All-cause and cause-specific mortality in patients with Behçet disease versus the general population.

BACKGROUND: The comparative risk of cause-specific mortality in patients with Behçet disease (BD) vs. the general population is not known. OBJECTIVES: To compare the risk of all-cause and cause-specific mortality in patients with BD vs. the general population. METHODS: Using data from the Korea National Health Insurance Service database for the period 2002-20, we conducted a cohort study comparing patients with BD with the general population, matched according to age and sex (1 : 4 ratio). We used Cox proportional hazard models to estimate hazard ratios (HRs) and 95% confidence intervals (CIs) for all-cause and cause-specific mortality. Subgroup analyses by age and sex were done. RESULTS: We included 24 669 patients with BD and 98 676 age- and sex-matched controls [mean (SD) age 40.5 (12.9) years; 34% male]. During a mean follow-up of 11.9 years, the incidence rate (IR) of death per 100 person-years was 0.36 in patients with BD and 0.29 in controls [hazard ratio (HR) 1.28, 95% confidence interval (CI) 1.20-1.38]. The risk of mortality was highest in the first year after BD diagnosis (HR 2.66, 95% CI 2.09-3.40). Patients with BD died more often in this period as a result of malignancy (HR 1.96, 95% CI 1.30-2.98); cardiovascular (HR 2.68, 95% CI 1.45-4.97), gastrointestinal (HR 3.50, 95% CI 1.35-9.07) and respiratory disease (HR 5.00, 95% CI 1.34-18.62); and infection (HR 3.33, 95% CI 1.02-10.92). Mortality as a result of neurological (HR 1.58, 95% CI 1.06-2.35) or genitourinary disease (HR 2.20, 95% CI 1.43-3.37) was also more common in patients with BD during the overall follow-up. Subgroup analyses showed consistent results. The risk of cardiovascular mortality vs. the general population was higher in younger patients (P = 0.006) and the risk of gastrointestinal mortality was increased in women vs. men (P = 0.04). CONCLUSIONS: This population-based cohort study revealed that the first year after diagnosis is the highest risk period for excess mortality in people with BD. The mortality burden in BD derives from a wide spectrum of organ involvement and should serve as a warning to clinicians about the systemic nature of the disease.

Behçet disease (BD) is a multisystem vasculitis (inflammation of the blood vessels) of unknown origin that commonly results in oral and genital ulcers, uveitis (eye inflammation) and skin lesions. BD is most prevalent in people from the Mediterranean to East Asia, affecting 0.4% of people in this area. Most lesions go away with time, but more severe forms that involve the cardiovascular and neurological systems can lead to death. It is estimated that people with BD have 1.4 times the risk of dying than the general population. Using large insurance databases in Korea, we investigated the risk of death in people with BD versus age- and sex-matched controls (i.e. people without the disease) from the general population. We found that patients with BD had a 28% greater risk of death than controls over 11.9 years of follow-up, with the highest risk being in first year after diagnosis. Top causes of death in people with BD included cancer, and cardiovascular, gastrointestinal, neurological, genitourinary, respiratory and infectious disease. Further analyses of the data showed that the risk of death in BD is affected by age and sex. In particular, younger patients were more susceptible to death as a result of cardiovascular disease and women were more susceptible to dying of gastrointestinal disease. Our study suggests that there could be an increased risk of death within the first year of being diagnosed with BD and highlights how BD is a systemic disease (i.e. the involvement of any internal organ system could lead to an increase in mortality). Finally, there were unique patterns of cause-specific deaths across subgroups of people with BD.

Autologous Hematopoietic Stem Cell Transplantation for Behçet's Disease: A Retrospective Survey of Patients Treated in Europe, on Behalf of the Autoimmune Diseases Working Party of the European Society for Blood and Marrow Transplantation.

