Systemic Connective Tissue Diseases Research Articles

An expert opinion of the Polish Cardiac Society Working Group on Pulmonary Circulation and the Polish Society for Rheumatology on the diagnosis and treatment of pulmonary hypertension in patients with connective tissue disease.

Systemic connective tissue diseases (CTDs) comprise a large group of diseases that are auto-immune in nature and characterized by the involvement of multiple systems and organs. Pul-monary hypertension (PH) of various etiologies may develop in the course of CTD, including pulmonary arterial hypertension (PAH), PH secondary to the lung disease, postcapillary PH in the course of left heart disease, and chronic thromboembolic pulmonary hypertension (CTEPH). In addition, the different forms of PH may coexist with each other. Among patients with CTD, PAH occurs most commonly in those with systemic sclerosis, where it affects ap-proximately 8%-12% of patients. The prognosis in patients with untreated PAH is very poor. It is particularly important to identify the high-risk CTD-PAH population and to perform effi-cient and accurate diagnostics so that targeted therapy of the pulmonary arteries can be intro-duced. Echocardiography is used to screen for PH, but clinical and echocardiographic suspicion of PH always requires confirmation by right heart catheterization. Confirmation of PAH ena-bles the initiation of life-prolonging pharmacological treatment in this group of patients, which should be administered in referral centers. Drugs available for pharmacological management include endothelin receptor antagonists, phosphodiesterase-5 inhibitors, and prostacyclins.

Inflammatory rheumatic diseases and pregnancy.

Pregnancy in a patient diagnosed with systemic connective tissue disorder is a challenge that requires a close co-operation between a rheumatologist and gynaecologist. Good control over the activity of the underlying condition and the choice of appropriate time for planning a pregnancy have direct effect on the pregnancy results in these patients. Applying gynaecological supervision adequate to the increased risk of complications is also very important. The aim of this study is to present the current knowledge on the care over pregnant patients with systemic connective tissue diseases and to draw attention to the importance of pregnancy planning in this group of patients.

Predictors of Quality of Life in Patients With Rheumatoid Arthritis in Pakistan: A Cross-Sectional Study.

Retracted

Satisfaction with analgesic therapy in patients with rheumatic diseases in real clinical practice, according to the KOMPAS study (Quality of Pain Relief in the Opinion of Patients with Arthritis and Back Pain)

Patient's satisfaction with treatment is a fundamental indicator of the quality of medical care, which is especially important for assessing the effectiveness of therapy for musculoskeletal pain in rheumatic diseases (RD).Objective: to determine satisfaction of patients with RD with pain relief therapy and to analyze the factors influencing the subjective assessment of analgesic therapy.Patients and methods. Anonymous survey of 1040 patients (age 55.8±14.0 years, 76.8% were women) with RD, rheumatoid arthritis (RA), osteoarthritis (OA), spondyloarthritis, systemic connective tissue diseases and gout, was carried out. The presence of pain and its therapy, satisfaction with treatment, and patient's opinion about the reasons of low pain relief effectiveness were assessed. The dependence of the presence of dissatisfaction with treatment on a number of demographic and clinical factors was analyzed.Results and discussion. 71.5% of patients experienced pain in one or more joint areas and/or in the back. 70.6% of patients used non-steroidal antiinflammatory drugs (NSAIDs), 1.6% – paracetamol, 40.0% – non-drug modalities and methods of alternative medicine. 15.6% of the respondents were completely satisfied with the treatment, 64.0% were partially satisfied, and 20.4% were completely dissatisfied. The main subjective reasons for the insufficient effectiveness of analgesic therapy were: fear of taking prescribed medications due to possible complications (45.4%), low effectiveness of drugs (15.7%), insufficient attention of doctors (20.3%). Male gender, body mass index >30 kg/m2 , severe pain, pain in several areas, and the diagnosis of OA were statistically significantly associated with treatment dissatisfaction. In contrast, patients with RA showed greater satisfaction with treatment.Conclusion. Most patients with RD are dissatisfied with the results of pain therapy. Educational work with patients and a personalized approach to prescribing analgesic therapy is needed.

Cardiac involvement in primary Sjӧgren's syndrome.

