Idiopathic inflammatory myopathies: one year in review 2021

Idiopathic inflammatory myopathies (IIMs) are a heterogeneous group of rare and complex connective tissue diseases, mainly characterised by inflammatory involvement of skeletal muscles. Several other organs may be affected, particularly lungs, heart, skin, gastrointestinal tract and joints, often determining the morbidity and mortality associated with these autoimmune disorders. The course is generally chronic and the onset subacute. This latter aspect, together with the rarity of these conditions, can result in a clinical challenge for the physician with a considerable diagnostic delay. The scientific literature makes continuous advances in the understanding of these diseases, in particular with regards to the pathogenesis, serological findings, diagnostic strategies and therapeutic approaches. The aim of this review is to highlight the most relevant literature contributions published on this topic over the last year.

Unattended compared to traditional blood pressure measurement in patients with rheumatoid arthritis: a randomised cross-over study.

BACKGROUND: Hypertension is characterised by a high prevalence, low awareness and poor control among rheumatoid arthritis (RA) patients. Correct blood pressure (BP) measurement is highly important in these subjects. The "unattended" BP measurement aims to reduce the "white-coat effect," a phenomenon associated with cardiovascular risk. Data on "unattended" BP measurement in RA and its impact on hypertensive organ damage are very limited. METHODS: BP was measured in the same patient both traditionally ("attended" BP) and by the "unattended" protocol (3 automated office BP measurements, at 1-min intervals, after 5 min of rest, with patient left alone) by a randomised cross-over design. Patients underwent clinical examination, 12-lead electrocardiography and trans-thoracic echocardiography to evaluate cardiac damage. RESULTS: Sixty-two RA patients (mean age 67 ± 9 years, 87% women) were enrolled. Hypertension was diagnosed in 79% and 66% of patients according to ACC/AHA and ESC/ESH criteria, respectively. Concordance correlation coefficients between the two techniques were 0.55 (95%, CI 0.38-0.68) for systolic BP and 0.73 (95%, CI 0.60-0.82) for diastolic BP. "Unattended" (121.7/68.6 mmHg) was lower than "attended" BP (130.5/72.8 mmHg) for systolic and diastolic BP (both p < .0001). Among the two techniques, only "unattended" systolic BP showed a significant association with left ventricular mass (r = 0.11; p = .40 for "attended" BP; r = 0.27; p = .036 for unattended BP; difference between slopes: z = 3.92; p = .0001). CONCLUSIONS: In RA patients, "unattended" BP is lower than traditional ("attended") BP and more closely associated with LV mass. In these patients, the "unattended" automated BP measurement is a promising tool which requires further evaluation.KEY MESSAGES"Unattended" automated blood pressure registration, aimed to reduce the "white-coat effect" is lower than "attended" value in rheumatoid arthritis patients."Unattended" blood pressure is more closely associated with left ventricular mass than "attende" registration.

One year in review 2020: idiopathic inflammatory myopathies.

The study of idiopathic inflammatory myopathies (IIMs) is acquiring growing importance among systemic autoimmune diseases and every year several articles are published about this group of diseases. Despite this growing interest, the management of IIMs is still critical due to the relative rarity of the condition. The availability of up-to-date knowledge of the evidence on this subject is essential to correctly understand this condition and provide the best care for the patients. The purpose of this review is to provide an overview of the most relevant literature contributions published in the last year.

One year in review 2019: idiopathic inflammatory myopathies.

The idiopathic inflammatory myopathies (IIMs) are a rare group of immune, systemic diseases characterised by muscle inflammation and frequently by extramuscular involvement. IIMs are heterogeneous with generally a chronic or subacute onset, which vary from less severe to more serious manifestations, not always easy to diagnose and even less to manage. In the past year, many studies have been published in order to clarify disease pathogenesis and improve patient management and treatment.The purpose of this review article is to provide an overview of the new insights in pathogenesis, serological findings, clinical manifestations and treatment of IIMs, summarising the most relevant studies published over the last year.

One year in review 2018: systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is a systemic autoimmune condition characterised by a wide spectrum of clinical manifestations, partly related to the disease itself, but also linked to its comorbidities and drugs adverse reactions. Following the previous annual reviews, we focused on new insights in SLE clinical features, pathogenic pathways, biomarkers of specific organ involvement and therapeutic strategies. We finally concentrated on SLE aspects that could significantly influence patients' quality of life and that need to be investigated in detail through the development and validation of disease-specific patient-reported outcomes.

Discrepancy between subjective symptoms, objective measures and disease activity indexes: the lesson of primary Sjögren's syndrome.

Mucosal dryness is a key clinical feature in primary Sjögren's syndrome (pSS) and its assessment relies on both objective measurement of residual secretion and subjective symptoms reported by patients. However, while the objective assessment and grading of glandular dysfunction can be easily performed, the spectrum of clinical symptoms encompassed by the terms 'dry eye' and 'dry mouth' is wide and heterogeneous. Therefore, patient reported outcomes (PROs) for dryness in pSS poorly correlate with the amount of glandular secretion. In addition, subjective dryness is not correlated with the severity of systemic disease and severely affects the patient quality of life even in presence of active extraglandular manifestations. The purpose of this review article is to provide an overview of glandular dysfunction in pSS as well as the impact of discrepancy between objective assessment, subjective symptom and extraglandular disease activity on disease management.

