Granulomatous polyangitis (Wegener granulomatosis): Clinical findings and results of long-term follow-up.

INTRODUCTION: To evaluate long-term outcome of patients with granulomatous polyangitis (GPA) followed up in a tertiary university hospital. PATIENTS AND METHODS: We reviewed medical records of 22 patients with GPA diagnosis confirmed by tissue biopsies between 2004 and 2014. RESULT: The mean time from the onset of symptoms to diagnosis was 7.8 ± 12.3 months [interquartile range (IR)= 4.0]. The most commonly involved organs were the upper respiratory tract (URT) (72.7%), lower respiratory tract (81.8%) and kidneys (72.7%). URT involvement indicated good prognosis (p= 0.046). Survival in the patients with and without URT involvement was 124.6 ± 6.9 months and 59.7 ± 22.9 months, respectively. End-stage renal failure (ESRF) requiring dilaysis and cardiac involvement were associated with mortality (p= 0.022 and p= 0.026, respectively). Of the 12 dialysis-dependent patients at diagnosis, 11 survived > 3 months and seven regained renal function permanently. Dialysis dependency was significantly lower in patients who received plasmapheresis (p= 0.047). Overall mortality rate was 18% (4/22). Mean survival was 55.9 ± 42.8 months (IR= 84.0). CONCLUSIONS: Diagnosis of GPA may be delayed by the nonspecific nature of its symptoms. URT involvement was associated with good prognosis, whereas cardiac involvement and ESRF requiring dialysis were associated with poor outcome. Plasmapheresis may increase the rate of renal recovery in the patients with ESRF requiring dialysis.

Impact of rheumatoid arthritis in Turkey: a questionnaire study.

OBJECTIVES: Unmet needs of rheumatoid arthritis (RA) patients regarding physician/patient communication, treatment preferences and quality of life issues were investigated in a Turkish survey study. METHODS: The study was conducted with the contribution of 33 rheumatologists, and included 519 RA patients. The study population included patients who had been on biologic therapy for >6 months and were still receiving biologic therapy (BT group), and those who were biologic naive, but found eligible for biologic treatment (NBT group). Of the RA patients, 35.5% initially had a visit to an internal disease specialist, 25.5% to a physical therapy and rehabilitation specialist, and 12.2% to a rheumatology specialist for their RA complaints. The diagnosis of RA was made by a rheumatologist in 48.2% of patients. RESULTS: The majority of RA patients (86.3%) visit their doctor within 15-week intervals. Most of the physician-patient communication focused on disease symptoms (99.0%) and impact of the disease on quality of life (61.8%). The proportion of RA patients who perceived their health status as good/very good/excellent was higher in the BT group than in the NBT group (74.3% vs. 51.5%, p<0.001). However, of those RA patients in the NBT group, only 24.8% have been recommended to start a biologic treatment by their doctors. With respect to dose frequency options, once-monthly injections were preferred (80%) to a bi-weekly injection schedule (8%). CONCLUSIONS: In conclusion, RA patients receiving biologic therapy reported higher rates of improved symptoms and better quality of life and seemed to be more satisfied with their treatment in our study.

Antisynthetase syndrome with refractory lung involvement and myositis successfully treated with double filtration plasmapheresis.

