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1.
Lupus ; 33(3): 298-311, 2024 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-38258530

RESUMEN

OBJECTIVE: This retrospective study aimed to conduct a comprehensive analysis of Turkish Systemic Lupus Erythematosus (SLE) patients of Caucasian ethnicity, focusing on their clinical, immunological, and therapeutic characteristics, damage accural and mortality. PATIENTS AND METHODS: We carried out a retrospective assessment of 300 SLE patients diagnosed between 2001 and 2017 at Kocaeli University Rheumatology Clinic. Demographic data, clinical manifestations, immunological profiles, treatment approaches, disease-related damage, and survival information were collected. RESULTS: The study population had a significant female predominance (89%) with a mean age of disease onset of 35.4 (SD:13.3) years. Hematological (72.6%) and mucocutaneous (72%) manifestations were the most common clinical findings, followed by arthritis (66.3%). Females had higher frequency of photosensitivity (p = 0.019), malar rash (p < 0.001), and alopecia (p = 0.014). Anti-dsDNA antibodies were detected in 61.6% of patients, while 57% of patients had hypocomplementemia. Secondary antiphospholipid syndrome was observed in 15.3% of patients; the most common manifestations included deep venous thrombosis (32.6%) and cerebrovascular accidents (30.4%). Lupus Nephritis (LN) affected 40.3% of the cohort. The most common pathologic finding was Class IV LN (30.5%). Eventually, 13 (4.3%) patients developed chronic kidney disease (CKD) and 4 had renal replacement therapies. Patients with LN had higher usage of pulse steroids, azathioprin, mycophenolate mofetil, cyclophosphamide, and rituximab (p < 0.001 for each).  In the juvenile-onset group (n = 31, 10.3%), an increased occurrence of malar rash (p = 0.009), nephritis (p = 0.034), hypocomplementemia (p = 0.001), positive anti-dsDNA (p = 0.007), anti-Sm (p = 0.046), anti-rib-P (p = 0.014) antibodies were observed. At least one damage parameter was observed in 32.6% patients with musculoskeletal manifestations being the most common. Thirteen patients were diagnosed with various malignancies, with cervical cancer being the most common (4 cases). The total 5 and 10-year survival rates were 92.5% and 86.7%, respectively. However, patients with CKD, had lower survival rates; 75% at 3 years and 60% at 15 years. Regression analysis demonstrated an association of CKD and  history of infections with decreased survival (p = 0.02, each). CONCLUSION: Ethnicity and geography influence the clinical diversity of SLE. Recognizing these disparities is crucial for tailoring patient care. Future inception cohort studies in Turkish SLE patients are necessary to address the limitations of retrospective research.


Asunto(s)
Exantema , Lupus Eritematoso Sistémico , Nefritis Lúpica , Insuficiencia Renal Crónica , Humanos , Femenino , Adolescente , Masculino , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/epidemiología , Lupus Eritematoso Sistémico/diagnóstico , Estudios Retrospectivos
2.
J Sex Med ; 21(3): 248-254, 2024 Feb 27.
Artículo en Inglés | MEDLINE | ID: mdl-38366657

RESUMEN

BACKGROUND: Sjögren's syndrome (SS) is an autoimmune systemic disease affecting many organs and systems, such as genital system. AIM: This study aimed to present the gynecological symptoms of patients who were followed up in an outpatient clinic because of primary Sjögren's syndrome (pSS) and secondary Sjögren's syndrome (sSS) and to show how the disease affected sexuality. METHODS: This study is a cross-sectional study conducted between 2019 and 2020. The study sample consisted of 60 pSS patients, 42 sSS patients, and 52 healthy control subjects. OUTCOMES: All the participants were questioned about sexuality, and completed the 36-item Short Form Survey, Hospital Anxiety and Depression Scale, Health Assessment Questionnaire, and Modified Hill questionnaire. RESULTS: The patients had a mean age of 55.6 ± 11.85 years in pSS, 59.39 ± 11.18 years in sSS, and 56.1 ± 10.46 years in healthy control subjects. Vaginal and vulvar dryness and dyspareunia were present at a significantly higher rate in SS, especially in pSS, compared with the control subjects. The Health Assessment Questionnaire score was significantly lower in the pSS group than in the sSS group. Arthralgia, myalgia, and fatigue were prominent in all SS patients. CLINICAL IMPLICATIONS: Gynecological symptoms, sexual ability, and the effects of the disease on sexuality should be questioned in all SS patients. STRENGTHS AND LIMITATIONS: It is very important that we evaluate the gynecological symptoms of both pSS and sSS patients and the effect of the disease on these symptoms. The small number of patients and healthy control subjects is a limitation. CONCLUSION: The gynecological and musculoskeletal symptoms negatively affected sexuality in patients with pSS and sSS, and the negative effect of the disease on sexuality was more pronounced in the pSS group.


