Reumatismo最新文献

In the field of obstetric antiphospholipid syndrome (APS), studies in mouse models and case reports support the potential benefit of tumor necrosis factor-α inhibitors (TNF-α-i ) in preventing pregnancy loss associated with APS. We present the case of a 36-year-old woman with a diagnosis of Takayasu arteritis who suffered from antiphospholipid antibody (aPL)-related thrombotic microangiopathy/probable catastrophic APS during her first pregnancy and who had a subsequent successful pregnancy while on treatment with certolizumab pegol (CZP), heparin, and low-dose aspirin. The report is followed by the literature review of published cases of APS or aPL-positive pregnancies treated with CZP or other TNF-α-i . A total of 20 pregnancies, including the one here presented, were reported in high-risk APS patients exposed to TNF-α-i , showing a favorable outcome in most pregnancies (80% live births, 69% absence of adverse pregnancy outcomes). Despite the limited available evidence, TNF-α-i could represent a promising option for high-risk patients in obstetric APS.

Objective: Systemic sclerosis (SSc) is a multisystem autoimmune disease of heterogeneous pathogenesis, including vascular, immunologic, genetic, epigenetic, and environmental factors. Progressive fibrosis is the hallmark of SSc. Intense research has been conducted to unveil new tools for early diagnosis and management, thus reducing morbidity and mortality. miR-21 has recently been considered to play an important role in the fibrosis of SSc. The objective of this study was to evaluate miR-21 levels in SSc patients and study its correlation to the extent of skin fibrosis and association with various clinical characteristics.

Methods: A total of 25 patients with SSc who fulfilled the American College of Rheumatology/European Alliance of Associations for Rheumatology 2013 classification criteria, as well as 25 controls, were enrolled in a cross-sectional study. The extent of skin fibrosis was evaluated using the modified Rodnan skin score, and disease severity was assessed using the Medsger severity score. The levels of miR-21 were measured by quantitative real-time polymerase chain reaction. The 2-ΔΔCt method was used for analysis. SSc patients affected by diabetes mellitus, hypertension, renal impairment, heart disease, malignancy, other autoimmune diseases, or a history of serious acute infection within 6 weeks were excluded.

Results: There was a high statistically significant difference in miR-21 levels between cases and controls (p<0.001). At a cut-off level of 2.55, miR21 could discriminate between SSc patients and controls with sensitivity 92% and specificity 100%. There was no significant correlation between miR-21 levels and the degree of skin fibrosis. There was a significant positive association between miR-21 levels and the presence of arthritis in SSc patients (p=0.007).

Conclusions: miR-21 was suggested as a robust diagnostic biomarker in SSc with superiority over the traditionally utilized antibodies. Additionally, due to its association with arthritis, it is supposed to play a proinflammatory role in addition to its pronounced profibrotic effects. Interestingly, the profibrotic miR-21 may not reflect the extent of skin fibrosis.

Objective: This study aimed to determine the prevalence and associated factors of depressive symptoms, poor sleep, and life quality among patients with systemic sclerosis (SSc).

Methods: This was a cross-sectional study including 120 SSc patients. Demographic and clinical data were obtained. The Short Form Health Survey 36 (SF-36), Pittsburgh Sleep Quality Index (PSQI), and short form of the Beck Depression Questionnaire were used to evaluate life quality, sleep quality, and self-reported depressive symptoms, respectively. The obtained data were analyzed to identify the demographic and clinical risk associations for depressive symptoms, poor sleep, and life quality.

Results: Of 120 participants, 108 patients (90%) were female. The mean age was 42.23 years, and the mean disease duration was 13.58 years. Most of the patients were married, unemployed, or housekeepers. Most of them had moderate economic conditions and tertiary education. The total scores of the SF-36 and PSQI questionnaires were 93.25±3.7 and 9.02±4.51, respectively, which showed good life quality but poor sleep quality. The prevalence of depressive symptoms was 44.16% (n=53), and most of them had mild to moderate depressive symptoms. The factors that correlated with life quality were occupational status and cough. The factors that negatively correlated with sleep quality were the presence of digital ulcers, cough, and dysphasia. The presence of cough, dyspnea, and gastroesophageal reflux disease was associated with depressive symptoms.

Conclusions: Our study showed a high prevalence of poor sleep quality and depressive symptoms among SSc patients. We found that gastrointestinal symptoms, respiratory symptoms, and digital ulcers affected patients' life quality, sleep quality, and mental status. Our results also demonstrated that depression was correlated with poor sleep quality, and they were both risk factors for diminished life quality. Identification of these factors would help to make pharmacological and non-pharmacological interventions to improve the quality of life and sleep in SSc patients.

Objective: To comprehensively trace the historical journey of adrenal gland research from the first anatomical descriptions in the 16th century to the development and clinical application of cortisone in the mid-20th century.

Methods: This review examines key phases in adrenal gland research, including anatomical discoveries, microscopic studies, experimental physiology, biochemical advancements, and clinical applications. We analyzed primary historical sources, scientific papers, and medical records to construct a chronological narrative of adrenal gland understanding and cortisone development.