Primary Sjӧgren's syndrome (pSS) is an autoimmune-mediated, inflammatory, and systemic connective tissue disease (CTD), especially in middle-aged women, which often involves multiple systems and organs of the body. In fact, the heart is an important target organ in patients with pSS. In recent years, it has been confirmed that the morbidity of cardiac involvement has increased in patients with pSS, and cardiovascular disease (CVD) is one of the main causes of death. The increased risk of CVD in pSS patients is associated with a great variety of risk factors, such as age, gender, hypertension, diabetes mellitus, dyslipidemia, disease duration, extra-glandular manifestations, therapeutic drugs of pSS, and so on. Early recognition and effective treatment of CVD may play a crucial role in improving adverse cardiovascular prognosis. Whereas cardiac involvement is closely related to patient prognosis and survival, the cardiac involvement of patients with pSS remains poorly studied. Therefore, this article reviews the cardiovascular risk factors, clinical manifestations of cardiac involvement, cardiovascular biomarkers, and therapeutic strategies of pSS patients.

ІМУНОЗАЛЕЖНІ МЕХАНІЗМИ ТА МОЛЕКУЛЯРНО-ГЕНЕТИЧНІ ОСОБЛИВОСТІ У ХВОРИХ НА СИСТЕМНІ ХВОРОБИ СПОЛУЧНОЇ ТКАНИНИ З КРІОГЛОБУЛІНЕМІЧНИМ СИНДРОМОМ

Introduction: Cryoglobulinemic syndrome (CGS) is an immune-related process caused by cryoglobulins composition in the blood in small or medium vessels. Most frequently, CGS is triggered by lymphotropic viruses, immune-related and oncological diseases. Objectives: Studying the immune-related mechanisms, molecular and genetic characteristics of patients with systemic autoimmune diseases (SAD) against the cryoglobulinemic syndrome. Methods: Among 380 patients with SAD, in 94 (57.6%) progressing chronic EBV-infection was diagnosed, and 22.1% of patients were diagnosed with progressing chronic HSV 1/2-infection based on DNA virus identification through the polymerase chain reaction (PCR) in three biological media (blood, saliva, mucus membrane scraping). Results: Analysis of the cryoglobulins in such patients showed that CGS was diagnosed in 118 (31.1%) patients with the mean concentration of CG1.68±0.33 g/l at a rate of 0.48 ±0.10g/l. The patients with the systemic connective tissue diseases with CGS demonstrated statistically lower miR-146а expression which resulted in the abnormal production of pro-inflammatory cytokines, the highest TLR9 expression on monocytes, slightly lower on lymphocytes, and the lowest on granulocytes; the increase in the relative amount of cytolytic T-lymphocytes, IL2 receptor lymphocytes, activated CD HLA DR+-lymphocytes against the reduction of NK-cells and regulatory suppressor CD4+/25+-cells was observed. The idiopathic and initiated oxidative monocyte capacity in CGS patients distinctly tended to increase, as compared to patients without CGS and normal individuals. Conclusions. Cryoglobulins may act as the so-called bridge between viral infections and the autoimmune processes. CGS was diagnosed in 31.1% of patients. Despite a substantial number of studies dedicated to the cryoglobulinemic syndrome, the peculiarities of the immune reaction of such patients need further research, since they create the risks of secondary vasculitis against SAD

The effect of vitamin D3 and thyroid hormones on the capillaroscopy-confirmed microangiopathy in pediatric patients with a suspicion of systemic connective tissue disease-a single-center experience with Raynaud phenomenon.

Raynaud phenomenon (RP) may be the first manifestation of a systemic connective tissue disease (SCTD). Early detection of dysfunction of small vessels called microangiopathy is essential for the diagnostic process. The focus of this single-center, retrospective study was to investigate the potential dependencies between microvascular image and laboratory markers measured in children with RP. The study analyzed the nail-fold video-capillaroscopy (NVC) findings and laboratory results of 81 children between the ages 6 and 17 who were referred to pediatric rheumatologist with a suspicion of SCTD. Out of 52 patients presenting with RP at the time of evaluation, abnormalities in capillary microscopic imaging were found in 34. NVC findings were then compared to levels of specific biomarkers in serum. Vitamin D3 serum levels have been significantly decreased in patients with RP (23.4 ng/mL ± 8.76 vs. 30.0 ng/mL ± 12.66, P = 0.0148). There were positive significant correlations between levels of vitamin D3 and acute-phase reactants in serum, such as C-reactive protein (P = 0.0292). Furthermore, free thyroxine levels (fT4) in patients with both RP (P = 0.0126) and micro-angiopathy (P = 0.05496) persisted in the lower range of the normal limit (< 1.0 ng/dL). Regular oral supplementation of vitamin D3 should be always considered in children with RP if deficiency is found. Additionally, low fT4 level (< 1.0 ng/dL) should be considered as an indication to perform NVC in patients suspected of SCTD even when they do not present RP.