Asunto(s)
Síndrome de Sjögren , Femenino , Humanos , Adulto , Persona de Mediana Edad , Anciano , Síndrome de Sjögren/complicaciones , Estudios Transversales , Conducta Sexual , Sexualidad , Vagina
3.
Adv Exp Med Biol ; 1436: 87-108, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-36648750

RESUMEN

The plasma membrane is not a uniform phospholipid bilayer; it has specialized membrane nano- or microdomains called lipid rafts. Lipid rafts are small cholesterol and sphingolipid-rich plasma membrane islands. Although their existence was long debated, their presence in the plasma membrane of living cells is now well accepted with the advent of super-resolution imaging techniques. It is interesting to note that lipid rafts function to compartmentalize receptors and their regulators and substantially modulate cellular signaling. In this review, we will examine the role of lipid rafts and caveolae-lipid raft-like microdomains with a distinct 3D morphology-in cellular signaling. Moreover, we will investigate how raft compartmentalized signaling regulates diverse physiological processes such as proliferation, apoptosis, immune signaling, and development. Also, the deregulation of lipid raft-mediated signaling during tumorigenesis and metastasis will be explored.


Asunto(s)
Caveolas , Microdominios de Membrana , Microdominios de Membrana/metabolismo , Membrana Celular/metabolismo , Caveolas/metabolismo , Transducción de Señal/fisiología , Fosfolípidos/metabolismo
4.
Rheumatol Int ; 42(3): 529-534, 2022 03.
Artículo en Inglés | MEDLINE | ID: mdl-34091705

RESUMEN

Takayasu arteritis (TA) is a large-cell vasculitis, and is not usually associated with avascular necrosis (AVN). The objective of the study was to investigate any association between TA and AVN, including the possible pathogenic effect of glucocorticoid (GCs) use. The study design was retrospective and cross sectional. TA patients were enrolled in the study. Demographic variables, disease activity, treatments, physician global assessment, Indian Takayasu Clinical activity score 2010, and Kerr criteria were recorded. Logistic regression analysis was performed to identify predictors of AVN. A total of 29 patients were assessed. AVN was observed in four (13.8%) patients with TA. Male gender and elevated C-reactive protein (CRP) were found to be significantly associated with AVN (p = 0.001 and p = 0.006, respectively). While type IIb TA was more common in patients with AVN (n = 2, 50%), type V was more likely in the absence of AVN (n = 13, 52%). Descending aorta and thoracic aorta were usually involved in patients with AVN (both, n = 3, 75%). In multivariate logistic regression, increased CRP levels were the only predictor for AVN (OR = 1.183, 95% Cl = 1.025-1.364, p = 0.021). No association was identified between AVN in TA patients and either duration or cumulative dose of GCs. The present study found that higher CRP levels and male gender were associated with AVN in patients with TA.


Asunto(s)
Osteonecrosis/complicaciones , Arteritis de Takayasu/complicaciones , Adulto , Proteína C-Reactiva/análisis , Causalidad , Estudios Transversales , Femenino , Glucocorticoides/uso terapéutico , Humanos , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Factores de Riesgo , Arteritis de Takayasu/tratamiento farmacológico
5.
Aging Clin Exp Res ; 34(4): 913-925, 2022 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-34731449

RESUMEN

BACKGROUND: Even though studies have indicated the usefulness and safety of endobronchial ultrasound-transbronchial needle aspiration (EBUS-TBNA), elderly patient data are limited due to the small sample sizes. AIM: We aimed to evaluate usage and safety of EBUS-TBNA in elderly population. METHODS: This single-center retrospective study was conducted with patients who underwent an EBUS-TBNA procedure between September 2011 and December 2019. The patients were categorized into two groups: those aged 65 years or older (elderly group) and those younger than 65 years (younger group). RESULTS: 2444 patient data, 1069 of which were in the elderly group, were analyzed. The cytological examination of EBUS-TBNA identified specimen adequacy in 96.8% of patients. One hundred and thirty patients (5.3%) experienced complications, with similar complication rates recorded in both the elderly and younger groups (5.4% vs 5.2%, p: 0.836). Logistic regression analyses revealed that age, and presence of hypertension, diabetes mellitus, coronary artery disease and malignancy are associated significantly with complication-related EBUS-TBNA. For the lymph nodes with a final diagnosis of malignancy, the sensitivity, specificity, positive predictive value, negative predictive value and accuracy of EBUS-TBNA revealed a diagnostic performance in excess of 90% except for metastasis and lymphoma. CONCLUSION: EBUS-TBNA can be considered a safe and effective technique in patients aged 65 years and over.


Asunto(s)
Biopsia por Aspiración con Aguja Fina Guiada por Ultrasonido Endoscópico , Ganglios Linfáticos , Anciano , Biopsia por Aspiración con Aguja Fina Guiada por Ultrasonido Endoscópico/efectos adversos , Biopsia por Aspiración con Aguja Fina Guiada por Ultrasonido Endoscópico/métodos , Humanos , Ganglios Linfáticos/diagnóstico por imagen , Ganglios Linfáticos/patología , Valor Predictivo de las Pruebas , Estudios Retrospectivos , Ultrasonografía
6.
Scott Med J ; 67(1): 18-27, 2022 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-35147461

