% per year thereafterThen 0.5-1% per year thereafter15% BMD lost in first 5y post 15% BMD lost in first 5y post

menopausemenopause

Osteopenia occurs in 40-50%Osteopenia occurs in 40-50%Osteoporosis occurs in 2-30%Osteoporosis occurs in 2-30%

Lifetime risk of fractures in IBD = Lifetime risk of fractures in IBD = 41%41%

CrD women have 2.5 fold increase CrD women have 2.5 fold increase fracture risk fracture risk

OsteoporosisOsteoporosisPreventionPrevention

1)1) Weight bearing exerciseWeight bearing exercise2)2) Stop smokingStop smoking3)3) Reduce weightReduce weight4)4) Moderate Xol intakeModerate Xol intake5)5) Ca intake (1000-1500mg/d) = 1 pint of semi skimmed is 700mgCa intake (1000-1500mg/d) = 1 pint of semi skimmed is 700mg6)6) Stop steroids ASAPStop steroids ASAP

1)1) Bone loss starts rapidly Bone loss starts rapidly 2)2) Occurs even with low dosesOccurs even with low doses3)3) Fracture risk improves on cessationFracture risk improves on cessation

7)7) Ca + Vit D = All patients on steroidsCa + Vit D = All patients on steroids8)8) Bisphosphonates = steroids >3m, those >65y or low impact Bisphosphonates = steroids >3m, those >65y or low impact

(fragility) fractures(fragility) fractures9)9) HRT eg testosterone in steroid induced hypogonadismHRT eg testosterone in steroid induced hypogonadism

BSG Mx of OsteoporosisBSG Mx of Osteoporosis

Calcium + Vit DCalcium + Vit D PO Bisphosphonates (eg alendronate, residronate)PO Bisphosphonates (eg alendronate, residronate) IV Bisphosphonates (eg. pamidronate)IV Bisphosphonates (eg. pamidronate)

In those with difficult side effects eg. oesophagitisIn those with difficult side effects eg. oesophagitis Poor mucosal absorptionPoor mucosal absorption Avoids the problems Avoids the problems

HRT (in PMP women) - risk of clots / breast+gynae HRT (in PMP women) - risk of clots / breast+gynae cancercancer

Raloxifene - modulator of OR, without increased of Raloxifene - modulator of OR, without increased of breast Cabreast Ca

SacroilitisSacroilitis Prevalence = 47%Prevalence = 47% Sacro-iliac painSacro-iliac pain Hazziness of Hazziness of

sacro-iliac jointsacro-iliac joint Can be one sidedCan be one sided

Rx = COX II Rx = COX II inhibitorsinhibitors

Try to avoid Try to avoid NSAIDSNSAIDS

Steroids / IFXSteroids / IFX Mx = Treat the IBDMx = Treat the IBD

IBD ArthropathyIBD Arthropathy 10-20% of IBD patients (esp in Colonic disease, EN, Eyes)10-20% of IBD patients (esp in Colonic disease, EN, Eyes) Not to be confused with Not to be confused with arthralgia secondary to steroid arthralgia secondary to steroid

withdrawal, AZA or steroid induced myopathy.withdrawal, AZA or steroid induced myopathy.

1) Type 1 (Large Joint) Arthropathy = 5%1) Type 1 (Large Joint) Arthropathy = 5% 6 joints, (typically 1 large joint eg. knee)6 joints, (typically 1 large joint eg. knee) Attacks assoc with active inflammatory relapses, EN + IritisAttacks assoc with active inflammatory relapses, EN + Iritis Usually self limiting, no role for NSAIDS Usually self limiting, no role for NSAIDS Treat the IBD = 5ASAs, Steroids, MTX, AZA, ColectomyTreat the IBD = 5ASAs, Steroids, MTX, AZA, Colectomy

2) Type 2 (Small Joint) Arthropathy = 3-4%2) Type 2 (Small Joint) Arthropathy = 3-4% Affects >5 joints, (typically small joints of hands and feet)Affects >5 joints, (typically small joints of hands and feet) No direct assoc with IBD activity or RxNo direct assoc with IBD activity or Rx

Rx Algorithm for IBD Rx Algorithm for IBD ArthropathyArthropathy1st 1st LineLine

Physical exercisesPhysical exercisesSimple analgesiaSimple analgesiaIntra-articular injectionsIntra-articular injections Steroids + LignocaineSteroids + Lignocaine

2nd 2nd LineLine

Sulfasalazine or Pentasa Sulfasalazine or Pentasa (sb)(sb)NSAIDS!!! / Codeine !!!NSAIDS!!! / Codeine !!!MTX (esp. Crohns)MTX (esp. Crohns)(No evidence for (No evidence for AZA/Cyclo)AZA/Cyclo)

Bonner G.F. AmJG. 2002Bonner G.F. AmJG. 2002Thompson GT. JRheum Thompson GT. JRheum 20002000

3rd 3rd LineLine

IFX (Type 1)IFX (Type 1)Thalidomide (80% Thalidomide (80% AnkSpon)AnkSpon)BisphosphonatesBisphosphonates

EGIM of IBDEGIM of IBDCrDCrD UCUC BothBoth ActivityActivity IBD RxIBD Rx

OFGOFG ++ ++ +/-+/-GallstoneGallstone + sb+ sb -- --PSCPSC ++ -- --PBCPBC ++ -- --AIPAIP ++ -- --Epi/Epi/ScleritisScleritis

++ ++ ++

Iritis/Iritis/UveitisUveitis

++ ++ ++

ENEN ++ ++ ++PGPG ++ +/-+/- +/-+/-SerositisSerositis ++ ++ ++SacroilitisSacroilitis ++ ++ ++T1 ArthroT1 Arthro ++ ++ ++T2 ArthroT2 Arthro ++ -- --AnkSpondAnkSpond ++ -- --