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OBJECTIVES: Glucocorticoids (GC) are widely accepted as the standard first-line treatment for giant cell arteritis (GCA). However, relapse rates are reported up to 80% on GC-only protocol arms in controlled trials of tocilizumab and abatacept in 12-24 months. Herein, we aimed to assess the real-life relapse rates retrospectively in patients with GCA from Turkey. METHODS: We assembled a retrospective cohort of patients with GCA diagnosed according to ACR 1990 criteria from tertiary rheumatology centres in Turkey. All clinical data were abstracted from medical records. Relapse was defined as any new manifestation or increased acutephase response leading to the change of the GC dose or use of a new therapeutic agent by the treating physician. RESULTS: The study included 330 (F/M: 196/134) patients with GCA. The mean age at disease onset was 68.9±9 years. The most frequent symptom was headache. Polymyalgia rheumatica was also present in 81 (24.5%) patients. Elevation of acute phase reactants (ESR>50 mm/h or CRP>5 mg/l) was absent in 25 (7.6%) patients at diagnosis. Temporal artery biopsy was available in 241 (73%) patients, and 180 of them had positive histopathological findings for GCA. For remission induction, GC pulses (250-1000 methylprednisolone mg/3-7 days) were given to 69 (20.9%) patients, with further 0.5-1 mg/kg/day prednisolone continued in the whole group. Immunosuppressives as GC-sparing agents were used in 252 (76.4%) patients. During a follow-up of a median 26.5 (6-190) months, relapses occurred in 49 (18.8%) patients. No confounding factor was observed in relapse rates. GC treatment could be stopped in only 62 (23.8%) patients. Additionally, GC-related side effects developed in 64 (24.6%) patients, and 141 (66.2%) had at least one Vasculitis Damage Index (VDI) damage item present during follow-up. CONCLUSIONS: In this first multi-centre series of GCA from Turkey, we observed that only one-fifth of patients had relapses during a mean follow-up of 26 months, with 76.4% given a GC-sparing IS agent at diagnosis. At the end of follow-up, GC-related side effects developed in one-fourth of patients. Our results suggest that patients with GCA had a low relapse rate in real-life experience of a multi-centre retrospective Turkish registry, however with a significant presence of GC-associated side effects during follow-up.
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BACKGROUND: The aim of this study was to evaluate the value of the PET vascular activity score (PETVAS) during the follow-up of patients with Takayasu arteritis. METHODS: Takayasu arteritis patients who underwent 18F-Fluorodeoxyglucose (FDG) PET imaging were evaluated retrospectively. In 8 patients both 1 and 2-h imagings were also performed prospectively. For PETVAS, 9 arterial areas were scored between 0-3 according to the FDG uptake. RESULTS: Forty-six images of 34 patients were evaluated. PETVAS was higher in patients with clinically active disease (p = 0.03) and in the C-reactive protein (CRP) elevated group among clinically inactive patients (p = 0.0015). PETVAS correlated with CRP (p = 0.003, r = 0.53) and erythrocyte sedimentation rate (p = 0.005, r = 0.41), whereas age, disease duration, immunosuppressive, and glucocorticoid (GC) treatments were not associated with PETVAS. First vs. 2nd-h PETVAS was similar in patients who had both 1st and 2nd h PET scans (p = 0.67). DISCUSSION: We observed higher PETVAS in patients with active disease and elevated acute phase reactants. Although scores in our study (performed at one-h) were lower compared to the original PETVAS study performed at two h, PETVAS seems to be a reliable tool to quantify FDG PET scores in routine practice.