RESUMEN

BACKGROUND: Guidelines recommend performing biomarker tests for epidermal growth factor receptor (EGFR), anaplastic lymphoma kinase (ALK), BRAF and ROS proto-oncogene-1(ROS1) genes and protein expression of programmed death ligand-1(PD-L1) in patients with non-small lung cell carcinoma (NSCLC). Studies reported that endobronchial ultrasound-transbronchial needle aspiration (EBUS-TBNA) can provide sufficient material for cancer biomarker analyses, but there are still concerns about the subject. AIM: The purpose of the study was to assess the adequacy of EBUS-TBNA for testing lung cancer biomarkers. METHODS: We retrospectively reviewed patients with NSCLC whose EBUS-TBNA was analysed for EGFR, ALK, ROS-1, BRAF and PD-L1 expression between December 2011 and December 2020. RESULTS: A total of 394 patients were enrolled in the study. EGFR mutation and ALK fusion were the most common studied biomarkers. EBUS-TBNA adequacy rate for biomarker tests was found 99.0% for EGFR, 99.1 for ALK, 97.2% for ROS1, 100% for BRAF and 99.3% for PD-L1 testing. Multivariate analysis revealed the histological type, history of treatment for NSCL, size, or 18-fluorodeoxyglucose uptake of sampled lesion did not show any association with TBNA adequacy for biomarker testing. CONCLUSION: EBUS-TBNA can provide adequate material for biomarker testing for EGFR, ALK, ROS-1, BRAF and PD-L1 expression.


Asunto(s)
Carcinoma de Pulmón de Células no Pequeñas , Neoplasias Pulmonares , Antígeno B7-H1/genética , Antígeno B7-H1/metabolismo , Biomarcadores de Tumor/metabolismo , Carcinoma de Pulmón de Células no Pequeñas/genética , Carcinoma de Pulmón de Células no Pequeñas/metabolismo , Carcinoma de Pulmón de Células no Pequeñas/patología , Biopsia por Aspiración con Aguja Fina Guiada por Ultrasonido Endoscópico , Receptores ErbB/genética , Humanos , Pulmón/metabolismo , Pulmón/patología , Neoplasias Pulmonares/patología , Proteínas Tirosina Quinasas , Proteínas Proto-Oncogénicas/genética , Proteínas Proto-Oncogénicas B-raf/genética , Especies Reactivas de Oxígeno , Estudios Retrospectivos
7.
Turk J Med Sci ; 52(5): 1737-1743, 2022 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-36422505

RESUMEN

BACKGROUND: Sjögren's syndrome is a systemic, autoimmune disease and can affect many organs and systems. In this study, we aimed to evaluate the respiratory manifestations, and the association between interstitial lung disease (ILD) and disease activity in primary SS (pSS) patients. METHODS: The study design was retrospective cross-sectional, and 151 patients followed up with a diagnosis of pSS between 2004 and 2019 were included in the study. Demographic and clinical data, laboratory results, chest radiographs and thorax computed tomography (CT) results were obtained from patient files and hospital imaging system. Thorax CT was requested from all patients with respiratory complaints and abnormalities in physical examination and pulmonary function test. Disease activity was calculated with EULAR pSS disease activity index (ESSDAI) and clinical European League Against Rheumatism SS Disease Activity Index (clin-ESSDAI). RESULTS: In our study, 97% of pSS patients were female, and the mean age was 55.9 ± 12 years, disease onset age was 45.5 ± 12.1 years, disease duration was 10.7 (1-38) years. According to CT findings of 120 patients, 35% had nodules, and 13.3% had ILD (62.5% nonspecific interstitial pneumonia, 25% lymphocytic interstitial pneumonia, 12.5% usual interstitial pneumonia). Bronchiectasis, emphysema, sequelae fibrotic changes, and pleural thickening was found in 3.3%, 5.8%, 15.8%, and 1.7% of patients, respectively. It was observed that there was a significant relationship between the presence of ILD and persistent cough, mediastinal LAP, low DLCO, high ESSDAI and clin-ESSDAI scores reflecting disease activity. DISCUSSION: The most common pulmonary manifestation in our patients was ILD. ILD was observed more frequently in patients with moderate and severe disease activity. Some of the ILD patients were diagnosed while they were asymptomatic. Even if they are asymptomatic, it is important to follow up the patients with physical examination, spirometry, DLCO and thorax CT.


Asunto(s)
Enfermedades Pulmonares Intersticiales , Síndrome de Sjögren , Humanos , Femenino , Adulto , Persona de Mediana Edad , Anciano , Masculino , Estudios Retrospectivos , Estudios Transversales , Enfermedades Pulmonares Intersticiales/diagnóstico por imagen , Enfermedades Pulmonares Intersticiales/etiología , Pulmón/diagnóstico por imagen
8.
Int J Clin Pract ; 75(1): e13645, 2021 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-32757291

