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Bogna Grygiel-Górniak,1 Iwona Ziółkowska-Suchanek,2 Elżbieta Kaczmarek,3 Maria Mosor,2 Jerzy Nowak,2 Mariusz Puszczewicz1 1Department of Rheumatology and Internal Diseases, Poznan University of Medical Sciences, Poznan, Poland; 2Institute of Human Genetics, Polish Academy of Sciences, Poznan, Poland; 3Department of Bioinformatics and Computational Biology, Poznan University of Medical Sciences, Poznan, Poland Background: The aim of the research genetic study was to investigate the association between variants (C1431T and Pro12Ala) of the peroxisome proliferator-activated receptor (PPARgamma-2) gene, Trp64Arg polymorphism of the beta-3-adrenergic receptor gene and lipid profile in Polish population including group of 103 patients with connective tissue disease (CTD) and 103 sex- and age-matched controls in context of statin use. Methods: Anthropometric and biochemical parameters were measured by routine methods, followed by genotyping (TagMan® Genotyping Assays, PCR-restriction fragment length polymorphism analysis). Nearly 30% of CTD patients used statins and 10% of the control group. Results: Although there were no differences between alleles and genotypes prevalence between CTD vs control groups, interesting lipid-gene associations were noted in this study. A higher level of triglycerides (TAG) and TAG/high-density lipoprotein (HDL) ratios was observed in CTD patients compared to controls. Similar differences were noted in CTD and control groups without statin treatment. Atherogenic markers: the atherogenic index of plasma, TAG/HDL and low-density lipoprotein/HDL ratio were low in the analyzed groups. Of the six analyzed polymorphisms, the Pro12Pro or C14131C or Trp64Trp genotypes were related to higher TAG and TAG/HDL ratios in patients with CTD; however, the highest TAG values were observed in the presence of the Trp64Trp genotype. Conclusion: Lipid disorders were present in both groups independent of statin treatment (mixed dyslipidemia and hypercholesterolemia were observed in the CTD and control groups, respectively). The risk of dyslipidemia increases with age. The presence of Pro12Pro, C14131C and Trp64Trp genotypes is related to higher TAG level in CTDs, and of these the Trp64Trp variant most reliably predicts hypertriglyceridemia. Keywords: C1431T, Pro12Ala, Trp64Arg polymorphisms, lipoproteins, rheumatic diseases, statins

Background: The exact role of cytokines in inflammation and metabolic disorders in case of connective tissue diseases (CTDs) is under discussion. Methods: In this study, we intended to find the relationship between the selected cytokines in inflammatory and metabolic disorders in patients with CTDs (n = 55) and compared the results with those of control group subjects (n = 25) matched by age and body mass. We estimated their nutritional status by the bioimpedance method. The levels of basic biochemical parameters and the levels of adiponectin, resistin, and chemerin were also estimated. Multiple regressions and area under the curve in receiver operating characteristic (AUC-ROC) curve were used to find the associations of aforementioned parameters. Results: Patients with CTDs exhibited higher levels of chemerin than that of control group subjects. We found an inverse relationship between chemerin, RBC count, and hemoglobin levels. The concentration of adiponectin inversely correlated with the levels of platelets and concentrations of glucose and triglycerides as well as the erythrocyte sedimentation rate, whereas the concentration of resistin was positively correlated with WBC count, C-reactive protein (CRP), and the amount of used oral glucocorticosteroids. The mean ± standard deviation for the AUC-ROC curve in case of chemerin was the highest (AUC-RO C=0.714, p=0.0005) than that of both resistin and adiponectin. Conclusions: Chemerin and resistin levels are related to the inflammatory state in patients with CTDs, whereas adiponectin levels seem to be correlated with a protective effect. Chemerin can be considered as a marker differentiating a proinflammatory state present in CTDs.

