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OBJECTIVES: Recurrent monoarthritis (RM) is a major challenge of many rheumatic diseases. Ablation is a well-known technique in the treatment of benign or malign lesions of different etiologies. We aimed to to investigate the success and safety of microwave ablation (MWA) as an adjunctive therapy in a cohort of medical treatment-resistant RM. METHODS: Patients with RM associated with different inflammatory diseases were included. MWA was performed after measuring the size of synovial hypertrophy with 15 or 20-watt power and different durations until microbubbles were shown indicating necrosis. Both clinical and radiologic data were recorded. RESULTS: We applied MWA in total of 24 knee joints of 10 female and 12 male patients aged between 22-71 years. Median intra-articular aspiration (IAA) need in the last 6 months before MWA was 5 (0-15). The median follow-up was 10 (3-16) months. Overall IAA count in the last 6 months before MWA in total of 144 months was 129 and decreased to 7 in post-MWA in total of 226 months (0.89 vs 0.03 per month, p< 0.001). The second MWA session was needed for 3 patients and a third session for 1. Functional disability and pain scores were improved significantly (median score from 9 to 1, p< 0.00001, in both). In magnetic resonance imaging, follow-up significant regression in synovial hypertrophy size was shown especially after 6th month. No complication was observed during the procedure or follow-up. CONCLUSION: As a less invasive technique compared with the surgical approach, MWA of synovial hypertrophy showed significant clinical improvement in RM safely. MWA seems promising as a treatment option candidate in the management of RM.
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Rheumatoid arthritis (RA) is a chronic, inflammatory systemic disorder of synovial joints and results in polyarthritis, chronical degeneration, and finally deformities and ankylosis in severe cases. Synovitis and pannus formation are results of inflammatory changes and lead into restriction in joint movement. Shoulders are among the later affected and larger joints and formation of synovitis in early active stages and pannus in later stages might be concluded with frozen shoulder and severe impairment in functionality. These late-term changes cannot be controlled with systemic or local anti-inflammatory agents and synovectomy is chosen in some cases. However, the results are not satisfactory and recurrence is common. In this case report, we presented a case of RA with severe shoulder pain, restricted movement due to synovial hypertrophy, and pannus formation which are resistant to local and systemic interventions and not suitable for surgical or chemical synovectomy. Microwave ablation (MWA) was performed successfully without any complication and she well responded in terms of DAS-28, functional, and pain scores. Range of motion and funcitonal restriction were recovered. This case report describes the use and promising results of MWA in RA with severe synovial hypertrophy and pannus formation even in the absence of active arthritis and effusion. MWA is a safe and minimally invasive technique that can be easily performed in coordinance of rheumatologists and interventional radiologists in proper cases.
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Artritis Reumatoide , Hipertrofia , Microondas , Humanos , Artritis Reumatoide/complicaciones , Artritis Reumatoide/cirugía , Artritis Reumatoide/diagnóstico por imagen , Femenino , Microondas/uso terapéutico , Articulación del Hombro/diagnóstico por imagen , Articulación del Hombro/cirugía , Persona de Mediana Edad , Técnicas de Ablación/métodos , Imagen por Resonancia Magnética/métodos , Rango del Movimiento Articular , Membrana Sinovial/diagnóstico por imagen , Membrana Sinovial/patologíaRESUMEN
OBJECTIVE: The skin pathergy test (SPT) is an important tool in the diagnosis of Behçet disease (BD), but its decreasing sensitivity over years has resulted in less frequent use in the clinical practice. This study aimed to improve the sensitivity of the SPT without compromising its specificity. METHODS: BD patients, patients with other inflammatory diseases, recurrent aphthous stomatitis, and healthy controls comprised the study group. The SPT was conducted using 20G needle and 21G lancet pricks, or with additional application of 23-valent polysaccharide pneumococcal vaccine (PS-23), Alum, or ATP to the prick site. Development of erythema and induration at 24 h/48 h were evaluated by the same observer. Induration (≥2 mm) with erythema at 48 h was accepted as a positive reaction. Proinflammatory cytokine production following stimulation with lipopolysaccharide or PS-23 was investigated by whole-blood assay (WBA) in a subgroup. RESULTS: Stimulation of the forearm skin by PS-23 and a 20G needle prick showed the highest sensitivity and specificity in BD (64.3% and 100%, respectively), especially in patients with active disease (80.3% and 100%, respectively), compared with a sensitivity of 4.8% in all and 6.1% in active patients using a single 20G prick. A positive result was associated with active disease and no use of immunosuppressives. In WBA, increased IL-1ß and IL-1Ra production in response to PS-23 was observed in the group with active BD, while the cytokine response to lipopolysaccharide was similar in all groups. CONCLUSIONS: The SPT conducted using a 20G needle prick and PS-23 antigens was shown to be a promising tool for the diagnosis of BD owing to its improved sensitivity compared with the standard approach.
