Canan Akman

Pulmonary Artery Involvement and Associated Lung Disease in Behçet Disease: A Series of 47 Patients

AbstractPulmonary 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.

Normal Diaphragmatic Motion and the Effects of Body Composition Determination With M-Mode Sonography

Objective. To evaluate the quantitative measurement of diaphragmatic motion in healthy subjects and to investigate the effects of different variables such as body mass index and waist circumference on the diaphragmatic motion. Methods. The study included 164 healthy subjects. The subjects were grouped according to age, sex, body mass index, and waist circumference. Measurements of diaphragmatic motion were made by a 3.5‐MHz sonographic unit in the M‐mode of the system. The posterior diaphragm on both sides was identified, and measurements were performed during deep inspiration. Results. The mean diaphragmatic motion measurements ± SD were 49.23 ± 10.98 and 50.17 ± 11.73 mm on right and left sides, respectively. Female subjects had statistically significantly (P < .05) decreased diaphragmatic motion (right, 46.93 ± 10.37 mm; left, 47.57 ± 10.36 mm) than male subjects. The mean diaphragmatic motion (right, 40.90 ± 8.89 mm; left, 39.37 ± 9.15 mm) was less in subjects who were underweight (P < .05) when compared with subjects who were of normal weight, overweight, and obese. Subjects who had a waist circumference of less than 70 cm showed a statistically significant decrease (P < .05) in diaphragmatic motion (right, 42.55 ± 9.12 mm; left, 42.24 ± 9.73 mm) when compared with subjects who had a waist circumference of 70 to 85, 85 to 100, and greater than 100 cm. Also, subjects younger than 30 years had statistically significantly (P < .05) decreased diaphragmatic motion (right, 44.57 ± 10.57 mm; left, 44.44 ± 11.37 mm). Conclusions. Sex, body mass index, waist circumference, and age may affect the diaphragmatic motion to some extent. Healthy persons of younger age with a smaller body mass index and waist circumference may show a decreased amount of diaphragmatic motion.

Emergency Endovascular Management of Pulmonary Artery Aneurysms In Behçet’s Disease: Report of Two Cases and a Review of the Literature

his report describes two patients with a known history of Behçet’s disease in whom massive hemoptysis developed from rupture of pulmonary artery aneurysms. The high recurrence rate of complications related to pulmonary artery aneurysms and even the aneurysms themselves due to inadequacy of medical therapy and the disadvantages of surgical treatment make these aneurysms candidates for endovascular management. The pulmonary artery aneurysms reported here were successfully treated with endovascular embolization using n-butyl cyanoacrylate. Pulmonary artery aneurysm embolization in Behçet’s disease has been reviewed in the light of relevant literature.

Progression of coronary artery calcification in renal transplant recipients

BACKGROUND Cardiovascular disease is the leading cause of mortality among renal transplant recipients. In the general population, coronary artery calcification (CAC) and progression of CAC are predictors of future cardiac risk. We conducted a study to determine the progression of CAC in renal transplant recipients; we also examined the factors associated with progression and the impact of the analytic methods used to determine CAC progression. METHODS We used multi-detector computed tomography to examine CAC in 150 prevalent renal transplant recipients, who did not have a documented cardiovascular disease. A baseline and a follow-up scan were performed and changes in CAC scores were evaluated in each patient individually, to calculate the incidence of CAC progression. Multivariate logistic regression analysis was used to evaluate the determinants of CAC progression. RESULTS Baseline CAC prevalence was 35.3% and the mean CAC score was 60.0 ± 174.8. At follow-up scan that was performed after an average of 2.8 ± 0.4 years, CAC prevalence increased to 64.6% and the mean CAC score to 94.9 ± 245.7. Progression of individual CAC score was found between 28.0 and 38.0%, depending on the method used to define progression. In patients with baseline CAC, median annualized rate of CAC progression was 11.1. Baseline CAC, high triglyceride and bisphosphonate use were the independent determinants of CAC progression. CONCLUSIONS Renal transplantation does not stop or reverse CAC. Progression of CAC is the usual evolution pattern of CAC in renal transplant recipients. Beside baseline CAC, high triglyceride level and bisphosphonate use were associated with progression of CAC.