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Fluorodesoxiglucosa F18 , Arteritis de Takayasu , Proteína C-Reactiva/metabolismo , Glucocorticoides/uso terapéutico , Humanos , Tomografía de Emisión de Positrones/métodos , Radiofármacos , Estudios Retrospectivos , Arteritis de Takayasu/diagnóstico por imagenRESUMEN
OBJECTIVES: To determine the prevalence of vitamin D deficiency in patients with small and medium vessel systemic vasculitis. METHODS: In this cross-sectional study, 25-hydroxy (OH) vitamin D3 levels were measured in adult patients with systemic small and medium vessel vasculitis including antineutrophil cytoplasmic antibody-associated vasculitis (AAV), cryoglobulinaemic vasculitis (CryV), IgA vasculitis (IgAV) and polyarteritis nodosa (PAN), and age- and sex-matched healthy subjects (HS) and patients with rheumatoid arthritis (RA) as control groups. 25OH vitamin D3 levels<30ng/ml and <20ng/ml were regarded as insufficiency and deficiency, respectively. RESULTS: Fifty-seven patients (42 AAV, 2 CryV, 8 IgA vasculitis, 5 PAN) with systemic vasculitis, 101 HS, and 111 RA patients were included. The mean 25OH vitamin D3 level was 21.8±14.2ng/mL in patients with vasculitis, 42.7±27.6ng/mL in HS (p<.001) and 20.1±18.47ng/mL in patients with RA (p=.54). Vitamin D insufficiency and deficiency were significantly higher in patients with systemic vasculitis compared to HS (75.4% vs 33.7%, p<.001; %50 vs 21.8%, p<.001, respectively). Vitamin D status was not different in patients with systemic vasculitis compared to RA. There was a negative correlation between vitamin D status and CRP levels (=-.364, p=.007). The multivariate logistic regression analysis showed that renal involvement was significantly associated with vitamin D deficiency/insufficiency in patients with vasculitis (OR 22.5 [95% CI 1.6-128.9]. CONCLUSION: Vitamin D deficiency and insufficiency are more frequent in patients with systemic small and medium vessel vasculitis and RA than HS. Renal involvement is one of the factors associated with vitamin D deficiency/insufficiency in patients with vasculitis.
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Artritis Reumatoide , Vasculitis , Deficiencia de Vitamina D , Adulto , Artritis Reumatoide/complicaciones , Artritis Reumatoide/epidemiología , Estudios Transversales , Humanos , Vasculitis/complicaciones , Vasculitis/etiología , Vitamina D , Deficiencia de Vitamina D/complicaciones , Deficiencia de Vitamina D/epidemiologíaRESUMEN
OBJECTIVE: To compare the clinical features, laboratory findings, and prognosis of Behçet's disease (BD) patients with and without Budd-Chiari syndrome (BCS). METHODS: This multicenter retrospective study investigated 61 (M/F: 41/20) patients with BD, having coexistent BCS, and 169 (M/F:100/69) BD patients as the control group without BCS from 22 different centers of Turkey diagnosed between 1990 and 2017. RESULTS: Of the total 61 BD patients with BCS, the onset of the first symptom and the median age of diagnosis were earlier in contrast to BD patients without BCS (p = 0.005 and p = 0.007). Lower extremity deep vein and inferior vena cava (IVC) thrombosis were more common in patients with BCS (all; p < 0.01) compared to the control group. Mortality was significantly higher in BD-BCS patients with IVC thrombosis than in the controls (p = 0.004). Since most of the cases in our cohort had chronic and silent form of BCS, mortality rate was 14.8%, which was on the lower range of mortality rate reported in literature (14-47%). While all BD-BCS patients received immunosuppressive (IS) agents, only half of them received additional anticoagulant treatments. Among IS agents, interferon treatment was more frequently used in this cohort (19%), compared to other series reported in literature (2.3%). CONCLUSION: To our knowledge, this is the largest series of BD patients with BCS. Our patients had earlier disease onset and diagnosis, higher frequency of IVC thrombosis, and higher mortality rate, compared to BD patients without BCS. Mortality was significantly higher in BD-BCS patients with IVC thrombosis compared to controls. Key Points ⢠Mortality rate is higher in BD-associated BCS patients with IVC involvement. ⢠Chronic and silent form of BD-associated BCS has a better prognosis. ⢠The main treatment options are corticosteroids and immunosuppressive agents, whereas anticoagulant treatment remains controversial.
