Comparison of demographic, clinic and radiological features of patients with axial spondyloarthritis accompanying familial Mediterranean fever to patients with each condition alone.

OBJECTIVE: To compare the demographic, clinical, and radiological features of patients with axial spondyloarthritis (axSpA) accompanying familial Mediterranean fever (FMF) to patients with each condition alone. METHOD: Hacettepe University Hospital database was screened regarding ICD-10 codes for FMF (E85.0) and axSpA (M45). The diagnosis of FMF was confirmed by Tel-Hashomer criteria, and axSpA by the presence of sacroiliitis according to the modified New York criteria or active sacroiliitis on magnetic resonance imaging. As control groups, 136 gender-matched, consequent FMF patients without axSpA and 102 consequent axSpA patients without FMF previously treated with any biological agents were included in the analysis. RESULTS: In patients with FMF + axSpA compared to the axSpA group, age at axSpA symptom onset and age at diagnosis were lower [median with interquartile range (IQR): 21 (17-30) vs 27 (21-37), p < 0.001; 23 (21-38) vs 32 (24-43) years, p = 0.001], moderate to severe hip disease and total hip replacement were more prevalent (23.4% vs 4.7%, p < 0.001; 11.2% vs 2.8%, p = 0.016). In patients with FMF + axSpA compared to the FMF group, age at FMF symptom onset and age at diagnosis were higher [13 (6-30) vs 11 (5-18), p = 0.057; 23 (13-33) vs 18 (10-31) years, p = 0.033] and amyloidosis was more prevalent (6.6% vs 2.2%, p = 0.076). Although the M694V variant (in one or two alleles) was more prevalent in the FMF + axSpA group, the difference was not statistically significant. CONCLUSION: In patients with FMF + axSpA, the age of onset of axSpA was significantly earlier, moderate to severe hip involvement and amyloidosis were more common than in patients with each condition alone.

Comparing immunoglobulin A vasculitis (Henoch-Schönlein purpura) in children and adults: a single-centre study from Turkey.

OBJECTIVE: Immunoglobulin A vasculitis/Henoch-Schönlein purpura (IgAV/HSP) is a systemic vasculitis involving small vessels with the deposition of immune complexes containing IgA. It is the most common primary systemic vasculitis of childhood and is much less common in adults. Our aim was to investigate the differences and similarities between adult and paediatric patients with IgAV/HSP. METHOD: We retrospectively evaluated the medical records of 35 adult and 159 paediatric (˂ 18 years old) patients with a clinical diagnosis of IgAV/HSP who were seen at the Departments of Rheumatology and Pediatric Rheumatology, Hacettepe University, Ankara, Turkey. The paediatric and adult patients were classified with IgAV/HSP according to the Ankara 2008 and American College of Rheumatology 1990 criteria, respectively. RESULTS: Upper respiratory tract infection was a common predisposing factor for both adults (34.3%) and children (21.4%). Creatinine and C-reactive protein were higher; and skin biopsy, hypertension, renal involvement, haematuria, proteinuria, and renal insufficiency at diagnosis were more frequent in adults than in children. Thrombocyte count was higher in children than in adults. Follow-up without treatment and complete recovery were more frequent in children, while persistent haematuria, chronic renal failure, relapse, and the use of corticosteroids/azathioprine were more frequent in adults. The only independent predictive factor for relapse was persistent haematuria. CONCLUSION: Various clinical and laboratory characteristics differ between children and adults with IgAV/HSP. Overall, IgAV/HSP has a self-limiting course in children but represents a more severe form of disease in adults, with more severe renal involvement. Persistent haematuria is a predictive factor for relapse.

Assessment of the HScore for reactive haemophagocytic syndrome in patients with rheumatic diseases.

