İsmail Doğan

Combined Long-Term Steroid and Immunosuppressive Treatment Regimen in Granulomatous Mastitis

Background: Idiopathic granulomatous mastitis (IGM) is a rare benign inflammatory disease of the breast. It is related to various etiological factors. The treatment of IGM is challenging as there is a lack of consensus in the literature and treatment options vary widely. Conservative treatment with antibiotics, glucocorticoids and immunosuppressive drugs, and surgery are used in the management of the disease. In this article we report our experience with IGM patients receiving immunosuppressive treatment. Patients and Methods: The medical records of patients with IGM receiving systemic therapy at the Hacettepe University Hospital between October 2007 and May 2010 were reviewed. 15 cases of histopathologically proven IGM were identified. The data was examined for risk factors and success of treatment. Results: 14 patients were given prednisolone together with azathioprine, and 1 patient who was pregnant at the time of diagnosis received only prednisolone (30 mg/day). 11 (73%) patients had a complete response to systemic therapy. 2 patients had a relapse, of whom 1 required surgical drainage and 1 was treated with a higher dose of glucocorticoids. Conclusion: Systemic therapy is a safe and effective treatment for IGM. The addition of azathioprine to glucocorticoid therapy permits quick tapering of the steroid doses and increases the treatment success.

Pulmonary hypertension in systemic lupus erythematosus: pulmonary thromboembolism is the leading cause.

BackgroundPulmonary hypertension (PH) is a life-threatening complication of systemic lupus erythematosus (SLE). Pulmonary hypertension in SLE has a variety of causes. Diagnosing early and defining the cause of PH accurately can provide better clinical outcome in SLE. We investigated the causes and characteristics of PH in patients with SLE. MethodsOne hundred twenty-one patients with SLE who had a visit in a 6-month period were assessed retrospectively. Patients who ever had a systolic pulmonary arterial pressure of 40 mm Hg or greater by Doppler echocardiography were considered to have PH. ResultsAmong 122 patients, 65 had echocardiography for some reason, and 10 (8.2%) were diagnosed as having PH by echocardiographic examination. This number reduced to 9 (7.4%) when we excluded the patient with normal pulmonary artery pressure at right heart catheterization. Causes of PH were as follows: thromboembolic events in 4 patients (44.4%) (2 of them had chronic thromboembolic PH), left-sided heart disease in 2 patients (22.2%), pulmonary arterial hypertension in 1 patient (11.1%), high cardiac output state in 1 patient (11.1%), and transient elevation of systolic pulmonary artery pressure in 1 patient (11.1%) who had a history of venous thromboembolism. Venous thromboembolic disease was significantly higher in patients with SLE with PH in comparison to patients with SLE without PH (7 patients [6.3%] vs 5 patients [50.0%]; P = 0.001). All patients improved clinically during their short-term follow-up. ConclusionsPatients with SLE are at increased risk for PH. This study highlights the complexity of the differential diagnosis of PH in patients with SLE once again and emphasizes the importance of pulmonary thromboembolism as a cause of PH. One should investigate patients with SLE with unexplained symptoms and/or signs related to PH for possible treatable causes.

Inflammatory bowel diseases and Takayasu's arteritis: coincidence or association?

Takayasus arteritis (TA) and inflammatory bowel disease (IBD) are rare diseases but there are case reports presenting their co‐existence in the literature. The aim of this study was to investigate the relation between IBD and TA.

Aspirin resistance in systemic lupus erythematosus. A pilot study

Systemic lupus erythematosus (SLE) patients are at increased risk of thrombosis and cardiovascular diseases. Aspirin is an effective treatment option for these patients. The aim of this study was to investigate the presence of aspirin resistance in SLE patients. We studied aspirin resistance in 33 SLE patients and nine healthy controls by using a Multiplate® impedance aggregometer (Dynabyte GmbH, Munich, Germany). Twenty-six SLE patients were on regular aspirin treatment. Aspirin resistance was found in five (19.2%) out of 26 patients who were on aspirin treatment. When the tests were repeated by adding acetylsalicylic acid in the medium, all of these patients became responsive to the aspirin. SLE disease activity, body mass index, smoking status, and the presence of anticardiolipin antibodies or positive lupus anticoagulant test results were no different in patients with or without aspirin resistance. (p > 0.05 for all). Our results suggest that there may be a considerable number of SLE patients with aspirin resistance.

