Eftal Yucel

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.

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

We report a case of an 18-year-old female, who was diagnosed as having systemic Behçets 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 Behçets 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.

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.

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

Background. Behcets 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 (β = −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.

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. J. Clin. Apheresis, 28:422–425, 2013.

Granulomatous anterior uveitis in a patient with CREST syndrome

The CREST syndrome is a variant form of progressive systemic sclerosis. Apart from the occurrence of keratoconjunctivitis sicca, other types of ocular involvement associated with this variant are quite rare. We present the case of a 73-year-old woman with the CREST variant of progressive systemic sclerosis who developed unilateral granulomatous anterior uveitis. Systemic and laboratory testing failed to suggest evidence for any other associated systemic disease as a possible cause of the granulomatous uveitis. The inflammation was successfully controlled with topical steroids and mydriatics. While a small number of cases of uveitis have been reported in other variant forms of progressive systemic sclerosis, to date there have been no descriptions of uveitis associated with the CREST syndrome.

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.