Background: Behçet's Disease (BD) is an autoimmune disease mostly presenting with recurrent oral and genital aphthosis, and uveitis. Patients are rarely refractory to immunosuppressive treatments. Autologous hematopoietic stem cell transplantation (aHSCT) is a standard of care in other autoimmune diseases. Some patients with BD have been treated with aHSCT based on compassionate use. Objectives: Evaluate the outcome of aHSCT in adult patients with BD treated in member centers of the European Society for Blood and Marrow Transplantation (EBMT). Methods: Adults who received aHSCT primarily for BD were identified retrospectively in the EBMT registry and/or in published literature. Data were extracted from either medical records of the patient or from publications. Results: Eight out of 9 cases reported to the registry and extracted data of 2 further patients from literature were analyzed. Four were female, median age at onset of BD was 24y (range 9-50). Median age at aHSCT was 32y (27-51). Patients had received median 4 (2-11) previous lines of therapy (89% corticosteroids, 50% methotrexate, anti-TNFα therapy or cyclophosphamide). All patients had active disease before mobilization. Conditioning regimen was heterogeneous. Median follow-up was 48 months (range 6-240). No treatment-related mortality was reported. This procedure induced complete remission (CR) in 80%, partial remission in 10% and lack of response in 10% of the patients. Relapse rate was 30% (2 relapses in patients in CR and 1 relapse in the patient in PR) with panuveitis (n=1), aphthosis (n=2) and arthralgia (n=1). Six patients were in CR. No late complications were reported. Conclusion: aHSCT has an acceptable safety profile and represents a feasible and relatively effective procedure in severe and conventional treatment-resistant cases of BD and has the potential to stabilize BD in patients with life-threatening involvements.

Surgical Experience of Behcet's Disease Involving the Peripheral Artery.

BACKGROUND: Behcet's disease with arterial involvement shows rapid poor progression compared with Behcet's disease without arterial involvement, such that it may be thought of as a different disease. With arterial involvement, high rates of postoperative recurrence and anastomotic complications are observed. METHODS: The medical records of 1,361 patients diagnosed with Behcet's disease who visited our hospital from January 2007 to December 2019 were reviewed. Overall, 118 patients with blood vessel involvement were considered. Those with only vein involvement, intracerebral arterial involvement, and coronary arterial involvement were excluded. The 8 patients who underwent surgical treatments and the 9 patients who underwent conservative treatments were included and reported in this study. RESULTS: The mean age and mean follow-up duration of the 17 patients with peripheral arterial involvement were 49.82 ± 13.24 years and 89.85 ± 52.70 months, respectively. In all cases where the operations were performed, the first operation was an emergency operation for rupture of the aneurysm, impending sign of rupturing, or acute ischemia. Eight patients received a total of 18 operations. The average number of operations per patient and the reoperation rate were 2.25 ± 0.89 times and 75%, respectively. The 5-year and 10-year survival rates of the patients who underwent surgery were 75.0% and 30.0%, respectively. When arterial involvement occurs in the form of an aneurysm, the hazard ratio for death is 9.644 (P = 0.040, confidence interval 1.11-83.74). CONCLUSIONS: When the artery invades in the form of an aneurysm, the mortality rate is higher regardless of surgery. The main cause of mortality is complications that occur in the anastomosis sites after the operation. Postoperative short-term and regular imaging of the anastomosis area can be beneficial to detect such complications early to decrease the frequency of emergency surgeries, which will help reduce mortality.

Coronary involvement in behçet's disease: what are its risks and prognosis? (rare cases and literature review)

Abstract Objective: In our clinic, we aimed to investigate the effect of preoperative risk factors and postoperative complications on reoperation and mortality in cases with Behçet's disease which presents very rare coronary artery involvement. Methods: Thirteen patients with Behçet's Disease who had undergone coronary artery bypass grafting in our center between 2003 and 2015 were analyzed. We evaluated the clinical and laboratory findings, complications and mortality rates of our patients in light of the literature. Results: The mean age was 38.5 (30-55; 3 women). The mean time from onset of Behçet's disease to coronary artery disease was 4,7 (3-11) years. Fifty-four percent of the patients were asymptomatic. Coronary artery disease of these was exposed while peripheral vascular surgery was planned due to complications of Behçet's disease. Symptomatic patients presented angina pectoris (31%), acute coronary syndrome (8%) and arrhythmia (8%). In coronary pathology of patients, distal type obstruction (31%), aneurysm and pseudoaneurysm (31%), proximal segment thrombus (15%), chronic type stenosis and occlusions (31%) were present. Early mortality (15%) was due to acute myocardial infarction while the late mortality (15%) was due to cerebral and gastrointestinal bleeding. Reoperation was due to bleeding in one case on the 1st postoperative day and due to acute pulmonary embolism in another case in the 3rdpostoperative year. Conclusion: In Behçet's disease, coronary artery bypass grafting is a procedure with high mortality, especially in the acute period. The on-pump surgery technique in these cases can be safely performed for multiple bypasses and in patients above 40 years old.

