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