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Síndrome de Behçet , Síndrome de Budd-Chiari , Síndrome de Behçet/complicaciones , Síndrome de Behçet/tratamiento farmacológico , Síndrome de Behçet/epidemiología , Síndrome de Budd-Chiari/complicaciones , Síndrome de Budd-Chiari/epidemiología , Estudios de Cohortes , Humanos , Inmunosupresores/uso terapéutico , Estudios Retrospectivos , Vena Cava InferiorRESUMEN
OBJECTIVES: To determine the prevalence of vitamin D deficiency in patients with small and medium vessel systemic vasculitis. METHODS: In this cross-sectional study, 25-hydroxy (OH) vitamin D3 levels were measured in adult patients with systemic small and medium vessel vasculitis including antineutrophil cytoplasmic antibody-associated vasculitis (AAV), cryoglobulinaemic vasculitis (CryV), IgA vasculitis (IgAV) and polyarteritis nodosa (PAN), and age- and sex-matched healthy subjects (HS) and patients with rheumatoid arthritis (RA) as control groups. 25OH vitamin D3 levels<30ng/ml and <20ng/ml were regarded as insufficiency and deficiency, respectively. RESULTS: Fifty-seven patients (42 AAV, 2 CryV, 8 IgA vasculitis, 5 PAN) with systemic vasculitis, 101 HS, and 111 RA patients were included. The mean 25OH vitamin D3 level was 21.8±14.2ng/mL in patients with vasculitis, 42.7±27.6ng/mL in HS (p<.001) and 20.1±18.47ng/mL in patients with RA (p=.54). Vitamin D insufficiency and deficiency were significantly higher in patients with systemic vasculitis compared to HS (75.4% vs 33.7%, p<.001; %50 vs 21.8%, p<.001, respectively). Vitamin D status was not different in patients with systemic vasculitis compared to RA. There was a negative correlation between vitamin D status and CRP levels (=-.364, p=.007). The multivariate logistic regression analysis showed that renal involvement was significantly associated with vitamin D deficiency/insufficiency in patients with vasculitis (OR 22.5 [95% CI 1.6-128.9]. CONCLUSION: Vitamin D deficiency and insufficiency are more frequent in patients with systemic small and medium vessel vasculitis and RA than HS. Renal involvement is one of the factors associated with vitamin D deficiency/insufficiency in patients with vasculitis.
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OBJECTIVE: Major salivary gland ultrasonography (SGUS) is a widely used imaging technique to evaluate salivary gland involvement in primary Sjögren syndrome (pSS). The aim of this study was to evaluate the relationship between SGUS, salivary flow rate (SFR) as an objective measure of the gland function, and oral health-related quality of life (OHRQOL) as a patient-reported outcome measure (PROM) in a pSS cohort. METHODS: Sixty-six patients with pSS were examined by SGUS according to Hocevar and Milic scoring systems. Patients with inhomogeneity/hypoechoic areas with scores ≥ 2 in parotid and submandibular glands were classified separately as "severe glandular involvement." Further, oral health, SFR, and Oral Health Impact Profile-14 (OHIP-14) for OHRQOL were assessed. RESULTS: Both total Hocevar and Milic scores were higher in 21 pSS patients with low unstimulated whole salivary flow rate (U-WSFR) than 45 pSS patients without low U-WSFR (P = 0.001 and P < 0.0001, respectively). Increased scores of homogeneity, hypoechoic areas and glandular border visibility were observed in patients with low U-WSFR (P < 0.05). Among these variables, homogeneity score was found to be an independent risk factor for low U-WSFR in pSS according to logistic regression analysis (OR 1.586, P = 0.001). Moreover, a higher OHIP-14 score was observed in severe parotid involvement compared to nonsevere cases (23.26 ± 21.19 vs 8.32 ± 13.82, P = 0.004). CONCLUSION: High Milic and Hocevar SGUS scores are associated with reduced SFR and poor OHRQOL as a PROM. The inhomogeneity component of the SGUS score is associated with low U-WSFR and is an indicator of severely affected gland function.
