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Nah, Hong, Lee, Oh, Kim, Jeon, Moon, Koh, and Yoo: Effect of Immunosuppressive Agents on Recurrence of Cardiovascular Involvement of Behcet's Disease
Original Article

J Korean Rheum Assoc 2007;14(4):345-353.

Published online: 23 December 2007

DOI: https://doi.org/10.4078/jkra.2007.14.4.345

Effect of Immunosuppressive Agents on Recurrence of Cardiovascular Involvement of Behcet's Disease

Seong-Su Nah, M.D., Seok Chan Hong, M.D., Chang-Keun Lee, M.D., Ji Seon Oh, M.D., Yong Gil Kim, M.D., Chan Hong Jeon, M.D.∗, Hee-Bom Moon, M.D., Eun-Mi Koh, M.D., Bin Yoo, M.D.

Division of Allergy and Rheumatology, Department of Internal Medicine, Seoul, Korea

Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea

Division of Rheumatology, Department of Internal Medicine, Seoul, Korea

∗Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea

Received 26 August 200725 October 2007

Copyright © 2007 The Korean Rheumatism Association

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Objective:

Despite the high risk for disease-related morbidity and mortality in Behcet's disease (BD) with cardiovascular (CV) manifestations, only a few studies concerning BD with CV involvements are available. We conducted study to evaluate the clinical manifestations of CV BD (cardiovascular Behcet's disease) and the clinical outcome according to the different treatment modalities, especially focusing on the immunosuppressive agents.

Methods:

We retrospectively reviewed 1,812 patients diagnosed with BD at tertiary hospital. All patients with vascular involvements were classified into three groups by lesion site. We assessed clinical characteristics, treatments, outcome and recurrence in each group.

Results:

Of 1,812 patients, 79 patients showed CV involvements. Male to female ratio was 65 (82.3%) to 14 (17.7%). Venous involvements occurred in 57 cases (72.2%), arterial lesions in 22 (27.8%), cardiac involvements 16 (20.3%). In clinical manifestation, only hypertension and arthritis were more frequently found in cardiac lesion than in venous lesion (p=0.01, p=0.01,respectively). CV lesions recurred in 16 patients (20.3%), mostly at the same sites as previous involvements. There was no association of recurrence with site of lesion (p=0.49). Recurrent rate was significant different in three medication group (p=0.028). Recurrences were more frequent in patients treated with no immunosuppressive agent and colchicines only or colchicines with prednisolone than in patients treated with additional immunosuppressive agent (p=0.024, R.R, 7.16 (95% CI, 1.55 to 32.99)).

Conclusion:

Recurrence rate was lower in patients with aggressive immunosuppressive treatment. Although most of patients improved, more efforts to decrease the relatively high rate of the recurrence (20.3%) would be needed.

Keywords: Behcet's disease, Cardiovascular diseases, Immunosuppressive agents, Recurrence

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Table 1.
Frequency and types of involvement in cardiovascular Behcet's disease
Venous lesion Arterial lesion Cardiac lesion
Femoral V 28 a. Aneurysm AR only 7
Iliac V 22 Aorta 5 RV thrombi only 4
Popliteal V 20 Pulmonary A 4 TR+RV thrombi 2
Pulmonary thromboembolism 9 Femoral A 2 MR+AR 1
IVC 9 Celiac axis 1 TS+PS+RV thrombi 1
SVC 8 Iliac A 1 AR+MI 1
Jugular V 4 Carotid A 1
Hepatic V 2 b. Occlusion
Subcutaneus thrombophlebitis 2 Popliteal A 6
Subclavian V 2 Iliac A 3
Cerebral sinus thrombosis 2 Femoral A 3
Portal V 1 Cerebral A 2
Renal V 1 Peroneal, tibial AA 1

V: vein, A: artery, AA: arteries, IVC: inferior vena cava, SVC: superior vena cava, AR: aortic valve regurgitation, RV: right ventricle, TR: tricuspid valve regurgitation, MR: mitral valve regurgitation, TS: tricuspid valve stenosis, PS: pulmonary valve stenosis, MI: myocardial infarction

Table 2.
Clinical characteristics of patients with cardiovascular Behcet's disease
Venous (%) Arterial (%) Cardiac (%) Total p-value
Hypertension 7 (12.3) 2 (9.1) 7 (43.8) 12 (15.2) 0.011
Obesity 7 (12.3) 4 (18.2) 1 (6.3) 9 (11.4) NS
Diabetes 2 (3.5) 0 2 (12.5) 4 (5.0) NS
Oral ulcer 57 (100) 21 (100) 16 (100) 79 (100) NS
Genital ulcer 41 (71.9) 13 (59.1) 12 (75.0) 55 (69.6) NS
Skin lesion 53 (93.0) 21 (95.5) 16 (100) 75 (94.9) NS
Erythema nodosum 43 (75.4/81.1) 13 (59.1/61.9) 9 (56.6/56.3) 56 (70.9/74.7) NS
Pseudofolliculitis 14 (24.6/26.4) 9 (36.4/42.9) 9 (56.6/56.3) 24 (30.4/32.0) NS
Papulopustular lesion 3 (5.3/5.7) 3 (18.9/14.3) 2 (12.5/15.4) 7 (8.9/9.3) NS
Eye lesion 16 (28.1) 4 (18.2) 4 (25.0) 20 (25.3) NS
Uveitis 14 (24.6/56.3) 3 (13.6/75.0) 4 (25.0/100) 19 (24.0/95.0) NS
Retinal vasculitis 1 (2.4) 1 (4.5/25.0) 0 1 (1.3/5) NS
Pathergy test 15/26 (57.7) 7/10 (70.0) 4/8 (50.0) 26/44 (59.1) NS
Arthritis 7 (12.3) 5 (18.2) 7 (43.8) 18 (22.8) 0.022
Enthesitis 0 1 (4.5) 0 1 (1.3) NS
Epididymitis 4 (7.0) 1 (4.5) 1 (6.3) 5 (6.3) NS
Fever 8 (14.0) 1 (4.5) 3 (18.8) 9 (12.3) NS
Central nerve lesion 3 (5.3) 1 (4.5) 1 (6.3) 5 (6.3) NS
Gastrointestinal lesion 3 (5.3) 1 (4.5) 1 (6.3) 4 (5.0) NS
Total 57 22 16
Table 3.
Analysis of recurred cardiovascular Behcet's disease
Site of lesion (case) Medication (case)
Venous Arterial Cardiac P value None C, P, CP Additional immunosuppressant P value Relative risk (95% CI) P value
Recurrence (%) 10 (17.5) 6 (27.3) 4 (25.0) 9 (32.1) 10 (32.3) 1 (4.0)
Recurrence free (%) 47 (82.5) 16 (72.7) 12 (75.0) 0.494 19 (67.9) 31 (67.7) 25 (96.0) 0.028 7.16 (1.6 to 33)∗ 0.024∗
Total 57 22 16 28 41 26 8.36 (1.8 to 39.6)∗∗ 0.036∗∗

∗None + C, P, CP vs. additional immuno-suppressant, ∗∗None vs. additional immuno-suppressant C: colchicines, P: prednisolone, CP: colchicine and prednisolone

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