RESUMEN

OBJECTIVE: Familial Mediterranean Fever (FMF) is an autoinflammatory disease that is commonly present with recurrent episodes of fever, peritonitis, pleuritis or arthritis. Enthesitis and sacroilitis can also be seen in FMF. Spondylitis is a less common manifestation of joint involvement in FMF and there are controversial publications about whether this involvement is FMF-related or coincidentally. The aim of this study was to provide a comparison between ankylosing spondylitis (AS) patients and FMF patients with AS. METHODS: A total of 404 patients who 360 of them was AS and 44 was FMF patients with AS (in accordance with Tel Hashomer) patients with AS (in accordance with modified New York criteria) were included in this study. All cases were evaluated retrospectively and patient's demographic and clinical data were recorded. RESULTS: The mean age was 34.5 ± 8.6 years and 61.4% of patients were female in FMF group. In AS group, the mean age was 41.2 ± 10.8 years and 67.8% of patients were male. In AS group, 92% of patients had inflammatory back pain, 51% had hip pain, 30% had heel pain, 14% had peripheral arthritis and 11% had uveitis. In FMF group, 98% of patients had inflammatory back pain, 59% had hip pain, 48% had heel pain, 43% had peripheral arthritis and 4.5% had uveitis. Syndesmophyte and enthesitis on X-ray were seen in 18% and 22% of AS patients, and 7% and 41% of FMF patients with AS, respectively. There were significant differences between AS patients and FMF patients with AS in terms of heel pain (P: .017), peripheral arthritis (P: .000) and enthesitis (P: .006). CONCLUSION: Peripheral arthritis and enthesitis were more frequent, and uveitis and syndesmophyte were less frequent in FMF patients with spondylitis than AS patients. When we look at gender differences, clinical and genetic features, it seems to be different condition from AS.


Asunto(s)
Artritis Reumatoide , Fiebre Mediterránea Familiar , Espondilitis Anquilosante , Adulto , Fiebre Mediterránea Familiar/complicaciones , Femenino , Humanos , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Factores Sexuales , Espondilitis Anquilosante/complicaciones , Espondilitis Anquilosante/epidemiología
9.
J Clin Rheumatol ; 26(6): 242-247, 2020 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-31094932

RESUMEN

OBJECTIVES: Tumor necrosis factor-related apoptosis-inducing ligand (TRAIL) belongs to the tumor necrosis factor (TNF) superfamily and is reported to play a role in autoimmune diseases. In this study, we aimed to measure serum TRAIL receptor 1 (TRAIL-R1) concentration and assess any phenotypic relationship in patients with ankylosing spondylitis (AS). METHODS: Fifty-three patients with AS were recruited from August 2014 to December 2014 cross-sectionally. Fifty-three sex- and age-matched healthy controls were also recruited. Serum TRAIL-R1 concentrations were measured using an enzyme-linked immunosorbent assay. The association between serum TRAIL-R1, TNF-α, disease activity indices, markers of systemic inflammation, and clinical features were evaluated. RESULTS: Serum TRAIL-R1 and TNF-α levels were increased in patients with AS compared with healthy controls (4.5 ± 2.3 vs 3.5 ± 2.3 pg/mL, p = 0.036; 3.8 [1.6-7.7] vs 2.0 [0.21-5.7] pg/mL, p = 0.048, respectively). Serum TRAIL-R1 displayed a medium positive correlation with serum TNF-α concentrations (r = 0.412; p = 0.002). Serum TRAIL-R1 concentration was higher in human leucocyte antigen (HLA)-B27-positive patients compared with non-HLA-B27 patients (5.5 ± 2.2 vs 3.1 ± 1.6 pg/mL, p < 0.001). No relationship was found between serum TRAIL-R1 concentration and disease activity scores. CONCLUSIONS: This study confirms that serum TRAIL-R1 levels are higher in AS patients than healthy controls. The persistence of significantly elevated serum TRAIL-R1 levels, even in patients with low disease activity or after excluding biologic treatment, and the association with HLA-B27 positivity, warrants further investigation due to the unclear role of TRAIL-R1 in the pathophysiology of AS.


Asunto(s)
Receptores del Ligando Inductor de Apoptosis Relacionado con TNF/sangre , Espondilitis Anquilosante , Apoptosis , Humanos , Espondilitis Anquilosante/diagnóstico
10.
Rheumatol Int ; 39(7): 1191-1200, 2019 07.
Artículo en Inglés | MEDLINE | ID: mdl-31025137

RESUMEN

Systemic sclerosis is associated with an increased prevalence/incidence of coronary artery disease. The aim of this study was to investigate epicardial adipose tissue (EAT) thickness which may contribute to cardio-metabolic risk in systemic sclerosis (SSc) patients without overt cardiac disease. EAT thickness was measured by transthoracic conventional Doppler echocardiography and compared in SSc patients (n = 47) and age- and sex-matched healthy controls (n = 36). The relationships between EAT thickness and markers of cardio-metabolic risk in SSc were examined. EAT thickness was significantly greater in patients with SSc compared to healthy controls (6 [7-5] vs 5 [6.75-3.25], p = 0.041). Compared to controls, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), leukocyte, neutrophil, B-type natriuretic protein (BNP), fasting plasma insulin and HOMA-IR were elevated (18 [31-10] vs 8.5 [18-4], p < 0.001; 0.4 [0.67-0.18] vs 0.21 [0.48-0.09], p = 0.012; 7510 [8731-5990] vs 6435 [7360-5195], p = 0.002; 4350 [5440-3570] vs 3390 [4168-2903], p < 0.001; 111 [185-74] vs 70 [127-70], p = 0.010; 6.7 [10.5-4.7] vs 4.7 [6.8-4.1], p = 0.008; 1.7 [2.6-1] vs 1.1 [1.7-0.9], p = 0.015, respectively). The total and low-density lipoprotein (LDL)-cholesterol were decreased in SSc patients (197 ± 45 vs 284 ± 36, p = 0.005; 118 [148-84] vs 140 [180-115], p = 0.003, respectively). In patients with SSc, the EAT thickness correlated positively with age, ESR, CRP, insulin, hemoglobin A1c and total and LDL-cholesterol (r = 0.574, p < 0.001; r = 0.352, p = 0.015; r = 0.334, p = 0.022; r = 0.290, p = 0.048; r = 0.317, p = 0.030; r = 0.396, p = 0.006 and r = 0.349, p = 0.016, respectively). Our study confirms that EAT thickness is greater in SSc patients compared to healthy controls using echocardiographic measurements. The results of our study suggest that EAT thickness is a candidate for atherosclerotic risk assessment in SSc.


