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Coatomer subunit α (COPA) syndrome is a mendelian autosomal dominant immune dysregulation disease characterized by early onset lung disease in the form of diffuse alveolar hemorrhaging or interstitial lung disease, frequently associated with arthritis, glomerulonephritis, and high titer autoantibodies usually mimicking other autoimmune diseases. While immunosuppressive medication has been effective in controlling arthritis, data on long-term lung disease control remains scarce, which poses a real challenge as the progression of lung disease is the main cause of poor life expectancy in COPA patients. Nevertheless, JAK inhibitor therapy seems to be the most promising therapeutic choice now. [ABSTRACT FROM AUTHOR]

Overlap syndrome of systemic sclerosis and idiopathic inflammatory myopathies is an increasingly frequent entity, but the association with immune-mediated necrotizing myositis has rarely been described. While myositis or myopathy may be features of scleroderma, it is imperative to correctly diagnose an overlap syndrome of these two, since it can be considered a different entity with specific management and a worse prognosis. Anti-signal recognition particle (anti-SRP) antibodies target the striated muscle fiber and inhibit myoblast regeneration, resulting in myofiber atrophy and necrosis. Anti-SRP antibodies are specific in immune-mediated necrotizing myopathy characterized by myonecrosis and minimal inflammatory reaction, with proximal muscle weakness and typical extra-muscular manifestation. There are controversial data on the association of cardiac manifestations and the presence of these antibodies, and recent studies cannot prove a significant correlation between the two. Myocarditis is a complication with an unpredictable, potentially severe outcome from heart failure and dilated cardiomyopathy to fatality. It can be difficult to diagnose, and a myocardial biopsy can be problematic in daily practice; thus, most practitioners rely on cardiac magnetic resonance with suggestive images for the correct diagnosis. This paper seeks to address the challenges associated with the diagnosis and treatment of collagen diseases by evaluating the role of anti-SRP antibodies in the pathogenesis of cardiac involvement. [ABSTRACT FROM AUTHOR]

Gout is a chronic metabolic disease, characterized by joint inflammation caused by monosodium urate monohydrate crystals. The main objective of this study was to identify the prevalence of gout in a rheumatology department in Romania and secondary to describe the characteristics of patients diagnosed with this condition, with emphasis on the comorbidities. This is a longitudinal, retrospective study on 280 patients from the Department of Internal Medicine and Rheumatology, Dr I Cantacuzino Hospital, from January 2017 to May 2019, diagnosed with chronic or acute gout. The prevalence of gout in our hospital in 2 years period was 0.97%. Gouty attack was diagnosed in 38.2% of cases, while 61.8% were evaluated for chronic gout. Male frequency was 69.6%, alcohol consummation was observed in more than half of the cohort (53.2%) and 72.1% were retired persons. The most frequent comorbidity was hypertension (HBP) (82.1%) followed by dyslipidemia (65.3%), atherosclerotic disease(ATS) (55.0%) and chronic kidney disease (53.9%). There was a significant association between HBP, ATS and dyslipidemia with chronic gout (p=0.038, p=0.022 and p=0.009, respectively). The rate of gouty attack significantly increased with the serum level of uric acid (p<0.001). The therapeutic approach complies with international recommendations. [ABSTRACT FROM AUTHOR]

The paper presents the special follow-up (category of importance of the dam: B) of the time behaviour of the Cinciș dam by evaluating (through direct observations and measurements), some parameters that define the state and evolution of the safety of the dam, in relation to actions to which he is subjected. Thus, it was verified if the variables that identify the behaviour of the structure as well as those related to the environmental and operational conditions are in the range of values obtained previously and if they are consistent. [ABSTRACT FROM AUTHOR]

