Abdominal aortic aneurysm healed with calcification in a female patient with Behçet's syndrome: A case report with a 2-decade follow-up.

Abdominal aortic aneurysms (AAA) are rare in Behçet syndrome (BS) but may result in life-threatening complications. AAA are generally treated with surgical intervention following aggressive immunosuppressive therapy. Healing with wall calcification and mural thrombus for AAA is scarcely reported in the literature. Herein, we present a female patient who developed a large saccular aneurysm in the infra-renal abdominal aorta 6 months after the diagnosis. Endovascular intervention was not found to be possible because of the dimensions of the aneurysm and the patient did not consent for open surgery. Therefore, she received only immunosuppressive treatment. By the second year of follow-up, we detected reduction in the size of AAA, along with circumferential wall calcification and mural thrombus. The patient was followed-up for 20 years without rupture and currently doing well. This case demonstrates that calcified large saccular AAA might not necessitate surgical intervention and be followed-up for many years without any complication.

Current surgical approaches in Takayasu's arteritis: a single-centre experience.

OBJECTIVES: We investigated cardiovascular surgical interventions in a group of patients with Takayasu's arteritis (TAK) diagnosed and followed by a single centre. METHODS: . Twenty patients with TAK (5 males, 15 females, mean current age: 38.1±10.7) who were operated for a broad spectrum of cardiovascular diseases ranging from coronary heart disease to coeliac stenosis or aneurysm between July 2008 and April 2016 were studied. One patient underwent operation related to aneurysm of ascending aorta and aortic insufficiency, 2 patients had operations for both coronary arteries originating from aortic arch, 6 patients for only arteries originating from aortic arch, 1 patient for both carotid and infra-inguinal artery, 5 patients for aorta-iliac or femoral revascularisation, 5 patients for renal artery and/or coeliac or superior mesenteric artery revascularisations. Three of these interventions were endarterectomy and patch plasty. RESULTS: The mean time between diagnosis and surgical intervention was 6.1±3.1 years (range: 3 months-12 years). A total of 4/32 (12.5%) grafts were occluded during the follow up period of mean 39.2±24.6 months. Secondary interventions like cross-femoral, or graft to superficial femoral artery bypasses were needed in 2 patients who underwent aorta-bifemoral bypasses to keep patency. There was no operative mortality. We did not observe any anastomotic aneurysm. One patient died due to graft infection 3 months after the operation. Stroke occurred in 2 patients who underwent re-vascularisations of the arteries originating from aortic arch. CONCLUSIONS: In our series, we have a relatively good midterm patency rates in patients with TAK and did not observe any anastomotic pseudoaneurysm. Stroke developed in 2 patients and mortality occurred in one patient due to the graft infection 3 months after the operation. In patients with limited carotid or aorta-iliac stenosis, chance for endarterectomy should be evaluated. Well-controlled disease activity with intensive medical treatment and multi-disciplinary approach could be associated with a favourable long-term outcome.

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.

Clinical efficacy and safety of a new 1000-mg suspension versus twice-daily 500-mg tablets of MPFF in patients with symptomatic chronic venous disorders: a randomized controlled trial.