Asunto(s)
Tejido Adiposo/diagnóstico por imagen , Pericardio/diagnóstico por imagen , Esclerodermia Sistémica/diagnóstico por imagen , Adulto , Anciano , Glucemia/análisis , Sedimentación Sanguínea , Proteína C-Reactiva/análisis , Ecocardiografía , Femenino , Humanos , Insulina/sangre , Resistencia a la Insulina/fisiología , Masculino , Persona de Mediana Edad , Factores de Riesgo , Esclerodermia Sistémica/sangre
12.
Clin Rheumatol ; 43(1): 259-267, 2024 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-38044416

RESUMEN

OBJECTIVES: Radiological alterations in psoriatic arthritis (PsA) are an established phenomenon frequently observed throughout the disease course. Our goal was to investigate the changes in the bone structure of PsA patients by conventional radiography. METHODS: This study designed as a retrospective cohort study and cross-sectional evaluation for disease activity. The disease activity and the severity of skin and nail involvement were assessed. The Simplified Psoriatic Arthritis Radiographic Score (SPARS) was used to investigate the radiological progression. Logistic regression analysis was used to determine the predictors of radiological changes. RESULTS: Joint space narrowing and bone proliferation in hands (p = 0.001 and p = 0.001, respectively) and joint space narrowing in feet (p = 0.047) were more common at the final evaluation than at the baseline assessment. Total scores of joint space narrowing and bone proliferation in hands and feet were higher at the last visit than at the initial assessment (p < 0.001). Male gender (p = 0.030, OR 4.32 (95%CI 1.15-16.15)], older age (for joint space narrowing [p = 0.026 OR 1.08 (95%CI 1.01-1.56)] and for proliferation [p = 0.025 OR 1.08 (95%CI 1.01-1.44)]), high Disease Activity index for Psoriatic Arthritis (DAPSA) scores at baseline [p = 0.032 OR 6.21 (95%CI 1.17-32.92)], and symmetrical polyarticular involvement at baseline [p = 0.025 OR 5.3 (95% CI 1.23-22.4)] were found as predictors of structural changes. CONCLUSION: By the end of the decade, joint space narrowing and proliferation were observed to be more common than erosion. Male gender, older age, higher initial DAPSA scores, and initial polyarticular involvement were identified as predictors of radiological damage. Key Points • The radiological changes of Psoriatic arthritis are a well-known entity. However, studies investigating the progression of joint involvement over time are scarce. • This study reveals that joint space narrowing and proliferation are the most prominent radiological alterations in Psoriatic Arthritis patients at the end of the decade. • Male gender, older age, higher baseline DAPSA scores, and initial polyarticular involvement are predictive factors influencing the progression of bone destruction  in Psoriatic Arthritis patients.


Asunto(s)
Artritis Psoriásica , Humanos , Masculino , Artritis Psoriásica/diagnóstico por imagen , Estudios Retrospectivos , Estudios Transversales , Radiografía , Mano , Índice de Severidad de la Enfermedad , Progresión de la Enfermedad
13.
Int J Rheum Dis ; 27(1): e14857, 2024 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-37578023

RESUMEN

Hyperimmunoglobulin D syndrome (HIDS) is a rare autoinflammatory disorder with autosomal recessive inheritance. It is caused by specific mutations in the mevalonate kinase gene (MVK). No treatment specific to HIDS has been approved to date; however, nonsteroidal anti-inflammatory drugs, steroids, colchicine, tumor necrosis factor-α inhibitors, and anti-interleukin-1 treatments are used, based on case reports and observational studies. Herein, we report a case with recurrent fever and arthritis attacks who did not respond to anakinra and was successfully treated with canakinumab. Long-term remission was achieved without any side effects with 300 mg canakinumab treatment every 4 weeks for 5 years.


