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Abstract Number: 804

Vasculitis and Inflammatory Bowel Diseases: A Study of 32 Patients with Both Conditions and Systematic Review of the Literature

Alice Sy1, Natasha Dehghan2, Nader A. Khalidi3, Lillian Barra4, Simon Carette5, David Cuthbertson6, Gary S. Hoffman7, Curry L Koening8, Carol A. Langford9, Carol McAlear10, Paul A. Monach11, Larry W. Moreland12, Philip Seo13, Ulrich Specks14, Steven R. Ytterberg15, Gert Van Assche16, Peter A. Merkel10 and Christian Pagnoux5, 1Medicine Division, London, ON, Canada, 2Rheumatology Division, Vancouver, BC, Canada, 3Internal Medicine/Rheumatology, McMaster University, Hamilton, ON, Canada, 4Rheumatology, St. Joseph's Health Care, London, ON, Canada, 5Division of Rheumatology, University of Toronto, Toronto, ON, Canada, 6Department of Biostatistics, University of South Florida, Tampa, FL, 7Center for Vasculitis Care and Research, Cleveland Clinic Foundation, Cleveland, OH, 8Division of rheumatology, George E. Wahlen Department of Veterans Affairs Medical Center Salt Lake City and University of Utah, University of Utah School of Medicine, Salt Lake City, UT, 9Center for Vasculitis Care and Research, Cleveland Clinic, Cleveland, OH, 10University of Pennsylvania, Philadelphia, PA, 11Rheumatology, Boston University, Boston, MA, 12Medicine, Division of Rheumatology and Clinical Immunology, University of Pittsburgh, Pittsburgh, PA, 13Johns Hopkins Vasculitis Center, Rheumatology Division, Johns Hopkins University, Baltimore, MD, 14Mayo Clinic, Rochester, MN, 15Rheumatology Division, Mayo Clinic, Rochester, MN, 16Mount Sinai Hospital, Toronto, ON, Canada

Meeting: 2014 ACR/ARHP Annual Meeting

Keywords: ANCA, Cutaneous manifestations, inflammatory bowel disease (IBD), takayasu arteritis and vasculitis

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Session Information

Title: Vasculitis

Session Type: Abstract Submissions (ACR)

Background/Purpose:  Small case series suggested that vasculitis and inflammatory bowel disease (IBD; Crohn’s disease [CD] or ulcerative colitis [UC]) can co-occur more commonly than the prevalences of the individual diseases suggest. This study aimed to describe this association through the analysis of a large cohort of carefully studied patients and a systematic literature review.

Methods:  Clinical data was available from patients with both IBD and vasculitis with follow-up >6 months enrolled in the Vasculitis Clinical Research Consortium (VCRC) Longitudinal Studies, followed in Canadian Vasculitis research network (CanVasc) centers, and/or in the University of Toronto’s IBD clinic. Individuals in which ANCA-associated vasculitis (AAV) and IBD were diagnosed within the same 12-month period were excluded because diagnostic misclassification as IBD is common at initial presentation of ileocolitis due to vasculitis. A systematic review of the literature (through 02/2014) for patients with IBD and vasculitis was conducted through a PubMed search. The main characteristics of patients with Takayasu arteritis (TAK) were compared to those patients in the VCRC with TAK but no IBD.

Results:  32 patients (17 VCRC, 15 CanVasc) with vasculitis and IBD satisfying our study criteria were identified. The main group included 13 patients with large vessel vasculitis (LVV): 12 TAK and 1 giant cell arteritis; 8 patients had CD and 5 had UC. Eight patients had AAV (6 granulomatosis with polyangiitis, GPA), 2 eosinophilic granulomatosis with polyangiitis, EGPA, 5 isolated cutaneous vasculitis, and 6 other vasculitides (Kawasaki, IgA nephropathy, polyarteritis nodosa, or central nervous system vasculitis). Patients with LVV and AAV were mostly female (18/21) with a median age of 20 (8 to 52) and 27 (17 to 58) years at diagnosis of IBD and vasculitis, respectively. The diagnosis of IBD preceded that of vasculitis in 12/13 LVV and 8/8 AAV patients, 3/5 with cutaneous vasculitis and 3/6 with other vasculitides. 

   305 other patients with IBD and vasculitis were identified in the literature, distributed among 4 similar subsets: LVV (n=143, 116 female, 69 CD, 74 UC, 132 TAK, 87 with IBD preceding vasculitis), cutaneous vasculitis (n=66, 33 with IBD preceding vasculitis), AAV (n=19, 13 GPA, 3 MPA, 3 EGPA), and other vasculitides (n=77, including IgA vasculitis, retinal vasculitis, CNS vasculitis, polyarteritis nodosa, Kawasaki disease, vasculitic neuropathy).

   As shown in the Table, no differences other than in ethnicity (likely due to center or publication biases) and age at TAK diagnosis were observed between patients with TAK with or without IBD. Mortality was low.

Characteristic

Patients with TAK but no IBD (VCRC)

N = 152

Patients with TAK and IBD from this series

N = 12

Patients with TAK and IBD from this series and literature

N = 144

Female/Male, No.

143/9

11/1

110/24*

Median age at diagnosis of TAK, years (range)

31 (4 to 63)

19 (8 to 52)

23 (8 to 69)

Ethnicity, No. (%)

 

 

 

                Caucasian

127 (84%)

8 (67%)

18 (24.7%)

                Asian

14 (9%)

3 (25%)

48 (65.8%)

Clinical manifestations, No (%)

 

 

n  = 75*

                Claudication of extremities

96 (63%)

8 (67%)

26 (35%)

                Decreased brachial pulse

92 (61%)

9 (75%)

47 (63%)

                Blood pressure difference between arms

70 (46%)

7 (58%)

45 (62%)

                Bruit over subclavian artery or aorta

77 (51%)

5 (42%)

40 (55%)

                Renal hypertension

20 (13%)

4 (33%)

15 (21%)

Median number of ACR criteria met

4 (1 to 6)

4.5 (2 to 6)

4 (2 to 6)

Median duration of follow-up, years

6.25 (0.25 to 31)

4.7 (0.75 to 28)

0.6 (0.1 to 28)

Outcomes, No (%)

 

 

n = 75*

                Remission of vasculitis at last follow-up

108/122 (89%)*

5/8 (63%)

59 (79%)

Stroke

Myocardial infarction       

Deaths

9 (7%)

4 (3%)

0

0

0

0

4 (5%)

1 (1%)

6 (8%)

* Complete information was not available for all patients, especially those from the literature.

Conclusion: These findings highlight the risk in patients with IBD (both CD and UC) to develop vasculitis, especially TAK. Further investigation of patients with both vasculitis and IBD may provide intriguing insights into common underlying mechanisms.


Disclosure:

A. Sy,
None;

N. Dehghan,
None;

N. A. Khalidi,
None;

L. Barra,
None;

S. Carette,
None;

D. Cuthbertson,
None;

G. S. Hoffman,

Roche Pharmaceuticals,

9;

C. L. Koening,
None;

C. A. Langford,
None;

C. McAlear,
None;

P. A. Monach,
None;

L. W. Moreland,
None;

P. Seo,
None;

U. Specks,
None;

S. R. Ytterberg,
None;

G. Van Assche,
None;

P. A. Merkel,

Genentech and Biogen IDEC Inc.,

2,

Bristol-Myers Squibb,

2,

GlaxoSmithKline,

2,

Actelion Pharmaceuticals US,

2,

Actelion Pharmaceuticals US,

5,

Sanofi-Aventis Pharmaceutical,

5,

Chemocentryx,

5;

C. Pagnoux,
None.

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