H2 FPEF score predicts atherosclerosis presence in patients with systemic connective tissue disease.

BackgroundCardiovascular diseases are common cause of morbidity and mortality in patients with systemic connective tissue diseases (SCTD) due to accelerated atherosclerosis which couldn't be explained by traditional risk factors (CVDRF).HypothesisWe hypothesized that recently developed score predicting probability of heart failure with preserved ejection fraction (H2FPEF), as well as a measure of right ventricular‐pulmonary vasculature coupling [tricuspid annular plane systolic excursion (TAPSE)/pulmonary artery systolic pressure (PASP) ratio], are predictive of atherosclerosis in SCTD.Methods203 patients (178 females) diagnosed with SCTD underwent standard and stress‐echocardiography (SE) with TAPSE/PASP and left ventricular (LV) diastolic filling pressure (E/e') measurements, carotid ultrasound and computed tomographic coronary angiography. Patients who were SE positive for ischemia underwent coronary angiography (34/203). The H2FPEF score was calculated according to age, body mass index, presence of atrial fibrillation, ≥2 antihypertensives, E/e' and PASP.ResultsMean LV ejection fraction was 66.3 ± 7.1%. Atherosclerosis was present in 150/203 patients according to: 1) intima‐media thickness>0.9 mm; and 2) Agatstone score > 300 or Syntax score ≥ 1. On binary logistic regression analysis, including CVDRF prevalence, echocardiographic parameters and H2FPEF score, only H2FPEF score remained significant for the prediction of atherosclerosis presence (χ2 = 19.3, HR 2.6, CI 1.5‐4.3, p < 0.001), and resting TAPSE/PASP for the prediction of a SE positive for ischemia (χ2 = 10.4, HR 0.01, CI = 0.01‐0.22, p = 0.004). On ROC analysis, the optimal threshold value for identifying patients with atherosclerosis was a H2FPEF score ≥2 (Sn 60.4%, Sp 69.4%, area 0.67, SE = 0.05, p < 0.001).ConclusionsH2FPEF score and resting TAPSE/PASP demonstrated clinical value for an atherosclerosis diagnosis in patients diagnosed with SCTD.

Methods for assessment of microcirculation

To date, many methods have entered rheumatological practice in order to evaluate more accurately the microcirculation. Most of them are non-invasive or minimally invasive, easily accessible, providing different information depending on the specifics of the study.&#x0D; Over the years, some of them (such as chromametry and volumetry) have lost their routine application and have acquired rather historical significance, while others have become an integral part of the rheumatological armentarium. An example of this is video capillaroscopy, which, through its dynamic development over the last 20 years, has evolved in the knowledge of changes in microcirculation in systemic sclerosis, as well as in other systemic connective tissue diseases. The expansion of knowledge in this area has led to the updating of the classification criteria for systemic sclerosis, allowing the addition of capillaroscopic changes as recognized by the European League against Rheumatism (EULAR).&#x0D; Another important indication for performing video capillaroscopy is the differentiation of primary from secondary Raynaud's phenomenon. Laser Doppler perfusion imaging is also used to detect secondary Raynaud's phenomenon in order to distinguish whether reduced blood flow is due to systemic autoimmune disease. Thermography also has a helpful role in diagnosing Raynaud's phenomenon, but unlike the other two methods, it has a much more limited application due to the lack of discriminative ability to distinguish nutritional from thermoregulatory blood flow, which leads to erroneous conclusions in pathological conditions.&#x0D; Venous occlusive plethysmography is one of the "gold standards" in the assessment of vascular function in health and disease and is an accurate, reproducible and convenient method to assess the effect of new vasoactive drugs. However, its application in everyday rheumatological practice is quite limited.