Asunto(s)
Fiebre Mediterránea Familiar , Deficiencia de Mevalonato Quinasa , Humanos , Deficiencia de Mevalonato Quinasa/diagnóstico , Deficiencia de Mevalonato Quinasa/tratamiento farmacológico , Deficiencia de Mevalonato Quinasa/genética , Anticuerpos Monoclonales Humanizados/uso terapéutico , Antiinflamatorios no Esteroideos/uso terapéutico , Proteína Antagonista del Receptor de Interleucina 1/uso terapéutico , Fiebre Mediterránea Familiar/tratamiento farmacológico
14.
Exp Clin Transplant ; 22(5): 373-380, 2024 May.
Artículo en Inglés | MEDLINE | ID: mdl-38970280

RESUMEN

OBJECTIVES: We aimed to reveal the incidence of lateonset noninfectious pulmonary complications and bronchiolitis obliterans syndrome and risk factors involved in development. MATERIALS AND METHODS: In this cross-sectional study, we retrospectively investigated 745 patients who underwent allogeneic hematopoietic stem cell transplantation in our hospital between January 2000 and December 2020. We evaluated demographic characteristics, comorbidities, and hematopoietic stem cell transplantation characteristics to determine possible risk factors affecting development of lateonset noninfectious pulmonary complications and bronchiolitis obliterans syndrome. RESULTS: Of 745 patients, 8.9% (n = 66) had late-onset noninfectious pulmonary complications. Complications included 38 patients with bronchiolitis obliterans syndrome, 13 with venous thromboembolism, 8 with cryptogenic organizing pneumonia, 5 with pneumothorax, 4 with interstitial lung disease-restrictive graft-versus-host disease, 5 with bronchiectasis, 2 with pneumomediastinum, and 1 with pleural effusion. Patients with and without complications were not significantly differentin terms of smoking history, hematopoietic stem cell transplantation characteristics, and conditioning regimens. Patients with complications had higher busulfan and lower antithymocyte globulin use than those without complications (both P<.05). Patients with complications more commonly had hematopoietic stem cell transplantation from related donors and chronic graft-versus-host disease (P < .05). Patients with bronchiolitis obliterans syndrome had more frequent use of busulfan (P <.05) but less frequent use of total body irradiation (P <.05) and antithymocyte globulin (P <.05) than those without this syndrome. Rate of hematopoietic stem cell transplantation from a related donor (P < .05) and frequency of chronic graftversus-host disease (P < .001) were significantly higher in patients with bronchiolitis obliterans syndrome, presented with bronchiectasis (78.6%), air trapping (67.9%), bronchial wallthickening (53.6%), and mosaic attenuation (39.3%) in thorax computed tomography. Pretransplant spirometry did not predict bronchiolitis obliterans syndrome development. CONCLUSIONS: Determining risk factors for late-onset noninfectious pulmonary complications is needed to aid in prevention and follow-up.


Asunto(s)
Bronquiolitis Obliterante , Trasplante de Células Madre Hematopoyéticas , Humanos , Trasplante de Células Madre Hematopoyéticas/efectos adversos , Estudios Retrospectivos , Masculino , Femenino , Factores de Riesgo , Adulto , Estudios Transversales , Persona de Mediana Edad , Factores de Tiempo , Incidencia , Bronquiolitis Obliterante/etiología , Bronquiolitis Obliterante/epidemiología , Bronquiolitis Obliterante/diagnóstico , Resultado del Tratamiento , Adulto Joven , Enfermedades Pulmonares/etiología , Enfermedades Pulmonares/diagnóstico , Enfermedades Pulmonares/epidemiología , Medición de Riesgo , Adolescente , Turquía/epidemiología , Enfermedad Injerto contra Huésped/etiología , Enfermedad Injerto contra Huésped/diagnóstico , Enfermedad Injerto contra Huésped/epidemiología
15.
Diagn Cytopathol ; 2024 May 01.
Artículo en Inglés | MEDLINE | ID: mdl-38690675

RESUMEN

BACKGROUND: In patients with extrathoracic malignancies (ETM), granulomatous lymph adenopathy called sarcoid-like reactions (SLR) can be seen in the regional or draining lymph nodes. We hypothesized that SLR may be a sign of imminent metastasis and investigated the clinical course and rate of recurrence in patients with ETM and granulomatous mediastinal lymphadenopathy (MLN). METHODS: In this retrospective observational study, we reviewed the medical files of patients with known ETM and who underwent EBUS-TBNA for initial staging or detection of recurrence from 2011 to 2023. Patients with granulomatous MLN were included. RESULTS: Forty-one patients (29 female) enrolled in the study. Breast and colorectal carcinomas were the most common malignancies. A total of 81 lymph nodes were sampled. The final diagnosis of patients was five sarcoidosis, one tuberculosis, one second primary, one drug reaction, and 33 SLR. Among patients with SLR, in one patient lymph nodes progressed during the follow-up and were accepted as false-negative without confirmatory biopsy. The negative predictive value (NPV) of granulomatous MLN for metastasis was 97.05%. CONCLUSION: Granulomatous MLN may be due to tuberculosis, drug reaction, sarcoidosis, or SLR in patients with ETM. SLR has a high NPV in patients with ETM. Follow-up imaging rather than confirmatory biopsy is reasonable in these patients.