Antisynthetase syndrome (ASS) is characterized by inflammatory muscle disease, pulmonary and joint involvement, and antisynthetase autoantibodies, with anti-Jo-1 antibody being the most common. Despite the use of immunosuppressive drugs, the prognosis of lung involvement seems poor. Herein, we report a case of refractory ASS, which maintained long-term remission by double filtration plasmapheresis (DFPP) combined with immunosuppressive therapy. For a 65-year-old woman, who was diagnosed with ASS, immunosuppressive therapy was initiated and plasmapheresis (PP) was performed five times due to acute interstitial pulmonary disease and inflammatory myopathy. She remained in remission for eight months following PP. Increase in interstitial involvement was identified by lung tomography when the patient presented again with complaint of progressive increase in dyspnea and muscle pain. Although the immunosuppressive therapy was increased for the patient with elevated creatine phosphokinase (CPK) (2776 IU/mL), a rapid decrease in diffusion capacity of the lung for carbon monoxide (DLCO) was observed and the patient underwent PP. After four sessions of therapy, insufficient clinical and laboratory response was obtained (control CPK 1797 IU/mL) and because of that issue DFPP using a 2A filter was performed to the patient. There was a marked improvement in complaints of the patient, DLCO, and laboratory findings (control CPK 508 IU/mL) after three sessions of DFPP. The patient, who continued the immunosuppressive therapy after DFPP procedure, is being followed for 12 months in remission. Although our experience is limited with only one patient, DFPP seems promising as a treatment option for ASS with severe lung involvement.

Assessment of disease activity and progression in Takayasu's arteritis with Disease Extent Index-Takayasu.

OBJECTIVE: Disease Extent Index-Takayasu (DEI.Tak) is a new index developed for the follow-up of Takayasu's arteritis (TA), assessing only clinical findings without the requirement of imaging. We investigated the effectiveness of DEI.Tak in assessing disease activity and progression by comparing with physician's global assessment (PGA) and active disease criteria defined by Kerr et al. METHODS: The initial DEI.Tak forms were filled in for 145 TA patients cross-sectionally to detect the baseline damage and after 29.8 (31) months (n = 105, 144 visits) only by including the new/worsening symptoms within the past 6 months. RESULTS: At baseline, all patients had a DEI.Tak >0 [mean (s.d.): 7.6 (4.2)]. At this evaluation, 62% of the patients had active, 16.2% had persistent and 21.8% had inactive disease according to the PGA. At follow-up, in 69% of the patients the DEI.Tak score was 0. However, 14% of them were accepted as having active and 17% persistent disease according to PGA. In contrast, 18% with a DEI.Tak ≥ 1 were inactive according to PGA. Patients with active or persistent disease with PGA had higher DEI.Tak compared with inactives [1.3 (1.9), 1 (1.3) vs 0.2 (0.6), respectively; P < 0.001]. According to Kerr's criteria 27% were active. The total agreement between DEI.Tak and Kerr's criteria was 94% (κ = 0.85). Compared with PGA, Kerr's criteria had a total agreement of 74% and DEI.Tak 68%. CONCLUSION: During follow-up, most TA patients showed no clinical activity with DEI-Tak. Although the agreement between Kerr's criteria and DEI.Tak seemed very good, using Kerr's criteria instead of DEI.Tak increased the consistency with PGA, which could be explained by the influence of imaging data and acute-phase reactant levels on the physician's decisions.

Endothelial functions are more severely impaired during active disease period in patients with Behcet's disease.

Oral apthous ulcers and skin lesions are the primary symptoms of Behcet's disease (BD). To date, there is no study to investigate possible associations between these lesions and endothelial functions. We have hypothesized that active BD period with oral and skin lesions might have more deteriorating effect on endothelial functions. Thirty-five patients with BD were registered for the study. Each subject was evaluated two times in both active and inactive disease periods. The subject with at least 30 days of lesion-free period was regarded in the inactive disease period, and the subject with any oral and/or skin lesions was regarded in the active disease period. For the control group, 35 healthy age- and sex-matched subjects were registered. In each subject, flow-mediated dilation (FMD) of the brachial artery after transient ischemia was evaluated in both active and inactive disease periods. High-sensitivity C-reactive protein (hsCRP) values (3.30 +/- 5.76 vs 14.19 +/- 13.55 vs 1.82 +/- 1.31, P < 0.001) and FMD values (13.89 +/- 5.57 vs 8.53 +/- 4.78 vs 15.83 +/- 5.29, P < 0.001) were significantly different among the BD patients in inactive and active disease periods and among control subjects. FMD values in inactive and active disease periods modestly correlated to hsCRP and low-density lipoprotein cholesterol values. Brachial FMD is more prominently impaired in BD patients within the active disease period. BD patients are possibly more vulnerable to cardiovascular manifestations when they are in the active disease period.

