Transcript Title

IBD Mimics: Treating the
“Refractory IBD Patient”
Who Doesn’t Have IBD
Mark T. Osterman, MD MSCE
Assistant Professor of Medicine
University of Pennsylvania
Case #1
History
• 22M UC Dx’d 3y prior w/ initial response to
steroids but soon after developed toxic
megacolon s/p emergent STC c/b abscess
• Later that year had completion TPC / IPAA
• Did well for 2y except pouchitis once
• Then started having episodic mod-severe
LUQ pain radiating to rest of abdomen a/w
occasional N / V for several mo
• No change in BMs, no bleeding, no fever
History
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Meds: none, no NSAIDs
FH: no IBD
SH: no smoking, EtOH, or drugs
PE: mild tenderness in LUQ
Subsequent Course
• Biopsies
– Ulcers and body / antrum: moderate chronic
inflammation, H. pylori negative
– Duodenum: minimal chronic inflammation
• Pouchoscopy normal (biopsies normal)
• SBFT normal
Subsequent Course
• Started BID PPI without benefit after 1mo
• Then also started having bilateral knee /
ankle pain
• Admitted soon after that when noticed this
rash…
Subsequent Course
• Urinalysis negative for blood / protein,
serum creatinine and BP normal
• Started IV steroids with improvement in all
symptoms within several days
• Sent home on long Prednisone taper
• Doing well on no meds 4y later and has
not developed renal disease
Henoch-Schönlein
Purpura (HSP)
Clinical Characteristics
• Small vessel IgA vasculitis
• Typically children (ages 3-15y, mean 6-7y)
• Rash (leukocytoclastic vasculitis)
– Palpable purpura, petechiae: dependent areas
– Most common initial symptom
• GI symptoms
– Colicky abd pain, N/V, precedes rash up to 1/3
– Small bowel (including TI), stomach, colon
– Can get bleeding / ischemia / perforation
– Intussusception (esp ileoileal) in children
Piram M and Mahr A, Curr Opin Rheumatol 2013
Clinical Characteristics
• Arthralgia / arthritis
– Oligo, large jts (esp LE), transient, migratory
• IgA nephropathy
– Adults and older children
– Hematuria / mild proteinuria to nephrotic
syndrome / GN / interstitial fibrosis / HTN
• Rare symptoms
– Pancreatitis, cholecystitis, protein-losing
enteropathy, ILD, pulm hemorrhage, uveitis,
keratitis, hemorrhagic CVA, orchitis
Piram M and Mahr A, Curr Opin Rheumatol 2013
Diagnosis and Management
• Diagnosis clinical
– Can confirm with skin and / or renal biopsy
with immunofluorescence
• Skin biopsy ideally within 24h of lesion appearance
(leakage of all Ig’s after 24h)
– Urinalysis in all, serum creatinine in adults
– Abdominal U/S, not contrast study, for
ileoileal intussusception in children
• Typically self-limited
– Supportive care
– NSAIDs or acetominophen
McCarthy HJ and Tizard EJ, Eur J Pediatr 2010
Diagnosis and Management
• Corticosteroids
– Mostly hospitalized patients
– Shorten duration of abdominal / joint pain
– Does not prevent renal disease
– Slow taper (at least 1-2 months)
• IgA nephropathy
– More severe in adults (10-30% ESRD at 15y)
– Not preventable with medication
– Corticosteroids, AZA, CsA, plasmapharesis,
IVIG, transplant in severe cases
McCarthy HJ and Tizard EJ, Eur J Pediatr 2010
Weiss PF et al, Pediatrics 2010
Chartapisak W et al, Cochrane Database Syst Rev 2009
Davin JC, Clin J Am Soc Nephrol 2011
Diagnosis and Management
• Recurrence
– 33% if no renal disease, milder / shorter
– Higher with renal disease
• Follow-up: no initial renal disease
– Children: 90-97% renal disease by 2-6 mo
• Urinalysis and BP Q2wk x 2mo, then Qmo x 4mo,
then Q2mo x 6mo, then at well visits after
• Serum creatinine if hematuria / mild proteinuria
– Adults: less clear, ?follow urinalysis / BP /
serum creatinine indefinitely
McCarthy HJ and Tizard EJ, Eur J Pediatr 2010
Davin JC, Clin J Am Soc Nephrol 2011
Narchi H, Arch Dis Child 2005
HSP and IBD
• Can mimic IBD
– 1st described in 1973 as mimicking CD and