16.
J Investig Med ; 72(1): 88-99, 2024 01.
Artículo en Inglés | MEDLINE | ID: mdl-37840192

RESUMEN

The generalizability of artificial intelligence (AI) models is a major issue in the field of AI applications. Therefore, we aimed to overcome the generalizability problem of an AI model developed for a particular center for pneumothorax detection using a small dataset for external validation. Chest radiographs of patients diagnosed with pneumothorax (n = 648) and those without pneumothorax (n = 650) who visited the Ankara University Faculty of Medicine (AUFM; center 1) were obtained. A deep learning-based pneumothorax detection algorithm (PDA-Alpha) was developed using the AUFM dataset. For implementation at the Health Sciences University (HSU; center 2), PDA-Beta was developed through external validation of PDA-Alpha using 50 radiographs with pneumothorax obtained from HSU. Both PDA algorithms were assessed using the HSU test dataset (n = 200) containing 50 pneumothorax and 150 non-pneumothorax radiographs. We compared the results generated by the algorithms with those of physicians to demonstrate the reliability of the results. The areas under the curve for PDA-Alpha and PDA-Beta were 0.993 (95% confidence interval (CI): 0.985-1.000) and 0.986 (95% CI: 0.962-1.000), respectively. Both algorithms successfully detected the presence of pneumothorax on 49/50 radiographs; however, PDA-Alpha had seven false-positive predictions, whereas PDA-Beta had one. The positive predictive value increased from 0.525 to 0.886 after external validation (p = 0.041). The physicians' sensitivity and specificity for detecting pneumothorax were 0.585 and 0.988, respectively. The performance scores of the algorithms were increased with a small dataset; however, further studies are required to determine the optimal amount of external validation data to fully address the generalizability issue.


Asunto(s)
Aprendizaje Profundo , Neumotórax , Humanos , Inteligencia Artificial , Neumotórax/diagnóstico por imagen , Reproducibilidad de los Resultados , Estudios Retrospectivos , Algoritmos
17.
Semin Arthritis Rheum ; 66: 152446, 2024 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-38669786

RESUMEN

BACKGROUND: Immunosuppressive (IS) agents are recommended for the first-line treatment of patients with active Takayasu's arteritis (TAK) together with glucocorticoids (GCs). However, there is limited data comparing the efficacy and outcomes of different IS agents for this purpose. OBJECTIVES: In this study, we aimed to compare the outcomes of two most frequently used first-line IS agents, namely methotrexate (MTX) and azathioprine (AZA) in TAK patients. METHODS: TAK patients who received any IS agent in addition to GCs as the initial therapy were included in this multicentre, retrospective cohort study. Clinical, laboratory and imaging data of the patients were assessed. In addition, a matched analysis (cc match) using variables 'age', 'gender' and 'diffuse aortic involvement' was performed between patients who received MTX or AZA as the first-line IS treatment. RESULTS: We recruited 301 patients (F/M: 260/41, mean age: 42.2 ± 13.3 years) from 10 tertiary centres. As the first-line IS agent, 204 (67.8 %) patients received MTX, and 77 (25.6 %) received AZA. Less frequently used IS agents included cyclophosphamide in 17 (5.6 %), leflunomide in 2 (0.5 %) and mycophenolate mofetil in one patient. The remission, relapse, radiographic progression and adverse effect rates were similar between patients who received MTX and AZA as the first-line IS agent. Vascular surgery rate was significantly higher in the AZA group (23% vs. 9 %, p = 0.001), whereas the frequency of patients receiving ≤5 mg/day GCs at the end of the follow-up was significantly higher in the MTX group (76% vs 62 %, p = 0.034). Similarly, the rate of vascular surgery was higher in AZA group in matched analysis. Drug survival was similar between MTX and AZA groups (median 48 months, MTX vs AZA: 32% vs 42 %, p = 0.34). IS therapy was discontinued in 18 (12 MTX, 6 AZA) patients during the follow-up period due to remission. Among those patients, two patients had a relapse at 2 and 6 months, while 16 patients were still on remission at the end of a mean 69.4 (±50.9) months of follow-up. CONCLUSIONS: Remission, relapse, radiographic progression and drug survival rates of AZA and MTX were similar for patients with TAK receiving an IS agent as the first-line f therapy. The rate of vascular surgery was higher and the rate of GC dose reduction was lower with AZA compared to MTX at the end of the follow-up.


Asunto(s)
Azatioprina , Inmunosupresores , Metotrexato , Arteritis de Takayasu , Humanos , Arteritis de Takayasu/tratamiento farmacológico , Arteritis de Takayasu/diagnóstico por imagen , Femenino , Masculino , Adulto , Azatioprina/uso terapéutico , Metotrexato/uso terapéutico , Inmunosupresores/uso terapéutico , Estudios Retrospectivos , Persona de Mediana Edad , Resultado del Tratamiento , Glucocorticoides/uso terapéutico , Glucocorticoides/administración & dosificación
18.
Vaccines (Basel) ; 11(3)2023 Mar 02.
Artículo en Inglés | MEDLINE | ID: mdl-36992164

