Racial differences in systemic sclerosis disease presentation: a European Scleroderma Trials and Research group study

Objectives. Racial factors play a significant role in SSc. We evaluated differences in SSc presentations between white patients (WP), Asian patients (AP) and black patients (BP) and analysed the effects of geographical locations.Methods. SSc characteristics of patients from the EUSTAR cohort were cross-sectionally compared across racial groups using survival and multiple logistic regression analyses.Results. The study included 9162 WP, 341 AP and 181 BP. AP developed the first non-RP feature faster than WP but slower than BP. AP were less frequently anti-centromere (ACA; odds ratio (OR) = 0.4, P < 0.001) and more frequently anti-topoisomerase-I autoantibodies (ATA) positive (OR = 1.2, P = 0.068), while BP were less likely to be ACA and ATA positive than were WP [OR(ACA) = 0.3, P < 0.001; OR(ATA) = 0.5, P = 0.020]. AP had less often (OR = 0.7, P = 0.06) and BP more often (OR = 2.7, P < 0.001) diffuse skin involvement than had WP.AP and BP were more likely to have pulmonary hypertension [OR(AP) = 2.6, P < 0.001; OR(BP) = 2.7, P = 0.03 vs WP] and a reduced forced vital capacity [OR(AP) = 2.5, P < 0.001; OR(BP) = 2.4, P < 0.004] than were WP. AP more often had an impaired diffusing capacity of the lung than had BP and WP [OR(AP vs BP) = 1.9, P = 0.038; OR(AP vs WP) = 2.4, P < 0.001]. After RP onset, AP and BP had a higher hazard to die than had WP [hazard ratio (HR) (AP) = 1.6, P = 0.011; HR(BP) = 2.1, P < 0.001].Conclusion. Compared with WP, and mostly independent of geographical location, AP have a faster and earlier disease onset with high prevalences of ATA, pulmonary hypertension and forced vital capacity impairment and higher mortality. BP had the fastest disease onset, a high prevalence of diffuse skin involvement and nominally the highest mortality

Diet in Scleroderma: Is There a Need for Intervention?

Systemic sclerosis (SSc) patients exhibit a plethora of risk factors for nutritional decline, including the presence of chronic inflammation and the progressive nature of disease-related multisystem involvement. The prevalence and consequences of nutritional decline in scleroderma are frequently underestimated, its management currently remaining a subject of debate. The main objective of the present study was to perform a detailed assessment of scleroderma patients’ diet as well as their eating habits and to describe the relationships with weight loss and malnutrition risk in the absence of professional nutritional counseling. Methods: We used a translated and validated version of the EPIC-Norfolk FFQ (European Prospective Investigation into Cancer and Nutrition Norfolk Food Frequency Questionnaire) to evaluate the patients’ diet and MUST (Malnutrition Universal Screening Tool) to investigate the risk of malnutrition. Disease activity was estimated using the EUSTAR-AI (European Scleroderma Trials and Research group Activity Index). Results: We included 69 patients with SSc, of which 42 underwent a detailed dietary assessment. Dietary factors were connected to body composition and digestive symptoms. We found high sodium intake and frequent suboptimal energy consumption in our study group, including patients with cardiopulmonary involvement. Liver transaminases were inversely correlated with the consumption of nuts and seeds. Malnutrition and weight loss were significantly associated with pulmonary hypertension, heart failure, albumin levels, and the extent of skin fibrosis, but not advanced age. Although the patients with EUSTAR-AI ≥ 2.5 were more frequently included in the moderate and high malnutrition risk categories, these results did not reach statistical significance. Conclusions: Currently, there is an unmet need for longitudinal and interventional research focusing on the long-term significance, ramifications, and management of nutritional impairment in SSc patients with various clinical manifestations. Our results indicate that scleroderma patients could benefit from personalized nutritional counseling in an interdisciplinary setting

THE SIGNATURE OF INTESTINAL DYSBIOSIS IN INFLAMMATORY RHEUMATIC DISEASES

The study of intestinal microbiota is an important and current subject. It is well known that the gut microbiota plays a decisive role in the development of intestinal function, contributes to the defense against different infections, gives tolerance to ingested foods, regulating and maintaining the function of the intestinal barrier. The gut microbiota is different from individual to individual, determining, through the molecular profile, an “individual profile”. Intestinal dysbiosis is associated with multiple diseases such as IBD, irritable bowel syndrome, nosocomial infections or rheumatic inflammatory disorders. By characterizing intestinal dysbiosis in patients, a link could be made between these bacteria and the pathogenic mechanisms of the diseases, assigning these structures key roles in the onset of systemic disorders. This allows a better understanding of the pathophysiological mechanisms of the diseases and allow having a targeted treatment aimed at improving dysbiosis and restoring the normal microbial gut profile