We evaluated the prevalence of systemic sclerosis (SSc)-related autoantibodies and their clinical significance and compared the sensitivity of two line immunoblot assays on a prospective study group of 96 Polish SSc patients (ACR-EULAR 2013 criteria) whose sera were assessed by indirect immunofluorescence (HEp-2 and monkey liver) and line immunoblot assays: ANA Profile 3 and Systemic Sclerosis Profile by EUROIMMUN (Lübeck, Germany). Organ involvement was evaluated according to the EUSTAR Minimal Essential Data Set. The following autoantibodies’ prevalence was found: Scl-70 (36%), Ro-52 (28%), CENP-B (22%), CENP-A (20%), PM-Scl-75 (20%), PM-Scl-100 (14%), fibrillarin (7%), Th/To (7%), RNA polymerase III 11 kDa (5%), RNA polymerase III 155 kDa (3%), PDGFR (3%), NOR-90 (2%), and Ku (1%). Significant associations between the autoantibodies’ presence and organ involvement were found: ATA (dcSSc > lcSSc, less prevalent muscle weakness), Ro-52 (gangrene, DLCO < 60), CENP-B and A (lcSSc > dcSSc, normal CK), CENP-B (rarer digital ulcers and joint contractures), PM-Scl-100 and 75 (PM/SSc overlap, CK increase, muscle weakness, muscle atrophy), PM-Scl-100 (dcSSc unlikely), PM-Scl-75 (lung fibrosis), fibrillarin (muscle atrophy, proteinuria, conduction blocks, palpitations), Th/To (proteinuria, arthritis, muscle weakness, and rarer esophageal symptoms), RNA Polymerase III 11 kDa (arterial hypertension, renal crisis), RNA polymerase III 155 kDa (renal crisis), and PDGFR (dcSSc, tendon friction rubs). Additionally, the Systemic Sclerosis Profile was significantly more sensitive in detecting SSc-related autoantibodies than ANA Profile 3 (p = 0.002). In conclusion, individual autoantibodies associated with specific characteristics of SSc.

Introduction: Microvascular changes play a significant role in systemic sclerosis (SSc) and mixed connective tissue disease (MCTD). The most serious complications of SSc and MCTD are lung fibrosis (LF) and pulmonary hypertension (PH)., Aim: To determine the relationship of the changes observed in capillaries with the serological profile, LF, PH, and finger ulcerations in patients with SSc and MCTD., Material and Methods: The tested group comprised 80 persons (61 SSc, 19 MCTD); mean age 53.6 ±13.6 years. Patients were qualified to the LF group based on HRCT. Likelihood of PH was determined using echocardiography. The presence of antinuclear antibodies (ANA) was assessed using indirect immunofluorescence, while ANA profile, and sclerosis profile were assessed using EUROIMMUN kits, and antiphospholipid antibodies (aPL) using the ELISA method. Capillaroscopy was performed using the Nikon CPS 160 optical microscope., Results: The following were found: a relationship between occurrence of anti-SS-A ( p = 0.006) and anti-centromere B antibodies ( p = 0.012) and ramified vessels, between anti-SS-B and capillary haemorrhages ( p = 0.019), a positive correlation between NOR90 antibodies and winding loops ( p = 0.021), PM-Scl 100 antibodies and enlarged vessels ( p = 0.033), a negative correlation between Scl-70 antibodies and winding loops ( p = 0.033), and a relationship between aCL and winding loops ( p = 0.002). No relationship between the capillaroscopy image and PH risk was found. A positive correlation was found between avascularisation areas and LF and between giant capillaries and finger ulcerations. A negative correlation was found between U1-RNP antibodies and finger ulcerations ( p = 0.009), and a positive correlation between antibodies to fibrillarin and ulcerations ( p = 0.028)., Conclusions: SS-A, SS-B and anti-centromere antibodies are associated with the late phase of sclerodermic microangiopathy. Avascularisation areas significantly correlate with a higher prevalence of LF. U1-RNP antibodies have a protective role, while anti-fibrillarin antibodies are the risk factor for finger ulcerations., Competing Interests: The authors declare no conflict of interest., (Copyright: © 2022 Termedia Sp. z o. o.)