BACKGROUND: Chronic venous disorders (CVD) is estimated to affect 30% to 50% of women and 10% to 30% of men. The most widely prescribed treatment for CVD worldwide is micronized purified flavonoid fraction 500 mg (MPFF). The aim of this clinical trial was to develop a new once daily 1000-mg oral suspension of MPFF. METHODS: In an international, randomized, double-blind, parallel-group study, symptomatic individuals classified CEAP C0s to C4s were randomized in either treatment arm and treated for 8 weeks. Lower limb symptoms (discomfort, pain and heaviness) were assessed using Visual Analog Scales (VAS), and quality of life (QoL) was measured with the CIVIQ-20 Questionnaire. RESULTS: A total of 1139 patients were included in the study. Both MPFF treatment regimens were well tolerated and associated with a significant reduction in lower limb symptoms. A non-inferiority of MPFF 1000-mg oral suspension once daily compared to MPFF 500-mg tablet twice daily (P<0.0001) was found for lower limb discomfort (-3.33 cm for MPFF 1000 mg and -3.37 cm for MPFF 500 mg), leg pain (-3.27 cm for MPFF 1000 mg and -3.31 cm for MPFF 500 mg) and leg heaviness (-3.41 cm for MPFF 1000 mg and -3.46 cm for MPFF 500 mg). The patients' QoL was improved by about 20 points on the CIVIQ scale in both groups (19.33 points for MPFF 1000 mg and 20.28 points for MPFF 500 mg). CONCLUSIONS: MPFF 1000-mg oral suspension and MPFF 500-mg tablets treatments were associated with similar reductions in lower limb symptoms and QoL improvement. The new once daily MPFF1000-mg oral suspension has a similar safety profile to two tablets of MPFF 500 mg, with the advantage of one daily intake, potentially associated with improved patient adherence and easier CVD management.

Bronchial artery enlargement may be the cause of recurrent haemoptysis in Behçet's syndrome patients with pulmonary artery involvement during follow-up.

OBJECTIVES: Haemoptysis occurring in a Behçet's syndrome (BS) patient with pulmonary artery involvement (PAI) during follow-up is usually regarded as PAI relapse. However, bronchial artery enlargement (BAE) may be the source of haemoptysis in some patients. METHODS: A chart review at the end of December 2014 revealed 118 patients with PAI in our centre since 1979. Nine (all men) had recurrent haemoptysis during follow-up which could not be explained with relapse of PAI. RESULTS: Haemoptysis recurred a median of 1.5 years (IQR: 9 months-5 years) during follow-up. Thorax CT scans did not show relapse of PAI or emergence of BAE. The patients were treated empirically but continued to complain of occasional haemoptysis thereafter. BAE was detected in 8 patients after a median follow-up of 9 years (IQR: 5-12 years). Six patients underwent bronchial artery embolisation that was repeated in 3. One patient with severe pulmonary hypertension died 3 weeks later. The remaining 5 are under follow-up for between 5 months-9 years. Pulmonary infarction and mild hemiparesis occurred in 2 patients after embolisation. One patient died with haemoptysis before undergoing embolisation. Another one with small BAE is under follow-up for 8 years without embolisation. The source of bleeding could not be determined in 1 patient who is now haemoptysis free for 5 years. CONCLUSIONS: BAE may be the source of recurring and fatal haemoptysis in BS patients with PAI during follow-up. Embolisation appears to be a life-saving procedure.

Clinical and Ultrasonographic Evaluation of Lower-extremity Vein Thrombosis in Behcet Syndrome: An Observational Study.

Vascular involvement can be seen in up to 40% of patients with Behcet syndrome (BS), the lower-extremity vein thrombosis (LEVT) being the most common type. The aim of the current study was to compare venous Doppler findings and clinical features between BS patients with LEVT and control patients diagnosed as having LEVT due to other causes.All consecutive 78 patients (71 men, 7 women; mean age 38.6 ±â€Š10.3 years) with LEVT due to BS and 50 control patients (29 men, 21 women; mean age 42.0 ±â€Š12.5 years) who had LEVT due to other causes, or idiopathic, were studied with the help of a Doppler ultrasonography after a detailed clinical examination. Patterns of venous disease were identified by cluster analyses. Clinical features of chronic venous disease were assessed using 2 classification systems. Venous claudication was also assessed.Patients with BS were more likely to be men, had significantly earlier age of onset of thrombosis, and were treated mainly with immunosuppressives and less frequently with anticoagulants. Furthermore, they had significantly more bilateral involvement, less complete recanalization, and more frequent collateral formation. While control patients had a disorganized pattern of venous involvement, BS patients had a contiguous and symmetric pattern, involving all deep and superficial veins of the lower extremities, with less affinity for crural veins. Clinical assessment, as measured by the 2 classification systems, also indicated a more severe disease among the BS patients. In line, 51% of the BS patients suffered from severe post-thrombotic syndrome (PTS) and 32% from venous claudication, whereas these were present in 8% and 12%, respectively, among the controls. Among BS patients, a longer duration of thrombosis, bilateral femoral vein involvement, and using no anticoagulation along with immunosuppressive treatment when first diagnosed were found to be associated independently with severe PTS.Lower-extremity vein thrombosis associated with BS, when compared to LEVT due to other causes, had distinctive demographic and ultrasonographic characteristics, and had clinically a more severe disease course.