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Calidad de Vida , Síndrome de Sjögren , Humanos , Saliva/metabolismo , Glándulas Salivales/diagnóstico por imagen , Índice de Severidad de la Enfermedad , UltrasonografíaRESUMEN
OBJECTIVES: Gastrointestinal (GI) system is commonly affected in sytemic sclerosis (SSc) patients who are also known to be at risk for malnutrition. We aimed to investigate the relationship between severity of GI disease, malnutrition and severity of organ involvement including microvasculopathy. METHODS: A hundred and thirty-four SSc patients were included into the study; disease activity and severity, the University of California, Los Angeles, Scleroderma Clinical Trials Consortium Scleroderma Gastrointestinal scale 2.0 (UCLA SCTC GIT 2.0) and malnutrition universal screening tool (MUST) were cross-sectionally assessed. Nailfold video-capillaroscopy(NVC) was performed to evaluate microvasculopathy. RESULTS: SSc patients who are at medium to high risk for malnutrition (n=20); had more frequently limited pulmonary function, lung involvement, pulmonary hypertension, capillary rarefaction and NVC late pattern than those at low risk for malnutrition. Capillary rarefaction (≤6/mm) was shown to be independently associated with medium to high risk for malnutrition defined by using MUST. Capillary rarefaction and severe skin involvement were found to be independently related to 'severe' or 'very severe' GI disease defined by using UCLA SCTC GIT 2.0. UCLA SCTC GIT 2.0 scores were not found to be good discriminative in patients at risk for malnutrition allowing for a ROC curve of area under the curve (AUC)<0.8). CONCLUSIONS: Assessment of gastrointestinal complaints and nutritional status by using symptom based questionnaires reflected the severity of GI disease and malnutrition including some limitations. Capillary rarefaction and severe skin involvement might be determining factors for malnutrition risk and severe GI disease.
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Enfermedades Gastrointestinales , Desnutrición , Rarefacción Microvascular , Esclerodermia Sistémica , Humanos , Microcirculación , Índice de Severidad de la Enfermedad , Encuestas y CuestionariosRESUMEN
Purpose: To describe posterior segment findings of antiphospholipid syndrome (APS) and compare them with systemic lupus erythematosus (SLE).Methods: A total of 11 patients with primary APS, 29 secondary APS patients, and 29 SLE patients without APS were included. All patients were referred from rheumatology clinic for detailed ophthalmologic examination. When patients had suspicious lesions, fundus fluorescein angiography was performed (n = 56).Results: The most common retinal examination finding was peripheral venous tortuosity (17.5%) in APS, which was not observed in SLE group. Common FFA findings were pigment epithelial window defects (10%) and vascular filling delays (7.5%) in APS, which were observed in 27.5% and 3.5% of patients with SLE consecutively.Conclusion: Venous tortuosity was significantly more in patients with APS. There was no significant difference for other ocular findings between the groups. Ocular complication rate was lower compared to earlier reports, probably due to better management of disease activity with current treatment protocols.