Competing Interests: The authors declare no conflict of interest.

Objectives: In rheumatic diseases, vitamin D supply is recommended as part of the prophylaxis and treatment of osteoporosis, especially in patients undergoing glucocorticoid therapy, but also due to its immunoregulatory and anti-inflammatory properties. We aimed to evaluate serum 25-hydroxyvitamin D [25(OH)D 3 ] levels in Polish patients with systemic sclerosis (SSc), systemic lupus erythematosus (SLE) and granulomatosis with polyangiitis (GPA), in relation to various clinical parameters, and to assess the initial range of doses for the purpose of further research., Material and Methods: 112 patients (39 with SLE, 44 with SSc and 29 with GPA), referred to the Department of Rheumatology and Internal Medicine in Poznan, Poland, were enrolled in this retrospective study. Demographic and clinical data were collected, including 25(OH)D 3 serum levels, vitamin D supplementation doses and season of blood sampling., Results: Mean (SD) serum 25(OH)D 3 concentrations were 31 (19.4) ng/ml for SLE, 28.8 (12.5) ng/ml for SSc and 28 (15.2) ng/ml for GPA, and they did not significantly differ between the groups. Vitamin D levels below the optimal range were found in 43.8% of SLE, 65.9% of SSc and 72.4% of GPA patients. 80% of patients reported vitamin D intake, with a mean daily dose of 1398 IU for SLE, 1345 IU for SSc and 1689 IU for GPA. Vitamin D insufficiency and deficiency were frequent among patients with rheumatic diseases, independently of the diagnosis and season., Conclusions: Patients with rheumatic diseases seem to require higher doses of vitamin D than recommended for the general population. The present results indicate the necessity to use higher initial doses of vitamin D in this group of patients (2000 to 4000 UI) and to maintain the dose of vitamin D regardless of the change of seasons., Competing Interests: The authors declare no conflict of interest., (Copyright: © 2021 Narodowy Instytut Geriatrii, Reumatologii i Rehabilitacji w Warszawie.)

Systemic lupus erythematosus (SLE) is a chronic and systemic autoimmune disease. SLE is described by production of autoantibodies and causes damage of many organs. T-cells play a crucial role in SLE pathogenesis. T-cells intensify inflammation through a number of processes, which leads to autoimmunization. CCR5 and MECP2 genes are linked with T-cells and pathogenesis of SLE. Polymorphisms in these genes are related with the prognostic factors of risk of disease onset and disease severity. The aim of this study was to estimate the influence of polymorphisms in MECP2 and CCR5 genes on the development and course of systemic lupus erythematosus. We examined 137 SLE patients and 604 healthy controls. We studied polymorphisms for CCR5 gene: rs333 and for MECP2: rs2075596, rs1734787, rs17435, and rs2239464. We genotyped our MECP2 samples and we performed a restriction fragment length polymorphism (RFLP) analysis for CCR5 samples. We showed a risk factor for allele T in rs17435 and for allele A in rs2075596 in MECP2. We noticed that MECP2 rs2075596 G/A, rs1734787 C/A, rs17435 A/T, and rs2239464 G/A polymorphisms are more prevalent in SLE patients than in healthy controls. We believe that above-mentioned MECP2 polymorphisms can be considered as SLE susceptibility factor., Competing Interests: The authors declare no conflict of interest.