Transverse myelitis caused by varicella zoster: case reports.

Transverse myelitis is a group of disorders characterized by focal inflammation of the spinal cord and results in loss of motor and sensory function below the level of injury. Occurrence of this condition during or following varicella infection is uncommon. This report describes two cases of transverse myelitis caused by varicella zoster.

Abnormal electronystagmography in rheumatoid arthritis.

OBJECTIVE: Although sensorineural hearing loss has been widely investigated in those with rheumatoid arthritis, studies assessing the vestibular system in patients with rheumatoid arthritis are limited. The aim of this study was to assess the vestibular system in patients with rheumatoid arthritis. METHODS: The study consisted of 43 patients with rheumatoid arthritis and 30 healthy controls. Otorhinolaryngologic and neurotologic examinations, pure-tone audiometry, impedancemetry, electronystagmography including smooth pursuit, saccade, positional, and caloric tests were performed in all patients with rheumatoid arthritis and in controls. The erythrocyte sedimentation rate, C-reactive protein level, and rheumatoid factor level were evaluated in all patients with rheumatoid arthritis. RESULTS: Audiograms revealed sensorineural hearing loss in 12 patients (27.9%) with rheumatoid arthritis and in two controls (6.6%); the difference was statistically significant (P=.033). In those with rheumatoid arthritis, the results of electronystagmography revealed central abnormalities in nine patients (20.9%), peripheral abnormalities in three (6.9%), and mixed abnormalities in three (6.9%). Smooth pursuit and saccade tracing impairments were significantly higher in patients with rheumatoid arthritis (P>.05). Canal paresis in patients with rheumatoid arthritis were significantly higher than those in the control group (P=.039). No association was found between electronystagmographic abnormalities in patients with rheumatoid arthritis and age, sex, duration of disease, the results of laboratory testing, sensorineural hearing loss, or medication use. CONCLUSION: Our results suggest an association of rheumatoid arthritis with vestibular system dysfunction as well as auditory impairment.

Assessment of Patients with Takayasu Arteritis in Routine Practice with Indian Takayasu Clinical Activity Score.

OBJECTIVE: To assess the Indian Takayasu Clinical Activity Score (ITAS2010) in followup of Takayasu arteritis (TA). METHODS: ITAS2010 forms were filled in prospectively (n = 144). Clinical activity was assessed with physician's global assessment (PGA) and criteria defined by Kerr, et al. RESULTS: ITAS2010 was significantly higher in patients with active disease. Total agreement between ITAS2010 and PGA was 66.4%, and between ITAS2010 and Kerr, et al was 82.8%. During followup, 14 of 15 patients showing vascular progression with imaging were categorized as having inactive disease according to ITAS2010. CONCLUSION: ITAS2010 was discriminatory for activity during the followup, but the agreement between PGA and ITAS2010 was moderate. Future work should include the incorporation of advanced vascular imaging and demonstration of ITAS2010 as a scalable measure and not simply a dichotomous measure of activity/flare versus remission.

Myositis and scleritis associated with Behcet's disease: an atypical presentation.

We report a case of an 18-year-old female, who was diagnosed as having systemic Behcet's disease with chief complaints of vision loss in the right eye and redness and pain in both eyes. In addition to an anterior necrotizing scleritis, she had several attacks of retrobulbar neuritis and lateral rectus myositis. She responded well to i.v. and topical prednisolone and her subsequent attacks were treated with i.v. cyclophosphamide (500 mg on presentation and on Days 7, 10, 12, 15, and 20; the cycle was repeated every 20 days). She benefited on this treatment and her vision improved dramatically. Her final acuity of 20/50 was thought to be due to presumed previous attacks of optic neuritis and anterior segment inflammation. During the five-month follow-up period, she had no other attacks. Ischemia and inflammation are common in Behcet's disease and sometimes thrombosis and vasculitic changes may cause a decrease in vision. In this case, the immunosuppressive therapy helped attenuate the inflammatory vasculitic episodes during the disease course.