Pulmonary artery involvement in Behçet׳s syndrome: Effects of anti-Tnf treatment.

OBJECTIVES: Anti-TNF agents are being increasingly used in patients with Behçet׳s syndrome (BS) when conventional immunosuppressives fail. However, experience with anti-TNF treatment on pulmonary artery involvement (PAI) of BS is limited. METHODS: A chart review revealed 13 patients with PAI (all men) treated with anti-TNF agents (12 infliximab and 1 adalimumab) following an inadequate response to immunosuppressives for 12.2 ± 9.5 SD months and 2 male patients who developed PAI while receiving infliximab for large vein thrombosis for 10 months and for parenchymal central nervous system involvement for 2 years, respectively. RESULTS: The first patient developing PAI while receiving infliximab responded to cyclophosphamide and prednisolone but the second died with hemoptysis within 1 month. At the end of the survey, 6 of the 13 patients with PAI were continuing these agents for 25.5 ± 16.2 SD months with good response, 4 stopped anti-TNF treatment after a mean of 23 ± 9.8 SD months after achieving clinical and radiologic response and 1 patient with good response went to another center after receiving infliximab for 10 months and the remaining 2 experienced serious infections (lung tuberculosis and aspergillosis) necessitating early withdrawal. Two patients relapsed within 3 years after stopping anti-TNF agents and concomitant azathioprine. One developed mesenteric vein thrombosis necessitating bowel resection and the second developed new PAI that was controlled with cyclophosphamide and prednisolone after short courses of infliximab, adalimumab, and canakinumab. CONCLUSION: Anti-TNF treatment seems to be effective for refractory PAI of BS but may not prevent its development. Relapses can be seen after withdrawal. Caution is required for their serious adverse effects.

Venous claudication in Behçet's disease.

OBJECTIVE: We have previously shown that venous claudication is significantly more common among patients with Behçet's disease (BD) and had proposed that this was a "venous claudication" because it was specifically more common among men with lower extremity venous thrombosis (LEVT). With this study, we reassessed the presence of claudication prospectively by a questionnaire and a treadmill exercise. METHODS: We studied all men: 61 BD patients with LEVT, 40 BD patients without vascular involvement, and 56 healthy controls. Venous claudication was assessed by a standardized questionnaire. In addition, patients were asked to walk on a treadmill for 10 minutes. Patients who experienced symptoms consistent with venous claudication but still able to walk and those who had to give up the treadmill exercise were noted. Ankle-brachial pressure indices measured before and after the treadmill test did not indicate any peripheral arterial disease. RESULTS: Twenty-one BD patients with LEVT (34%), two BD patients without vascular involvement (5%), and none of the healthy controls described venous claudication when assessed with the questionnaire (P < .001). There were significantly more patients who described claudication during the treadmill exercise among patients with LEVT (21%) compared with those with no vascular disease (8%) and healthy controls (2%) (P = .002). Finally, only those with LEVT (6 of 61) had to stop the treadmill challenge because of claudication. CONCLUSIONS: Venous claudication is a severe and frequent symptom, being present in up to one third of BD patients with LEVT. It impairs walking capacity in 10% of these patients.

Long-term treatment results in soft tissue sarcomas of the thoracic wall treated with pre-or-postoperative radiotherapy--a single institution experience.