Radiologic examination of pharynx for oropharyngeal dysphagia in a patient with dermatomyositis (clinical observation)

Dermatomyositis – a systemic connective tissue disease, from the group of idiopathic inflammatory myopathies. Affection of the skeletal muscle tissue in inflammatory myopathies causes progressive muscle weakness. In dermatomyositis reduction in contraction ability of the muscles involved in the act of swallowing, leads to violation of the mechanism of respiratory protection from getting food masses inside and from development of dysphagia.The purpose of the study was to evaluate the changes detected in the process of radiologic examination of the dermatomyositis patient’s throat.Materials and methods. Patient K. 61 years old, he has dermatomyositis and clinical signs of oropharyngeal dysphagia. The patient underwent a pharyngeal radiologic examination, which allowed to confirm the disturbance of swallowing function and to establish the fact of aspiration.Results and discussion. Radiological examination of the pharynx showed signs of pharynx hypotony, pharyngeal ectasia, stasis, residual amount of radiocontrast agent in caudal section of larynx, penetration, aspiration.Conclusion. Radiological examination of the pharynx in patients with dermatomyositis is important method examination of studying swallowing disorders and predicting risk of serve complications.The peculiarity of this case is the demonstration of swallowing dysfunction dynamics in a patient with dermatomyositis at admission to the clinic and after treatment.

СИСТЕМНАЯ И СТОМАТОЛОГИЧЕСКАЯ ПАТОЛОГИЯ ПАЦИЕНТА С РЕВМАТОИДНЫМ АРТРИТОМ НА ФОНЕ МЕТОТРЕКСАТ-ТЕРАПИИ (клинический случай)

Background. One of the urgent problems of modern medicine is the formation and development of an interprofessional approach to the complex therapy of patients with combined pathology. An interdisciplinary approach to the examination and treatment of patients with concomitant diseases is due to the interconnection of the oral mucosa with systemic diseases. Literature data indicate the pathogenetic commonality of systemic connective tissue diseases, including rheumatoid arthritis with periodontitis and oral mucosa diseases, which is based on a diffuse inflammatory process.&#x0D; Objectives ― study of a clinical case of combined lesion of the oral mucosa of a patient with rheumatoid arthritis during treatment with methotrexate.&#x0D; Methods. We examined patient R. — an elderly man of 72-year-old with ulcerative and necrotic lesions of the oral mucosa, simultaneously suffering from rheumatoid arthritis and receiving methotrexate as maintenance therapy. The patient was sent to the Department of Therapeutic Dentistry of Omsk State Medical University, to the clinical base of dental clinic № 1 in Omsk in order to clarify the diagnosis and prescribe complex treatment.&#x0D; Results. Patient R. revealed a combined ulcerative-necrotic lesion of the oral mucosa and gums with rheumatoid arthritis. Joint general and local medical treatment by a dentist of lesions of the oral mucosa, gums, examination, observation in dynamics and the advice of a physician-therapist in terms of prescribing maintenance therapy for rheumatoid arthritis by reducing the dose of the drug (methotrexate) gave a joint positive effect in a comprehensive interdisciplinary approach to the treatment of a patient with comorbid pathology.&#x0D; Conclusions. Our research has shown that it is necessary to increase activity in the development of an interdisciplinary collaboration between dentists and internists. The continuity and friendship of doctors of different specialties make it possible to get a positive result in the treatment and improve the quality of life of patients.

548 Heterotopic Ossification or Dystrophic Calcinosis Cutis in Burn Scars - A Case Series

Abstract Introduction Heterotopic ossification of large joints is a commonly seen and described entity in Burn survivors. We have observed a rather uncommon phenomenon in a series of 4 Burn survivors who presented to our Burn center for scar treatment and open wounds many years after their injuries. A chronic, small, splinter-like subcutaneous irritation led to open wounds and infections in their burn scars. A similar condition is known as dystrophic calcinosis cutis in the dermatologic literature, but caused by calcium metabolism changes in otherwise healthy skin. Methods The demographics and history of illness was documented in all 4 patients. A literature review was performed. The calcified bone-like irritant was removed surgically in all patients, sometimes in more than one location. Pathologic evaluation was performed on the removed specimen. Strategies leading to healing of the wounds were evaluated. Results Four publications were found addressing this phenomenon in Burn scars, all case reports. All of our patients had undergone curettage of the lesions by a dermatologist in the past without resolution. All lesions were excised surgically and proved to extend subcutaneously far more extensively than the visible skin lesion. X rays proved not helpful in preoperative assessment of the extent. All wounds healed after the irritant was removed. Conclusions All lesions appeared to have formed along tendon or fascia remnants underneath the burn scars, exerting pressure on the overlying epithelium which eventually led to breakdown and necrosis. This finding prompted the term “heterotopic cutaneous ossification”. Chronic tension and inflammation were assumed in the etiology, rather than calcium metabolism abnormalities or systemic connective tissue disease, which were not present in these patients. Research is needed to establish the prevalence, cause, and prevention of this condition in Burn survivors.