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Síndrome Antifosfolípido/complicaciones , Lupus Eritematoso Sistémico/complicaciones , Enfermedades de la Retina/etiología , Epitelio Pigmentado de la Retina/patología , Vena Retiniana/patología , Adolescente , Adulto , Síndrome Antifosfolípido/diagnóstico , Femenino , Angiografía con Fluoresceína , Humanos , Lupus Eritematoso Sistémico/diagnóstico , Masculino , Persona de Mediana Edad , Enfermedades de la Retina/diagnóstico , Microscopía con Lámpara de Hendidura , Tonometría Ocular , Agudeza Visual , Adulto JovenRESUMEN
OBJECTIVE: Approximately 30-45% of patients with familial Mediterranean fever (FMF) have been reported to have attacks despite colchicine treatment. Currently, data on the treatment of colchicine-unresponsive or colchicine-intolerant FMF patients are limited; the most promising alternatives seem to be anti-interleukin-1 (anti-IL-1) agents. Here we report our experience with the off-label use of anti-IL-1 agents in a large group of FMF patients. METHODS: In all, 21 centers from different geographical regions of Turkey were included in the current study. The medical records of all FMF patients who had used anti-IL-1 treatment for at least 6 months were reviewed. RESULTS: In total, 172 FMF patients (83 [48%] female, mean age 36.2 years [range 18-68]) were included in the analysis; mean age at symptom onset was 12.6 years (range 1-48), and the mean colchicine dose was 1.7 mg/day (range 0.5-4.0). Of these patients, 151 were treated with anakinra and 21 with canakinumab. Anti-IL-1 treatment was used because of colchicine-resistant disease in 84% and amyloidosis in 12% of subjects. During the mean 19.6 months of treatment (range 6-98), the yearly attack frequency was significantly reduced (from 16.8 to 2.4; P < 0.001), and 42.1% of colchicine-resistant FMF patients were attack free. Serum levels of C-reactive protein, erythrocyte sedimentation rate, and 24-hour urinary protein excretion (5,458.7 mg/24 hours before and 3,557.3 mg/24 hours after) were significantly reduced. CONCLUSION: Anti-IL-1 treatment is an effective alternative for controlling attacks and decreasing proteinuria in colchicine-resistant FMF patients.
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Sistemas de Liberación de Medicamentos/métodos , Fiebre Mediterránea Familiar/tratamiento farmacológico , Fiebre Mediterránea Familiar/epidemiología , Interleucina-1/administración & dosificación , Uso Fuera de lo Indicado , Adolescente , Adulto , Anciano , Fiebre Mediterránea Familiar/diagnóstico , Femenino , Humanos , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Resultado del Tratamiento , Turquía/epidemiología , Adulto JovenRESUMEN
BACKGROUND: Radiographic severity of ankylosing spondylitis (AS) shows such great variance that some patients never develop syndesmophytes throughout the entire disease span, whereas some develop bamboo spine relatively early. OBJECTIVE: To study the association between ERAP1, IL23R and PTGER4 single nucleotide polymorphisms (SNPs) and radiographic severity in AS patients. METHODS: rs27044 and rs30187 (ERAP1), rs11209032 (IL23R) and rs10440635 (PTGER4) SNPs were genotyped in 235 AS patients fulfilling the modified New York criteria. Patients were classified as mild- and severe-AS according to modified Stoke AS spinal score (mSASSS). Mild-AS is defined as having mSASSS of "0" following at least 10 years of disease duration. Severe-AS is defined as having mSASSS of >20 (patients with mild vertebral changes (i.e. squaring or erosions) were omitted for clear stratification) regardless of disease duration. RESULTS: The genotype distributions and allele frequencies of ERAP1 rs27044 and rs30187, IL23R rs11209032 and PTGER4 rs10440635 SNPs were similar in mild- (n=171, mSASSS=0, 55.6% HLA-B27 positive) and severe-AS patients (n=64, mSASSS=48.5±17.8, 73.4% HLA-B27 positive). After adjustment for clinical differences between groups (gender, disease duration, HLA-B27 and smoking status) by logistic regression analysis, none of the alleles in the investigated SNPs were found to be associated with radiographic severity of AS. CONCLUSION: In radiographically well-categorized AS patients, ERAP1 rs27044 and rs30187, IL23R rs11209032 and PTGER4 rs10440635 SNPs are not found to be associated with radiographic severity of AS.
