High fatigue scores in patients with idiopathic inflammatory myopathies: a multigroup comparative study from the COVAD e-survey.

Idiopathic inflammatory myopathies (IIMs) confer a significant risk of disability and poor quality of life, though fatigue, an important contributing factor, remains under-reported in these individuals. We aimed to compare and analyze differences in visual analog scale (VAS) scores (0-10 cm) for fatigue (VAS-F) in patients with IIMs, non-IIM systemic autoimmune diseases (SAIDs), and healthy controls (HCs). We performed a cross-sectional analysis of the data from the COVID-19 Vaccination in Autoimmune Diseases (COVAD) international patient self-reported e-survey. The COVAD survey was circulated from December 2020 to August 2021, and details including demographics, COVID-19 history, vaccination details, SAID details, global health, and functional status were collected from adult patients having received at least one COVID-19 vaccine dose. Fatigue experienced 1 week prior to survey completion was assessed using a single-item 10 cm VAS. Determinants of fatigue were analyzed in regression models. Six thousand nine hundred and eighty-eight respondents (mean age 43.8 years, 72% female; 55% White) were included in the analysis. The overall VAS-F score was 3 (IQR 1-6). Patients with IIMs had similar fatigue scores (5, IQR 3-7) to non-IIM SAIDs [5 (IQR 2-7)], but higher compared to HCs (2, IQR 1-5; P < 0.001), regardless of disease activity. In adjusted analysis, higher VAS-F scores were seen in females (reference female; coefficient -0.17; 95%CI -0.21 to -13; P < 0.001) and Caucasians (reference Caucasians; coefficient -0.22; 95%CI -0.30 to -0.14; P < 0.001 for Asians and coefficient -0.08; 95%CI -0.13 to 0.30; P = 0.003 for Hispanics) in our cohort. Our study found that patients with IIMs exhibit considerable fatigue, similar to other SAIDs and higher than healthy individuals. Women and Caucasians experience greater fatigue scores, allowing identification of stratified groups for optimized multidisciplinary care and improve outcomes such as quality of life.

Respiratory failure due to concomitant interstitial lung disease and diaphragmatic involvement in a patient with anti-MDA5 dermatomyositis: a case report.

Diaphragm myositis is a rare manifestation of idiopathic inflammatory myopathies, barely portrayed in literature despite its potential severity. We describe a 57-year-old Caucasian male with anti-MDA5 positive dermatomyositis, that had a 4-month history of progressive dyspnoea requiring oxygen-therapy, scarcely responsive to prednisolone. Chest high resolution computed tomography (HRCT) showed mild interstitial lung disease (ILD), whereas pulmonary function tests evidenced severe restrictive syndrome with high lung ultrasound score. Diaphragm ultrasound revealed a marked diaphragm dysmotility, confirmed by electromyography (EMG). The patient was treated with intravenous immunoglobulins and mofetil mycophenolate with progressive improvement of dyspnoea, lung volumes and ILD at CT scan. Ultrasound examination also revealed marked improvement of the diaphragmatic disfunction and a reduction of lung ultrasound score. The use of ultrasound may provide a valuable tool in the diagnosis of diaphragm myositis, which may play a major role in the respiratory impairment of these patients. A combined lung and diaphragm examination allowed bedside monitoring of the improvements in both lung aeration and diaphragm contractility.

New developments in ultrasound in rheumatology: innovative tools and promising applications.

In the setting of rheumatology, the assessment of the involvement of joints and peri-articular structures was the first application of ultrasonography in the management of rheumatic diseases. In this field, the main novelties are related to the development of new technical tools to help overcome some of the limitations of conventional ultrasonographic assessment. In the meantime, there has been a growing interest in the application of ultrasound to extra-articular sites, relying on the definition of new indications. The present review aims to provide a critical analysis of the technical developments and new fields of application of ultrasonography in rheumatology.

Thrombocytopenia in idiopathic inflammatory myopathies: a case series analysis.

Idiopathic inflammatory myopathies (IIM) are a group of rare connective tissue diseases (CTDs) deeply affecting patients' prognosis. Extra-muscular involvement is not rare and skin, joints and lung are the most common targets. However, also dyserythropoiesis has been described, carrying relevant issues on patients' management and follow-up, as for example, lymphopenia has been associated with an increased risk of rapid progressive interstitial lung disease in anti-MDA5 positive dermatomyositis. Conversely to systemic lupus erythematosus, thrombocytopenia has only been rarely described in IIM and very few authors focused on its potential prognostic implications. We describe five cases of thrombocytopenia in IIM patients positive for myositis specific (MSA) or associated (MAA) autoantibodies. These reports extend the spectrum of haematological features associated to IIM, focusing also on potential risk factors for thrombocytopenia occurrence.

Insights Into the Concept of Rheumatoid Arthritis Flare.