RESUMEN

OBJECTIVE: Rotavirus (RV) is one of the most common and important causes of acute gastroenteritis (AGE) in newborns and children worldwide. The aim of this study was to evaluate the effect of the RV vaccine on the natural history of RV infections using the neutrophil-lymphocyte ratio (NLR), platelet-lymphocyte ratio (PLR), and systemic immune inflammatory index (SII) as hematological indexes, clinical findings, and hospitalization. METHOD: Children aged 1 month to 5 years who were diagnosed with RV AGE between January 2015 and January 2022 were screened, and 630 patients were included in the study. The SII was calculated by the following formula: neutrophil × platelet/lymphocyte. RESULTS: Fever and hospitalization were significantly higher and breastfeeding was significantly lower in the RV-unvaccinated group than in the RV-vaccinated group. The NLR, PLR, SII, and CRP were significantly higher in the RV-unvaccinated group (p < 0.05). The NLR, PLR, and SII were significantly higher both in the non-breastfed group than in the breastfed group and in the hospitalized group than in the not hospitalized group (p < 0.05). CRP was not significantly different in either the hospitalization group or the breastfeeding group (p > 0.05). SII and PLR were significantly lower in the RV-vaccinated group than in the RV-unvaccinated group in both the breastfed and non-breastfed subgroups. For NLR and CRP, while there was no significant difference according to RV vaccination status in the breastfed group, there was a significant difference in the non-breastfed group (p value: <0.001; <0.001). CONCLUSIONS: Despite the low level of vaccine coverage, the introduction of RV vaccination had a positive impact on the incidence of RV-positive AGE and related hospitalizations in children. These results showed that breastfed and vaccinated children were less prone to inflammation because their NLR, PLR, and SII ratios were lower. The vaccine does not prevent the disease 100%. However, it can prevent severe disease with exsiccation or death.

19.
Thorac Res Pract ; 2023 Nov 28.
Artículo en Inglés | MEDLINE | ID: mdl-38015163

RESUMEN

OBJECTIVE: Coronavirus disease 2019 (COVID-19) can cause hypoxic respiratory failure; long-term oxygen therapy (LTOT) duration is unknown. MATERIAL AND METHODS: The aim was to investigate which patients would need LTOT after COVID-19 pneumonia. This single-center, prospective study was conducted at the Ankara University Faculty of Medicine, Department of Chest Diseases, between May 2021 and December 2021. The 70 patients hospitalized for COVID-19 pneumonia and discharged with LTOT due to hypoxemic respiratory failure were included. Patients were divided into 2 groups as group I (LTOT requirement <3 months) and group II (LTOT requirement continued ≥3 months). RESULTS: The mean age was 64.4 ± 13.5 years, and 44 (62.9%) of them were male. The most frequently encountered comorbidities were cardiovascular disease (57.1%) and lung disease (22.9%). While PaO2 levels increased in both groups during the follow-up period, this increment was significantly higher in group I (PaO2: 66.6 ± 9.9 mm Hg, P < .001). The factors affecting the LTOT requirement were evaluated using binary logistic regression. On multivariate analyses of lymphocytes, ferritin, C-reactive protein, PaO2, SaO2, subpleural reticulation, and number of lobes affected (≥3 lobes), the SaO2 level and presence of subpleural reticulation were significantly different between the 2 groups [odds ratio (OR) (95% CI): 0.853 (0.749-0.971), P = .016] and [OR (95% CI): 0.171 (0.042-0.733), P = .017], respectively. CONCLUSION: A significant proportion of patients who develop respiratory failure due to COVID-19 recover within the first 3 months. Factors determining the LTOT requirement for more than 3 months were SaO2 and the presence of subpleural reticulation.

20.
Int J Rheum Dis ; 25(6): 685-691, 2022 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-35505570

RESUMEN

AIM: Although the importance of biopsychosocial models for chronic diseases is emphasized nowadays, the lack of assessment tools to meet this need draws attention. This study was planned to assess the validity, reliability, and responsiveness of the Cognitive Exercise Therapy Approach-Biopsychosocial Questionnaire (BETY-BQ) in patients with fibromyalgia. METHOD: Hospital Anxiety and Depression Scale (HADS), Mindful Attention Awareness Scale, Health Assessment Questionnaire, the Fibromyalgia Impact Questionnaire, and Short-Form 36 (SF-36) were used for the validity of BETY-BQ. For the reliability study, evaluations were repeated at 1-week intervals and for the responsiveness, the same scales were applied to patients under medical treatment at 3-month intervals. RESULTS: The correlations of BETY-BQ with the other scales were found to be moderate to high (respectively r = .591, P < .001 and r = .441, P < .001; r = -.419, P < .001; r = .617, P < .001; r = .722, P < .001; r = -.580 and -.374, P = .001 and P < .001) and with SF-36 sub-parameters were found to be weak-moderate. The test-retest method was used for reliability, and the correlation between the responses was very high (r = .901, P < .001). The intraclass correlation coefficient (ICC) was found to be high (ICC = 0.899, P < .001). Cronbach's alpha (α) value was found to be .947. In the correlation analysis of time-dependent changes, BETY-BQ correlated weakly with other scales and moderately correlated with HADS (HADS-anxiety r = .489, P = .006, HADS-depression r = .500, P = .005). CONCLUSIONS: BETY-BQ was presented in the literature as a valid, reliable, and sensitive scale that can be used both in the biopsychosocial evaluation and as an easily understood, practical scale that can be answered in a short time in the practices of healthcare professionals.


Asunto(s)
Fibromialgia , Cognición , Terapia por Ejercicio , Fibromialgia/diagnóstico , Fibromialgia/psicología , Fibromialgia/terapia , Humanos , Psicometría , Reproducibilidad de los Resultados , Encuestas y Cuestionarios
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