Results: The review highlights significant milestones, beginning with Bartolomeo Eustachio's 1564 discovery of the adrenal glands. It details the crucial microscopic phase, initiated by Moritz Nagel's 1836 study, which revealed the cortex-medulla distinction. Julius Arnold's 1866 description of the three-zone cortical structure and the identification of chromaffin cells are also discussed. The experimental physiology phase, featuring groundbreaking work by Thomas Addison and Charles-Édouard Brown-Séquard, established the vital role of the adrenal cortex. The biochemical phase, marked by the isolation and synthesis of cortisone, is examined in depth, with particular focus on the contributions of Edward Calvin Kendall, Tadeus Reichstein, and others. Finally, the clinical phase is detailed, emphasizing Philip Showalter Hench's revolutionary application of cortisone in rheumatoid arthritis treatment in 1948.

Conclusions: This historical journey demonstrates how advancements in anatomy, histology, physiology, and biochemistry synergistically contributed to our understanding of the adrenal glands. The development of cortisone, culminating from this collective knowledge, revolutionized the treatment of inflammatory diseases, marking a significant milestone in medical history and setting the stage for modern endocrinology and rheumatology.

Objective: In the absence of national and European guidelines on the treatment of rheumatoid arthritis (RA) with interstitial lung disease (ILD), the Italian Society of Rheumatology decided to develop national clinical practice guidelines on the management of patients with RA-ILD in accordance with the requirements of the National Guideline System of the National Institute of Health.

Methods: The development process included a systematic review of the available evidence and its adaptability to the Italian context, followed by a consultation with experts in rheumatology, respiratory diseases, radiology, and representatives of the health professions and patients.

Results: The panel decided to develop recommendations in three main scenarios. The first section of recommendations is focused on drugs indicated for RA to assess their safety and efficacy in RA-ILD. The second set of recommendations covered the drugs indicated for the treatment of ILD in patients with RA-ILD (to assess their efficacy and safety in patients with RA). The third part of these guidelines dealt with drugs indicated for the treatment of RA-ILD upon first-line failure. Moreover, the lack or absence of scientific evidence in literature on certain topics, such as the value of a multidisciplinary treatment approach and lung transplantation, led to the decision to proceed through expert consensus to develop good clinical practice guidelines.

Conclusions: These guidelines represent a fundamental step towards improving the health management of patients with rheumatological diseases in Italy by providing specific and evidence-based guidelines for the management of RA-ILD. Their use is intended to promote health and reduce the burden of morbidity and mortality in this vulnerable population.

Objective: IgG4-related disease (IgG4-RD) is a recently described fibroinflammatory disorder. The most common presentations include salivary and lacrimal gland hypertrophy, orbitary disease, autoimmune pancreatitis, retroperitoneal fibrosis, and tubulointerstitial nephritis. Lymphoplasmacytic infiltration, fibrosis, IgG4+ cell hyperplasia, and elevated IgG4 serum levels are the primary pathophysiological findings related to the disease. We described for the first time the epidemiology and clinical manifestations in our country.

Methods: A descriptive study of patients from a retrospective cohort based on clinical records of adults with IgG4-RD treated between 2014 and 2021 in a high-complexity national center.

Results: 23,381 patients with IgG4-RD diagnosis from 12 centers nationwide until December 2021 provided demographical data. We limited the search through ICD-10 coding: M358, M359, and M368, other specified systemic involvement of connective tissue; L948, other specified localized connective tissue disorders; D472, monoclonal gammopathy; K861, other chronic pancreatitis; H051, H059, and H063, chronic inflammatory disease of orbit; and C488, malignant neoplasm of overlapping sites of retroperitoneum and peritoneum. Thirty-three patients were identified with IgG4-RD based on comprehensive diagnostic criteria. A definitive diagnosis was obtained in 48.48% of patients, a probable diagnosis in 27.7%, and a possible diagnosis in 24.24%. A total of 21 patients were female, with a male/female ratio of 1/1.75. The median age at diagnosis was 53.87 years (interquartile range of 27.06), the minimum age of diagnosis was 11.53 years, and the maximum was 79.18. Regarding clinical presentation at diagnosis, ocular/orbitary affectation was present in 16 patients (48.48%), followed by head and neck in 10 patients (30.30%), and biliary tract/gastrointestinal in 9 (27.27%). A single-organ compromise was identified in 15 patients (45.45%), and 18 patients (54.55%) had two or more organs affected, with lymphatic and ocular/orbitary being the most commonly reported.

Conclusions: Epidemiologic and demographic data on IgG4-RD in our country are similar to those in world medical literature. The higher frequency of the disease in males above 65 years and females under 65 years suggests distinct pathophysiologic factors related to sex and age. This work has a limitation of subreports or misreports, which physicians can make when registering ICD-10 codes in clinical records. Nonetheless, it is the legal record in Colombia and requires an analysis like the one made in this study.

