scholarly journals Hematological Manifestations of SLE at Initial Presentation: Is It Underestimated?

2012 ◽  
Vol 2012 ◽  
pp. 1-5 ◽  
Author(s):  
P. K. Sasidharan ◽  
M. Bindya ◽  
K. G. Sajeeth Kumar

SLE can present with hematological manifestations alone or along with features of other system involvement. With a low index of clinical suspicion or inadequate follow up the diagnosis may be delayed or missed at the time of presentation, in those with hematological abnormalities as the initial manifestation. An observational study was conducted among patients of SLE, in a tertiary referral centre of North Kerala, with the purpose of estimating the proportion of patients with hematological manifestations as the initial presentation of the disease and to study their nature. It was observed that 82% of the patients had hematological manifestations at presentation. It is the most common presenting manifestation of SLE in people of North Kerala. Autoimmune hypothyroidism was one of the common coexisting abnormalities in these patients, which is not included in the American College of Rheumatology (ACR) criteria for diagnosis. Arthritis was uncommon among those who presented with hematological manifestations. A significant number of patients do not satisfy the ACR criteria at the time of diagnosis but do so on follow up. The ACR criteria are weak to diagnose such patients and therefore need revision. We therefore propose an alternative to ACR criteria as “Kozhikode criteria for SLE”.

2020 ◽  
Vol 14 (Supplement_1) ◽  
pp. S252-S253
Author(s):  
U N Shivaji ◽  
A Bazarova ◽  
T Critchlow ◽  
S C Smith ◽  
O M Nardone ◽  
...  

Abstract Background In clinical practice, patients with IBD have their biologic therapies withdrawn due to variety of reasons. The aim of the study was to report on predictors of prognosis in IBD patients after biologics have been discontinued, with a minimum follow-up of 24 months. Methods All IBD patients who discontinued their first-use biologic were identified between January 2013 and Dec 2016 from EMR at a tertiary referral centre, to ensure at least 24 months follow-up. Reasons for discontinuation and pre-defined adverse outcomes (steroid and other rescue therapies, hospitalisations, surgery including perianal) were recorded. The data were analysed using multivariable and univariable logistic regressions within a machine learning technique in order to predict adverse outcomes, within the stated timeframe. We tested the significance of the identified predictors and performed Kaplan–Meier survival analysis to compare patients with elective vs. non-elective discontinuation of biologics. Results 147 patients who discontinued biologics (M = 74, median age 39y; CD = 110) were identified. Follow-up ranged from 24 to 60 months (median 40 months). The reasons for non-elective discontinuation included side effects (n = 21, 14%), primary or secondary non-response (n = 33, 22%) and patient choice (n = 10, 7%), among others. 59 (40%) patients had elective discontinuation. In this cohort, elective discontinuation resulted in fewer IBD-related adverse outcomes (AO) compared with non-elective. Figure 1 shows a Kaplan–Meier curve comparing the two (p = 0.003). Using data from all 147 patients, multivariable logistic regression analysis was done to identify significant predictors of prognosis. These are represented in Table 1. Overall, a significant number of patients (n = 80, 54%) had AO within 6 months of discontinuation, and 96 (65%) patients needed biologics to be restarted by the end of the study follow-up period. Conclusion Among IBD patients who discontinued biologics, there were fewer AO when they were electively discontinued. However, majority of patients required restart of biologics to manage their disease during the follow-up period. Clinicians need to be cautious when considering biologic discontinuation given the high proportion of AO and re-escalation to biologics therapy.


2017 ◽  
Vol 76 (11) ◽  
pp. 1911-1914 ◽  
Author(s):  
Gina Hetland Brinkmann ◽  
Ellen S Norli ◽  
Pernille Bøyesen ◽  
Désirée van der Heijde ◽  
Lars Grøvle ◽  
...  

ObjectiveTo determine how the European League Against Rheumatism (EULAR) definition of erosive disease (erosion criterion) contributes to the number of patients classified as rheumatoid arthritis (RA) according to the 2010 American College of Rheumatology/EULAR RA classification criteria (2010 RA criteria) in an early arthritis cohort.MethodsPatients from the observational study Norwegian Very Early Arthritis Clinic with joint swelling ≤16 weeks, a clinical diagnosis of RA or undifferentiated arthritis, and radiographs of hands and feet were included. Erosive disease was defined according to the EULAR definition accompanying the 2010 RA criteria. We calculated the additional number of patients being classified as RA based on the erosion criteria at baseline and during follow-up.ResultsOf the 289 included patients, 120 (41.5%) fulfilled the 2010 RA criteria, whereas 15 (5.2%) fulfilled only the erosion criterion at baseline. 118 patients had radiographic follow-up at 2 years, of whom 6.8% fulfilled the 2010 RA criteria and only one patient fulfilled solely the erosion criterion during follow-up.ConclusionFew patients with early arthritis were classified as RA based on solely the erosion criteria, and of those who did almost all did so at baseline.