This paper discusses the problem of multidimensional databases, with the purpose of demonstrating the efficient support offered by them for the management of OLAP processing and motivation of decisions, especially the ones related to environmental risks. The OLAP (On-Line Analytical Processing) systems that implement or use multidimensional databases are oriented towards decision makers and allow them to an easily process, sectioning and grouping of data, as well as the visualization of data from different perspectives, for the discovery of the factors that would influence a certain behavior. OLAP systems offer advanced statistical analysis facilities, such as the one based on time series or forecasts, which are based on predefined models. The calculation of the environmental risk offers an image of the degree of air pollution due to specific polluting factors, obtained especially as result of the industrial processes that have taken place in the Targoviste, in Dambovita area. An OLAP system, consisting of decision support components, was implemented as part of the PN2 three years research project: "Sustainable Management System of Resources Used for Monitoring and Evaluating the Environmental Risks in Order to Prevent the Negative Effects and to Manage Crises Situations - MEMDUR", code D11-037/18.09.2007. [ABSTRACT FROM AUTHOR]

Rheumatoid arthritis (RA) is an inflammatory, chronic and systemic disease that primarily affects the synovial joints. Anemia is a common extra-articular manifestation in the absence of an effective treatment. The main mechanisms involved include shortening the lifespan of erythrocytes, inadequate bone marrow and abnormalities in iron metabolism. Eighty-eight patients over 18 years with definite diagnosis of rheumatoid were included in this study. The prevalence, respectively the characteristics of anemia were analyzed, together with demographic data, the type of symptoms, the type of comorbidities, the hematological indices and treatments. The mean age of the study population was 65.31 ± 12.57 years. Treat to target was achieved in one third of the patients (36.4%). The prevalence of anemia was 55% with higher prevalence in males (57%) than females (50%). Anemia was associated with higher disease activity (p=0.036). Out of the anemic patients, 7.14% had megaloblastic anemia, 40.48% had anemia of chronic disease and 21.43% suffered from iron deficiency anemia. Microcytic normochromic and normocytic hypochromic patterns can have mixed causes, belonging to both iron-pathophysiological processes and chronic inflammation. The prevalence of anemia at the 1-year check-up was 29.44% and the percentage of patients who achieved treat-to-target goals increased from 36.40% to 40.90%. The majority (48.80%) did not prove to have anemia neither at admission nor at follow-up. The results of the study suggest that anemic patients tend to have a higher level of RA activity, therefore screening for anemic syndrome should be part of the management of these patients, in an effort to establish the best therapeutic conduct. [ABSTRACT FROM AUTHOR]

Rheumatoid arthritis (RA) is a chronic inflammatory disease that predominantly affects middle-aged adults in the third to fifth decades of life, but can also occur at any age. Significant differences were observed between patients with the diagnose of the disease under the age of 65 years – young- onset of RA (YORA) and those with the onset over the age of 65 years -elderly-onset of RA (EORA). The literature has shown that patients in the EORA group, in comparison to the young, have more severe onset, shorter duration of morning stiffness, lower frequency of seropositivity and a more important biological inflammatory syndrome. Objective: Describe and compare the clinical characteristics, laboratory features, functional status, therapeutic approach and disease progression in elderly-onset and young-onset rheumatoid arthritis (RA) patients. Materials and methods: This retrospective, transversal study included 102 patients diagnosed with rheumatoid arthritis according to the ACR / EULAR criteria and who had at least 3 visits to our clinic, the last one during 2019- 2020. Depending on the age at disease onset, we divided them into 2 groups- EORA and YORA and analyzed them comparing the clinical, laboratory and treatment data obtained at diagnosis. Subsequently we studied the evolution of the disease activity and the therapy efficiency at 6 months of follow-up and at the last hospitalization for each group. Results: The percentage of women is similar and predominant in both groups, YORA and EORA (68.3% and 71.8%). YORA was associated with a longer disease length and a prolonged symptom duration prior to the diagnosis in comparison to EORA (p<0.001 and P=0.002). Extra-articular manifestations were more frequent in elderly-onset RA patients at diagnosis, especially the presence of rheumatoid nodules (46.2% vs 22.2%. p=0.011) and weight loss (82.1% vs 34.9%, p<0.001). Anemia was statistically associated with the EORA group (p=0.037). Analyzing the radiological findings, there was a greater number of patients who showed erosions (48.7%) and geodes (28.2%) in EORA, than in YORA group (33.3% and 19.0%). The prevalence of specific auto-antibodies positivity as antiCCP was higher in YORA (76.2% vs 53.8%, p=0.019), as well as the positivity of Rheumatoid factor (RF) (84.1% vs 61.5%, p=0.010). The majority of patients began treatment with synthetic DMARD monotherapy, 54.0% of YORA and 64.1% of EORA. Methotrexate was the main drug administrated in both groups (61.5% in EORA and 54.0% in YORA, p-value= 0.602). Other medications, such as Sulfasalazine and Leflunomide, were less preferred in the two groups. Biologic therapy was preferred in younger patients than in those with RA at 65 years of age or over (69.8% vs 35.9%, p=0.001). Disease activity measured with DAS28(CRP) score was similar between the two groups at baseline, but significantly lower for YORA patients measured at the last hospitalization (p=0.020), treat to target (low disease activity and remission) being achieved in 53% of cases in the YORA group versus 23% of EORA patients (p=0.002). Conclusions: The definite diagnosis of RA was delayed in YORA patients in comparison to EORA patients and the extra-articular manifestations of the disease were more frequently found in the EORA group. Seropositivity was statistically significantly associated with the YORA group. Anemia was predominant in patients with disease onset over 65 years old. Both groups underwent DMARDs therapy in the early stages of the disease, but biologic therapy was more often administered in younger patients. Disease activity at diagnosis was similar in both groups, but in dynamic, the treat to target endpoint was achieved more frequent in YORA population. [ABSTRACT FROM AUTHOR]