Peroxisome proliferator-activated receptors ( PPAR gamma-2 ) and beta-3-adrenergic receptors ( ADRB3 ) are involved in the risk of hypertension. But their exact role in blood pressure modulation in patients with connective tissue diseases (CTD) is still not well defined. In this study, 104 patients with CTD and 103 gender- and age-matched controls were genotyped for Pro12Ala and C1431T polymorphisms of the PPAR gamma-2 gene and Trp64Arg polymorphism of the ADRB gene. Anthropometric and biochemical measurements were evaluated, followed by genotyping using TaqMan® SNP genotyping assays and polymerase chain reaction-restriction fragment length polymorphism. The prevalence of analyzed genotypes and alleles was comparable between patients with CTD and the control group, as well as hypertensive and normotensive subjects. Patients with CTD have lower body fat and higher body water amount, serum glucose, and triglyceride (TG) levels. Hypertensive subjects are older and have higher body mass, BMI, waist circumference (WC), body water content, glucose, and TG concentration. The multivariate analysis revealed that hypertensive subjects with Ala12/X or Trp64Trp have higher body mass and WC when compared to normotensive subjects. Trp64Trp polymorphism was also characterized by a higher TG level, while T1431/X subjects had higher WC. The presence of CTD, visceral fat distribution, and increased age are the predictors of hypertension development. Hypertensive patients with CTD and Trp64Trp polymorphism have an increased risk of visceral obesity development and metabolic complications, which in turn affects the value of blood pressure. In addition, either Ala12/X or T1431/X predicts the visceral body fat distribution in hypertensive subjects., Competing Interests: The authors have declared no conflicts of interest., (Copyright © 2020 Bogna Grygiel-Górniak et al.)

Objectives: The aim of the study was to explore whether TGF-β and IL-6 gene polymorphisms may be associated with SLE and assess the frequency of HLA-DRB1 alleles in Polish systemic lupus erythematosus (SLE) patients., Methods: 216 SLE patients and 552 healthy individuals were examined for TGF-β rs1800469 and rs1800470 by TaqMan SNP genotyping assay and for and IL-6(rs2069827 and rs1800795 using the PCR- RFLP method., Results: An increased frequency of TT genotype and T allele of the TGF β -509 C/T was found in SLE patients (p=0.02). The TGF-β 869 C allele was more frequent in SLE patients. The genotype-phenotype analysis showed association between the TGF β -509 C/T and mean value of CRP, ESR, haemoglobin, APTT, Pt and INR (p=0.05, p=0.03, p<0.001, p=0.03, p=0.03 and p=0.05, respectively) as well as anti-SSA and anti-Sm presence (p=0.04 and p=0.03, respectively); the TGF- β 869 T/C and mean value of APTT and INR (p=0.01 and p=0.05, respectively); the IL-6 -174 G/C and SLICC (p=0.05), anti-SSA (p=0.05) and anti-SSB (p=0.05). A higher TGF-β and IL-6 serum level were found in SLE patients compared to controls (both p<0.0001). In SLE patients with the TGF-β -509 TT genotype have shown positive association with the TGF-β serum levels. Polish SLE patients have strong positive association with HLA-DRB1*52.1, and negative with the HLA-DRB1*07:01 allele. HLA-DRB1*52.1 was also associated with higher TGF-β serum levels in the Polish population., Conclusions: Our results suggested that the TGF β -509 C/T variant may be considered as a genetic marker for SLE in the Polish population.

Competing Interests: The authors declare no conflict of interest.

Background: Granulomatosis with polyangiitis (GPA) is an antineutrophil cytoplasmic antibodies (ANCA)-associated necrotizing granulomatous vasculitis that affects small to medium size vessels. While the classical form with renal and respiratory tract involvement is mainly seen, a limited form (i.e., with no renal disease) may also occur. We present an unusual case of GPA manifesting merely as a bilateral ocular involvement and complete heart block., Case Presentation: We report a case of a 60-year-old male patient with a limited form of GPA who initially presented with bilateral chronic conjunctivitis and complete atrioventricular block. His visual acuity subsequently declined due to progression to bilateral panuveitis with exudative retinal detachment. The laboratory investigation revealed the elevation of acute phase reactants and strongly positive cytoplasmic ANCA (c-ANCA). Despite negative conjunctival and musculocutaneous biopsy results, the positive c-ANCA, and the clinical manifestation, i.e., heart and ocular involvement, led to the diagnosis of GPA. The remission was achieved with cyclophosphamide and methylprednisolone systemic therapy., Conclusions: A limited form of GPA may be a diagnostic chameleon. Though rare, it is essential to consider even extremely uncommon findings. Our patient is the first case of such a unique demonstration of the limited GPA manifesting as a bilateral ocular involvement and complete heart block.