ETIOPATHOGENIC MECHANISMS OF TOBACCO CONSTITUENTS IN RHEUMATOID ARTHRITIS

Active smoking is considered a risk factor for rheumatoid arthritis. Smokers show respiratory extra-articular manifestations and complications, such as interstitial lung disease and chronic obstructive pulmonary disease. Smokers may receive a more intensive drug therapy than non-smokers, but they have a poor prognosis. Smoker’s resistance to therapy may be caused by the pharmacokinetic interactions between drugs and tobacco constituents. The present account of some of those thousands of components of the gas and tar phase of cigarette smoke (polynuclear aromatic hydrocarbons, quinones, cyanide, heavy metals, bacterial endotoxins, nicotine and carbon monoxide) may help explain the inconclusive incrimination of tobacco use in the development of rheumatoid arthritis and convince more clinicians to recommend smoking cessation

PSORIATIC ARTHRITIS “SINE PSORIASIS” – A RARE FORM OF DISEASE THAT RAISES POSITIVE AND DIFFERENTIAL DIAGNOSIS PROBLEMS

Psoriatic arthritis is part of the big group of spondylarthropaties, presenting numerous clinical forms and having both musculoskeletal and extraarticular manifestations. Usually, the disease diagnosis is set after the presence of clinical signs of skin psoriasis. In that case, the diagnosis is made quite easily. The condition puts a lot of problems regarding differential diagnosis especially in the situation when it precedes the onset of skin lesions. Regarding the presented case, a correct diagnosis of psoriatic arthritis “sine psoriasis” was made after about 20 years of disease evolution, at which time the specific nail lesions of psoriasis appeared

Non-Traditional Pro-Inflammatory and Pro-Atherosclerotic Risk Factors Related to Systemic Lupus Erythematosus

Cardiovascular diseases (CVD) are one of the leading causes of high mortality in patients with systemic lupus erythematosus (SLE). The Framingham risk score and other traditional risk factors do not fully reflect the CVD risk in SLE patients. Therefore, in order to stratify these high-risk patients, additional biomarkers for subclinical CVD are needed. The mechanisms of atherogenesis in SLE are still being investigated. During the past decades, many reports recognized that inflammation plays a crucial role in the development of atherosclerosis. The aim of this report is to present novel proinflammatory and pro-atherosclerotic risk factors that are closely related to SLE inflammation and which determine an increased risk for the occurrence of early cardiovascular events

What can be hidden behind a persistent fever syndrome?

Fever is a defence mechanism of the body that occurs in various pathological situations, most often being secondary to an infectious disease. A prolonged febrile syndrome can hide many clinical problems, thus delaying a correct diagnosis and treatment. We present the case of a 52-year-old patient who addressed with a high fever associated with a generalized skin rash, arthralgia, myalgia and fatigue. Initially, the patient was referred to the infectious disease clinic where numerous paraclinical investigations were performed which ultimately ruled out an infectious cause of fever. Prompt response to corticosteroid therapy after performing numerous combinations of antibiotics, led to a possible autoimmune disease, the patient being redirected to the rheumatology clinic. Following the biological, immunological and radiological investigations, the diagnosis of adult Still’s disease was supported and the corresponding immunosuppressive treatment was initiated with good clinical-biological evolution

Ischemic Heart Disease and Rheumatoid Arthritis—Two Conditions, the Same Background

Rheumatoid arthritis (RA) is one of the most frequent inflammatory rheumatic diseases, having a considerably increased prevalence of mortality and morbidity due to cardiovascular disease (CVD). RA patients have an augmented risk for ischemic and non-ischemic heart disease. Increased cardiovascular (CV) risk is related to disease activity and chronic inflammation. Traditional risk factors and RA-related characteristics participate in vascular involvement, inducing subclinical changes in coronary microcirculation. RA is considered an independent risk factor for coronary artery disease (CAD). Endothelial dysfunction is a precocious marker of atherosclerosis (ATS). Pro-inflammatory cytokines (such as TNFα, IL-1, and IL-6) play an important role in synovial inflammation and ATS progression. Therefore, targeting inflammation is essential to controlling RA and preventing CVD. Present guidelines emphasize the importance of disease control, but studies show that RA- treatment has a different influence on CV risk. Based on the excessive risk for CV events in RA, permanent evaluation of CVD in these patients is critical. CVD risk calculators, designed for the general population, do not use RA-related predictive determinants; also, new scores that take into account RA-derived factors have restricted validity, with none of them encompassing imaging modalities or specific biomarkers involved in RA activity

IS THERE A PLACE FOR ANTI-NUCLEOSOME ANTIBODY ASSESSMENT IN SCLERODERMA?