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BACKGROUND/AIM: The aim of this study was to assess the relationships between the course of Behçet's disease (BD), disease-specific fears, and work productivity and activity impairment. MATERIALS AND METHODS: In this cross-sectional study, 110 consecutive BD patients were included. The Work Productivity and Activity Impairment questionnaire was used. RESULTS: In the group of employed patients, 30.41% had missed work during the previous week. The mean percentages of daily activity impairment were higher in patients with musculoskeletal involvement (39.81 ± 33.61%) compared to those without (23.48 ± 32.45%) (P = 0.008). A greater decrease in working hours was observed in patients with eye involvement (45.52 ± 15.29 h) compared to those without (54.15 ± 15.29 h) (P = 0.007). More of the male patients (67.8%) were afraid of losing their jobs compared to females (30%) (P = 0.000). CONCLUSION: The highest levels of lost productivity and the most severe effects on daily life are consequences of eye and musculoskeletal involvement in the study population. More effective therapeutic approaches are required to improve the working lives of patients with BD. Moreover, male patients had a higher fear of losing their jobs, suggesting a match between the expected clinical course and the predictions of BD patients.
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Síndrome de Behçet , Eficiencia/fisiología , Absentismo , Adulto , Síndrome de Behçet/epidemiología , Síndrome de Behçet/fisiopatología , Síndrome de Behçet/psicología , Estudios Transversales , Femenino , Actividades Humanas/psicología , Actividades Humanas/estadística & datos numéricos , Humanos , Masculino , Persona de Mediana Edad , Dolor , Evaluación de Capacidad de TrabajoRESUMEN
OBJECTIVES: Interleukin-17 (IL-17) has been associated with the pathogenesis of various autoimmune/inflammatory diseases. The aim of this study was to investigate the expression of Th17-related immunity in an innate immunity-dominated vasculitis, namely Behcet's disease (BD). METHODS: Peripheral blood mononuclear cells from 37 patients (age: 38.5 ± 9.8 years) with BD, and 25 healthy controls (HC) (age: 39.1 ± 9.3 years), were cultured in Th17-inducing conditions (IL-6, Phytohemagglutinin (PHA), IL-1ß, and IL-23) for 6 days. Cultured cells were stained with CD4, CD8, CD3, TCR gamma/delta, CD19, interferon-γ (IFN-γ), and IL-17 antibodies to determine the intracellular cytokine secretion by flow cytometry. RESULTS: IL-17 expression by CD8+ and γδ+ T cells was higher in BD compared to HC (p = 0.004, p = 0.003, respectively). No differences were observed between the groups in the IL-17 production by B cells. Under Th17-inducing conditions, production of IFN-γ by CD4+, CD8+, and γδ+ T cells was also higher in BD compared to HC (p < 0.05 in all). CONCLUSION: Our results suggest that under Th17-stimulating conditions, T cells express both IL-17 and IFN-γ in BD. More prominent IL-17 and IFN-γ production by all lymphocyte subsets in BD might be associated with the increased innate responses, early tissue neutrophil infiltrations and late adaptive immunity in BD.
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Síndrome de Behçet/inmunología , Medios de Cultivo/farmacología , Leucocitos Mononucleares/efectos de los fármacos , Células TH1/efectos de los fármacos , Células Th17/efectos de los fármacos , Adulto , Linfocitos B/efectos de los fármacos , Linfocitos B/inmunología , Linfocitos B/patología , Síndrome de Behçet/patología , Estudios de Casos y Controles , Medios de Cultivo/química , Femenino , Humanos , Inmunidad Innata , Inmunofenotipificación , Interferón gamma/biosíntesis , Interferón gamma/metabolismo , Interleucina-17/biosíntesis , Interleucina-17/metabolismo , Interleucina-1beta/farmacología , Interleucina-23/farmacología , Interleucina-6/farmacología , Leucocitos Mononucleares/inmunología , Leucocitos Mononucleares/patología , Activación de Linfocitos/efectos de los fármacos , Masculino , Persona de Mediana Edad , Fitohemaglutininas/farmacología , Cultivo Primario de Células , Células TH1/inmunología , Células TH1/patología , Células Th17/inmunología , Células Th17/patologíaAsunto(s)