2019 ◽  
Vol 13 (2) ◽  
pp. 80-83
Author(s):  
I. V. Menshikova ◽  
V. V. Strogonova

Objective: to analyze the efficacy of tofacitinib (TOFA) in patients with an advanced stage of rheumatoid arthritis (RA) and previous methotrexate (MTX) failure according to comprehensive clinical and laboratory examination.Patients and methods. Nineteen patients (11 women and 8 men aged 29 to 71 years (mean age 53.47±10.53 years)) with a reliable diagnosis of RA according to the 2012 American College of Rheumatology (ACR) criteria were followed up. Due to a high disease activity (DAS28 averaged 5.84±0.89), the patients were prescribed TOFA 10 mg/day taken for 12 months. The disease activity indicators were assessed at baseline, 3, 6, and 12 months after the start of the study.Results and discussion. The effect of TOFA developed quite quickly: at 3 months of therapy, there were positive clinical, laboratory, and instrumental changes in most patients; 5 (26.3%) patients achieved the goal of treatment. At 12 months of therapy, 8 patients achieved DAS28 remission and 3 patients had a low disease activity; moderate RA activity persisted in 7 patients; and a high disease activity was seen in 1 patient; these SDAI activity indicators were observed in 2, 8, 7, and 2 patients, respectively. According to the ACR criteria, 20, 50, and 70% improvements were achieved by 8, 6, and 3 patients, respectively. No serious adverse reactions were recorded. The findings confirm the good efficacy and safety of TOFA in the treatment of patients with an advanced stage of RA.Conclusion. The innovative mechanism of TOFA allows a significant number of patients, in whom MTX therapy was not effective enough, to achieve the goal of treatment. 


2020 ◽  
Vol 8 ◽  
Author(s):  
Nora Manz ◽  
Andreas H. Krieg ◽  
Michael Buettcher ◽  
Nicole Ritz ◽  
Ulrich Heininger

Background: Acute hematogenous osteomyelitis (OM) and septic arthritis require immediate diagnosis and treatment by an interdisciplinary team of pediatric infectious disease specialists and pediatric orthopedic surgeons. Adverse outcomes such as growth disturbance, bone deformity, and chronic infections have been described in older studies. However, there is only little known about long-term follow-up of patients of the last two decades. Therefore, we aimed to evaluate subjective and objective long-term outcomes of these children with osteoarticular infections treated in the millennial years.Methods: Cross-sectional study performed in two pediatric centers including patients admitted for OM and/or SA between 2005 and 2014 and follow-up consultations in 2019. Patients with symptoms of ≤2 weeks duration at initial presentation were contacted. Subjective outcomes were assessed by standardized interview, objective outcomes by clinical examination. Medical charts were used to extract data from the initial presentations. Statistical analysis was performed by non-parametric tests and Fisher's exact test.Results: Of 147 eligible patients 77 (52%) agreed to participate, of which 68 (88%) had an interview and physical examination and 9 (12%) an interview only. Thirty-three (39%) had OM, 26 (34%) SA, and 21 (27%) combined OM/SA. Median (IQR) age at follow-up was 13.3 (10.5–18.0) years with a median (IQR) follow-up of 7.1 (6.1–8.6) years. Persistent complaints including pain, functional differences and scar paresthesia, reported by 21 (28%) patients, were generally mild and only 3 (5%) required ongoing medical care. Objective sequelae including pain, limited range of motion, unilateral axis deformity or asymmetric gait were found in 8 (12%) participants. Older age, female sex, joint involvement, surgical intervention, persistent fever, and C-reactive protein elevation were associated with adverse clinical outcome.Conclusions: Adverse outcomes were observed in a considerable number of patients, most of which were minor, and only few required ongoing medical care. Long-term follow up is advisable for patients with risk factors identified during the initial presentation.This study was registered on ClinicalTrials.gov (NCT03827980).


2021 ◽  
Vol 8 (12) ◽  
pp. 666-672
Author(s):  
Derya Kaşkari ◽  
Ahmet Eftal Yücel

Objective: Fibromyalgia syndrome (FMS) is a chronic disorder characterized by widespread, unexplained pain in the muscles, including the head, neck, and sides of the hips, and fatigue. We aimed to evaluate the familiarity of physical medicine and rehabilitation and rheumatology physicians with fibromyalgia syndrome (FMS) in Turkey by means of a survey and to determine if these physician groups followed the 1990 FMS diagnostic criteria and 2010 FMS classification criteria for diagnosis. Material and Methods: The survey questions consisted of two parts; the first part consisted of 10 questions about demographics and professional experience, as well as the number of patients who had been diagnosed, treated, and followed up with in the prior 3 months by physicians. The second part consisted of 15 questions about perspectives on the 1990 FMS diagnostic criteria and 2010 FMS classification criteria. Results: One hundred and seventy one physicians participated in this survey. The majority of physicians 105 (99.1%) from physical medicine and rehabilitation and 59 (90.8%) rheumatologists could diagnose FMS. The rate of diagnosis and the rate of follow-up for FMS patients were significantly higher with physical medicine and rehabilitation specialists than with rheumatologists (p= 0.013 and p = 0.000; respectively) and were statistically significant. Conclusion: Differences in the awareness and descriptions of as well as approaches to FMS by physical medicine rehabilitation physicians and rheumatologists were examined in this study.


