Systemic sclerosis is a chronic autoimmune disease of still not fully understood pathogenesis. Fibrosis, vascular wall damage, and disturbances of innate and acquired immune responses with autoantibody production are prominent features. Systemic sclerosis has specific subsets with different autoantibodies, and differences in the affected skin areas. The suspicion of systemic sclerosis and establishing the diagnosis will be facilitated by the criteria created by EULAR/ACR experts. The treatment of this autoimmune disease remains a challenge for clinicians and new therapeutic options are constantly sought. The occurrence of various symptoms and the involvement of many organs and systems make systemic sclerosis a multidisciplinary disease and require a holistic approach. The present article summarizes different clinical features of systemic sclerosis and the profile of autoantibodies and discusses recent rules and future perspectives in disease management.

Primary Sjögren’s syndrome (pSS) is an autoimmune connective tissue disease affecting the exocrine glands, leading to damage of their structure and impairment of their function. In the course of pSS the internal organs may be involved and the symptoms may concern any system. Neurological disorders are one of the most common extraglandular manifestations of pSS. Available literature data estimate the prevalence of neurological symptoms as about 8.5–70% of patients diagnosed with pSS. The most common neurological complication of pSS is peripheral neuropathy, and in particular sensory polyneuropathy. Central nervous system involvement is much less common. There are also reports of various symptoms connected with damage to cranial nerves and the autonomic nervous system. A careful neurological evaluation, combined with neurophysiological tests, is recommended in patients with pSS. This review summarizes the neurological manifestations of pSS, their possible pathogenic mechanisms, diagnostic evaluation and potential treatment.

Polymyositis (PM) is an idiopathic inflammatory myopathy that affects striated muscles. Dermatomyositis (DM) is an idiopathic inflammatory myopathy with presence of skin symptoms. Both are characterized by acute or subacute onset, symmetrical proximal muscle weakness, the presence of mononuclear cell infiltrates of the muscles and increased activity of muscle enzymes. The treatment still remains glucocorticoids and disease-modifying drugs. Symptoms of PM/DM can be a signal of developing cancer. Known risk factors for cancer in patients with PM/DM are older age, male gender, dysphagia, skin necrosis, cutaneous vasculitis, rapid onset of the disease, elevated creatinine kinase (CK) and C reactive protein (CRP), and an increase in the erythrocyte sedimentation rate (ESR). Recently three new myositis-specific autoantibodies (MSA) predicting the risk of cancer have been discovered: melanoma differentiation-associated protein 5 (anti-MDA-5), transcription intermediary factor 1γ (TIF-1γ), and nuclear matrix protein NXP-2.

Primary Sjögren's syndrome is an autoimmune disorder with external exocrine glands dysfunction and multiorgan involvement. The pathogenesis of primary Sjogren’s syndrome is still unclear; however, our knowledge of the involvement of different cells (e.g., B and T cells, macrophages and dendritic cells) and pathways (BAFF/APRIL and interferons) leading to the development of autoimmunity is continually expanding. For clinicians, the most frequent symptoms are dryness of eyes and mouth, but often the patients have musculoskeletal symptoms and systemic manifestations. However, the increased risk of lymphoproliferative disorders in this group of patients, most commonly B-cell marginal zone lymphoma, is particularly important. Recent separation of IgG4-related diseases and attempts to create further diagnostic criteria for pSS testify to the difficulties, and at the same time a large interest, in understanding the disease so as to allow the effective treatment. This article draws attention to the problems faced by the clinician wishing to securely identify pSS by using accurate laboratory biomarkers and useful imaging tools and predict the development of complications associated with this, still not fully understood, autoimmune disease.