Coronary Involvement in Behçet's Disease: what are its Risks and Prognosis? (Rare Cases and Literature Review).

OBJECTIVE: In our clinic, we aimed to investigate the effect of preoperative risk factors and postoperative complications on reoperation and mortality in cases with Behçet's disease which presents very rare coronary artery involvement. METHODS: Thirteen patients with Behçet's Disease who had undergone coronary artery bypass grafting in our center between 2003 and 2015 were analyzed. We evaluated the clinical and laboratory findings, complications and mortality rates of our patients in light of the literature. RESULTS: The mean age was 38.5 (30-55; 3 women). The mean time from onset of Behçet's disease to coronary artery disease was 4,7 (3-11) years. Fifty-four percent of the patients were asymptomatic. Coronary artery disease of these was exposed while peripheral vascular surgery was planned due to complications of Behçet's disease. Symptomatic patients presented angina pectoris (31%), acute coronary syndrome (8%) and arrhythmia (8%). In coronary pathology of patients, distal type obstruction (31%), aneurysm and pseudoaneurysm (31%), proximal segment thrombus (15%), chronic type stenosis and occlusions (31%) were present. Early mortality (15%) was due to acute myocardial infarction while the late mortality (15%) was due to cerebral and gastrointestinal bleeding. Reoperation was due to bleeding in one case on the 1st postoperative day and due to acute pulmonary embolism in another case in the 3rdpostoperative year. CONCLUSION: In Behçet's disease, coronary artery bypass grafting is a procedure with high mortality, especially in the acute period. The on-pump surgery technique in these cases can be safely performed for multiple bypasses and in patients above 40 years old.

Predictors of paravalvular aortic regurgitation after surgery for Behcet's disease-related severe aortic regurgitation.

BACKGROUND: Behcet's disease (BD)-related aortic regurgitation (AR) is known to be associated with paravalvular leakage (PVL) after successful aortic valve (AV) surgery. This study aimed to determine predictors of PVL after successful AV surgery in BD patients. We retrospectively collected data of 35 patients (42.1 ± 9.1 years, 27 men) who underwent surgery for severe BD-related AR at two tertiary centers. The diagnosis was established based on echocardiographic, surgical, and/or pathological findings in conjunction with the International Study Group criteria for BD. A total of 76 cases of AV surgery in 35 patients were analyzed. RESULTS: A median follow-up duration was 8.0 years (interquartile range, 5.4-14.3 years). PVL developed in 18 patients (51.4%) within 2 years after the first surgery. Six patients who met the diagnostic criteria for BD did not develop PVL, in whom 5 patients took immunosuppressive therapy (IST). However, 4 of 9 patients (44.4%) who did not meet the diagnostic criteria developed PVL, in whom four (44.4%) patients took IST. On multivariable analysis, postoperative IST and concomitant aortic root replacement (ARR) were two independent predictors for less PVL development (HR 0.38, 95% CI 0.17-0.89, p = 0.025 for postoperative IST; HR 0.17, 95% CI 0.08-0.36, p < 0.001 for concomitant ARR). Preoperative IST use did not determine PVL development (p = 0.75). CONCLUSIONS: Postoperative, but not preoperative, IST and concomitant ARR were independent predictors of less development of PVL. Special attention is required for early diagnosis BD-related AR, especially in patients not satisfying the current diagnostic criteria.

Surgical Treatment of Pulmonary Complications in Behçet's Syndrome.

We described disease characteristics, management and outcome of a group of Behçet's syndrome (BS) patients who underwent pulmonary lobectomy, segmentectomy or various pleura interventions for complications due to pulmonary artery or descending aorta involvement. There were 9 patients with BS (8 M/1 F; mean age: 24.8 ±â€¯7.5 and mean disease duration: 4.3 ±â€¯3.8 years) who underwent lung surgery from 2000 to 2017. Their medical files including operation details and radiological and pathological studies were reviewed retrospectively. Lobectomy was done in 6 patients, decortications and pleura interventions in the remaining 3. The reason for the surgical procedures were giant pulmonary arterial aneurysms refractory to the medical treatment (n = 4), pneumothorax due to large cavities (n = 2), pleural effusions refractory to the medical treatment (n = 1), a bronchopleural fistula after embolization (n = 1) and bronchiectasis (n = 1). All patients received medical treatment with cyclophosphamide and corticosteroids before the surgical interventions. The histological examination in patients with pseudo-aneurysms showed destruction of medial elastic fibers, inflammatory cell infiltration of adventitial tissues and penetration into the adjacent bronchi with adherent thrombi. Two patients died: one due to Budd-Chiari syndrome 1 year after the surgery, another because of a massive hemoptysis 3 months after lobectomy. The remaining 7 patients are still alive with a median follow-up of 8 years (inter-quartile range [IQR]: 4-11). Lobectomies in BS patients with giant pulmonary aneurysms refractory to medical treatment can be done with favorable outcome. Also, complications such as large cavities causing pneumothorax, refractory pleural effusions, bronchiectasis, and embolization complications can be managed effectively with surgical interventions.