Research in the past two decades has revolutionized our understanding of depressive illnesses. Proinflammatory cytokines have become a point of interest in the interconnecting areas of neuropsychiatric and autoimmune diseases. The cytokine hypothesis of depression suggests that pro-inflammatory cytokines play a primary role in the mediation of the pathophysiological characteristics of major depression, in which an inflammatory process may be induced by external and internal stressors, such as psychological and inflammatory diseases, respectively. The higher prevalence of depression, particularly in patients with chronic autoimmune connective tissue disorders (CTD), suggests that depression may present a dysfunctional adaptation of cytokine-induced sickness, which could manifest in times of an exacerbated activation of the innate immune system. Inflammation is thought to contribute to the development of clinical depression through its ability to induce sickness behaviors corresponding to the neurovegetative features of depression, through the dysregulation of the hypothalamic-pituitary-adrenal axis, alterations in neurotransmitter synthesis and reuptake, and through its involvement in the neuroprogression pathways. This review explores the complex interrelationships in which inflammatory responses alter neuroendocrine and neuropsychological regulation contributing to depressive symptoms in CTD. The prevalence and characteristics of depression, and its correlation to the levels of inflammatory cytokines and disease activity among different CTD will be reviewed., (© 2019 The Authors. Psychiatry and Clinical Neurosciences © 2019 Japanese Society of Psychiatry and Neurology.)

Biological disease-modifying antirheumatic drugs target specific components of the immune response related to pathogenesis of autoimmune and inflammatory diseases. Introduction of biologic therapies has enabled better disease control than conventional drugs and thus a reduction in comorbidity and mortality. However, there is concern about adverse effects of these drugs including infections, cancers and drug-induced autoimmune diseases. Patients undergoing biologic treatment are at small but significant risk of serious infections. The overall risk of malignancies in patients on biologics compared with the general population is not increased, but there is evidence of a higher risk of individual cancers. Surprisingly, biological treatment may induce autoantibody production and, rarely, development of autoimmune diseases. A growing body of literature has evaluated the risk of adverse effects during biologic therapies. This paper outlines adverse effects of biological disease-modifying antirheumatic drugs related to immune system disorders, both immunodeficiency and autoimmunity., Competing Interests: The authors declare no conflict of interest.

Systemic sclerosis (SSc) and mixed connective tissue disease (MCTD) are 2 conditions in which pulmonary hypertension (PH) can develop.We retrospectively analyzed the probability of PH in case of 83 patients (69 SSc and 14 MCTD). The European Society of Cardiology/European Respiratory Society (ESC/ERS) echocardiographic guidelines of 2015 were used for the evaluation.On the basis of an echocardiography, the patients were divided into 2 subgroups: patients with elevated probability of PH (EP) (n = 16) versus the group with a low probability of PH (LP) (n = 67). Of the 16 patients in the EP group, 15 were SSc patients and 1 was an MCTD patient, respectively, that is, 21.7% and 7.1% of all patients. Of the 16 patients with EP, 10 with SSc had right-heart catheterization, which excluded PH in 7 patients; hence, PH was estimated to be 11.6% in the SSc group. The distribution of the individual causes of PH was arterial PH 2.9%, PH associated with interstitial lung disease 4.3%, PH associated with left ventricular disease 1.5%, and PH of unknown origin 2.9%. Further, there was a significant difference between EP and LP in the incidence of the right bundle branch block in standard electrocardiography, left atrial and right ventricular dimension, tricuspid annular plane systolic excursion, and Doppler-derived tricuspid lateral annular systolic velocity (S') in echocardiography.Echocardiography, particularly those evaluating the parameters included in the ESC/ERS guidelines of 2015, appears to be a useful tool in the detection of patients with a high PH probability. Additional tissue Doppler echocardiography seems to be a good option.