OBJECTIVE: To evaluate the long term results among patients with soft tissue sarcoma of the thoracic wall. MATERIALS AND METHODS: Twenty-six patients who were treated with pre-or postoperative radiotherapy between December 1980-December 2007, with a diagnosis of soft tissue sarcoma of the thoracic wall were retrospectively evaluated. RESULTS: The median age was 44 years (14-85 years) and 15 of them were male. A total of 50% of patients were grade 3. The most common histologic type of tumor was undifferentiated pleomorphic sarcoma (26.9%). Tumor size varied between 2-25 cm (median 6.5 cm). Seventeen of the cases had marginal and 9 had wide local resection. Four cases received preoperative radiotherapy and 22 postoperative radiotherapy. Six of the patients with large and high grade tumors received chemotherapy. Median follow-up time was 82 months (9-309 months). Local recurrence and metastasis was detected in 34.6% and 42.3% of patients, respectively. Five- year local control (LC), disease-free survival (DFS), overall survival (OS), and disease-specific survival (DSS) were 62%, 38%, 69%, and 76% respectively. On univariate analysis, the patients with positive surgical margins had a markedly lower 5-year LC rate than patients with negative surgical margin, but the difference was not significant (43% vs 78%, p=0.1). Five-year DFS (66% vs 17%) and DSS (92% vs 60%) rates were significantly worse for the patients who had high grade tumors (p=0.01, p=0.008 respectively). CONCLUSIONS: Tumor grade and surgical margin are essential parameters for determining the prognosis of thoracic wall soft tissue sarcoma both in our series and the literature.

Unilateral absence of the left pulmonary artery with patent ductus arteriosus and interrupted inferior vena cava.

Unilateral absence of the pulmonary artery and interrupted inferior vena cava are rare congenital disorders. The clinical presentation is variable, and many patients can be asymptomatic for many years and even throughout their lives. We report the case of a 44-year-old female patient with a history of hemoptysis. She was referred to our clinic with a diagnosis of pulmonary artery agenesis. Computed tomography revealed absence of the left main pulmonary artery and long-tract patent ductus arteriosus (PDA). Blood supply to the left lung was provided by major aortopulmonary collateral arteries (MAPCAs). Right heart catheterization through the right femoral vein was problematic, as the catheter could not be negotiated from the inferior vena cava to the right atrium. We revealed the interrupted inferior vena cava, which continued as a dilated azygos vein to the superior vena cava. The magnitude of the shunt flow from the PDA was too small, and the calculated shunt fraction was not significant. Hemoptysis was possibly due to MAPCAs. However, as the patient had no active bleeding when she was referred, the exact source of the hemoptysis could not be identified.

An unusual case with vasculo-Behçet's disease: peripheral arterial aneurysm in an elderly woman.

Behçet's disease (BD) is a chronic, multi-systemic disorder that can affect all sizes of arteries and veins. Vascular involvement of BD is generally observed in young men and is an important cause of morbidity and mortality. Arterial lesions can appear as aneurysms, stenosis and occlusions in BD. We, here, present the case of a woman who developed a peripheral arterial aneurysm in her sixth decade, 20 years after disease onset. BD patients with aneurysms should be consulted at any age for pre- and post-operative assessment for immunosuppressive treatment to reduce recurrences, complications and disease activation.

Pulmonary artery involvement and associated lung disease in Behçet disease: a series of 47 patients.

