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IBD Mimics: Treating the “Refractory IBD Patient” Who Doesn’t Have IBD Mark T. Osterman, MD MSCE Assistant Professor of Medicine University of Pennsylvania.

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Presentation on theme: "IBD Mimics: Treating the “Refractory IBD Patient” Who Doesn’t Have IBD Mark T. Osterman, MD MSCE Assistant Professor of Medicine University of Pennsylvania."— Presentation transcript:

1 IBD Mimics: Treating the “Refractory IBD Patient” Who Doesn’t Have IBD Mark T. Osterman, MD MSCE Assistant Professor of Medicine University of Pennsylvania

2 Case #1

3 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

4 History Meds: none, no NSAIDs FH: no IBD SH: no smoking, EtOH, or drugs PE: mild tenderness in LUQ

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6 Subsequent Course Biopsies –Ulcers and body / antrum: moderate chronic inflammation, H. pylori negative –Duodenum: minimal chronic inflammation Pouchoscopy normal (biopsies normal) SBFT normal

7 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…

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10 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

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12 Henoch-Schönlein Purpura (HSP)

13 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

14 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

15 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

16 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 Chartapisak W et al, Cochrane Database Syst Rev 2009 Davin JC, Clin J Am Soc Nephrol 2011 McCarthy HJ and Tizard EJ, Eur J Pediatr 2010 Weiss PF et al, Pediatrics 2010

17 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

18 HSP and IBD Can mimic IBD –1 st described in 1973 as mimicking CD and acute appendicitis 1 –Can involve TI, stricture, mimic PG 1-5 Can be mimicked by IBD –Palpable purpura, purpura fulminans in UC 6,7 Can coexist with IBD –Case reports of HSP + CD 8,9 6 de Oliveira GT et al, J Crohns Colitis Kempton CL et al, Inflamm Bowel Dis Saulsbury FT and Hart MH, J Pediatr Gastroenterol Nutr Cassater D et al, J Nephrol Yentis I, Br J Radiol Scherbaum WA et al, Clin Investig Ortego-Centeno N et al, Dig Dis Sci Samuel S et al, Dig Dis Sci Yavuz A et al, J Crohns Colitis 2011

19 HSP and Anti-TNF Therapy 1 report of HSP after 6 mo of IFX in UC 1 2 reports of HSP after 7-18 mo of ADA in CD 2,3 2 reports of HSP after Etanercept (psoriasis, RA) 4,5 Rapid resolution after stopping anti-TNF Mechanism unclear 1 Nobile S et al, J Clin Rheumatol Rahman F et al, World J Gastrointest Pharmacol Ther Marques I et al, J Crohns Colitis Lee A et al, J Clin Rheumatol Duffy TN et al, Clin Exp Rheumatol 2006

20 Case #2

21 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

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23 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

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25 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

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27 Subsequent Course 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

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29 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

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32 Subsequent Course Underwent ileostomy reversal Still doing well almost 2y later

33 Idiopathic Myointimal Hyperplasia of the Mesenteric Veins (IMHMV)

34 Epidemiology 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

35 Differential Diagnosis 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

36 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 Blaszyk H et al, Am J Gastroenterol 2005 Sherman H et al, Pathol Res Pract 2006 Abu-Alfa AK et al, Am J Surg Pathol 1996 Kao PC et al, J Clin Gastroenterol 2005


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