Antiphospholipid syndrome (APS) in patients with systemic lupus erythematosus (SLE) displays a severe disease sub-phenotype with vascular manifestations ranging from peripheral thrombosis to neurologic and ophthalmic symptoms. The prevalence of morbidities including thrombosis, renal lesions, and cognitive impairment contributes to a higher risk of organ damage and a reduced quality of life in patients. In addition to the clinical heterogeneity, the diagnostic challenge is heightened in elderly patients as APS-related SLE is primarily diagnosed in young females. Many patients reach menopause due to the clinical association of premature menopause and improvements in diagnostic and therapeutic strategies in recent years. Although obstetric morbidity is not a concerning feature of the disease within this age group, a number of manifestations which may contribute to a decreased quality of life are present and must therefore not be disregarded. An improved prognosis derives from successful therapeutic regimens with minimal adverse effects in individual patients. The multifaceted management involves patient evaluation and risk stratification, followed by thromboprophylaxis efforts through the correction of modifiable risk factors, lifestyle recommendations, and pharmacological therapy. This review highlights the role of estradiol in the disease pathogenesis as well as the clinical complications and management of APS-related SLE in perimenopausal and postmenopausal patients.

Chikungunya virus (CHIKV) is an arthropod-borne alphavirus, transmitted by Aedes aegypti and Aedes albopictus mosquitoes. It is responsible for a febrile illness, typically accompanied by maculopapular rash and severe, incapacitating arthralgia. The disease, although generally self-limiting, frequently evolves into a long-lasting, debilitating rheumatic disorder, which shares many clinical features with rheumatoid arthritis (RA). The underlying mechanism by which CHIKV induces persistent arthritis remains under investigation, however, currently, attention is drawn to the fact, that chronic chikungunya (CHIK) and RA have many common cellular and cytokine pathways involved in their pathogenesis. Over the past decades, the virus has dispersed unexpectedly from tropical and subtropical regions of Africa and Asia, affecting millions of people worldwide. No licensed vaccine, nor antiviral drug against CHIKV is yet available. Treatment of acute CHIK is symptomatic, whereas in chronic stages, different disease-modifying anti-rheumatic drugs (DMARDs) have been used with variable success. Hence, chronic CHIK is an emerging rheumatic condition that rheumatologists have to deal with. This review provides brief insights into the epidemiology, pathogenesis, clinical features and management of Chikungunya disease, with special regard to post-chikungunya rheumatic disorder and its relationship with RA.

The presence of antinuclear antibodies (ANA) is mainly associated with connective tissue diseases (CTD). In addition, their presence is found in healthy people. These antibodies are more common in women and the elderly. Some drugs and xenobiotics are also important for the development of autoimmunity and ANA synthesis. Moreover, the deficiency of vitamin D in the body of patients correlates with occurrence of these antibodies. Unlike the healthy group, a positive ANA count was observed in patients with atopic dermatitis (AD) and in people with immune disorders. Antinuclear antibodies in low counts are also found in the course of chronic bacterial or viral infection and in patients with hematological malignancies. Also the possibility of false positive results, which may be caused by the choice of method used to determine antibodies, should be borne in mind. Taking into account all these factors, it is concluded that the ANA result itself has no diagnostic value., Competing Interests: The authors declare no conflict of interest.