The prevalence and relevance of traditional cardiovascular risk factors in primary Sjögren's syndrome.

Accelerated atherosclerosis is a distinct feature of some inflammatory and autoimmune disorders and several specific autoimmune mechanisms and persistent inflammation have been identified to exert a pivotal role in precocious atherosclerotic damage in these disorders. Although increased atherosclerotic risk has been well established in some rheumatic autoimmune systemic diseases, such as systemic lupus erythematosus and rheumatoid arthritis, reliable data regarding the prevalence and pathogenetic mechanisms associated with increased atherosclerotic damage in primary Sjögren's syndrome are scarse. Indeed, primary Sjögren's syndrome is an autoimmune disorder characterised by chronic inflammation and autoimmune dysregulation that shares many pathogenic mechanisms and clinical features with systemic lupus erythematosus and rheumatoid arthitis. Higher prevalence of subclinical atherosclerosis has been observed in primary Sjögren's syndrome patients and recent population-based studies demonstrated an increased risk of cardiovascular events in these patients in comparison to general population. Among mechanisms associated with atherosclerotic damage, the prevalence and the role of traditional cardiovascular risk factors have been poorly investigated. In particular, the issue of whether the presence of these cardiovascular risk factors is associated with an increased risk of cardiovascular events needs to be further explored.

Targeting Inflammation to Prevent Cardiovascular Disease in Chronic Rheumatic Diseases: Myth or Reality?

Evidence for increased risk of cardiovascular morbidity and mortality in chronic inflammatory rheumatic diseases has accumulated during the last years. Traditional cardiovascular risk factors contribute in part to the excess of cardiovascular risk in these patients and several mechanisms, including precocious acceleration of subclinical atherosclerotic damage, inflammation, and immune system deregulation factors, have been demonstrated to strictly interplay in the induction and progression of atherosclerosis. In this setting, chronic inflammation is a cornerstone of rheumatic disease pathogenesis and exerts also a pivotal role in all stages of atherosclerotic damage. The strict link between inflammation and atherosclerosis suggests that cardiovascular risk may be reduced by rheumatic disease activity control. There are data to suggest that biologic therapies, in particular TNFα antagonists, may improve surrogate markers of cardiovascular disease and reduce CV adverse outcome. Thus, abrogation of inflammation is considered an important outcome for achieving not only control of rheumatic disease, but also reduction of cardiovascular risk. However, the actual effect of anti-rheumatic therapies on atherosclerosis progression and CV outcome in these patients is rather uncertain due to great literature inconsistency. In this paper, we will summarize some of the main mechanisms linking the inflammatory pathogenic background underlying rheumatic diseases and the vascular damage observed in these patients, with a particular emphasis on the pathways targeted by currently available therapies. Moreover, we will analyze current evidence on the potential atheroprotective effects of these treatments on cardiovascular outcome pointing out still unresolved questions.

One year in review 2017: primary Sjögren's syndrome.

Primary Sjögren's syndrome (pSS) is a complex and heterogeneous disease. Last year, a great deal of basic and clinical research was carried out in pSS. Following the previous reviews of this publishing series, we will herewith provide a critical digest of the most recent literature on pSS pathogenesis, clinical manifestations and treatment. More specifically, we will focus on the heterogeneity of the disease, on the underlying pathogenetic pathways and on the possible new targeted treatments on the horizon.

One year in review 2016: Sjögren's syndrome.

Sjögren's syndrome (SS) is a complex heterogeneous disease charactered by a broad spectrum of clinical and serological manifestations, including non-Hodgkin's lymphoma (NHL). Last year, 2015, was an exciting year for research into SS with novel insights into disease pathogenesis, clinical aspects and long-term outcomes. In addition, the use of biologic therapy in SS is rapidly expanding, with new evidence emerging regarding potential therapeutic targets. In this article, we will provide an overview of the recent literature on the pathogenesis, clinical features and novel treatments of SS.

Ciliate-adenovirus interactions in experimental co-cultures of Euplotes octocarinatus and in wastewater environment.

The aim of the present work was to study the dynamics of the interactions between human adenovirus and ciliates under both experimental and field conditions. Experimental co-cultures of the ciliated protozoan Euplotes octocarinatus and human adenovirus (HAdV) type 2 were established and virus internalization was investigated using nested PCR and direct immunofluorescence (IF). In addition, to study protozoa-virus interactions in the field, wild ciliates were isolated from active sludges of a wastewater treatment plant and analyzed for the presence of adenovirus using direct IF. In vitro experiments revealed HAdV type 2 inside Euplotes cells after 15min of contact and its persistence until at least 35 days post infection. In addition, our results showed the adsorption of adenovirus on the surface of wild ciliates. We conclude that HAdV is taken up by ciliates, however more studies are necessary in order to better investigate the mechanisms, the infectivity of internalized virus and the protective effects of internalization against disinfection.