OBJECTIVES: Reactive haemophagocytic syndrome (RHS) is a hyperinflammatory disorder often occurring in the background of several disorders such as infections, malignancies, and rheumatic diseases. Recently, a score known as the HScore was developed for the diagnosis of RHS. In the original study, most of the patients had underlying haematological malignancy or infection and the best cut-off value for the HScore was 169 (sensitivity 93%; specificity 86%). In this study we aimed to analyse the performance of the HScore in rheumatic disease-related RHS. METHOD: The patients with rheumatic disorders evaluated in the Departments of Rheumatology and Paediatric Rheumatology at Hacettepe University, Ankara, Turkey between 2002 and 2014 were reviewed retrospectively. The first group (n = 30) consisted of patients with RHS; the control group (n = 64) included patients with active rheumatic diseases without RHS. RESULTS: In the RHS group, 14 (46.7%) had adult-onset Still's disease (AOSD), 10 (33.3%) systemic juvenile idiopathic arthritis (SJIA), and six (20%) systemic lupus erythematosus (SLE). The control group (n = 64) consisted of 32 (50%) AOSD, 13 (20.3%) SJIA, and 19 (29.7%) SLE patients. Applying the HScore to the RHS patients, the best cut-off value was 190.5 with a sensitivity of 96.7% and specificity of 98.4%. When we excluded the patients from the control group who had not had bone marrow aspiration (n = 23), the same cut-off (190.5) performed best (sensitivity 96.7%; specificity 97.6%). Applying the 2004 haemophagocytic lymphohistiocytosis (HLH-2004) criteria gave a sensitivity of 56.6% and a specificity of 100% in the whole study group. CONCLUSIONS: In our study, a cut-off value for the HScore different from the original study performed better. Further studies are warranted to determine optimum cut-off values in different studies.

Risk factors for malignancy in systemic sclerosis patients.

Systemic sclerosis (SSc) is an autoimmune connective tissue disease with multisystem involvement. An increased incidence of cancer in SSc patients compared with the general population has been reported in several reports. Our aims in this study were to determine the most common malignancies and to investigate the possible risk factors for the development of malignancy in patients with SSc. Three hundred forty SSc patients from 13 centers were included to the study. Data of the patients were obtained by evaluating their medical records retrospectively. A total of 340 patients with SSc were evaluated. Twenty-five of the patients had 19 different types of malignancy. Bladder cancer was the most common type of cancer with four patients and was followed by breast cancer with three patients, and cervix cancer and ovarian cancer with two patients each. Other types of cancers such as squamous cell skin cancer, adenocancer with an unknown origin, multiple myeloma, chronic myeloid leukemia, papillary thyroid cancer, larynx cancer, non-small cell lung cancer, follicular type non-Hodgkin lymphoma (NHL), endometrium cancer, colon cancer, uterus cancer, neuroendocrine tumor, glioblastoma multiforme, and soft tissue sarcoma were diagnosed in one patient each. The only cancer type that showed an association with cyclophosphamide dose was bladder carcinoma. Other malignancies did not show a correlation with age, sex, smoking, type and duration of the disease, autoantibodies, organ involvement, and dose and duration of cyclophosphamide therapy. Cancer may develop in any organ in patients with SSc. Continuous screening of the patients during a follow-up period is necessary for the early detection of the tumor development.

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.

Infliximab, a TNF-α antagonist treatment in patients with ankylosing spondylitis: the impact on depression, anxiety and quality of life level.

The objective of this study was to assess the effect of infliximab on depression, anxiety and quality of life in patients with active ankylosing spondylitis (AS). In this 6-week longitudinal study, 16 patients with AS were assessed. Active disease as defined by BASDAI ≥4.0 was sought for inclusion. Infliximab was administered 5 mg/kg at 0, 2 weeks and 6 weeks. Collected data included age, sex and date of onset of rheumatologic disease. Activity of disease was measured using Bath Ankylosing Spondylitis Disease Activity Index (BASDAI). Biological activity was evaluated with erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP). ESR and CRP were assessed at baseline and day 42. The Hospital Anxiety and Depression scale (HADS), Beck Depression Inventory (BDI) and 36-item Short Form Health Survey (SF-36) were used to evaluate anxiety, depression and quality of life. BASDAI, SF-36, HADS and BDE were assessed prior to the initial infliximab dose and at 2nd, 14th and 42nd day. Seven (43.8%) AS patients had depression scores above the cut off value for both the HADS depression (HADS-D) and BDI and 4 (25 %) had high HADS anxiety scores at baseline. Significant time effect for BDI and HADS-D scores were observed. Although significantly lower BDI scores were found after first, second and third infusions of infliximab, compared to initial score, the significant decrease in HADS-D appeared after second and third infusions. A significant time effect for HADS-anxiety scores were found as well. All of the subscales of SF-36 improved significantly during the course, with an exception of role emotional, for which the difference approached to the significance. The change in BASDAI scores and CRP and ESR, in the treatment process, were not correlated with the change in depression and anxiety scores. Infliximab which is an anti-TNF-α drug, may be effective in the treatment of depression accompanying AS. Possible implications for the treatment of major depressive disorder were discussed, as well.