Microorganisms inhabiting human body form a complex ecosystem. The mutual influence of the microbiome and the immune system of the host constitute the basis for numerous diseases, e.g. pseudomembranous colitis, inflammatory bowel disease, type 1 diabetes, atopic diseases, obesity, reactive arthritis. New molecular diagnostic methods and multi-center studies may help in understanding of the role of microbiota in health and disease. Rheumatoid arthritis has a multi-faceted etiology, and its causes are not entirely understood. There are indications for the influence of microbiomes of oral cavity, intestines, lungs and urinary tract on the development of rheumatoid arthritis. Interactions between microorganisms and human immune system play role in the pathogenesis of the disease., Competing Interests: The authors declare no conflict of interest.

Antinuclear antibodies (ANA) are primarily significant in the diagnosis of systemic connective tissue diseases. The relationship between their occurrence in allergic diseases is poorly documented. However, the mechanism of allergic and autoimmune diseases has a common thread. In both cases, an increased production of IgE antibodies and presence of ANA in selected disease entities is observed. Equally important is the activation of basophils secreting proinflammatory factors and affecting the differentiation of TH17 lymphocytes. Both autoimmune and allergic diseases have complex multi-pathogenesis and often occur in genetically predisposed individuals. The presence of antinuclear antibodies was confirmed in many systemic connective tissue diseases and some allergic diseases. Examples include atopic dermatitis, non-allergic asthma, and pollen allergy. Co-occurring allergic and autoimmune disorders induce further search for mechanisms involved in the aetiopathogenesis of both groups of diseases., Competing Interests: The authors declare no conflict of interest.

Retroperitoneal fibrosis (RPF) is a rare disease, characterized by inflammation and deposition of fibrotic tissue in the vicinity of the abdominal aorta and iliac arteries. We present a report of five patients admitted to our department between January 2014 and February 2017, diagnosed with RPF. Abdominal pain was the most common presenting symptom; however, in one patient, RPF was identified accidentally in routinely performed ultrasonography. In 4 cases, corticosteroids (CS) in combination with azathioprine were applied as first-line therapy, whereas one patient was treated with intravenous methylprednisolone pulses followed by oral CS. In this paper, clinical features as well as laboratory and radiographic findings together with management and treatment outcomes in patients with RPF are discussed. Given the rarity of the condition, it seems important to report every single case of RPF to help establish its management algorithm., Competing Interests: The authors declare no conflict of interest.

Retroperitoneal fibrosis (RPF) is a rare disease, hallmarked by inflammation and deposition of fibrous tissue around the abdominal aorta. This process may spread contiguously and involve adjacent structures, leading to many complications, among which the most frequent and most severe is ureteral obstruction. The condition usually has idiopathic origin (idiopathic retroperitoneal fibrosis - IRF), but can also develop secondarily to a number of factors. The etiology of the disease remains unclear. Current research suggests that about half of the cases of IRF may be a symptom of a recently discovered, clinically heterogeneous immunoglobulin G4-related disease (IgG4-RD). Corticosteroids are the first-line treatment for IRF, but effective attempts to use immunosuppressants are also made. This paper presents the current state of knowledge on the etiopathogenesis, clinical presentation, diagnosis and therapeutic possibilities in different forms of RPF. Based on the latest research, an analysis of the relationship between IRF and IgG4-RD was performed.

The majority of rheumatic diseases belong to the group of autoimmune diseases and are associated with autoantibody production. Their etiology is not fully understood. Certain medications and environmental factors may have an influence on the occurrence of rheumatic diseases. Establishing a cause-effect relationship between a certain factor and disease induction is not always simple. It is important to administer the drug continuously or monitor exposure to a given factor in the period preceding the onset of symptoms. The lack of previously diagnosed autoimmune disease, or finally the lack of symptoms within a few weeks/months after discontinuation of the drug/cessation of exposure, is also important. The most frequently mentioned rheumatic diseases caused by drugs and environmental factors include systemic lupus erythematosus, scleroderma, systemic vasculitis, polymyositis, dermatomyositis, and Sjögren's syndrome. The objective of this study is to summarize current knowledge on rheumatic diseases induced by drugs and environmental factors.