Identification of a pathological change in the course of systemic chronic immune-inflammatory diseases is key to delivering effective treatment strategies. In this context, one of the most compelling issues is the concept of flare. The multifaceted expression of disease activity in rheumatoid arthritis (RA) makes it challenging to provide an omni-comprehensive definition of flare, encompassing the pathology's different objective and subjective domains. Our incomplete understanding of the pathophysiological mechanisms underlying this process contributes to the partial comprehension of its potential clinical expression. This review focuses on the proposed pathophysiological processes underlying disease recrudescence in RA and the variable definitions adopted to capture flare in clinical practice through its objective, subjective, and temporal domains. Overall, what emerges is a complex landscape far from being unraveled.

Endoglin and Systemic Sclerosis: A PRISMA-driven systematic review.

Background: Systemic Sclerosis (SSc) is a rare autoimmune disease whose pathogenesis is still poorly understood. The Transforming Growth Factor ß superfamily is considered pivotal and a crucial role has been suggested for the type III receptor, Endoglin (ENG). The aim of this systematic review is to investigate and combine the current clinical and molecular available data, to suggest novel hints for further studies. Methods: We followed PRISMA guidelines; the search was performed on three databases (MEDLINE, Web of Science, Embase) in date November 2nd, 2021. Subsequent to the exclusion of duplicates, we applied as inclusion criteria: 1. focus on the relationship between ENG and SSc; 2. English language. As exclusion criteria: 1. ENG exclusively as a cellular biomarker; 2. no focus on ENG-SSc relationship; 3. review articles and 4. abstracts that did not add novel data. Eligibility was assessed independently by each author to reduce biases. We divided records into clinical and molecular works and subgrouped them by their study features and aim. Results: We selected 25 original papers and 10 conference abstracts. Molecular studies included 6 articles and 4 abstracts, whereas clinical studies included 17 articles and 6 abstracts; 2 articles presented both characteristics. Molecular studies were focussed on ENG expression in different cell types, showing an altered ENG expression in SSc-affected cells. Clinical studies mainly suggested that different disease phenotypes can be related to peculiar disregulations in soluble ENG concentrations. Discussion: Concerning the possible limits of our search, boolean operators in our strings might have been uneffective. However, the use of different strings in different databases should have reduced this issue at a minimum. Another bias can be represented by the selection step, in which we excluded many articles based on the role of Endoglin as a histological vascular marker rather than a signaling receptor. We tried to reduce this risk by performing the selection independently by each author and discussing disagreements. Our systematic review pointed out that ENG has a pivotal role in activating different TGFß-stimulated pathways that can be crucial in SSc pathogenesis and progression.

Multidisciplinary Approach in the Early Detection of Undiagnosed Connective Tissue Diseases in Patients With Interstitial Lung Disease: A Retrospective Cohort Study.

Interstitial lung disease (ILD) encompasses a wide range of parenchymal lung pathologies with different clinical, histological, radiological, and serological features. Follow-up, treatment, and prognosis are strongly influenced by the underlying pathogenesis. Considering that an ILD may complicate the course of any connective tissue disease (CTD) and that CTD's signs are not always easily identifiable, it could be useful to screen every ILD patient for a possible CTD. The recent definition of interstitial pneumonia with autoimmune features is a further confirmation of the close relationship between CTD and ILD. In this context, the multidisciplinary approach is assuming a growing and accepted role in the correct diagnosis and follow-up, to as early as possible define the best therapeutic strategy. However, despite clinical advantages, until now, the pathways of the multidisciplinary approach in ILD patients are largely heterogeneous across different centers and the best strategy to apply is still to be established and validated. Aims of this article are to describe the organization of our multidisciplinary group for ILD, which is mainly focused on the early identification and management of CTD in patients with ILD and to show our results in a 1 year period of observation. We found that 15% of patients referred for ILD had an underlying CTD, 33% had interstitial pneumonia with autoimmune feature, and 52% had ILD without detectable CTD. Furthermore, we demonstrated that the adoption of a standardized strategy consisting of a screening questionnaire, specific laboratory tests, and nailfold videocapillaroscopy in all incident ILD proved useful in making the right diagnosis.

The pathological diagnosis of the height of fatal falls: A mathematical approach.

The authors analyzed the injury pattern of 385 victims of fall from a height which underwent a complete autopsy, with the objective to investigate whether it was possible to construct a mathematical model to be used for height of the fall diagnosis. The cases were selected and enrolled according to a balanced stratification of the heights of the fall, allowing a subdivision into seven classes consisting of 55 subjects each: 6m or less, 9m, 12m, 15m, 18m, 21m, 24m or more (maximum 36m). For each case anthropologic and necroscopic data was collected and analyzed to obtain a standardized description of the injury pattern was obtained, dividing the body into 4 major anatomical areas (Head, Thorax, Abdomen, Skeleton), each of them further divided in 5 major organs. Every organ was finally divided into 5 objective degrees of injury. Statistical analysis was performed on all the available data using IBM SPSS Statistics 20, to test the performance of the "injury pattern assessment table" in the diagnosis of the height of the fall and to develop a related mathematical model. Our findings confirm that the height of the fall is significantly associated with age, weight of the body and the injury pattern. An Injury Pattern Assessment Table and two mathematical models which correlates the height of the fall with analyzed variables are presented.