2019 ◽  
Vol 9 (02) ◽  
Author(s):  
Haider S Al-Hadad ◽  
Aqeel Abbas Matrood ◽  
Maha Abdalrasool Almukhtar ◽  
Haider Jabur Kehiosh ◽  
Riyadh Muhi Al-Saegh

Background: Systemic lupus erythematosus (SLE) is an autoimmune disease. Few biomarkers for SLE have been validated and widely accepted for the laboratory follow-up of inflammatory activity. In SLE patients, with lupus nephritis (LN), complement activation leads to fluctuation of serum C3 and C4 that are frequently used as clinicalm biomarker of disease activity in SLE. Patients and Methods: In this study the number of patients were 37, seven patients were excluded for incomplete data collection, 28 were females ,2 were males. The duration of the study is two years from 2015 to 2017. Patients were considered to have SLE and LN according to American College of Rheumatology (ACR) criteria, and International Society of Nephrology/ Renal Pathology Society (ISN/RPS). All patients were evaluated withm clinical presentation, laboratory investigations. Our patients underwent kidney biopsy according to standard procedure by Kerstin Amann, and their tissue specimens were studied in the laboratory with light microscope (LM) and immunofluorescence microscope reagents. The relationship between the serological markers and immunofluorescence deposits in kidney biopsy of all patients were studied using the statistical analysis of Pearson correlation and single table student's T test. A P value 0.05 was considered statistically significant. Results: The granular pattern of IF deposits was present in all LN patients, and in more than two third of patients these IF deposits presented in glomerular, tubular, and mesangium sites. While less than one third of patients had IF deposits in the mesangium only. There was no statistically significant correlation between serum ANA, anti-dsDNA, and IF deposits of different types. There was significant correlation between serum C3 and C4 hypocomplementemia and IgG immune deposits in kidney biopsy, and there was significant relationship between serum C3 hypocomplementemia and full house immunofluorescence (FHIF) deposits inm kidney biopsy.Conclusions:Immunofluorescence deposits is mainly granular pattern in LN patients. There was no significant association between serum ANA, anti-dsDNA, and immune deposits in kidney tissue. Immunofluorescence deposits of IgG type correlates significantly with serum C3 and C4 hypocomplemetemia, and these immune deposits in association with low complement levels correlates with LN flare. There was significant correlation between C3 hypocomplementemia and FHIF.


Author(s):  
Shmakova O.P.

Prevention of disability is one of the most significant tasks of child and adolescent psychiatry. Obtaining data on the dynamics of the number of people with disabilities and the factors affecting this indicator seems to be one of the relevant aspects. Aim: to trace the dynamics of the number of children with disabili-ties and to assess the change in the structure of early disability over the past decades. Materials and Meth-ods. A comparative analysis of two cohorts of patients was carried out: 1st - patients born in 1990-1992. (1203 patients (men - 914, 76%; women - 289, 24%)) who applied to the district neuropsychiatric dispensa-ry for outpatient care in childhood and adolescence; II - children and adolescents born in 2005 - 2018 (602 patients (male - 410, 68%; female - 192, 32%), ob-served at the time of the study by a child psychiatrist in the neuropsychiatric dispensary. Research methods: clinical and psychopathological; follow-up; statisti-cal. Results. Comparison of the number and nosologi-cal distribution of disabled children in two cohorts showed that over the 15th year there has been a shift towards an increase in the proportion of disabled children among patients observed by child and ado-lescent psychiatrists. The increase in the number of children with disabilities was due to those suffering from childhood autism and other disorders of general development. There were no statistically significant differences in the number of people with disabilities who received benefits before the age of 7, as well as differences in gender ratios among disabled people in the two cohorts. Conclusion. Early disability is a mul-tifactorial phenomenon, prevalence, dynamics, the structure of which depends not only on clinical, but also on socio-administrative realities. Children with autism require increased attention, since there has been a multiple increase in the number of patients with this diagnosis.


2018 ◽  
Vol 68 (suppl 1) ◽  
pp. bjgp18X697469
Author(s):  
Rebecca Ward ◽  
Fahmy W Hanna ◽  
Ann Shelley-Hitchen ◽  
Ellen Hodgson ◽  
Adrian Heald ◽  
...  