Autonomic neural control of the cardiovascular system in patients with Behçet's disease in the absence of neurological involvement.

Behçet's disease (BD) is a chronic multi-system disease presenting with recurrent oral and genital ulceration, and relapsing uveitis. Heart rate recovery (HRR) after exercise is a marker of parasympathetic activity. A delayed recovery of systolic blood pressure (SBP) after exercise might reflect sympathetic hyperactivity. The analysis of variations in heart rate has also been used to determine the balance between sympathetic and vagal nerve activities in the heart. Our objective was to determine HRR, the SBP response to exercise and heart rate variability (HRV) in patients with BD in the absence of neurological involvement. The study population consisted of 32 patients with BD and 30 healthy controls who were matched with respect to age, sex, and physical activity. Heart rate recovery was calculated as the difference between heart rate at peak exercise and heart rate at 1, 2, and 3 min of recovery. Blood pressure recovery indexes were determined by dividing the systolic blood pressure at 2 and 3 min in recovery to the systolic blood pressure at peak exercise. In patients with BD, mean HRR at 1 min (HRR1) were not significantly different than that of controls (21 ± 7 vs 20 ± 7 bpm, p = 0.50). Although, resting mean SBP of patients with BD was higher than controls (121 ± 13 vs 115 ± 12 mmHg, p = 0.039), the SBP recovery indices of the patients with BD at 2 and 3 min were similar to those of controls (0.84 ± 0.07 vs 0.84 ± 0.09, p = 0.89 and 0.78 ± 0.09 vs 0.78 ± 0.08, p = 0.93, respectively). Both time domain and frequency domain parameters of patients with BD were similar to that of controls. This study shows that the patients with BD have normal HRR1 and normal SBP response to exercise and normal HRV. These findings might suggest unaltered autonomic neural control of the cardiovascular system in this disorder in the absence of neurological involvement.

Effect of prayer on osteoarthritis and osteoporosis.

Prayer is one kind of worship that is composed of repetitive action during praying in Islam. The prayer is performed five times a day, every Friday, bairams and death ceremonies. The aim of this study is to search the role of this repetitive action on knee, hip osteoarthritis and osteoporosis. Forty-six patients who had been performing the prayer at least for 10 years, and 40 patients who had not performed the prayer, were included in this prospective study. Each patient was evaluated with standard questionnaire form, joint examination was done and various laboratory parameters were studied. Anterior-posterior radiography of the pelvis and weight-bearing knees of each patient were examined. Each film was evaluated by two investigators separately and first scored by using Kellgren and Lawrence (K&L) scale, then the width of the joint space of hips and knees were measured directly using a steel ruler and recorded to the nearest half millimeter. Bone mineral density (BMD) of lumbar spine and femur was measured. Patients having Heberden's nodes, Bouchard's nodes, and carpometacarpal disease were frequent in worshiper group. Joint space width measurements and assessment according to K&L scale did not differ between worshipers and non-worshipers. BMD of lumbar spine was decreased in worshipers and also decreased with patients having Heberden's nodes, Bouchard's nodes, female gender and age. Prayer has no effect on knee and hip osteoarthritis, and may be related with hand osteoarthritis. It seems to have negative effect on lumbar BMD, but further investigations are needed.

Vestibular evoked myogenic potentials in Behcet's disease.