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Síndrome de Behçet , Humanos , Síndrome de Behçet/diagnóstico , Lipopolisacáridos , Pruebas Cutáneas/métodos , Streptococcus pneumoniae , Vacunas Neumococicas , CitocinasRESUMEN
INTRODUCTION: Imatinib is an orally administered tyrosine kinase inhibitor with wide clinical use in different indications from solid tumors to hematologic malignancies. Inclusion body myositis (IBM) is an acquired myopathy of both inflammatory and degenerative nature. CASE REPORT: We present an 81 years old male with a history of gastrointestinal stromal tumor (GIST) operated 8 years ago and was evaluated for the progressive loss of weight and muscle strength leading to total immobilization in 6 months. He was under imatinib for 8 years despite the remission of GIST. Physical examination disclosed diffuse loss of muscle strength, most prominently involvement of distal upper and proximal lower extremity in an asymmetrical pattern with normal serum creatinine kinase level (CK). Further investigations including bilateral thigh MRI, electromyography (EMG), and PET/CT suggested myositis and degenerative myopathy and ruled out any malignancy. Quadriceps femoris biopsy proved the diagnosis of IBM and no trigger except for imatinib was displayed. MANAGEMENT AND OUTCOME: Clinical improvement in terms of weight loss and muscle weakness was achieved after the discontinuation of imatinib. DISCUSSION: This is the first case of IBM associated with prolonged use of imatinib not reported in the literature so far. Since imatinib is widely used in different conditions, it is important to be aware of even its rare adverse effects. Poor response of IBM to conventional immunosuppressive agents enhances the value of etiology identification to relieve symptoms in addition to supportive care.
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Artritis , Reumatología , Sinovitis , Humanos , Microondas/uso terapéutico , Sinovitis/cirugíaRESUMEN
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/patologíaRESUMEN
BACKGROUND: Indomethacin is a nonsteroidal anti-inflammatory drug, which is known to produce serious side effects, causing ulcerative lesions. Nesfatin-1, a newly identified anorexigenic peptide, was recently shown to have neuroprotective effects. The aim of the study was to investigate the anti-inflammatory effects of nesfatin-1 on indomethacin-induced gastric ulcer. MATERIALS AND METHODS: After a 24-h starvation period, ulcer was induced in Sprague-Dawley rats by subcutaneous administration of indomethacin (25 mg/kg), whereas control group received vehicle. Fifteen minutes after ulcer induction, rats were treated with either saline or nesfatin-1 (0.1, 0.3, or 1 µg/kg, intraperitoneally). At the fourth hour, all rats were decapitated and their trunk blood was collected for tumor necrosis factor (TNF)-α and interleukin (IL)-6 measurements. Stomach samples were examined microscopically and analyzed for myeloperoxidase (MPO) activity, malondialdehyde (MDA), glutathione (GSH), luminol-, and lucigenin-enhanced chemiluminescence (CL) levels. RESULTS: Ulcer induction increased serum TNF-α; and IL-6 levels, gastric CL and MDA levels and MPO activity but decreased gastric GSH content (P < 0.05-0.001). On the other hand, 0.1 µg/kg dose of nesfatin-1 reduced microscopic and macroscopic damage scores, decreased MPO activity and MDA levels, CL and IL-6 levels, whereas gastric GSH was replenished (P < 0.01). However, indomethacin-induced increase in TNF-α level was abolished at only 1 µg/kg dose of nesfatin-1 (P < 0.01). CONCLUSIONS: Nesfatin-1 alleviated indomethacin-induced gastric injury, suggesting that the anti-inflammatory and gastroprotective effects of nesfatin-1 on oxidative gastric damage could be implemented by supporting the balance in oxidant and antioxidant systems while inhibiting the generation of pro-inflammatory mediators.