Atrium Advanta V12 Large Diameter Stent-Graft Applications for Infrarenal Abdominal Aortic Pseudoaneurysms (due to Behcet Disease): Safety and Efficacy.

BACKGROUND: The aim of the study was to determine the safety and effectiveness of Atrium Advanta V12 large diameter stent-graft applications for infrarenal abdominal aortic pseudoaneurysms (due to Behcet disease [BD]). METHODS: Data of Advanta V12™ (Atrium Europe B.V, Mijdrecht, the Netherlands) applied 12 female patients (mean age 30.5 ± 6.3, range 26-44) with infrarenal abdominal aortic pseudoaneurysms were analyzed retrospectively. All Advanta V12 large diameter stent grafts were implemented from right or left sided 12F femoral sheaths. Stent grafts with 12-16 mm in size and 29-61 mm in length were utilized. Technical success rate, procedure-related mortality and morbidity, and primary patency rate at 4 years were evaluated. RESULTS: Technical success rate was 100%. Neither procedure-related mortality nor morbidity was determined. The mean aortic diameter was 14.0 ± 0.8 mm for pseudoaneurysmatic abdominal aortas. The mean follow-up period was 46.5 ± 40.3 months (range 18-75). During follow-ups, only one recurrent aneurysm has evolved at the stenting site due to patients' withdrawal of immunosuppressive treatment. The advent of a new aneurysm proximal or distal to the stent-graft region or at the femoral access localization was not observed. There were no stent occlusions. Primary patency rate at 4 years was 100%. Complete aneurysm exclusion was achieved 100% at 48 months. CONCLUSIONS: The use of Advanta V12 large diameter stent grafts for infrarenal abdominal aortic pseudoaneurysms (due to BD), especially in female patients with small aortic diameter, is safe and efficient. Primary patency rate of the stent grafts at 4 years is excellent.

The value of endografts in the surgical management of arterial lesions secondary to Behçet disease.

BACKGROUND: This study summarizes our experience in the surgical management of arterial lesions secondary to Behçet disease (BD) and assesses the value of endografts. METHODS: Data from BD patients with arterial lesions managed surgically in our center from January 1998 to December 2015 were studied retrospectively. Surgical procedures, graft selection, graft-related complications, and retreatments were analyzed. RESULTS: We recruited 33 patients (29 men and 4 women; male-to-female ratio, 7.25:1) with an average age of 36.7 years (range, 25-51 years). The arterial lesions included 27 aneurysms in 24 patients and nine stenotic or occlusive lesions in nine patients. Immunosuppressive therapy was administered routinely preoperatively and postoperatively as recommended. Altogether, 15 great saphenous veins (GSVs), 8 synthetic grafts, and 13 endografts were used in 36 primary procedures. The mean follow-up duration was 3.8 ± 2.9 years. Graft-related pseudoaneurysm was seen in three GSVs (20%) and in three synthetic grafts (38%) at the anastomosis, but not in endograft implantations (log-rank, P = .171). Graft occlusions were observed in 1 GSV (7%), 2 synthetic (25%), and 2 endografts (15%; log-rank, P = .881). Graft infection occurred in one synthetic graft (13%) and in one endograft (8%) but not in the GSVs (log-rank, P = .689). Graft-related artery rupture occurred in only one endograft (8%). Two patients died, giving a mortality rate of 6.1%. CONCLUSIONS: In the surgical management of arterial lesions secondary to BD, endografts were superior to GSV and synthetic grafts in decreasing anastomotic pseudoaneurysm. However, improvements are needed to enhance the long-term patency and reduce infections.