Diabetes mellitus brings together several syndromes, all burdened by a high complexity and with the potential to generate vital impairment. The large number of complications result from the association of high blood glucose level with vascular damage, neuropathy, poor healing and overall increased atherosclerosis process. The clinical manifestation of these complications involves a wide range of manifestations from simple lesions to complex pathology, many of them requiring surgical treatment. Surgical implications of diabetes mellitus include diabetic foot syndrome, soft tissue infections, renal impairment and abdominal pathology. [ABSTRACT FROM AUTHOR]

Biological therapy is used in a wide range of medical settings. Adverse reactions to biological therapy can limit their widespread use, so early detection and treatment can adjust attempts to stop these molecules. TNF Alpha blockers may cause the following skin reactions in alpha patients: injection site reactions, infections, immune-mediated reactions (psoriasis, psoriasis, drug-induced lupus, vasculitis, hidradenitis, alopecia), allergic or neoplastic reactions. We present the case of a patient with RA who developed skin lesions during biological therapy and was diagnosed with drug-induced lupus based on clinical elements, associated autoimmunity, and dermatological evaluation. The skin lesions were attributed to the interaction of three medications (biosimilar Etanercept, Leflunomide, and Isoniazid), all of which have been implicated in causing these side effects. The solutions that saved the patient were temporarily discontinuing the immunosuppressive medication and replacing it with a local corticoid, followed by the continuation of Etanercept in associated with Methotrexate, and the patient was able to continue the biological medication and obtain a favorable response to the treatment. In conclusion, skin changes caused by TNF Alpha inhibitors are common, but vary in severity, and do not warrant therapy interruption. [ABSTRACT FROM AUTHOR]

The newly identified refractory adult-onset autoinflammatory syndrome known as VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is brought on by somatic mutations in the ubiquitin-like modifier-activating enzyme 1 (UBA1) gene in hematopoietic stem and progenitor cells that change the expression of the UBA1 isoform. As a result, patients have a variety of hematologic and systemic inflammatory symptoms. All types of medical professionals should treat VEXAS syndrome seriously due to the high fatality rate. To better comprehend the condition and enhance the prognosis for VEXAS syndrome, this review article describes the essential traits and clinical signs of the condition. The discussion of future directions in the study of systemic inflammatory disorders brought on by somatic mutations concludes., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2024, Padureanu et al.)