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Antioxidantes/farmacología , Depresores del Apetito/farmacología , Proteínas de Unión al Calcio/farmacología , Proteínas de Unión al ADN/farmacología , Indometacina/toxicidad , Proteínas del Tejido Nervioso/farmacología , Úlcera Gástrica/inducido químicamente , Úlcera Gástrica/tratamiento farmacológico , Animales , Antiinflamatorios no Esteroideos/toxicidad , Interacciones Farmacológicas , Femenino , Vaciamiento Gástrico/efectos de los fármacos , Glutatión/metabolismo , Inyecciones Intraperitoneales , Interleucina-6/sangre , Masculino , Malondialdehído/metabolismo , Nucleobindinas , Peroxidasa/metabolismo , Ratas Sprague-Dawley , Factor de Necrosis Tumoral alfa/sangreRESUMEN
Cardiac involvement (CI) is rare in Behçet syndrome (BS), but the important point is that CI may be the first manifestation of the disease. The presence of CI worsens the prognosis of BS, so early diagnosis and early initiation of immunosuppressive treatment (IST) are vital. Coronary aneurysm may develop spontaneously in these patients, or any vascular intervention may cause aneurysm with a pathergy-like reaction. The risk of restenosis is high after percutaneous coronary intervention or coronary artery bypass surgery applied without IST. Therefore, it should be kept in mind that IST constitutes the main step of treatment. Herein, we present a young male diagnosed with BS after acute coronary syndrome caused by coronary artery aneurysms and thrombosis.
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Síndrome Coronario Agudo , Síndrome de Behçet , Aneurisma Coronario , Humanos , Masculino , Síndrome de Behçet/complicaciones , Síndrome de Behçet/diagnóstico , Síndrome de Behçet/tratamiento farmacológico , Síndrome Coronario Agudo/diagnóstico por imagen , Síndrome Coronario Agudo/etiología , Aneurisma Coronario/etiología , Pronóstico , Puente de Arteria Coronaria/efectos adversos , Inmunosupresores/uso terapéuticoRESUMEN
PURPOSE: This study aims to evaluate the safety and effectiveness of genicular artery embolization (GAE) using lipiodol in comparison to imipenem/cilastatin (IPM-CS). MATERIALS AND METHODS: This retrospective study screened patients who underwent GAE between January 2022 and February 2023 for inclusion. Clinical outcomes were assessed at 1, 3, and 6 months post-procedure using the Visual Analog Scale (VAS) for pain and the Western Ontario and McMaster Universities Osteoarthritis Index (WOMAC) for pain, stiffness, functional capacity, and total scores. Technical and clinical success rates, complications, and patient-reported outcomes were assessed. RESULTS: A total of 42 patients were included in the study, with 13 patients treated with lipiodol and 29 with IPM-CS for GAE. Transient skin discoloration was noted in 23.1% of lipiodol patients and 31% of the IPM-CS group (p = 0.722). One patient (7.6%) in the lipiodol group developed knee edema and erythema due to drug-induced vasculitis (p = 0.309). Clinical success rates in the lipiodol group were 76.9% at 1 month, consistent at 3 months, and 69.2% at 6 months. For the IPM-CS group, success rates were 89.7, 86.2, and 75.9%, respectively, with no significant differences (p = 0.353, p = 0.657, p = 0.713). The median percentage change in WOMAC stiffness scores for the lipiodol group at 1, 3, and 6 months post-GAE were - 25%, - 16.7%, and - 16.7%, respectively, while the IPM-CS group showed decreases of - 40%, - 50%, and - 50%. Significant differences were found between the groups at all time points (p = 0.017, p = 0.009, and p = 0.002, respectively). CONCLUSION: Lipiodol shows comparable clinical success to IPM-CS in GAE.
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The triad of ascites, pleural effusion, and elevated cancer antigen-125 (CA-125) levels in the absence of ovarian malignancy in systemic lupus erythematosus patients is specifically named pseudo-pseudo Meigs' syndrome (PPMS) or Tjalma syndrome. In this case we reported a 33 years female patient with pleural effusion lasting for 3 years and new onset progressive massive ascites and increased level of CA-125. After she was evaluated for an underlying benign and malign ovarian tumor or any other malignancies, serologic tests were requested with respect to progressive renal dysfunction, proteinuria, lymphopenia, anemia, and effusion. She was diagnosed with systemic lupus erythamatosus (SLE) and renal biopsy showed class-V lupus nephritis. Immunosuppressive treatment led to improvement in both SLE activity and components of PPMS, including massive ascites and pleural effusion and without the need of diuretics. Co-existence of unexplained CA-125 increase, pleural effusion, and ascites might be related to PPMS and detailed examination to exclude malignancy and early and effective treatment of SLE are the mainstay of management.