Takayasu's arteritis in Turkey - clinical and angiographic features of 248 patients.

OBJECTIVE: Takayasu's arteritis (TA) is a chronic, inflammatory vasculitis affecting the aorta and its major branches. Although it is more prevalent in Far-East Asia, the distribution of the disease is worldwide with different vascular involvement patterns and clinical manifestations. The objective of this study was to evaluate the demographic, clinical, angiographic and prognostic features of TA patients in Turkey. METHODS: Clinical and angiographic findings of 248 TA patients (228 female, 27 male) followed at 15 Rheumatology Centers were prospectively evaluated according to a predefined protocol. RESULTS: The mean age was 40.1 years (30.2 years at the clinical onset). Clinical manifestations included constitutional symptoms in 66%, absent or diminished pulses in 88%, bruits in 77%, extremity pain in 69%, claudication in 48%, hypertension in 43% and cerebrovascular accidents (CVA) in 18% of the patients. Renal artery stenosis, aortic regurgitation and pulmonary hypertension were present in 26%, 33% and 12%, respectively. According to the new angiographic classification, type V (50.8%) and Type I (32%) were the most frequent types of involvement. Corticosteroids were the main treatment in 93% of the patients alone (9%) or in combination with immunosuppressive agents (84%). Most frequently preferred immunosuppressive agents were methotrexate (63%), azathioprine (22%) and cyclophosphamide (13%). Remission was observed at least once in 94% of the patients and sustained remission in 71% during follow-up. CONCLUSION: The demographical, clinical and angiographic findings of TA patients in our series were similar to those reported from Japan, Brazil and Colombia. Combination therapies with immunosuppressive agents were the preferred choice of treatment in Turkey.

Reliability of high-resolution ultrasonography in the assessment of Achilles tendon enthesopathy in seronegative spondyloarthropathies.

OBJECTIVE: The present study was mainly aimed at investigating the interobserver and intraobserver reproducibility of ultrasound (US) results in the assessment of Achilles tendon enthesopathy in patients with seronegative spondyloarthropathies (SpA). METHODS: A total of 28 patients with a diagnosis of SpA according to the European Spondyloarthropathy Study Group criteria were included. The patient female/male ratio was 1.8 (18/10), mean age was 42 (range 25-75) years and mean disease duration was 9 (range 1-35) years. Mean (SD) Bath Ankylosing Spondylitis Disease Activity Index (BASDAI) and Bath Ankylosing Spondylitis Functional Index (BASFI) scores were 32.4 (14.5) and 26.3 (9.2), respectively. Bilateral Achilles tendon US examinations were carried out independently by three investigators using a MyLab70 XVG (Esaote Biomedica, Genoa, Italy), equipped with a broadband 6-18 MHz linear probe. Each Achilles tendon was scanned for assessing the presence/absence of US findings indicative of enthesopathy according to the Outcome Measures in Rheumatoid Arthritis Clinical Trials (OMERACT) preliminary definition. The same findings were also scored on a 3-grade semiquantitative scoring system on which investigators reached a consensus prior to the study. Total additive scores per Achilles tendon were calculated. RESULTS: Moderate to excellent interobserver and intraobserver agreements were found for most of the US findings indicative of enthesopathy. Similar results were obtained using semiquantitative assessments, with weighted kappa values estimating the interobserver and intraobserver agreements for soft tissue inflammation of 0.696 and 0.816, respectively and for tissue damage 0.711 and 0.901, respectively. CONCLUSION: US assessment of Achilles tendon enthesopathy in patients with SpA, using the OMERACT preliminary definition, was found to be reliable. Bone irregularity and entheseal hypoechogenicity were the most difficult abnormalities to reach agreement on.

Thoracotomy in spine surgery.