Outcomes of surgery for patients with Behcet's disease causing aortic pseudoaneurysm: a shift from open surgery to endovascular repair.

OBJECTIVES: Behcet's disease is a form of systematic vasculitis that affects vessels of various sizes. Aortic pseudoaneurysm is one of the most important causes of death among patients with Behcet's disease due to its high risk of rupture and associated mortality. Our study aimed to investigate the outcomes of Behcet's disease patients with aortic pseudoaneurysms undergoing open surgery and endovascular aortic repair. METHODS: From January 2003 to September 2014, ten consecutive patients undergoing surgery for aortic pseudoaneurysm met the diagnostic criteria for Behcet's disease. Endovascular repair was the preferred modality and open surgery was performed as an alternative. Systemic immunosuppressive medication was administered after Behcet's disease was definitively diagnosed. RESULTS: Eight patients initially underwent endovascular repair and two patients initially underwent open surgery. The overall success rate was 90% and the only failed case involved the use of the chimney technique to reach a suprarenal location. The median follow-up duration was 23 months. There were 7 recurrences in 5 patients. The median interval between operation and recurrence was 13 months. No significant risk factors for recurrence were identified, but a difference in recurrence between treatment and non-treatment with preoperative immunosuppressive medication preoperatively was notable. Four aneurysm-related deaths occurred within the follow-up period. The overall 1-year, 3-year and 5-year survival rates were 80%, 64% and 48%, respectively. CONCLUSIONS: Both open surgery and endovascular repair are safe and effective for treating aortic pseudoaneurysm in Behcet's disease patients. The results from our retrospective study indicated that immunosuppressive medication was essential to defer the occurrence and development of recurrent aneurysms.

Outcomes of surgery for patients with Behcet's disease causing aortic pseudoaneurysm: a shift from open surgery to endovascular repair

OBJECTIVES: Behcet’s disease is a form of systematic vasculitis that affects vessels of various sizes. Aortic pseudoaneurysm is one of the most important causes of death among patients with Behcet’s disease due to its high risk of rupture and associated mortality. Our study aimed to investigate the outcomes of Behcet’s disease patients with aortic pseudoaneurysms undergoing open surgery and endovascular aortic repair. METHODS: From January 2003 to September 2014, ten consecutive patients undergoing surgery for aortic pseudoaneurysm met the diagnostic criteria for Behcet’s disease. Endovascular repair was the preferred modality and open surgery was performed as an alternative. Systemic immunosuppressive medication was administered after Behcet’s disease was definitively diagnosed. RESULTS: Eight patients initially underwent endovascular repair and two patients initially underwent open surgery. The overall success rate was 90% and the only failed case involved the use of the chimney technique to reach a suprarenal location. The median follow-up duration was 23 months. There were 7 recurrences in 5 patients. The median interval between operation and recurrence was 13 months. No significant risk factors for recurrence were identified, but a difference in recurrence between treatment and non-treatment with preoperative immunosuppressive medication preoperatively was notable. Four aneurysm-related deaths occurred within the follow-up period. The overall 1-year, 3-year and 5-year survival rates were 80%, 64% and 48%, respectively. CONCLUSIONS: Both open surgery and endovascular repair are safe and effective for treating aortic pseudoaneurysm in Behcet’s disease patients. The results from our retrospective study indicated that immunosuppressive medication was essential to defer the occurrence and development of recurrent aneurysms.

Preoperative immunosuppressive therapy reduces paravalvular leakage after aortic valve surgery in patients with aortic regurgitation attributable to Behçet's disease.

OBJECTIVES: Severe aortic regurgitation (AR) attributable to Behçet's disease (BD) is a rare but challenging problem in light of the surgical treatment notoriously complicated with paravalvular leakage (PVL) and high mortality. This study aims to test whether immunosuppressive therapy is effective in reducing the complication of the operation and improve the prognosis. METHODS: In a retrospective cohort of 644 hospitalised BD patients who were diagnosed and followed up between January 1998 and September 2014, 18 patients (2.8%) with severe AR due to BD were identified and their medical records were analysed. RESULTS: Among the 18 patients, 15 patients underwent the valve surgery and PVL developed in 7 (47%) at a median interval of 3.5 months. In the median follow-up of 32.5 months from first operations, patients with PVL had a significantly higher incidence of repeat surgery and death (71% vs. 0%, p=0.007). They less likely received preoperative immunotherapy (14% vs. 100%, p=0.001), had a lower cumulative dosage of cyclophosphamide before first operations (2.1±5.7g vs. 13.0±6.4g, p=0.004) and a higher preoperative erythrocyte sedimentation rate (44.4±20.7mm/first hour vs. 25.0±12.1mm/first hour, p=0.04) compared with those without developing PVL. Multivariate analysis showed preoperative immuno-suppressive therapy (hazard ratio 18.58; 95% confidence interval, 2.134-161.81; p=0.008) was an independent factor associated with the absence of PVL. The 5-year PVL-free survival rates were significantly higher in patients receiving preoperative immunotherapy (p=0.0004). CONCLUSIONS: In patients with severe AR due to BD, preoperative immunosuppressive therapy, especially cyclophosphamide in conjunction with glucocorticoid, could reduce PVL after the corrective surgery and improve the outcomes.