Effect of capillaroscopic patterns on the pulse oximetry measurements in systemic sclerosis patients

Nailfold capillaroscopy is a simple method for determining microvascular damage in systemic sclerosis (SSc). The pulse oximeter is a noninvasive tool that is used to estimate a patients arterial blood oxygen saturation. The aim of this study was to investigate the effect of capillaroscopic patterns on pulse oximetry measurements in SSc patients. The nailfolds from 2nd to 5th fingers in both hands were examined in all subjects by using capillaroscopy. Patients were categorized according to their capillaroscopy findings in 3 groups as having early, active or late patterns. Oxygen saturation (SpO2) measurements were performed from 2nd to 5th fingers in both hands with a finger probe. We studied 53 SSc patients (F/M: 48/5). According to capillaroscopy findings 18 patients were classified as having early pattern (34.0%), 19 active pattern (35.8%), and 15 late pattern (28.3%). Only 1 (1.9%) patient had normal capillaroscopy findings. SpO2 could not be measured in 47 (11.0%) fingers and in 20 (37.7%) SSc patients. There were 20 (37.7%) patients with a ≥4% difference between the minimum and maximum SpO2 measurements among fingers. There were no difference between the groups of SSc patients defined by capillaroscopy findings in terms of mean maximal SpO2 or mean minimal SpO2 measured from fingers (p NS, for all). Assessment of SpO2 values in patients with SSc is challenging. We did not detect any effect of capillaroscopic patterns on mean SpO2 values. On the other hand ≥4% difference between minimum and maximum SpO2 values measured from fingers of a patient may be considered as an indirect sign of microvascular damage. Assessment of the highest measured SpO2 values among the fingers of a patient may be more suitable in practice.

Recurrent uveitis due to sildenafil usage in a patient with Behçet’s disease

Behçet’s disease (BD) may cause uveitis and retinal vasculitis in nearly half of the patients. Uveitis is one of the most serious complications that can lead to blindness. Sildenafil (Viagra(R)) and the other phosphodiesterase type 5 (PDE5) inhibitors are the first-line options for the treatment of erectile dysfunction, but transient visual symptoms and serious ocular side effects have been reported in PDE5 inhibitor users. Herein, we report a case with BD who applied to our outpatient unit with recurrent uveitis after sildenafil therapy.

FRI0272 Five Factor Score (FFS) and its Relationship with Mortality in Granulomatous Poliangiitis (GPA)

Background Five Factor Score (FFS) has been used in evaluating prognosis in systemic vasculitis including GPA. A revised FFS was published in 2011.1 Objectives This study is aimed to investigate usefullness of revised FFS and its relationship with mortality in GPA. Methods Totally 77 (F/M: 41/36) GPA patients from database of Vasculitis center was retrospectively analyzed. Demographic and clinical characteristics were recorded. Five factor score of patients were calculated. Patients who had been lost from follow up was evaluated by phone call (n=14). Survival analysis was done by Kaplan-Meier method. Results Mean ages of patients were 50±15 years. Distribution of organ involvements were as; ear-nose –throat (ENT): 81.7%, pulmonary: 83.3%, renal: 59.5%, skin: 32.4%, musculoskeletal: 68.6%, eye: 23.3%. ANCA was positive in 74% of patients (cANCA/ pANCA:65%/14.7%). Most frequent treatment protocol in remission induction was corticosteroid + cyclophosphamide (75.5%). Rituximab was used in 12 (15.6%) patients. Seven of patients with renal involvement (n=23) were died, whereas three patients had renal transplantation and four had been on hemodialysis therapy. Totally 14 patients had died. Causes of mortality were co-infections in chronic renal failure (n=2), pulmonary hemorrhage (n=3), pulmonary hemorrhage and infection (n=2), gastrointestinal perforation (n=2), infective endocarditis (n=1), not known (n=4). Survival analysis was done with 63 patients. Median survival time was 36 (1-264) months. One-year and three-year survival rate was 92% and 81%, respectively. Twenty-three (36.5%) of patients had follow-up ≥5 years. Survival rate was statistically different between groups regarding to FFS scores (Table, p=0.003, Figure). 3-year survival rate was higher in patients with ENT involvement compared to without (87% vs 58%, p=0.052). Survival analysis according to Five Factor score Five Factor score 1-year survival, % 3-year survival, % p 0 97 93 1 96 81 0.003 2 60 30 Ear, nose and throat involvement (+) 93 87 0.052 Ear, nose and throat involvement (−) 82 58 Conclusions Three-year survival rate of GPA patients in our Vasculitis center is 81%. Survival rate was lower in patients with higher FFS scores concordant with literature. According to our results, FFS was found useful in evaluating prognosis in GPA. References Guillevin L ve ark. The Five Factor Score revisited. Medicine 2011; 90:19-27. Disclosure of Interest None declared