Pulmonary artery aneurysms (PAAs) are well known causes of mortality and morbidity in Behçet disease (BD). However, pulmonary artery involvement in BD is not limited to PAA; the other main type of pulmonary artery involvement is pulmonary artery thrombus (PAT), with or without associated PAA. In addition, other types of lung disease like nodules and cavities in the lung parenchyma are frequently associated with pulmonary artery involvement, and can be misinterpreted as being due to infection. We surveyed the clinical, radiologic, and laboratory characteristics and outcome of 47 BD patients with pulmonary artery involvement and the associated findings, all seen and followed at a single dedicated tertiary care center.We identified 47 (41 male, 6 female) patients in whom pulmonary artery involvement was diagnosed, who were registered in the multidisciplinary clinic at Cerrahpasa Medical Faculty between January 2000 and December 2007. Mean age at diagnosis was 29 ± 8 years, and mean disease duration to the onset of pulmonary artery involvement was 3.6 ± 4.8 years. Hemoptysis was the most common presenting symptom (79%) followed by cough, fever, dyspnea, and pleuritic chest pain. Thirty-four of 47 patients (72%) presented with PAA, including 8 with associated PAT. The remaining 13 patients (28%) had isolated PAT. Patients with isolated PAT in general have clinical features similar to patients with PAA. However, hemoptysis was less frequent and voluminous in patients with isolated PAT. Most (91%) of the patients had active disease outside the lungs when they presented with pulmonary artery involvement.Forty (85%) patients had nodules and 6 (13%) had cavities when first seen. Peripheral venous thrombosis was present in 36 of 47 (77%) patients, and intracardiac thrombi in 12 of the 36 (33%) patients. Nodules, cavities, and intracardiac thrombi were mainly present in the acute stages of pulmonary artery involvement.Pulmonary artery involvement is usually multiple, and involves mostly descending branches of the pulmonary artery. Pulmonary artery involvement may disappear, but arterial stenosis or occlusions usually develop at the same location. After a mean follow-up of 7 years, 12 of 47 (26%) patients were dead; patients with larger aneurysms were more likely to die. Sixteen of 47 (34%) patients were symptom free, and the remaining 40% had mild dyspnea (13/47) and/or small bouts of hemoptysis (8/47).Pulmonary artery pressure may be elevated, and may indicate a poor prognosis. Mediastinal lymphadenopathy and mild pleural and pericardial effusions may also be observed. Corticosteroids and immunosuppressive agents are the mainstays of treatment; however, refractory cases may require embolization, lobectomy, cavitectomy, and decortication.

Management and prognosis of nonpulmonary large arterial disease in patients with Behçet disease.

OBJECTIVE: The purpose of this study was to evaluate and report our treatment policies in the management of nonpulmonary arterial aneurysms in Behçet disease and to assess the prognosis in a cohort of 25 patients diagnosed between 1996 and 2007 by formally reassessing their outcome at the present time. METHODS: We identified 25 patients (24 men/1 woman) with Behçet disease with nonpulmonary aneurysms (n = 23) or occlusions (n = 2) between 1996 and 2007. All patients fulfilled the International Study Group Criteria for Behçet disease. Aneurysms were demonstrated with contrast-enhanced computed tomography (CT) or magnetic resonance angiography (MRA) after first-line ultrasonography. Standard surgical procedures were carried out in 22 patients. One patient with a nonruptured saccular aortic aneurysm and 2 patients with carotid aneurysms were managed only medically. For the patients with aneurysms located in the aortic bifurcation, we preferred aorto-bi-iliac bypasses; for the six extremity aneurysms, we were able to ligate the arteries; and for the other 10 extremity aneurysms we used polytetrafluoroethylene (PTFE) grafts for bypass procedures. All patients received immunosuppression with cyclophosphamide and corticosteroids before the operation and were continued in the postoperative period. All patients were examined between January and December 2010 paying special attention for new and anastomotic aneurysms and graft patency. RESULTS: There was one death and 1 patient was lost to follow-up. The remaining 23 patients (92%) were under follow-up after a mean of 7.4 ± 2.9 years after their operation. Four PTFE grafts (40%) inserted for extremity aneurysms occluded with no disabling consequences. Also, 6 patients who were treated with ligation postoperatively began to complain of mild to moderate claudication. In 2 patients, aneurysms recurred at the anastomotic site, whereas in 3 patients, new aneurysms developed at other sites. CONCLUSION: The surgical management of large, nonpulmonary arterial disease of Behçet disease is currently quite satisfactory. When the false aneurysm is in the infrarenal aorta, aorto-bi-iliac bypass is the preferred surgical intervention. Extremity aneurysms can be treated with synthetic graft insertion. In selected cases, ligation can give satisfactory results; however, postoperative claudication is common. In some patients with small intact saccular aneurysms, surgery may not be necessary. Patients must be prescribed immunosuppressive therapy with cyclophosphamide and corticosteroids before and after the surgical intervention in order to avoid Behçet disease activation.