Efficacy of anti-TNF alpha in severe and/or refractory Behçet's disease: Multicenter study of 124 patients.

OBJECTIVE: To report the efficacy and safety of anti-TNF agents in patients with severe and/or refractory manifestations of Behçet's disease (BD). METHODS: We performed a multicenter study of main characteristics and outcomes of anti-TNF alpha treatments [mainly infliximab (62%), and adalimumab (30%)] in 124 BD patients [48% of men; median age of 33.5 (28-40) years]. RESULTS: Overall response (i.e. complete and partial) rate was 90.4%. Clinical responses were observed in 96.3%, 88%, 70%, 77.8%, 92.3% and 66.7% of patients with severe and/or refractory ocular, mucocutaneous, joint, gastro-intestinal manifestations, central nervous system manifestations and cardiovascular manifestations, respectively. No significant difference was found with respect to the efficacy of anti-TNF used as monotherapy or in association with an immunosuppressive agent. The incidence of BD flares/patient/year was significantly lower during anti-TNF treatment (0.2 ± 0.5 vs 1.7 ± 2.4 before the use of anti-TNF, p < 0.0001). The prednisone dose was significantly reduced at 6 and 12 months (p < 0.0001). In multivariate analysis, retinal vasculitis was negatively associated with complete response to anti-TNF (OR = 0.33 [0.12-0.89]; p = 0.03). The efficacy and relapse free survival were similar regardless of the type of anti-TNF agent used. After a median follow-up of 21 [7-36] months, side effects were reported in 28% of patients, including infections (16.3%) and hypersensitivity reactions (4.1%). Serious adverse events were reported in 13% of cases. CONCLUSION: Anti-TNF alpha therapy is efficient in all severe and refractory BD manifestations. Efficacy appears to be similar regardless of the anti-TNF agent used (infliximab or adalimumab).

What the Cardiologist Should Know About Cardiac Involvement in Behçet Disease.

Behçet disease (BD) is a chronic multisystem inflammatory vasculitis affecting mainly young adults and is characterized by a remitting-relapsing course. In North America, the prevalence is 5.2 per 100,000 population. It is believed that cardiac involvement is one of the most severe complications in patients with BD despite its sporadic occurrence, being greatly correlated with mortality.

Prognosis of arterial aneurysm after surgery in patients with Behçet's disease.

AIM: Despite improvements in therapeutic modalities, the treatment of arterial aneurysms complicating Behçet's disease (BD) is still challenging. This study examined the long-term prognosis after surgery for arterial aneurysms in BD. METHODS: This study included 9 patients with BD (8 men and 1 woman) who underwent surgery for arterial aneurysms between 1989 and 2008. The outcomes after the surgical intervention were assessed, including procedure-related complications and survival. RESULTS: The initial surgical procedures were performed for aortic or iliac aneurysms in 5 patients and for lower-extremity aneurysms in 4 patients. There was no operative mortality. The mean follow-up period was 135±69 months, ranging from 53 to 259 months. Patients with aortic or iliac aneurysms underwent graft interposition with Dacron prostheses. Their postoperative courses were uneventful, and all patients were alive during the follow-up with no procedure-related complications. Those treated for lower-extremity aneurysms tended to show perioperative and postoperative complications, including aneurysmal degeneration of the autogenous vein graft in 2 patients. One patient who initially underwent surgery for a popliteal artery aneurysm died due to the rupture of a dissecting aortic aneurysm after serial surgical interventions for multiple aneurysms. Concomitant aortic or iliac aneurysms in 2 patients were followed up without any change in size under medical treatment using colchicine and corticosteroids. CONCLUSION: Although we cannot draw a firm conclusion because of the small number of cases in the present series, graft interposition can lead to a favorable prognosis in BD patients with aortic or iliac aneurysms, whereas surgical treatment of BD-related lower-extremity aneurysms is frequently associated with short- and long-term postoperative complications. Immunosuppressive therapy might possibly improve treatment outcomes.