Fármacos Dermatológicos/efectos adversos , Erupciones por Medicamentos/etiología , Infliximab/efectos adversos , Pitiriasis Rubra Pilaris/inducido químicamente , Fármacos Dermatológicos/administración & dosificación , Erupciones por Medicamentos/patología , Femenino , Humanos , Infliximab/administración & dosificación , Persona de Mediana Edad , Pitiriasis Rubra Pilaris/patología , Arteritis de Takayasu/tratamiento farmacológicoRESUMEN
Abstract/ Resumo Behcet's disease (BD) is a multisystem inflammatory disorder characterized by recurrent oral and genital ulcers, skin lesions and uveitis. The nervous system involvement of BD, neuro-Behcet's disease (NBD), is one of the important causes of mortality of the disease. Herein, we present a 29-year-old male with parenchymal NBD who has progressed rapidly and was managed with an uncommon aggressive immunosuppresive combination therapy. The patient first presented six years ago with vertigo and difficulty in talking and walking. On examination, he had oral ulcers, acneiform lesions on the torso, genital ulcer scar, dysartria, and ataxia. Along with the magnetic resonance imaging (MRI) findings, the patient was diagnosed as NBD. After pulse methylprednisolone (1g/day, 3 days) and 8 courses of 1g/month iv cylophosphamide therapy, he was put on azathioprine and oral methlyprednisolone. On the 4th year of the maintenance therapy, he was admitted with NBD relapse which was treated with 3 days of iv 1g pulse methlyprednisolone. One year after the last relapse, the patient voluntarily stopped medications and presented with global aphasia, right hemihypoesthesia and quadriparesis. MRI findings were suggestive of NBD relapse. After exclusion of infection, pulse methylprednisolone was started but no improvement was observed. Considering the severity of the NBD, the patient was put on methylprednisolone (1mg/kg/day), iv cylophosphamide (1g) and adalimumab 40 mg/14 days subcutaneously with appropriate tuberculosis prophylaxis. Neurological examination and MRI findings after 4 weeks showed dramatic improvement however patient developed pulmonary tuberculosis. Methylprednisolone dose was decreased (0.5mg/kg/day) and quadruple antituberculosis therapy was started. Patient was discharged with 5/5 muscle strength in extremities without any respiratory symptoms 2 months after first presentation. Prompt introduction of immunosuppressive therapy is crucial in NBD. Although combination of TNF inhibitors and cyclophoshamide is a rare therapeutic approach, it may be life-saving. However a higher awareness is required for opportunistic infections.
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Síndrome de Behçet/tratamiento farmacológico , Inmunosupresores/uso terapéutico , Enfermedades del Sistema Nervioso/tratamiento farmacológico , Inhibidores del Factor de Necrosis Tumoral , Adulto , Síndrome de Behçet/complicaciones , Quimioterapia Combinada , Humanos , Masculino , Enfermedades del Sistema Nervioso/etiologíaRESUMEN
Behçet's disease (BD) is characterized by recurrent oro-genital ulcers, mucocutaneous lesions, and serious organ involvement. We investigated the salivary microbiome in BD using high-throughput sequencing of the 16S rRNA V4 region. Stimulated saliva samples were collected from 31 BD patients and 15 healthy controls, and in 9 BD patients, a second saliva sample was collected following dental and periodontal treatment. Sequence analysis identified a total of 908 operational taxonomic units (OTUs) present across all samples. Patients had a microbial community structure that is significantly less diverse than healthy controls. The most overabundant species in BD was Haemophilus parainfluenzae, while the most depleted included Alloprevotella rava and species in the genus Leptotrichia. Periodontal treatment improved oral health indices in BD but had no short-term effect on bacterial community structure. Neither the BD-associated genetic risk locus within the HLA-B/MICA region nor being on immunosuppressive medications explained the differences between patients and controls.