OBJECTIVE: Aim of the study was the assessment of thoracotomies performed for spine surgery. METHODS: All patients undergoing spine surgery with thoracotomy between December 2004 and October 2007 were included and evaluated with regard to their age, gender, etiology, the level of the intervention and the types of procedures performed. RESULTS: A total of 29 patients undergoing spine surgery were included. Mean age was 45 years (range: 6-75) and the female to male ratio was 9 : 20. Trauma (14 patients, 48 %) and malignancy (5 patients, 17 %) were the most frequent reasons for surgery. A total of 7, 8 and 14 patients were operated at T3-T6, T7-T10, and T11-L2 levels, respectively. A left thoracotomy was performed in all but three patients (n = 26). The most frequent procedure was corpectomy and stabilization (n = 25, 86 %). CONCLUSION: An anterior approach with thoracotomy is needed for spine surgery in subjects with conditions such as lung cancer, trauma, hydatid cyst, etc. A good preoperative assessment of the vertebrae requiring intervention and the use of appropriate techniques for separating the diaphragm during thoracotomy may provide a good exposure up to the L3 level.

A case of brain abscess due to Entamoeba species, Eikenella corrodens and Prevotella species.

We report a case of brain abscess due to multiple pathogens including Entamoeba species, Eikenella corrodens and Prevotella species. Patient had odontogenic infection without any site of amoebic infestation. Complete recovery was achieved with surgical treatment and antimicrobial therapy.

Autoimmune polyendocrine syndrome with atrial septal defect.

Atrial septal defect is frequently reported with genetic syndromes. But, to the best of our knowledge, it has not been reported with autoimmune polyendocrine syndrome. Here, the case of a 44-year-old-woman with concomitant involvement of the salivary gland, thyroid, intestines, and, possibly endocrine pancreas, diagnosed with autoimmune polyendocrine syndrome type II, is reported with accompanying atrial septal defect. Celiac disease, Hashimoto thyroiditis, and Sjögren syndrome were symptomatic and laboratory confirmed diagnosis; anti-glutamic acid decarboxylase antibody was positive but asymptomatic for type-1 diabetes. She was known to have sinus venosus type atrial septal defect diagnosed at 38 years old, when she had tiredness and chest pain.

Posterior leukoencephalopathy after combination chemotherapy in a patient with lymphoma.

Posterior leukoencephalopathy syndrome is a recently described syndrome involving mainly parieto-occipital gray/white matter of the brain. It occurs secondary to various clinical entities, like hypertension and immunosuppressive therapy. Few cases after combination chemotherapy have been reported. This study describes a 36-year-old woman with primary refractory T-cell lymphoma, who developed central nervous system toxicity due to treatment with intrathecal methotrexate and intravenous ifosfamide, idarubicine and etoposide given as a salvage regimen. Both clinical features as well as magnetic resonance imaging findings were typical for posterior leukoencephalopathy syndrome. The patient died despite anti-hypertensive therapy and haemodialysis. Central nervous system toxicity related to chemotherapeutics and posterior leukoencephalopathy syndrome are discussed briefly.

Cervical spinal cord stimulation improves neurological dysfunction induced by cerebral vasospasm.

The effect of cervical spinal cord stimulation on the cerebral blood flow has been investigated both experimentally and clinically since 1986. Although the effect of the spinal cord stimulation on cerebral ischemia induced by cerebral vasospasm after subarachnoid hemorrhage has been investigated widely, neurological dysfunction induced by cerebral vasospasm and the effect of the spinal cord stimulation on neurological dysfunction have not been investigated so far. The aim of this study is to investigate the neurological dysfunction induced by cerebral vasospasm after subarachnoid hemorrhage and whether the spinal cord stimulation improves this neurological dysfunction or not in New Zealand albino rabbits. The animals were divided into sham and experiment groups: Sham group. Motor evoked potentials were recorded before experimental procedure was performed in this group. Just after, intracisternal saline was injected and 3 days later a stimulation electrode was placed in the cervical epidural space. Motor evoked potentials were recorded but electrical stimulation was not applied. Experiment group. Firstly, motor evoked potentials had been recorded before experimental procedure was performed in also this group. After then a stimulation electrode was placed in the cervical epidural space of the animals in which subarachnoid hemorrhage procedure was performed 3 days ago. Motor evoked potentials were recorded both before and after spinal cord stimulation. Motor evoked potential latencies and amplitudes did not change in the sham operation group. But, motor evoked potential latencies extended and the amplitudes decreased in the experiment group before spinal cord stimulation. Spinal cord stimulation improved the changes occurring in latencies and amplitudes in the experiment group. Spinal cord stimulation improves the neurological dysfunction induced by cerebral vasospasm and motor evoked potentials recording is a reliable electrophysiological method to detect cerebral vasospasm and to assess the effects of different treatments in cerebral vasospasm.