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PURPOSE: To determine the accuracy of final judgements of doctors at the emergency department (ED) and radiologists to differentiate between complicated and uncomplicated acute appendicitis, because these have different treatment options. METHODS: This prospective, multicenter study included adult patients with imaging-confirmed acute appendicitis, operated with intention to appendectomy. Both doctors at ED and radiologists assessed appendicitis severity as a final judgement of "uncomplicated" or "complicated" appendicitis. Doctors at ED integrated clinical, laboratory, and imaging findings. Radiologists relied solely on imaging findings. Outcomes were accuracy of these judgements for diagnosis of complicated appendicitis compared to the reference standard by an adjudication committee. RESULTS: After imaging, 1070 patients with confirmed acute appendicitis were included. Doctors at ED accurately labelled 656 of 701 (93.6%) patients with true uncomplicated appendicitis as uncomplicated, and 163 of 369 (44.2%) patients with true complicated appendicitis were labelled as complicated. Sensitivity, specificity, and positive and negative predictive values (PPV and NPV) for complicated appendicitis were 44.2%, 93.6%, and 78.4% and 76.1%, respectively. Comparable accuracy was found for the radiologist's assessment in 941 patients, with true positive rates of 92.2% (581 of 630 patients) for uncomplicated appendicitis and 46.6% (145 of 311 patients) for complicated appendicitis. CONCLUSION: More than half of all patients with true complicated appendicitis is incorrectly classified as uncomplicated appendicitis according to the judgements of doctors at ED, integrating clinical, laboratory, and imaging results, and of radiologists assessing diagnostic imaging. These judgements are thereby not sufficiently reliable in ruling out complicated appendicitis.
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Apendicitis , Servicio de Urgencia en Hospital , Radiólogos , Humanos , Apendicitis/diagnóstico por imagen , Apendicitis/diagnóstico , Apendicitis/cirugía , Estudios Prospectivos , Masculino , Adulto , Femenino , Diagnóstico Diferencial , Persona de Mediana Edad , Apendicectomía , Sensibilidad y Especificidad , Competencia Clínica , Enfermedad Aguda , Valor Predictivo de las Pruebas , Tomografía Computarizada por Rayos XRESUMEN
Pachydermaperiostosis (PDP) is a rare condition of connective tissue presenting with abnormal skin and skeletal findings that usually occur as a complication of an underlying disease, especially malignancies. We described a case of a patient with severe transfusion-dependent anemia and both skin and joint findings, diagnosed as PDP. The haematological assessment revealed myelofibrosis as the underlying disease, and treatment with ruxolitinib as the first-line choice was given by skipping hydroxyurea due to pancytopenia. The patient got benefits in arthralgia and amelioration of pancytopenia and a reduced spleen volume was observed, despite the negative result for JAK2 gene mutation. This is the first case of ruxolitinib being used in PDP with myelofibrosis, and it highlights the importance of evaluating PDP as not just a skin and joint problem but a result of systemic disease and individual-based treatment.
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Pancitopenia , Mielofibrosis Primaria , Humanos , Mielofibrosis Primaria/complicaciones , Mielofibrosis Primaria/diagnóstico , Mielofibrosis Primaria/tratamiento farmacológico , Nitrilos , PirimidinasRESUMEN
OBJECTIVE: The aim of present study is to compare ventricular and supraventricular arrhythmia incidences in subjects with and without mitral valve prolapse (MVP) syndrome and to examine if an association exists between ventricular arrhythmias and repolarization parameters in patients with MVP syndrome. METHODS: This cross-sectional study involved 41 subjects with MVP Syndrome and 41 subjects with palpitation but without MVP (control group). All subjects were subjected to lead-electrocardiogram, transthoracic echocardiography, and 24-h Holter monitoring to identify repolarization abnormalities, structural abnormalities, and supraventricular and ventricular arrhythmias. The QRS width, QTC interval, and Tpeak-Tend intervals were measured for each participant. RESULTS: The number of subjects who had premature ventricular contractions (PVCs), couplets, and non-sustained ventricular tachycardia (NSVTs) was significantly higher in the MVP group compared to the control group. Left ventricular end-systolic diameter (LVESD) and left ventricular end-diastolic diameter (LVEDD) and left atrial diameter were also significantly higher in the MVP group than the control group. QRS width and Tpeak-Tend interval were also significantly higher in subjects with MVP than the controls. Correlation analysis showed a positive correlation between the severity of mitral regurgitation (MR) and the number of PVCs and couplets, while there was a significant correlation between left atrium (LA) diameter and the number of the PVCs and NSVTs. CONCLUSION: Subjects with MVP experience ventricular arrhythmias more often including PVCs, couplets, and NSVTs compared to subjects without MVP. LVESD, LVEDD, LA diameter, QRS width, and Tpeak-Tend interval were increased in MVP subjects than those without MVP. There is an association between the severity of the MR and the frequency of the PVCs, couplets, or NSVTs.
