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8/2/2019 Behcet SvsIBDDr.M.sandhuSept21 04

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A diagnostic dilemma ofBehcets disease - a case

studyMonty Sandhu

PGY-1


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Case PresentationID:

39 y/o Iranian female with hx SLERFR:

Chronic diarrhea + rash to legs

PMH:SLE

joint pain/arthritis, ITP, alopecia, + ANA 1:320, + dsDNA22

July/04 B/W: N plt/ LFT/ Cre. dsDNA 60, N C3/C4/ ESR/ CRP. U/A + sm amt rbc and ++ wbcHTN

Meds:Plaquenil, prednisone, tylenol, demerol, gravol


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HPI2mo history watery stools

18lb wt loss 2 appetite

Denies N/V/abdo pain. Negative travel hx. No H/A. Nofevers, no cough

After diarrhea started (?), UTI developed and treated with 10dPenicillin

1mo ago developed leg rash, followed oral ulcers and andconjuctivitis/blepharitis


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Physical exam

BP 128/90, P105, RR 16, T 38.5, SaO2 99% R/AHEENT

No lymphadenopathy, N thyroid, blepharitis, conjuctivalinjection, + oral ulcers to tongue and lips. Neg hair pulltest

CVS

N S1, S2, no EHS/ murmurs, JVP 1-2cm ASA, no rubsResp

UnremarkableGI

Abdomen soft, non-tender, distended, N BS, no peritonealsigns, no HSM/ ascites, no masses

MSKNo active joints/ effusion/ ROM. N muscle bulk/ tone/power

Derm

Erythematous, swollen nodules bilaterally to L/E


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InvestigationsWbc 11.66 (neut 9.3), Hgb 130, plt 262, all lytes WNL, Ca/PO4/ Mg/ LFTs WNL except AST 48, albumin 28

ESR 92, CRP 96.2, TSH 0.34 ( ), free T4 N, CMV negative

Urine 0.3g/L protein, 125 wbc/uL 24hr urine 0.13g/d protein,Cre 47

Stool negative for O&P, yersinia, campylobacter, E.coli,Shigella, Salmonella

Anti ds DNA and ANA negative, C3 0.99, C4 0.2

CXR N, Abdo X-rays show air fluid levels, Abdo U/S-unremarkable

Urine and blood C&S- no growth, C.Dif toxin negative


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Investigations cont Colonoscopy

Sigmoid biopsyAcute + chronic inflammatory inflitratesNo granulomas, no dysplasia, no malignancy

no convincing evidence of vasculitis Possible etiologies: resolving infection or drugs.

Skin biopsy

Adipose tissue panniculitis with septal acute and chronicinflammationNo evidence of arteritisAppearance consistent with erythema nodosum


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Course in Hospital

Started on Solumedrol 20mg IV bid and switched to poafter 5d. Both diarrhea and EN rash resolved

D/C home on prednisone taper (60mg 50mg),plaquenil, alendronate, Vit D & Ca

F/U arranged with Rheumatology, GI


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Clinical QuestionsWhat are the clinical manifestations and diagnostic criteria forBehcets disease?

How do you differentiate Behcets disease with GIinvolvement from IBD?

Can Behcets disease and IBD exist as 2 separate entities inthe same patient?

Literature review:

Medline search of IBD combined with Behcets disease limited to english and human datamainly case reports and editorial comments found


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Behcets Disease (BD) Multisystem, chronic, relapsing vasculitis of unknown etiology

IncidenceRare in Western countries, but common in Mediterranean,Middle East and Japan

Mean age of onset 25-35yr

Clinical Manifestations:

Oral, apthous ulcers

Urogenital lesions

Cutaneous lesions


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Manifestations of BD cont Pathergy

Ocular disease (uveitis is most common)

Neurological involvement

Vascular disease

Arthritis (nonerosive, asymmetric, nondeformingoligoarthritis)

Mild renal disease (rare)

GI ulcerations


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Oral Aphthae


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GI Manifestations of BDMain effects are on ileocecal region and colon

Most common symptoms of intestinal Behcets areabdominal pain, N/V, diarrhea blood in stool,constipation

GI complications usually occur 4-6yr after onset of oralulcers

Intestinal lesions are resistant to medical treatment

Lower incidence in, India and Israel (0-5%)