acute appendicitis1
– Can involve TI, stricture, mimic PG1-5
• Can be mimicked by IBD
– Palpable purpura, purpura fulminans in UC6,7
• Can coexist with IBD
– Case reports of HSP + CD8,9
1Yentis
I, Br J Radiol 1973
WA et al, Clin Investig 1993
3Ortego-Centeno N et al, Dig Dis Sci 1999
4Samuel S et al, Dig Dis Sci 2011
5Yavuz A et al, J Crohns Colitis 2011
2Scherbaum
6de
Oliveira GT et al, J Crohns Colitis 2013
CL et al, Inflamm Bowel Dis 2001
8Saulsbury FT and Hart MH, J Pediatr Gastroenterol Nutr 2000
9Cassater D et al, J Nephrol 2006
7Kempton
HSP and Anti-TNF Therapy
• 1 report of HSP after 6 mo of IFX in UC1
• 2 reports of HSP after 7-18 mo of ADA in
CD2,3
• 2 reports of HSP after Etanercept
(psoriasis, RA)4,5
• Rapid resolution after stopping anti-TNF
• Mechanism unclear
1Nobile
S et al, J Clin Rheumatol 2009
F et al, World J Gastrointest Pharmacol Ther 2010
3Marques I et al, J Crohns Colitis 2012
4Lee A et al, J Clin Rheumatol 2006
5Duffy TN et al, Clin Exp Rheumatol 2006
2Rahman
Case #2
History
• 62F with PMH of HTN
• Presented to outside hospital with several
months of diarrhea and lower abd pain
• 10 watery BM/d with blood half the time
• Meds: Losartan-HCTZ, no NSAIDs
• FH: no IBD
• SH: no smoking, EtOH, or drugs
• PE: mod tenderness in lower abd L > R
Subsequent Course
• Stool Cx and C.diff negative
• Started Prednisone as well as oral and
topical 5-ASA and sent home
• Biopsies: cryptitis and ulceration but with
capillary thrombi and glandular dropout
possibly c/w ischemic colitis
• 1mo admitted to our hospital with worse
symptoms
• CT: continuous L-sided colitis
Subsequent Course
• Started IV steroids
• Did not improve after 3d
• I did repeat flex sig which showed same
findings of severe continuous ulceration
from anal verge to descending but normal
transverse
Subsequent Course
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CT angiography normal
Angiography normal
Serologic testing for vasculitis negative
Now felt confident that Dx was IMHMV
Made NPO, started TPN and IV abx
Improved somewhat and begged us to let
her go home
Subsequent Course
• Continued NPO and TPN
• 1mo later underwent elective lap-assisted
L colectomy with low rectal anastomosis
and diverting ileostomy
• Intraop, most of rectum to descending
edematous / thick with surrounding
mesenteric edema
Subsequent Course
• Underwent ileostomy reversal
• Still doing well almost 2y later
Idiopathic Myointimal
Hyperplasia of the
Mesenteric Veins
(IMHMV)
Epidemiology
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15 prior cases, all diagnosed postop
Avg age 52y, but 6 were < 40y
13 M, 3 F
Most involved rectosigmoid / sigmoid
Most thought to be IBD >> ischemia
Most underwent surgery within 6 mo
Preop complications: 2 perforation, 1 toxic
megacolon, 1 stricture with obstruction
• Surgery curative in all
Wangensteen KJ and Osterman MT, Submitted
Differential Diagnosis
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IBD
Ischemia
Vasculitis
Enterocolic lymphocytic phlebitis /
mesenteric veno-occlusive disease
– ?Overlap with IMHMV
– Can see myointimal hyperplasia but with
prominent lymphocytic phlebitis
– Can occur in other bowel segments
– Shorter time to surgery (weeks)
Haber MM et al, J Clin Gastroenterol 1993
Flaherty MJ et al, Am J Surg Pathol 1994
Etiology
• Likely not vasculitis
– Little to no inflammation on histology
– Lack of other organ involvement
– No recurrence after surgery
• Hemodynamic theory
– Acquired vascular anomaly (AVM) after trauma
– Saphenous grafts for CABG, AVF for dialysis,
pre-bowel resection trauma (bowel surgery)
– Sigmoid mobile → torsion → AVM → vessel
hyperplasia due to high blood flow / pressure
Abu-Alfa AK et al, Am J Surg Pathol 1996
Kao PC et al, J Clin Gastroenterol 2005
Blaszyk H et al, Am J Gastroenterol 2005
Sherman H et al, Pathol Res Pract 2006