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Vasculitis is the inflammatory changes in vessels of any size that usually have a systemic involvement with a quite variable clinical presentation affecting various organs. Although systemic presentation is more common, in some cases localized inflammation of vasculature of a single organ or limited branches of aorta are reported. Here we present, an isolated vasculitis of bilateral inferior thyroidal arteries in a female patient aged 49 years, who presented with neck pain and was diagnosed with ultrasonography and computed tomographic angiography. The clinical and imaging findings were managed successfully with glucocorticoid induction and addition of methotrexate to the treatment. Localized forms of vasculitis are rarer and the limited size of the affected area makes diagnostic investigations and management more complicated. Non-invasive imaging modalities rather than conventional angiography provide useful information in a safer and easier way. Isolated vasculitis of thyroidal arteries is an extremely uncommon site and should be excluded in case of unexplained neck pain, even in the presence of normal laboratory examinations, probably because of the size of the involved vessels.
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Dolor de Cuello , Vasculitis , Humanos , Femenino , Dolor de Cuello/diagnóstico por imagen , Dolor de Cuello/tratamiento farmacológico , Dolor de Cuello/etiología , Vasculitis/diagnóstico , Inflamación/complicaciones , Angiografía , ArteriasRESUMEN
Protracted febrile myalgia syndrome (PFMS) is a rare form of familial Mediterranean fever (FMF) characterised by prolonged myalgia. The duration of PFMS is much longer than a typical 2-5-day attack familial Mediterranean fever and lasts for 2-6 weeks until they treated with corticosteroids. Colchicine is not effective for control of PFMS's attacks. The attacks typically resolve with corticosteroid and/or IL-1 receptor blockers. Herein, we present a young adult without typical familial Mediterranean fever clinical features but presenting with atypical course and severe asymmetric muscle strength loss. Thigh magnetic resonance imaging confirmed inflammation and oedema and muscle biopsy showed no pathological findings. Electromyography revealed myopathic findings during attack-period, despite normal results in attack-free study. The patient was treated successfully with anakinra and remarkable rapid recovery in both muscular findings and acute phase reactants were observed. PFMS should be considered even in the absence of apparent FMF attack pattern and in the presence of unexpected severe muscle weakness, especially in areas endemic for FMF and long-lasting myalgia attacks.
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OBJECTIVE: Hyperinflammation (HI) that develops in week 2 of COVID-19 contributes to a worse outcome. Because week 2 laboratory findings can be relatively mild, the available criteria for classification of hemophagocytic lymphohistiocytosis or macrophage activation syndrome are not helpful. METHODS: Our study included a discovery cohort of patients from Turkey with symptomatic COVID-19 who were followed up while hospitalized during the initial wave and a replication cohort of hospitalized patients from a later period, all of whom required oxygen support and received glucocorticoids. Diagnosis of HI was made by an expert panel; most patients with COVID-19-associated HI (HIC) received tocilizumab or anakinra. Clinical and laboratory data from start day of treatment with tocilizumab or anakinra in HIC patients were compared with the data from day 5-6 in patients without HIC. Values maximizing the sensitivity and specificity of each parameter were calculated to determine criteria items. RESULTS: The discovery cohort included 685 patients, and the replication cohort included 156 patients, with 150 and 61 patients receiving treatment for HI, respectively. Mortality rate in HI patients in the discovery cohort (23.3%) was higher than the rate in patients without HI (3.7%) and the rate in patients in the overall replication cohort (10.3%). The 12-item criteria that we developed for HIC showed that a score of 35 provided 85.3% sensitivity and 81.7% specificity for identification of HIC. In the replication cohort, the same criteria resulted in 90.0% sensitivity for HIC; however, lower specificity values were observed because of the inclusion of milder cases of HIC responding only to glucocorticoids. CONCLUSION: The use of the 12-item criteria for HIC can better define patients with HIC with reasonable sensitivity and specificity and enables an earlier treatment start.