Many connective tissue diseases are characterized by fatigue, which is described in the literature as prostration, weakness, lassitude or asthenia. In many other diseases (autoimmune, neurologic or metabolic) fatigue impinges on daily activities and thus influences the quality of life. Different molecular backgrounds are involved in the development of fatigue. Not only does the immunosuppressive treatment of autoimmune diseases reduce fatigue, but also selective nutritional components may have an effect on secretion of cytokines which are responsible for development of the sensation of tiredness (e.g. secretion of interleukin-6). The beneficial influence of selected food components (such as polyunsaturated omega-3 fatty acids, nutritional antioxidants or adequate fat intake with the diet) on proinflammatory cytokine secretion has been demonstrated in many studies. In this review, the biochemical, neurological and nutritional aspects of fatigue in autoimmune diseases are underlined.

Systemic sclerosis (SSc) is an autoimmune connective tissue disorder of unknown etiology. This disease is characterized by a large variety of clinical patterns, which include the fibrosis of skin and visceral organs causing a variety of clinical manifestations. Genetic and environmental factors participate in the etiology of this disease; however, recently many studies underline the oxidative background influencing the course and complications of this disease. Reactive oxygen species (ROS) synthesized in SSc can mediate extra- and intracellular oxidative processes affecting endothelial cells and fibroblasts. The estimation of prooxidative markers in the pathogenesis of SSc can enable the identification of useful markers for disease activity and, thus, may help in planning appropriate therapy focusing on the fibrotic or vascular pattern. Recently, many attempts have been made to find antioxidative molecules (nutritional and pharmacological) reducing the prooxidant state in a variety of cells--mainly in endothelium and proliferating fibroblasts. This paper presents both the background of oxidative stress processes in systemic sclerosis mediated by different mechanisms and the evidence suggesting which of the dietary and pharmacological antioxidants can be used as therapeutic targets for this disease.

Three-dimensional imaging of the mucosa of the lower lip and labial minor salivary glands is demonstrated in vivo using swept source optical coherence tomography (OCT) system at 1310 nm with modified interface. Volumetric data sets of the inner surface of the lower lip covering ~230 mm(2) field are obtained from patients with Sjögren's syndrome and a control group. OCT enables high-resolution visualization of mucosal architecture using cross-sectional images as well as en-face projection images. Comprehensive morphometry of the labial minor salivary glands is performed, and statistical significance is assessed. Statistically significant differences in morphometric parameters are found when subgroups of patients with Sjögren's syndrome are analyzed.

The antiphospholipid syndrome (APS) is a systemic non-inflammatory and autoimmune connective tissue disease. ASP is characterized by formation of autoantibodies against cell membrane phospholipids which act as procaogulation factors. Association with recurrent pregnancy loss, intrauterine growth retardation, preeclampsia and placenta ablation makes ASP a serious perinatologic problem. The primary form can evaluate into lupus-like disease or full-symptomatic lupus erythematosus, both usually preceded by acute form of APS. In pregnant women this situation may present considerable diagnostic problems. The aim of this work was to present a patient with acute primary antiphospholipid syndrome during pregnancy and puerperium, which subsequently was diagnosed as systemic lupus erythematosus flare. Such progression is rare and, despite several previous reports, requires extensive explanations and research.

Antibodies to citrullinated proteins/peptides (ACPAs) are the second serological marker to have recently been included in the 2010 ACR/EULAR Rheumatoid Arthritis (RA) Classification Criteria, which are focused on early diagnosis and therapy. This review discusses their history and some clinical aspects of ACPAs, focusing on the diagnostic utility of anti-cyclic citrullinated peptide (anti-CCP) antibodies as a marker of RA as compared to the widely used rheumatoid factor (RF). Simultaneously, this review aims to raise physician awareness and interest in anti-citrullinated vimentin antibody (anti-Sa/anti-MCV), another member of the ACPA family, which appears to have a better predictive value as a marker of RA than anti-CCP or RF and correlates closely with disease activity and therapeutic response among patients with RA.