Renal amyloidosis in Behçet's disease: clinicopathologic features of 8 cases.

PURPOSE: Behçet's disease (BD) is a rare inflammatory disorder with variable articular and systemic manifestations. Amyloidosis is an uncommon complication of BD. The aim of this study is to present clinicopathological characteristics and outcome of 8 patients with renal amyloidosis secondary to BD. METHODS: A total of 220 cases of secondary-type renal amyloidosis diagnosed over 26-year period (1981-2007) in a single institution via needle biopsy were reviewed retrospectively, and accompanying BD was found in ten of them. Clinical and follow-up information of the patients was gathered from hospital records and computer-based data system. Two cases were excluded from the study due to another concomitant preamyloidotic condition. Biopsy slides of remaining 8 cases were culled from the archives and re-examined. Extent and pattern of renal amyloid deposition were explored. Amyloid typing was accomplished via immunohistochemistry. Factors acting on outcome and renal survival were explored. RESULTS: Patients were all male (age range 27-56). Duration of BD before diagnosis of amyloidosis varied from 24 to 192 months, and mean follow-up period was 89 months. Nephrotic syndrome was the most common presentation. Immunohistochemical typing revealed AA-amyloid in all cases. Two cases showed glomerular-dominant, one case vascular-dominant amyloid deposition, and rest were codominant glomerular and vascular, except one that was indeterminate. Patients were treated with steroids and/or different immunosuppressives, three progressed to end-stage kidney failure. CONCLUSION: Behçet's disease is one of the rare diseases that lead to AA amyloidosis. Morphology of renal amyloidosis due to BD has diversities in terms of differential amyloid deposition in renal compartments. Patients follow variable clinical courses accordingly.

Long-term clinical outcomes and risk factors for the occurrence of post-operative complications after cardiovascular surgery in patients with Behçet's disease.

OBJECTIVES: Cardiovascular surgery in patients with Behçet's disease (BD) frequently leads to postoperative complications such as anastomotic leakage, occlusion or pseudoaneurysm. We evaluated the clinical outcomes and related risk factors of postoperative complications in BD patients undergoing cardiovascular surgeries, as well as the long-term efficiency of postoperative immunosuppressive treatment. METHODS: Forty-one patients with BD who had undergone cardiovascular surgery between 1990 and 2009 were studied. We evaluated the patients' clinical data, postoperative complications, and survival rate. Risk factors related to the occurrence of postoperative complications were identified by univariate analysis using the Kaplan-Meier method with the log-rank test and multivariate analysis using the Cox proportional hazards regression model. RESULTS: Fifty-nine operations were performed in 41 patients. During the mean follow-up period of 65.3±48.1 months, complications such as paravalvular leakage, dehiscence, fistula, graft occlusion, or pseudoaneurysm occurred in 29 operations (49.2%). The cumulative occurrence rate of postoperative complication was 10.2% at three months, 32.8% at 12 months, and 43.8% at 24 months. Upon univariate analysis, young age, high Creactive protein levels, lack of postoperative immunosuppression, and short disease duration were identified as significant factors responsible for the occurrence of postoperative complications. In multivariate analysis, postoperative immunosuppression was found to independently lower the risk of complications. The 5-year survival rate was significantly higher in patients with postoperative immunosup immunosuppression than in those without (84.5% vs. 45.0%, p=0.011). CONCLUSIONS: The present study suggests that postoperative immunosuppressive therapy after cardiovascular surgeries in BD patients is important for reducing the development of serious postoperative complications.

Aortic regurgitation caused by Behçet's disease: surgical experience during an 11-year period.