Mammographic changes in women on hormonal replacement therapy

OBJECTIVES In the present retrospective study, we aimed to determine the frequency and the types of mammographic changes of breast parenchyma in women receiving hormone replacement therapy (HRT). We also investigated whether there was an association between mammographic changes and some clinical and hormonal characteristics of the women on HRT. METHODS One-hundred and eight women were included into the study. Of the 108 women, 19 were climacteric, four premature menopause, 50 spontaneous menopause and 35 surgical menopause. Prior to the start of HRT, an initial mammography was performed and it was repeated at 6- to 18-month intervals according to the womens status. Estrogen alone was started for 35 surgical menopause women and a combination of estrogen plus progesterone for the remaining 73 women. RESULTS Group I consisted of 96 women with no parenchymal changes or a decrease in parenchymal density on mammography, whereas group II consisted of 12 women with an increase in parenchymal density (11%) during the mean period of 24 months. Endogenous E2 levels were significantly higher in group II than in group I (52.4 +/- 42.3 pg/ml vs. 32.3 +/- 29.3 pg/ml, P < 0.05). Climacterium or types of menopause did not affect the mammographical density changes. Neither the type nor the duration of HRT had an effect on mammographic density increase. CONCLUSIONS We concluded that the endogenous E2 level might be an important role in screening the women mammographically. Long-term follow-up studies were concluded to be needed in order to evaluate the effects of HRT on mammographic changes.

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.

Coronary artery calcification and coronary ischaemia in renal transplant recipients

BACKGROUND Cardiovascular disease is the leading cause of mortality among renal transplant recipients. Data on the relationship between coronary artery calcification (CAC) and coronary ischaemia in renal transplantation patients are scant. We conducted a study to determine the prevalence and determinants of CAC in these patients; we also examined the frequency of coronary ischaemia in patients with moderate and severe CAC. METHODS We used multi-detector spiral computed tomography to examine CAC in 178 consecutive renal transplant recipients. Angina pectoris was sought with the Rose questionnaire. The extent of calcification was measured by Agatston score. Myocardial perfusion scintigraphy was performed in patients with moderate and severe CAC. Multivariate logistic and linear regression analysis was used to evaluate the determinants of CAC presence and CAC score, respectively. RESULTS CAC was present in 72 patients (40.4%), mean CAC score was 113.7±275.5 (median: 0 and range: 0-1712). Age, time on transplantation and Rose angina pectoris were the independent determinants of both CAC presence and high CAC scores in all multivariate models. Coronary ischaemia was detected in 17.1% of the patients with moderate-to-severe CAC. CONCLUSIONS CAC is highly prevalent in renal transplant recipients; it is associated with symptoms of coronary ischaemia. Time on transplantation is an independent determinant of CAC. Future studies to evaluate the prognostic significance of CAC in these patients are necessary.

Multiple pulmonary artery aneurysms due to infective endocarditis

Bilateral pulmonary artery aneurysms developed in the course of staphylococcal endocarditis in a 6-year-old girl with ventricular septal defect. Consecutive computed tomography scans revealed the progressive enlargement of one of the aneurysms. She underwent an urgent left upper lobectomy because of the impending rupture and a possible life-threatening hemorrhage. The second ipsilateral aneurysm was plicated in order to exclude the aneurysm sac. In the next operation the ventricular septal defect was closed and vegetations located on the tricuspid valve were removed. On follow-up spontaneous thrombotic resolution occurred in the right-sided aneurysms.

Pedunculated localized fibrous tumor of the pleura presenting as a moving chest mass.

Localized fibrous tumor of the pleura is a rare primary pleural neoplasm. The tumors are usually discovered incidentally on routine chest radiography. A change in the lesions position with respiration and/or posture of the patient indicates a pedunculated pleural lesion. Computed tomography and magnetic resonance imaging reveal the relation of the lesions to adjacent structures. Magnetic resonance imaging is also useful for the characterization of the lesions on the basis of signal features. These tumors usually consist of mature fibrous tissue.

Aortic and coronary calcifications in Takayasu arteritis

OBJECTIVES Atherosclerosis is well recognized in Takayasu arteritis (TAK) and the associated plaques tend to be more common in areas of arteritis. We now report arterial wall calcification in a large group of TAK patients and controls. We hypothesized that the degree of coronary artery calcification would point to a systemic effect of inflammation while that in the thoracic aorta more of local inflammation. METHODS A total of 47 patients with TAK, 43 patients with SLE and 70 healthy controls (HC) were studied. The presence of coronary artery and thoracic aorta calcifications (ToAC) was investigated by multi-detector computed tomography (MDCT). Atherosclerotic plaques in the carotid arteries were screened using B mode ultrasound. RESULTS The frequency of coronary artery calcification was significantly increased among patients with SLE as compared to the healthy controls while the increase in TAK did not reach statistical significance. There were more TAK patients with ToAC among the TAK as compared to the SLE patients [21/47 (45%) vs 10/43 (23%), P = 0.033]. In addition, a circumferential type of calcification, vs a punctuate or linear type, was the more common type in 67% of patients with TAK whereas only the linear or punctuate type was seen in SLE patients and HC. SLE and TAK patients were found to have increased risk for carotid artery plaques. Among TAK patients, coronary artery calcification, ToAC and carotid artery plaques tend to be at sites of primary vasculitic involvement. CONCLUSIONS There is increased atherosclerosis in TAK and SLE. Vessel wall inflammation seems to be also important in the atherosclerosis associated with TAK.