The aim of this study was to investigate vestibular evoked myogenic potentials (VEMPs) and their clinical significance in Behcet's disease. Twenty-six patients with Behcet's disease and 25 healthy volunteers were evaluated for pure tone audiometry, caloric response, and VEMPs. Sensorineural hearing loss was found in 53.8% of patients with Behcet's disease, which was significantly higher than controls. Four patients had canal paresis, but no controls; this difference was not significant. Although VEMP recordings were elicited in all study subjects, mean p13 and n23 latencies were prolonged in Behcet's patients compared with controls. Seven patients had delayed VEMP responses. There were no correlations regarding p13 values and age, duration or activity of disease, vertigo, or sensorineural hearing loss. The results of this preliminary study suggest an association between delayed VEMP responses and Behcet's disease. Further research with large samples is needed to confirm that VEMP testing is useful to diagnose and follow vestibular dysfunction in Behcet's disease.

Impaired coronary microvascular and left ventricular diastolic functions in patients with ankylosing spondylitis.

BACKGROUND: It has been shown that the patients with inflammatory rheumatic diseases such as systemic lupus erythematosus and rheumatoid arthritis have an increased risk of developing atherosclerosis. However, the association of ankylosing spondylitis (AS) to atherosclerosis and related diseases is still controversial. Accordingly, we investigated coronary flow reserve (CFR) and left ventricular (LV) diastolic function in patients with AS using transthoracic Doppler echocardiography. METHODS: CFR and LV diastolic function were studied in 40 patients with AS (38.9+/-10.2 years, 26 males) and 35 healthy volunteers (37.5+/-6.4 years, 23 males). Coronary diastolic peak flow velocities (DPFV) were measured at baseline and after dipyridamole infusion. CFR was calculated as the ratio of hyperemic to baseline DPFV. LV diastolic function was assessed by both standard and tissue Doppler imaging. RESULTS: Demographic features and coronary risk factors except diastolic blood pressure were similar between the groups. CFR were significantly lower in the AS group than in the control group (2.20+/-0.46 versus 3.02+/-1.50, P<0.0001). Reflecting LV diastolic function mitral A-wave and E/A ratio were borderline significant, and mitral E-wave deceleration time and isovolumic relaxation time were significantly different between the groups. Serum hsCRP and TNF-alpha levels were significantly higher in the patients with AS, and hsCRP and TNF-alpha levels independently correlated with CFR. CONCLUSION: These findings show that CFR reflecting coronary microvascular function and LV diastolic function are impaired in patients with AS, and severity of these impairments correlate well with hsCRP and TNF-alpha. These results suggest that impaired CFR may be an early manifestation of cardiac involvement in patients with AS.

Cardiovascular prognostic value of vascular involvement in Behcet's disease.

We measured carotid artery intima-media thickness (IMT), brachial artery flow mediated dilation (FMD), and coronary flow reserve (CFR) in 38 Behcet's disease (BD) patients without vascular involvement, 15 BD patients with vascular involvement, and 35 control subjects. BD patients with and without vascular involvement were similar regarding carotid IMT (0.52+/-0.14; 0.51+/-0.09; 0.46+/-0.09; P=0.051), and brachial FMD (16.3+/-6.8; 16.5+/-9.1). CFR values were significantly impaired in BD patients compared to the controls; however the BD patients with and without vascular involvement were similar regarding CFR (2.66+/-0.45; 2.62+/-0.48; 2.91+/-0.53, P=0.007 with and without vascular involvement and the controls, respectively). Endothelial and coronary microvascular functions are compromised in BD patients without vascular involvement as remarkably as that in patients with vascular involvement.

Impaired coronary microvascular function in familial Mediterranean fever.