BACKGROUND AND AIM OF THE STUDY: Aortic regurgitation (AR) caused by Behçet's disease is a rare clinical condition and surgical experience is limited. Many patients undergoing simple aortic valve replacement (AVR) required a second or third operation as a result of prosthetic valve dehiscence. We analyze our experience with surgical management for AR in Behçet's disease during an 11-year period. METHODS: From April 1997 through December 2008, 10 male patients (mean age, 38.4 ± 6.9 years) with AR caused by Behçet's disease underwent surgical treatment. The primary procedures were AVR in seven cases and aortic root replacement in three (two Bentall, one Cabrol). The duration of follow-up was 5.3 ± 3.6 years. RESULTS: In the seven patients with simple AVR, valve dehiscence occurred in six after an interval of 2.9 ± 1.7 months, necessitating 11 reoperations (four redo AVRs, one redo redo-AVR, four aortic root replacements, and two heart transplants) with one early death. In aortic root replacement patients, two Bentall patients survived and required no reintervention, and the Cabrol patient died from electromechanical uncoupling one year postoperatively. Of the seven survivors, the definite procedure was aortic root replacement in four cases, heart transplantation in two, and AVR in one. CONCLUSIONS: The rate of valve dehiscence was high after isolated AVR in patients with AR caused by Behçet's disease. At the first operation, aortic root replacement should be the procedure of choice, preferably with a homograft. Heart transplantation might be a feasible alternative to aortic root replacement in selected patients.

Mortality in Behçet's disease.

OBJECTIVE: To report the long-term mortality in patients with Behçet's disease (BD). METHODS: A cohort of 817 patients fulfilling the international criteria for BD from a single center in France were analyzed for causes of death, the standardized mortality ratio (SMR), and the factors associated with mortality. RESULTS: Among the 817 patients with BD, 41 (5%) died after a median followup of 7.7 years, of whom 95.1% were male. The mean ± SD age at death was 34.8 ± 11.9 years. Main causes of death included major vessel disease (mainly, arterial aneurysm and Budd-Chiari syndrome) (43.9%), cancer and malignant hemopathy (14.6%), central nervous system involvement (12.2%), and sepsis (12.2%). The mortality rate at 1 year and 5 years was 1.2% and 3.3%, respectively. There was an increased mortality among patients ages 15-24 years (SMR 2.99, 95% confidence interval [95% CI] 1.54-5.39) and those ages 25-34 years (SMR 2.90, 95% CI 1.80-4.49) as compared with age-and sex-matched healthy controls. The mortality decreased in patients older than age 35 years (SMR 1.23, 95% CI 0.75-1.92). In multivariate analyses, male sex (hazard ratio [HR] 4.94, 95% CI 1.53-16.43), arterial involvement (HR 2.51, 95% CI 1.07-5.90), and a high number of BD flares (HR 2.37, 95% CI 1.09-5.14) were independently associated with the risk of mortality. CONCLUSION: The overall mortality in our BD cohort was 5% after a median followup of 7.7 years. Male sex, arterial involvement, and the number of flares were associated with mortality in BD.

Behçet's disease as a frequently unrecognized cause of aortic regurgitation: suggestive and misleading echocardiography findings.

BACKGROUND: Behçet's disease (BD) is a systemic vasculitis that can be a possible cause of aortic regurgitation (AR). When aortic valve replacement is performed in patients with BD, unless preoperative immunosuppressive therapy and special operative techniques are used, the postoperative course is usually complicated by valve dehiscence, necessitating another operation. The preoperative diagnosis of BD is challenging because of its protean manifestations, but an early diagnosis is associated with a more favorable prognosis. METHODS: Between 1990 and 2008, 15 patients with final diagnoses of BD underwent aortic valve replacement. Medical records and echocardiographic findings were retrospectively analyzed. RESULTS: Of the 15 patients, only 2 were diagnosed as having BD prior to first operation. Twelve underwent repeat open-heart surgery because of valve dehiscence, and 6 of these 12 patients eventually died. In 5 patients with available echocardiographic findings of native valve morphology, echocardiography demonstrated characteristic aneurysmal changes and redundant coronary cusp motion in 4 patients. Vegetation-like mobile lesions and echo-free spaces mimicking abscess pockets led to misdiagnoses of infective endocarditis in 7 patients at least once during their clinical courses. Furthermore, complete atrioventricular block requiring permanent pacemaker insertion developed in 5 patients, especially those with echo-free spaces in the periannular area. CONCLUSIONS: Dismal prognoses are almost inevitable unless etiologic diagnoses are made prior to surgery in patients with BD and AR. It is important to diagnose BD preoperatively, and the diagnostic importance of echocardiographic findings should be kept in mind when evaluating patients with AR.