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Síndrome de Behçet/microbiología , Microbiota/genética , ARN Ribosómico 16S/genética , Saliva/microbiología , Adolescente , Adulto , Bacterias/clasificación , Bacterias/genética , Síndrome de Behçet/tratamiento farmacológico , Síndrome de Behçet/genética , Análisis Discriminante , Femenino , Genotipo , Antígenos HLA-B/genética , Haemophilus parainfluenzae/genética , Haemophilus parainfluenzae/fisiología , Interacciones Huésped-Patógeno/efectos de los fármacos , Humanos , Inmunosupresores/uso terapéutico , Masculino , Persona de Mediana Edad , Índice de Higiene Oral , Índice Periodontal , Análisis de Secuencia de ADN , Adulto JovenRESUMEN
OBJECTIVE: To assess the performance of the new 2012 provisional European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) polymyalgia rheumatica (PMR) clinical classification criteria in discriminating PMR from other mimicking conditions compared with the previous 5 diagnostic criteria in a multicenter prospective study. METHODS: Patients older than 50 years, presenting with new-onset bilateral shoulder pain with elevated acute-phase reactants (APR), were assessed for the fulfillment of the new and old classification/diagnostic criteria sets for PMR. At the end of the 1-year followup, 133 patients were diagnosed with PMR (expert opinion) and 142 with non-PMR conditions [69 rheumatoid arthritis (RA)]. Discriminating capacity, sensitivity, and specificity of the criteria sets were estimated. RESULTS: Discriminating capacity of the new clinical criteria for PMR from non-PMR conditions and RA as estimated by area under the curve (AUC) were good with AUC of 0.736 and 0.781, respectively. The new criteria had a sensitivity of 89.5% and a specificity of 57.7% when tested against all non-PMR cases. When tested against all RA, seropositive RA, seronegative RA, and non-RA control patients, specificity changed to 66.7%, 100%, 20.7%, and 49.3%, respectively. Except for the Bird criteria, the 4 previous criteria had lower sensitivity and higher specificity (ranging from 83%-93%) compared with the new clinical criteria in discriminating PMR from all other controls. CONCLUSION: The new 2012 EULAR/ACR clinical classification criteria for PMR is highly sensitive; however, its ability to discriminate PMR from other inflammatory/noninflammatory shoulder conditions, especially from seronegative RA, is not adequate. Imaging and other modifications such as cutoff values for APR might increase the specificity of the criteria.
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Artritis Reumatoide/diagnóstico , Polimialgia Reumática/diagnóstico , Dolor de Hombro/diagnóstico , Anciano , Diagnóstico Diferencial , Femenino , Humanos , Masculino , Persona de Mediana Edad , Polimialgia Reumática/clasificación , Estudios Prospectivos , Sensibilidad y EspecificidadRESUMEN
OBJECTIVE: The aim of this study is to evaluate the demographic characteristics of critically ill patients and to determine intensive care unit (ICU) mortality and its predictors. METHODS: This study was undertaken in the Istanbul Medeniyet University Göztepe Training and Research Hospital Medical ICU. Between May 2012 and January 2013, 111 patients (53 female, 58 male; mean age, 73.79±14.73, mean length of ICU length stay, 9.1±10.7; prevalence of geriatric patients, 77.5%) were admitted to the ICU. The common indications for ICU admission, prevalence of mechanical ventilation support, hematological and biochemical parameters and their effects on mortality were assessed. RESULTS: The common indications for ICU admission were hemodynamic instability (48.6%), respiratory failure (27.9%) and sepsis (15.3%). Hypertension (46.8%) was the most common comorbidity. Prevalance rates of heart failure and diabetes mellitus were 32.4% and 25.2% respectively. Mortality rate was 52.3% in all patients. Approximately 80% of all deaths was observed within the first fifteen-day. In additon, mortality rate (85.7%) was prominent within patients in need of the mechanical ventilation support. Mechanical ventilation requirement, increased ferritin and vitamin B12 levels were independent risk factors for mortality in critically ill patients (p<0.01, for all). CONCLUSION: Mortality rate was higher in medical ICU. Herein, increased prevalence of geriatric population, concomitant comorbidities and mechanical ventilation requirements may play role.