A diagnostic dilemma of Behcets disease a case study

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A diagnostic dilemma of Behcets disease- a case
study

• Monty Sandhu
• PGY-1

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Case Presentation

• ID
• 39 y/o Iranian female with hx SLE
• RFR
• Chronic diarrhea rash to legs
• PMH
• SLE
• joint pain/arthritis, ITP, alopecia, ANA 1320,
  dsDNA 22
• July/04 B/W N plt/ LFT/ Cre. dsDNA 60, N C3/C4/
  ESR/ CRP. U/A sm amt rbc and wbc
• HTN
• Meds
• Plaquenil, prednisone, tylenol, demerol, gravol

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HPI

• 2mo history watery stools
• 18lb wt loss 2 ?appetite
• Denies N/V/abdo pain. Negative travel hx. No
  H/A. No fevers, no cough
• After diarrhea started (?), UTI developed and
  treated with 10d Penicillin
• 1mo ago developed leg rash, followed oral ulcers
  and and conjuctivitis/blepharitis

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Physical exam

• BP 128/90, P105, RR 16, T 38.5, SaO2 99 R/A
• HEENT
• No lymphadenopathy, N thyroid, blepharitis,
  conjuctival injection, oral ulcers to tongue
  and lips. Neg hair pull test
• CVS
• N S1, S2, no EHS/ murmurs, JVP 1-2cm ASA, no rubs
• Resp
• Unremarkable
• GI
• Abdomen soft, non-tender, distended, N BS, no
  peritoneal signs, no HSM/ ascites, no masses
• MSK
• No active joints/ effusion/ ?ROM. N muscle bulk/
  tone/ power
• Derm
• Erythematous, swollen nodules bilaterally to L/E

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Investigations

• Wbc 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 OP, 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 CS- no growth, C.Dif toxin
  negative

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Investigations cont

• Colonoscopy
• Sigmoid biopsy
• Acute chronic inflammatory inflitrates
• No 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 chronic inflammation
• No evidence of arteritis
• Appearance consistent with erythema nodosum

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Course in Hospital

• Started on Solumedrol 20mg IV bid and switched to
  po after 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 Questions

• What are the clinical manifestations and
  diagnostic criteria for Behcets disease?
• How do you differentiate Behcets disease with GI
  involvement from IBD?
• Can Behcets disease and IBD exist as 2 separate
  entities in the same patient?
• Literature review
• Medline search of IBD combined with Behcets
  disease
• limited to english and human data
• mainly case reports and editorial comments found

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Behcets Disease (BD)

• Multisystem, chronic, relapsing vasculitis of
  unknown etiology
• Incidence
• Rare 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, nondeforming
  oligoarthritis)
• Mild renal disease (rare)
• GI ulcerations

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Oral Aphthae
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GI Manifestations of BD

• Main effects are on ileocecal region and colon
• Most common symptoms of intestinal Behcets are
  abdominal pain, N/V, diarrhea blood in stool,
  constipation
• GI complications usually occur 4-6yr after onset
  of oral ulcers
• Intestinal lesions are resistant to medical
  treatment
• Lower incidence in, India and Israel (0-5)

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Intestinal BD

• Oral ulcers
• Ddx- anemia, avitaminosis, viral infn, IBD,
  Reiter syndrome
• Esophagus
• Uncommon site of reported BD
• Ulcers similar to those in mouth
• Stomach
• Gastric mucosa least commonly involved. Apthous
  ulcers
• Duodenum
• Aphthous ulcers resistant to medical therapy
• Pancreas
• Vasculitis may cause pancreatitis

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Intestinal BD cont

• Intestine
• Presentation similar to Crohns disease (CD). In
  pt with intestinal BD, other stigmata of BD may
  appear later
• 2 forms Mucosal inflammation or
  ischemia/infarct
• S. intestine most commonly involved (terminal
  ileum and 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 oval ulcers 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
• Liver
• Most common complication is Budd-Chiari syndrome
• BCS is a common manifestation of BD in Turkey
• Spleen
• Splenomegaly

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Punch out lesion in colon
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Comparing IBD to BD

• BD
• Symptomatic diagnosis
• neutrophil hyperfunction, excess inflammatory
  cytokines (TNF)
• Trt with sulfasalazine, steroids is 1st line for
  GI vasculitis
• Anti TNF monoclonal antibody and thalidomide
  treatment
• HLA B51 phenotype
• Barium studies show preserved haustras, deep
  ulcers

• IBD
• Endoscopic diagnosis
• neutrophil hyperfunction, excess inflammatory
  cytokines (TNF)
• Trt with sulfasalazine, steroids is 1st line
• Anti TNF monoclonal antibody and thalidomide
  treatment
• DR4, DQ4 (CD), B52, DR2 (UC) phenotypes
• longitudinal ulcers, cobblestone appearance,
  stricture formation, fistulas

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Case Report

• History
• 15y/o Arabic male admitted to hosp for W/U
  fatigue, arthralgia, 5mo wt loss and recurring
  nodular skin lesions over shins x2yr
• History of painful oral, penile and perianal
  lesions x2yr
• Recent diagnosis of uveitis
• Physical exam
• Conjunctival erythema, photophobia, aphthous
  ulcer to buccal mucosa, tender lesions to penis
  and perineal area
• Erythema nodosum on lateral aspects of both
  ankles
• Pathergy test negative
• ESR 35, N LTFs CBC, ANA/ anti DNA/ anti ENA/
  RPR negative.
• 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 CS and OP 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
  multiple seripiginous and linear ulcers and
  pseudo polyps.
• Terminal ileum showed cobblestoning with
  seripiginous ulcers
• Biopsy of terminal ileum showed infiltration of
  lamina propria with plasma cells and lymphocytes
• Microgranulomas on biopsy of ascending colon
• Crohns disease

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Case Report discussion

• Discussion
• Several other case reports discussed which showed
  BD with UC
• Controversy as to whether these cases are dual
  diagnoses or simply intestinal BD
• Based on endoscopy and histopathology, authors
  concluded that CD was present
• Based on symptomatic criteria, BD was also
  present
• Case was not intestinal BD b/c imaging was not
  typical for this pattern
• Conclusion
• Several case reports of coexisting IBD not
  otherwise explained by intestinal BD
• Possibility of dual diagnosis should always be
  considered in BD patients with GI findings

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Conclusion

• There are specific symptomatic criteria for
  diagnosis of BD
• Our patient must present with 2 further episodes
  of oral ulcers within this 12mo period a solid
  ophthalmologic diagnosis to meet these criteria
• Findings on biopsy were not typical for IBD
• Neither IBD nor BD can be confidently diagnosed
  based on these findings 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 or recurrence of diarrhea with
  repeat colonoscopy for definitive diagnosis