Antihypertensive drugs and inflammation in acute ischemic stroke as a predictor factor of future cardiovascular mortality.

The beneficial effects of antihypertensive drugs in secondary stroke prevention could not be based on their effects on lowering hypertension only.In this prospective study, the status of blood pressure, treatment regimens, new atherothrombotic event, blood sampling for hs-CRP and fibrinogen were asked at initial visit, 6th, and 12th months in 226 patients with atherothrombotic stroke.Eighty-seven percent of patients had an antihypertensive regimen, but hypertension control was achieved in 34.1% of patients.Neither use of six different antihypertensive drug regimens nor the change in blood pressure levels showed any difference on new atherothrombotic events, outcomes or survival rates.On the other hand, the higher levels of hs-CRP at baseline were found to be associated with higher mortality rates (p=0.020).Our findings emphasize the predictive role of inflammation in future cardiovascular mortality in patients with acute ischemic stroke, indicating that inflammatory mediators underlying the atherothrombotic process play a more important role than it is assumed.

Unusual cause of dysphagia: inflammatory pseudotumor of the lung.

Inflammatory pseudotumor (IPT) is a rare disease that usually occurs in the lung. Patients with IPT are usually asymptomatic, with a solitary pulmonary nodule or mass detected on routine chest roentgenogram. IPT can behave as a malignant tumor both clinically and radiologically. Cough, fever, dyspnea, and hemoptysis are the usual presenting symptoms. This report describes the case of a 37-year-old man with a 4-month course of dysphagia secondary to lower esophageal invasion by the posterior mediastinal extension of a lung IPT.

Surgical treatment of Buerger's disease.

We aimed to evaluate the characteristics of 198 new patients with Buerger's disease treated surgically in the last decade. We also compared these results with our former series reported in 1993. The records of patients with Buerger's disease who were enrolled in an ongoing investigational protocol between 1991 and 2001 were reviewed. Sympathectomy was carried out in 161 patients and revascularization in 19 patients. The cumulative secondary patency rate was 57.9% for bypass grafts at a mean follow-up of 5.4 years. Clinical outcome following sympathectomy was considered improved in 52.3% of patients, stable in 27.8%, and worse in 19.8%. Seven major and 36 minor amputations were performed, with a limb salvage rate of 95.6%. The aggressiveness of the disease has increased compared with previous series, parallel to the expansion of cigarette consumption. Bypass surgery should be considered for patients with severe ischemia who have target vessels. Sympathectomy still has a role to improve distal flow.

Primitive neuroectodermal tumor of the pancreas. A case report of an extremely rare tumor.

Primitive neuroectodermal tumor (PNET) of the pancreas is extremely rare. Although the diagnosis of PNET is suggested by the light microscopic appearance of the tumor, it should be confirmed by the immunohistochemical evaluation of the c-myc expression and if possible, further determination of the particular chromosome translocation, t(11;22)(q24,q12). In this report, we present a male patient with pancreatic PPNET who had been followed up for 50 months. The related literature is also reviewed. In our case, the pathologic diagnosis was based on the positive immunoreactivity for CD99 in many of the tumor cells. The complementary cytogenetic studies were not possible in the private setting of the patient's treatment. The patient was 31 years old when first operated. Within 4 months of the first operation he had local recurrence. In the third year of his follow-up he had been discovered to have pulmonary metastases and another metastatic tumor in his lung was diagnosed the year after. The metastatic foci were primarily treated by surgical resections. He had chemotherapy after each resection of pulmonary metastatic foci. After 50 months of the initial surgical intervention, he succumbed to widespread thoracic and bone metastases. Because of the extreme rarity of PPNET in the pancreas, and its rather protracted course, we think our case may further contribute to the ever expanding database for this particular entity.