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Intestinal BDOral ulcers

Ddx- anemia, avitaminosis, viral infn, IBD, Reiter

syndrome

EsophagusUncommon site of reported BDUlcers similar to those in mouth

StomachGastric mucosa least commonly involved. Apthous ulcers

DuodenumAphthous ulcers resistant to medical therapy

Pancreas

Vasculitis may cause pancreatitis


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Intestinal BD cont

IntestinePresentation similar to Crohns disease (CD). In ptwith intestinal BD, other stigmata of BD may appearlater

2 forms: Mucosal inflammation or ischemia/infarct

S. intestine most commonly involved (terminal ileumand cecum)

Rectal and anal involvement rare

Ileocecal involvement common to Japan & Turkey.Colonic involvement common in Europe & N. America


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Intestinal BD cont..Intestine cont

Morphology of lesions: aphthous ulcers or deep round or ovalulcers with punched-out appearance. Longitudinal ulcers rare

Ulcers localize and appear in clusters

Concordance b/w number of ulcers and risk of perforation

Rectal involvement rare

LiverMost common complication is Budd-Chiari syndromeBCS is a common manifestation of BD in Turkey

SpleenSplenomegaly


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Punch out lesion in colon


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Comparing IBD to BDBD

Symptomatic diagnosis

neutrophil hyperfunction,excess inflammatorycytokines (TNF)

Trt with sulfasalazine,steroids is 1 st line for GIvasculitis

Anti TNF monoclonal

antibody and thalidomidetreatment

HLA B51 phenotype

Barium studies showpreserved haustras, deepulcers

IBDEndoscopic diagnosis

neutrophil hyperfunction,excess inflammatorycytokines (TNF)

Trt with sulfasalazine,steroids is 1 st line

Anti TNF monoclonalantibody and thalidomide

treatmentDR4, DQ4 (CD), B52, DR2(UC) phenotypes

longitudinal ulcers,cobblestone appearance,stricture formation, fistulas


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Case ReportHistory:

15y/o Arabic male admitted to hosp for W/U fatigue,arthralgia, 5mo wt loss and recurring nodular skin lesionsover shins x2yrHistory of painful oral, penile and perianal lesions x2yrRecent diagnosis of uveitis

Physical exam:Conjunctival erythema, photophobia, aphthous ulcer tobuccal mucosa, tender lesions to penis and perineal area

Erythema nodosum on lateral aspects of both anklesPathergy test negativeESR 35, N LTFs & CBC, ANA/ anti DNA/ anti ENA/ RPRnegative.

Tentative Dx = Behcets disease


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Case Report cont

1mo later.. Readmit for abdo pain, anorexia, diarrhea

Tenderness to RLQ but negative peritoneal signs

Stool C&S and O&P negative

Upper GI series and barium contrast enema normal

OGD normal


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Case Report cont Colonoscopy findings:

Normal rectal and sigmoid mucosa

Descending, transverse, ascending colon showed multipleseripiginous and linear ulcers and pseudo polyps.

Terminal ileum showed cobblestoning with seripiginousulcers

Biopsy of terminal ileum showed infiltration of laminapropria with plasma cells and lymphocytes

Microgranulomas on biopsy of ascending colon

Crohns disease


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Case Report discussionDiscussion

Several other case reports discussed which showed BD withUCControversy as to whether these cases are dual diagnoses orsimply intestinal BDBased on endoscopy and histopathology, authors concludedthat CD was presentBased on symptomatic criteria, BD was also presentCase was not intestinal BD b/c imaging was not typical forthis pattern

ConclusionSeveral case reports of coexisting IBD not otherwiseexplained by intestinal BD

Possibility of dual diagnosis should always be considered inBD patients with GI findings

C l i


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ConclusionThere are specific symptomatic criteria for diagnosis of BD

Our patient must present with 2 further episodes of oral ulcers withinthis 12mo period + a solid ophthalmologic diagnosis to meet thesecriteria

Findings on biopsy were not typical for IBD

Neither IBD nor BD can be confidently diagnosed based on thesefindings and history

Possibly a drug induced inflammatory response of the bowel(previous Abx use)?

Possibility of an infectious cause for her erythema nodosum?Close monitoring of patient for recurrence of oral lesions orrecurrence of diarrhea with repeat colonoscopy for definitivediagnosis

behcet svsibddr.m.sandhusept21 04

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