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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ACR Meeting Abstracts - https://acrabstracts.org/abstract/vasculitis-and-inflammatory-bowel-diseases-a-study-of-32-patients-with-both-conditions-and-systematic-review-of-the-literature/