BackgroundWomen with gestational diabetes (GDM) have an elevated risk of developing type 2 diabetes (T2DM). NICE Guidance recommends women who develop GDM are screened 6 weeks post-partum and annually thereafter.AimTo evaluate conformity to guidance of screening in women with GDM by 6-week post-partum fasting plasma glucose (FPG) and annual FPG and determine time between delivery and development of T2DM.MethodRecords at a tertiary referral centre were used to identify women (n = 54) diagnosed with GDM by antenatal oral glucose tolerance test between July 1999 and January 2007. Data from laboratory records were used to collect investigations of glycaemic status during the follow-up period (median follow-up 12.4 years, range 9.5–17.1 years).ResultsOf 252 women, 102 (40.2%) did not have a FPG at 6 weeks (+/−2 weeks). Of these, median time to first test was 1.2 years (range 0.04–10.8 years), with only 43.1% followed-up within 1 year. In those who had a 6-week FPG, 17 (11.3%) women had no further tests. A total of 84 (33% of those with gestational diabetes in the index pregnancy) women were diagnosed with T2DM; median time from delivery to diagnosis was 5.2 years (range 0.35–15.95). We found the only significant factor for a follow-up test at 1-year post-partum was the use of insulin.ConclusionOur data suggest an alternative approach is needed for monitoring women with a history of GDM. This needs to be appropriate for a generally healthy group in which traditional screening mechanisms may not be adequate or sufficient.


2018 ◽  
Vol 16 (05) ◽  
pp. 362-368 ◽  
Author(s):  
Federica Sullo ◽  
Agata Polizzi ◽  
Stefano Catanzaro ◽  
Selene Mantegna ◽  
Francesco Lacarrubba ◽  
...  

Cerebellotrigeminal dermal (CTD) dysplasia is a rare neurocutaneous disorder characterized by a triad of symptoms: bilateral parieto-occipital alopecia, facial anesthesia in the trigeminal area, and rhombencephalosynapsis (RES), confirmed by cranial magnetic resonance imaging. CTD dysplasia is also known as Gómez-López-Hernández syndrome. So far, only 35 cases have been described with varying symptomatology. The etiology remains unknown. Either spontaneous dominant mutations or de novo chromosomal rearrangements have been proposed as possible explanations. In addition to its clinical triad of RES, parietal alopecia, and trigeminal anesthesia, CTD dysplasia is associated with a wide range of phenotypic and neurodevelopmental abnormalities.Treatment is symptomatic and includes physical rehabilitation, special education, dental care, and ocular protection against self-induced corneal trauma that causes ulcers and, later, corneal opacification. The prognosis is correlated to the mental development, motor handicap, corneal–facial anesthesia, and visual problems. Follow-up on a large number of patients with CTD dysplasia has never been reported and experience is limited to few cases to date. High degree of suspicion in a child presenting with characteristic alopecia and RES has a great importance in diagnosis of this syndrome.


2021 ◽  
Vol 19 (1) ◽  
Author(s):  
Antonio Leon-Justel ◽  
Jose I. Morgado Garcia-Polavieja ◽  
Ana Isabel Alvarez-Rios ◽  
Francisco Jose Caro Fernandez ◽  
Pedro Agustin Pajaro Merino ◽  
...  

Abstract Background Heart failure (HF) is a major and growing medical and economic problem, with high prevalence and incidence rates worldwide. Cardiac Biomarker is emerging as a novel tool for improving management of patients with HF with a reduced left ventricular ejection fraction (HFrEF). Methods This is a before and after interventional study, that assesses the impact of a personalized follow-up procedure for HF on patient’s outcomes and care associated cost, based on a clinical model of risk stratification and personalized management according to that risk. A total of 192 patients were enrolled and studied before the intervention and again after the intervention. The primary objective was the rate of readmissions, due to a HF. Secondary outcome compared the rate of ED visits and quality of life improvement assessed by the number of patients who had reduced NYHA score. A cost-analysis was also performed on these data. Results Admission rates significantly decreased by 19.8% after the intervention (from 30.2 to 10.4), the total hospital admissions were reduced by 32 (from 78 to 46) and the total length of stay was reduced by 7 days (from 15 to 9 days). The rate of ED visits was reduced by 44% (from 64 to 20). Thirty-one percent of patients had an improved functional class score after the intervention, whereas only 7.8% got worse. The overall cost saving associated with the intervention was € 72,769 per patient (from € 201,189 to € 128,420) and €139,717.65 for the whole group over 1 year. Conclusions A personalized follow-up of HF patients led to important outcome benefits and resulted in cost savings, mainly due to the reduction of patient hospitalization readmissions and a significant reduction of care-associated costs, suggesting that greater attention should be given to this high-risk cohort to minimize the risk of hospitalization readmissions.


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