Objective: Leukemia inhibitory factor (LIF) is a multifunctional cytokine involved in numerous physiological processes, including inflammation and immune response regulation. Recent studies have highlighted its potential role in the pathogenesis and treatment of autoimmune diseases such as rheumatoid arthritis (RA) and multiple sclerosis (MS). This review aims to investigate the role of LIF in various autoimmune disorders and its impact on the recovery and treatment of these diseases.

Methods: A comprehensive literature search was conducted using Google Scholar, PubMed, and Scopus databases. Relevant studies published up to December 2023 were identified using keywords such as "leukemia inhibitory factor", "autoimmune diseases", "rheumatoid arthritis" and "multiple sclerosis".

Results: The literature indicates that LIF has a dual role in autoimmune diseases. In RA, LIF plays an important role in the progression of joint damage by increasing the inflammatory response. In MS, LIF has been shown to promote remyelination and neuroprotection, suggesting its potential as a therapeutic agent. However, the precise mechanisms by which LIF modulates immune responses in these conditions remain incompletely understood.

Conclusions: LIF represents a promising target for treating autoimmune diseases, particularly RA and MS. Further research is required to elucidate its mechanisms of action and develop targeted therapies that can control its beneficial effects while minimizing potential adverse outcomes.

Objective: Limited data in Latin America exists regarding the efficacy of switches from original biologicals to biosimilars in real-life scenarios. Currently, no studies assess this switch using imaging. The objective of this study was to evaluate clinical, functional, ultrasonographic, and radiological responses in a group of patients with rheumatoid arthritis (RA) and psoriatic arthritis (PsA) switched from original adalimumab (oADA) to biosimilar (bADA) (GP2017).

Methods: A prospective cohort study included diagnosed RA and PsA patients undergoing oADA treatment. At the baseline visit, blood analysis, X-rays, ultrasound, and an interview for sociodemographic and clinical data were conducted. Evaluators were unaware of each other's data. Patients switched to bADA during follow-up and were assessed in the same program within 3 to 12 months post-switch (only including patients with all evaluations).

Results: Out of 270 RA cohort patients, 35 met the criteria for complete pre- and post-control post-switch to bADA (GP2017), along with 15 PsA patients. The mean time between the switch and the second evaluation was 4.1 months (interquartile range 7). No statistical differences were observed in disease activity or functional capacity. Regarding imaging, no difference was found in X-ray erosion number; however, ultrasonography revealed decreased power Doppler (PD) activity, but not grayscale changes. No differences in acute phase reactants, joint count, or patient visual analog scale were observed between controls.

Conclusions: In this analysis of the switch between oADA and bADA, no differences were found in disease activity, functional capacity, or radiographic progression. Ultrasonography indicated improvement of PD findings.

Objective: Antiphospholipid syndrome (APS) is a disease characterized by recurrent thrombosis in the presence of antiphospholipid antibodies. The most uncommon events described in the literature have been peripheral neurological disorders. This paper aims to systematically review the cases of peripheral neuropathy (PN) in APS patients.

Methods: We systematically searched articles on PN and APS with English abstracts in PubMed from 1966 to August 2022.

Results: We found 10 articles on PN and APS with 100 patients. Age varied from 25 to 78 years; 86-100% of patients in these studies were female. Most patients had primary APS (n=9); one article considered secondary APS associated with other autoimmune diseases. Disease duration varied from 0 to 8.6 years, but three articles did not provide this information. Most studies showed positivity for anticardiolipin antibodies (n=5), followed by lupus anticoagulant (n=2). Regarding clinical NP features, mononeuritis multiplex (n=3) and autonomic neuropathy (n=3) were more common than peripheral polyneuropathy (n=2). Nerve biopsy was performed in 7 articles and resulted positive in all cases. Concerning treatment, most articles used anticoagulation (n=4), followed by glucocorticoids (n=3), intravenous immunoglobulin, and immunosuppressive drugs (n=1). Most cases improved after treatment (n=7).

Conclusions: This study demonstrates that PN is a rare complication in APS and occurs more frequently in females, associated with antiphospholipid antibody positivity. Most cases were confirmed by electroneurography or nerve biopsy and had a good outcome.

Thymic tumors are rare in the general population, and to the best of our knowledge, no cases of thymoma have been described in patients with rheumatic diseases treated with tumor necrosis factor (TNF)-α inhibitors, except for the case of a patient receiving infliximab for Crohn's disease (CD) who developed a B2 thymoma. We describe a 60-year-old Caucasian male with radiographic axial spondyloarthritis (r-axSpA) and CD who developed an AB-type thymoma without myasthenia gravis after 18 years of treatment with TNF-α inhibitors. The patient had received the same molecule since the r-axSpA/CD diagnosis and changed it 6 months before the diagnosis of thymoma due to a disease flare. At the time of the drug switch, no mediastinal mass was present on the chest X-ray. The thymoma was surgically removed, and no additional therapy was needed. Treatment with TNF-α inhibitors was reintroduced after surgery. This case raises some important questions that remain open and deserve to be addressed in the future, such as the association between immunosuppressive therapy and thymoma and the controversial relationship between TNF-α inhibitors and myasthenia gravis.