Cervical spinal cord stimulation increases cerebral cortical blood flow in an experimental cerebral vasospasm model.

BACKGROUND: Cerebral microcirculatory changes during cerebral vasospasm after aneurysmal subarachnoid haemorrhage (SAH) are still controversial and uncertain. The aim of our study is to demonstrate that spinal cord stimulation (SCS) augments cerebral cortical microcirculatory blood flow in an experimental cerebral vasospasm model by using Laser Doppler Flowmetry (LDF). METHOD: The experiments were carried out on 24 New Zealand rabbits. Three experimental groups were designed. In group 1, Cerebral cortical blood flow (CCoBF) was evaluated by LDF in 8 rabbits. In group 2, Intracisternal saline injection and cervical epidural electrode placement without SCS were performed in 8 animals before LDF. In group 3, LDF was performed before and after SCS on the 4th day of SAH in 8 rabbits. CCoBF parameters obtained from LDF data were compared. FINDINGS: The occurrence of vasospasm after SAH was demonstrated with significant changes in LDF values. In all SAH animals, SCS resulted in significant increase (approximately 30%) in CCoBF. This increase was observed to continue even after the cessation of the stimulation. CONCLUSIONS: These results indicate that SCS improves cortical ischemia due to vasospasm after induced SAH. The cervical SCS may constitute a new therapeutic modality in treating disturbed CCoBF due to vasospasm.

The effects of the electrical stimulation of the nasal mucosa on cortical cerebral blood flow in rabbits.

The cerebral vessels have sympathetic, parasympathetic, and sensory innervations. A sensory innervation of the cerebral vessels originating in the trigeminal ganglion has been described in a number of species by several investigations. It has been shown that the electrical stimulation of the trigeminal ganglion causes an increase of cerebral blood flow (CBF). The aim of our present study is to stimulate the trigeminal ganglion with an extracranial and non-invasive method. A stimulating electrode was put in the nasal mucosa via right nares of rabbits and trigeminal ganglion was stimulated orthodromically via nasociliary nerve (NCN). Variations in the cortical CBF were evaluated by laser Doppler flowmetry. In experiment group, CBF increased together with the beginning of electrical stimulation. The flow values were remained high as long as the stimulation. In post-stimulation period, the CBF was decreased gradually and returned to the baseline values at 120s. This study demonstrated that the electrical stimulation of the NCN branch of the trigeminal nerve increases the cortical CBF under physiological conditions.

Primary multiple cerebral hydatid cysts with unusual features.

We present a case of 45 years old female patient with two primary cerebral hydatid cysts, each localised in separate hemispheres. Detailed studies revealed no evidence of hydatid disease elsewhere in the body. The cysts were removed by two separate operations. Though the cyst in the right posterior parietal lobe was delivered unruptured, seven months later a new cyst was detected in the same cavity, which seems to show relatively rapid growth in a year, and was also removed unruptured. This case is presented here for its unusual features such as having two primary cerebral hydatid cysts simultaneously in separate hemispheres, one of which partly localised intraventricularly, and the occurrence of a new cyst in the same cavity following intact removal of the previous cyst.

Stab wound of the cervical spinal cord and ipsilateral vertebral artery injury.

Stab wound of the cervical spinal cord with ipsilateral vertebral artery injury is rare. We report a case of a penetrating injury by scissors to the cervical spinal cord and right vertebral artery. The management is discussed and literature reviewed.

Vertebral plasma cellular osteomyelitis.

Plasma cellular osteomyelitis is a chronic recurrent, unifocal or multifocal inflammatory disorder, which has an unknown aetiology. The infectious focus or foci may occur in different sites of the skeleton. This unusual clinical entity has been reported in over 200 cases in the relevant literature, but the involvement of the spine with plasma cellular osteomyelitis has not been reported before. Because of the rarity of this disease and the possible difficulty in differentiating plasma cell osteomyelitis, both clinically and histopathologically from the other similar afflictions of the spine, we describe our case.