BACKGROUND: Patients with inflammatory rheumatic diseases have an increased risk of developing atherosclerosis. However, the question of whether patients with familial Mediterranean fever (FMF) are at risk of atherosclerosis and related diseases remains controversial. OBJECTIVE: We aimed to use transthoracic Doppler echocardiography to investigate coronary flow reserve (CFR) and left ventricular (LV) diastolic function in patients with FMF. METHODS: CFR and LV diastolic function were studied in 33 patients with FMF (16 men, 17 women; mean age, 36.7+/-12.0 years) and 35 healthy volunteers (20 men, 15 women; mean age, 36.8+/-5.2 years). Coronary diastolic peak flow velocities (DPFV) were measured at baseline and after dipyridamole infusion. LV diastolic function was assessed by standard and tissue Doppler imaging. RESULTS: CFR was significantly lower in the FMF group than in the control group (2.27+/-0.38 versus 3.02+/-0.50, P<0.0001). Significant between-group differences were found regarding LV diastolic function mitral E/A ratio, mitral E-wave deceleration time, and lateral A(m). Serum high sensitivity C-reactive protein (hsCRP) levels were significantly higher in the patients with FMF, and hsCRP values independently correlated with CFR. CONCLUSIONS: Coronary microvascular function and LV diastolic function are impaired in patients with FMF. The severity of these impairments is correlated with hsCRP. Impaired CFR may be an early manifestation of cardiac involvement in patients with FMF.

Patients with Behcet's disease carry a higher risk for microvascular involvement in active disease period.

BACKGROUND: Behcet's disease (BD) is characterized with remissions and exacerbations. However, to date, there is no study to investigate a possible association of disease activity (active versus inactive disease period) with cardiovascular complications. METHODS: Forty patients with BD were evaluated in both active and in inactive disease period. For the control group 45 healthy volunteers, age and sex matched, were registered. Subjects with at least a 15-day lesion-free period were regarded in inactive disease period, and subjects with any oral, skin, and/or genital lesion was regarded as in active disease period. In each subject coronary diastolic peak flow velocities (DPFV) were measured at baseline and after dipyridamole infusion (0.84 mg/kg over 6 minutes) using an Acuson Sequoia C256 echocardiography system. Coronary flow reserve (CFR) was defined as the ratio of hyperemic to baseline DPFV. RESULTS: CFR values were significantly lower in BD patients compared to the controls (2.57+/-0.50 versus 2.87+/-0.53, P = 0.006). In active disease period, basal DPFV (24.6+/-7.5 versus 27.3+/-6.6, P = 0.019) was significantly higher than in the inactive disease period. In the active disease period hyperemic DPFV (61.7+/-14.9 versus 56.8+/-16.7, P = 0.015) values decreased significantly. Therefore, in the active disease period CFR significantly decreased from 2.57+/-0.50 to 2.09+/-0.46, P<0.001. The only independent predictor of CFR within the active disease period was the disease duration (beta = -0.384, P = 0.012). CONCLUSION: Within the active disease period, coronary microvascular function is more prominently impaired in BD patients. Therefore, BD patients are possibly more vulnerable to cardiovascular manifestations when they are in an active disease period.

Cochleovestibular dysfunction in ankylosing spondylitis.

Ankylosing spondylitis (AS) is a rheumatic disease characterized by chronic inflammation. The aim of this study was to evaluate the functions of the cochlea and the vestibular system in patients with AS. The study group consisted of 32 patients with AS and 30 healthy volunteers as a control group. Otorhinolaryngologic examinations were performed in all patients together with pure-tone audiometry, speech tests, impedancemetry, transient evoked otoacoustic emission (TEOAE) and electronystagmography (ENG). A significant difference was found between the 2 groups with regard to pure-tone averages at high frequencies in each ear (p < 0.05). The rates of reproducibility in TEOAE testing were significantly lower in patients with AS (p = 0.03). The signal-to-noise rates of the response values were lower at all frequencies in patients with AS, but a statistically significant difference was only observed at 2, 3 and 4 Hz (p < 0.05). ENG revealed pathologies in 11 patients with AS (34%), 8 of which were central (25%) and 3 of which were peripheral (9%). No correlation was found between cochleovestibular dysfunction and age, sex, disease duration, activity and medication taken. This study demonstrated that there is an association between AS and cochleovestibular dysfunction.

Transverse myelitis caused by varicella zoster: case reports

Transverse myelitis is a group of disorders characterized by focal inflammation of the spinal cord and results in loss of motor and sensory function below the level of injury. Occurrence of this condition during or following varicella infection is uncommon. This report describes two cases of transverse myelitis caused by varicella zoster.