Macrolides are a group of antibiotics with a distinctive macrocyclic lactone ring combined with sugars (cladinose, desosamine). The action of macrolides is to block protein synthesis by binding to the subunit of 50S ribosome of bacteria. Prototype macrolide was erythromycin, which came into clinical practice in the 50s of the 20th century. Its antimicrobial spectrum covers the scope of the penicillins but is extended to the impact of atypical bacteria. In the 90s more drugs of this group were synthesized—they have less severe side effects than erythromycin, extended spectrum of Gram-negative bacteria. Macrolides are effective in treating mycobacterial infections especially in patients infected with HIV. It is now known that in addition to antibacterial abilities, macrolides have immunomodulatory effects—they inhibit the production of proinflammatory cytokines (TNF, IL1, 6, and 8) affect transcription factors (NF-κB) as well as costimulaton (CD 80) and adhesion molecules (ICAM). This review article focused not only on the their antimicrobial abilities but also on efficacy in the treatment of several inflammatory disorders independent of the infectious agent. Their wider use as immunomodulators requires further study, which can lead to an extension of indications for their administration.

Purpose of review The purpose of this article is to draw attention to the role of Epstein–Barr virus (EBV) virus in the pathogenesis of the primary Sjögren's syndrome. The article introduces the problem of consequences of EBV acute infection, and its reactivation, in association with the immune response modulation by the virus and with an increased risk of developing systemic autoimmune diseases and EBV-associated cancers. Recent findings The knowledge about the mechanisms by which the virus may stay for years in a latent phase, unrecognized by the host response immune cells is constantly expanding. There are several mechanisms and theories about EBV influence on the autoimmune process in Sjogren's syndrome (pSS), including the similarity (molecular mimicry) between viral EBNA-2 protein and Ro-60 antigen or EBER-1 and EBER-2 viral proteins and La antigen. Summary The influence of EBV infection on the development and course of pSS has been proven. It has also been established that both EBV and pSS result in the increased risk of tumor (especially lymphoma) development. In the light of these findings, new ways to manage EBV infections are being sought. Optimal methods for assessing EBV infection status are being devised. Research also aims at finding therapies, which target EBV through the inhibition of the autoimmune process and of viral activity. The present article is an attempt to discuss the most important phenomena and elements linking EBV infection to the primary Sjögren's syndrome.

Rheumatic fever (RF) is an autoimmune disease associated with group A β-hemolytic streptococcal infection, in the course of which the patient develops carditis, arthritis, chorea, subcutaneous nodules and erythema marginatum.Rheumatic fever diagnosis is based on the Jones criteria, developed in 1944, then revised twice by the American Heart Association (AHA), in 1992 and recently in 2015.The last revision of the Jones criteria consists mainly in the supplementation of the major criteria with echocardiographic examination, the introduction of a concept of subclinical carditis and the isolation of low, medium and high risk populations among the patients.AHA recommends that all the patients with suspected RF undergo Doppler echocardiographic examination after the Jones criteria have been verified, even if no clinical signs of carditis are present.

ObjectiveThe objective of this study was to determine the length of delay in diagnosis of inflammatory rheumatic diseases, and to indicate the main factors responsible for such delays.Material and methodsA retrospective multi-centre questionnaire survey carried out among 197 patients with diagnosed inflammatory rheumatic diseases or undergoing the diagnostic process.ResultsThe most common early symptoms of inflammatory rheumatic disease included joint pain (94%), joint swelling (78%), morning joint stiffness (77%), fatigue (76%), and sleep disturbed by joint pain (74%). When asked about the reasons for seeking medical help, most patients indicated intensification of the symptoms (89%) and the fact that the symptoms made them unable to perform daily activities or work (86%). Limited access to specialists (70%) and the conviction that the symptoms will resolve spontaneously (57%) had the biggest impact on delaying a visit to a doctor. Before visiting a rheumatologist, the patients consulted their symptoms with their general practitioners (GPs, 95%), orthopaedicians (43%), and neurologists (29%). Almost half of the patients (48%) consulted their symptoms with at least 2 non-rheumatologists, whereas as many as 21% of patients visited 4 or more specialists. After the onset of symptoms of rheumatic disease, 28% of patients delayed seeing any doctor for 4 months or longer. 36% of patients waited 4 months or longer for a referral to a rheumatologist. The great majority of the patients (85%) made an appointment with a rheumatologist within a month of receiving a referral. 25% of patients waited 4 months or longer to see a rheumatologist.ConclusionsDiagnostic delays result from both the level of patients’ awareness (ignoring early symptoms) and improper functioning of the health care system. In the case of the health care system, the source of delays is not only “queues to rheumatologists”, but also referring patients to non-rheumatologists.