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COVID-19 , Humanos , Proteína Antagonista del Receptor de Interleucina 1/uso terapéutico , SARS-CoV-2 , Pandemias , Glucocorticoides/uso terapéuticoRESUMEN
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: The prognostic significance of complete cytogenetic response (CCyR) is well defined in patients with chronic phase chronic myeloid leukemia treated with imatinib as first-line therapy. However, the effect on outcomes of obtaining molecular response itself and the depth of it is not clear. In this study we aimed to determine the frequency of complete molecular response (CMR) during long-term follow-up and the clinical significance of CMR on patient outcomes and survival. PATIENTS AND METHODS: We retrospectively evaluated the files of 178 chronic phase chronic myeloid leukemia patients using imatinib therapy. Forty-seven patients with missing data were excluded from the study and the assessment was done in 131 patients. CMR was defined as undetectable BCR-ABL transcripts using real-time quantitative polymerase chain reaction with a sensitivity level of ≥ 104 in 2 consecutive analyses at least 3 months apart. Cytogenetic and molecular monitoring during treatment was performed according to the European LeukemiaNet recommendations criteria. Our primary objective was to analyze the association of deeper molecular response with differences in progression-free survival (PFS). RESULTS: Eighty-eight patients (67%) achieved CMR at any time in a median of 65 months of follow-up. The rate of CMR was higher in patients who achieved CCyR at 12 months and major molecular response (MMR) at 18 months. Fewer events occurred in the CMR group than the MMR group (26.1% vs. 50.0%). Overall survival was not different in both groups. CMR was associated with longer PFS with borderline significance. CONCLUSION: Prolonged imatinib therapy helps to achieve a deeper molecular response in the long-term. Achieving deeper molecular response at any time positively affects maintaining the cytogenetic and molecular responses, and decreases the transformation to accelerated and/or blastic phase. The slight prolongation in PFS did not reach statistical significance.
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Antineoplásicos/uso terapéutico , Mesilato de Imatinib/uso terapéutico , Leucemia Mielógena Crónica BCR-ABL Positiva/tratamiento farmacológico , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Citogenética/métodos , Supervivencia sin Enfermedad , Femenino , Proteínas de Fusión bcr-abl/metabolismo , Humanos , Leucemia Mielógena Crónica BCR-ABL Positiva/metabolismo , Leucemia Mielógena Crónica BCR-ABL Positiva/mortalidad , Masculino , Persona de Mediana Edad , Pronóstico , Estudios Retrospectivos , Adulto JovenRESUMEN
OBJECTIVE: To determine the frequency of subclinical atherosclerosis in patients with systemic sclerosis (SSc; scleroderma) compared to healthy subjects (HS) and rheumatoid arthritis (RA) patients and to determine the ability of cardiovascular (CV) risk indices in detecting SSc patients with subclinical atherosclerosis. METHODS: A total of 110 SSc patients (102 females and 8 males, mean ± SD age 50.5 ± 11.9 years), 110 age- and sex-matched RA patients, and 51 HS without CV disease were examined with ultrasonography (US). Carotid intima-media thickness (cIMT) >0.90 mm and/or carotid plaques were used as the gold standard for subclinical atherosclerosis (US+). Systematic Coronary Risk Evaluation (SCORE), QRisk II, and 2013 American College of Cardiology (ACC)/American Heart Association (AHA) CV risk indices were calculated. RESULTS: Twenty-one (19.1%) SSc patients, 24 (21.8%) RA patients, and 3 (5.9%) HS had subclinical atherosclerosis (SSc versus RA: P = 0.62, SSc versus HS: P = 0.029). cIMT in SSc was higher compared to HS (0.68 ± 0.15 mm versus 0.61 ± 0.10 mm; P = 0.008) but similar to RA patients (0.66 ± 0.14 mm; P = 0.82). Subclinical atherosclerosis in SSc was associated with age (odds ratio [OR] 1.07, P = 0.013), elevated erythrocyte sedimentation rate (OR 3.4, P = 0.045), and pulmonary arterial hypertension (OR 4.27, P = 0.012). Concerning CV risk indices, of the 21 US+ SSc patients only 0, 3 (14.2%), and 6 (28.6%) were classified as high CV risk according to SCORE, QRisk II, and ACC/AHA risk indices, respectively. CONCLUSION: Subclinical atherosclerosis in SSc patients is more frequent than in HS, but is as frequent as in RA patients in which accelerated atherosclerosis is clearly defined. CV risk indices for the general population are considerably insufficient to detect SSc patients with atherosclerosis.