Introduction. The hallmarks of systemic sclerosis (SSc) include microangiopathy, autonomic dysfunction, as well as immune disturbance and the widespread fibrosis of the skin and visceral organs. While the significance of SSc-specific autoantibodies such as anti-centromere and anti-topoisomerase I has long been demonstrated, the clinical relevance of non-specific autoantibodies remains a matter of debate. Our primary objective was to assess the relationships between non-SSc-specific antibody titers and the clinical characteristics of scleroderma patients. Secondary objectives included a comparison between SSc, SLE and healthy controls (HC) with respect to autoantibody values, as well as the analysis of the immune disturbance in elderly individuals in the 3 groups. Material and method. We conducted a cross-sectional study in which we recruited 67 adult patients with SSc, 67 age and gender-matched individuals with SLE and healthy controls (HC). Biological samples (venous blood) were collected in order to determine the levels of anti-SSA/Ro, anti-SSB/La, anti-U1RNP and anti-nucleosome antibodies (ELISA). We recorded the presence of digital ulcers (DUs), ILD (thoracic X-rays), and PAH (Doppler echocardiography) in the scleroderma cohort. Results. The frequency of anti-nucleosome antibody positivity in the scleroderma group exceeded our expectations, resembling that of lupus patients. Moreover, our findings indicate an association between serum anti-nucleosome antibody titers and SSc-related cardiopulmonary involvement. Anti-U1RNP antibodies were linked to PAH. We did not identify a notable relationship between the 4 autoantibodies studied and DUs. However, the latter were significantly more frequent in male patients. Although elderly individuals with scleroderma did not demonstrate a significantly decreased autoantibody production, lupus patients over 60 years of age exhibited a decline in anti-nucleosome antibody titers. Discussions. Earlier research reported an association between anti-nucleosome and anti-U1RNP antibodies with SSc-related cardiopulmonary impairment. Moreover, male gender is currently regarded as an important risk factor for the development of scleroderma DUs. Conclusions. Recent research provides new insights on the pathogenic processes of autoimmune rheumatic diseases, in an attempt to identify potential risk factors for organ involvement. Our study confirms the link between anti-nucleosome antibodies and cardiopulmonary involvement in the SSc population. Moreover, the impact of immunosenescence on the dynamics of autoantibody production in connective tissue diseases remains in need of further investigation

ARTICULAR INVOLVEMENT IN INFLAMMATORY BOWEL DISEASE – THE MOST FREQUENT EXTRAINTESTINAL MANIFESTATION

Objectives. Inflammatory bowel disease (IBD) are part of a pathology that has an ascending incidence both in Romania and around the world. It is well known that intestinal inflammation in IBD is not limited to the intestinal epithelium. Sometimes, extraintestinal manifestations (EIM) may have a more severe clinical expression than the intestinal disorder or may even encourage an increased morbidity. The research motivation focused on the development of specific clinical and epidemiological data for patients diagnosed with IBD who associate EIM. Material and methods. We performed a retrospective study including 517 patients with intestinal inflammation (Crohn disease - CD, ulcerative colitis - UC or undifferentiated colitis - NC) diagnosed during 1975-2016 in the N-E region of Romania. All the cases were extracted from the national database (IBD Prospect). Results. In our study the prevalence of UC versus CD cases prevailed. There were 368 cases (71.2%) of UC, 135 cases (26.1%) of CD and 10 cases of NC (1.9%). The prevalence of EIM in IBD patients in N-E Romania was 9,9% which was quite low. In the study group, EIMs occurred with a higher frequency in patients diagnosed with CD compared to UC. Thus, of the 51 cases of IBD and EIM, 27 (52.9%) belonged to the CD’s phenotype and 24 cases (47.1%) of the UC’s phenotype. Discussions. Both patients with CD and UC experienced a greater risk than the rest of patients for developing EIM. The most frequent EIMs were highlighted at the level of musculoskeletal system. Among EIM, there were 38 cases (74,5%) with articular manifestations (of which 26 had peripheral manifestations - arthritis, 12 cases developed axial manifestations – sacroiliitis/ankylosing spondylitis - SI/AS). Also, cases with multiple EIM had at least one articular manifestation. Conclusions. Our results sustain, once again, the fact that inflammation in IBD is not limited at the level of gastrointestinal tract. The presence of EIM, especially joint involvement, is a certainty validated by the results of many clinical trials