Rheumatoid Hand and Other Hand-deforming Rheumatic Conditions.

The hand and wrist are commonly involved in rheumatic conditions, particularly rheumatoid arthritis and other systemic connective tissue diseases. With spondyloarthritis, hand and wrist involvement frequently occurs in psoriatic arthritis but generally does not occur in the remaining subtypes. The hand and wrist may also be affected in various metabolic and endocrine diseases, but these lie beyond the scope of this review.Radiographs may demonstrate the presence of joint space narrowing, bone loss, cysts and erosions, malalignments, and osteolysis. They may also show regions of soft tissue swelling or thinning, and detect calcifications. Ultrasonography and magnetic resonance imaging (MRI) enable evaluation of the soft tissues, particularly the synovium, tenosynovium, and tendons. Furthermore, erosions are better demonstrated than on radiographs. MRI allows evaluation of periarticular bone marrow edema.This article discusses typical imaging features of the hand and wrist in rheumatologic conditions including the advantages and limitations of the various methods.

Analysis of clinical study of salivary glands and the rate of oral fluid secretion in children with systemic connective tissue diseases

This article reflects clinical studies on the cause of xerostomy in children with systemic connective tissue disease (CTS). For more than 20 years, we have provided comprehensive dental care to children with CTS and have found that the presence of xerostomy and destructive changes in the parotid salivary glands in children with CTS, are characteristic of the manifestation of Shegren syndrome and contributed to: development of multiple tooth decay in the cervical area, the presence of abundant plaque on the teeth, inflammatory diseases of the gums and mucous membranes of the mouth and lips, the joining of secondary infection.

Клинический случай развития ретроперитонеального фиброза (болезни Ормонда) в практике ревматолога

Забрюшинный (ретроперитонеальный) фиброз (РПФ) — редкое заболевание, характеризующееся разрастанием воспалительной и фиброзной ткани в забрюшинном пространстве. Обычно эти разрастания локализуются вокруг инфраренальной части брюшной аорты и подвздошных артерий, часто покрывая мочеточники или другие органы брюшной полости. Идиопатические заболевания составляют 70% случаев. РПФ может быть ассоциирован с иммуноглобулином G4 (IgG4), что является причиной двух третей случаев идиопатического РПФ. Вторичный РПФ может развиться вследствие инфекций, злокачественных новообразований, приема лекарственных препаратов, забрюшинного кровотечения или других заболеваний. Описан клинический случай идиопатического РПФ, вероятно, ассоциированного с IgG4, у пациента, прошедшего симптоматическое оперативное лечение в урологической клинике и продолжившего лечение у ревматолога патогенетической терапией (метилпреднизолон и мофетила микофенолат) со значительным улучшением. Для ревматологов данная патология представляет безусловный клинический интерес, поскольку, с одной стороны, в основе данных изменений лежат процессы, аналогичные таковым при системных заболеваниях соединительной ткани, а с другой стороны — это редкая патология, встречаемость РПФ составляет от 0,1 до 1,3 случая на 100000 человек в год. Однако чаще такие пациенты сразу попадают на прием к врачам смежных специальностей (урологам, нефрологам, хирургам, сосудистым хирургам), что подчеркивает важность разбора такого клинического случая.

Abstract P700: Stroke in Lupus Spectrum Disorder: A Single-Center Case Series in the Kentucky Appalachian Region

Background: Lupus spectrum disorders (LSDs) encompass systemic lupus erythematosus (SLE), lupus nephritis, antiphospholipid syndrome (APS), and mixed connective tissue disease. Neuro-vascular disorders associated with LSDs include headache, transient ischemic attack, vascular cognitive impairment, immune-mediated cerebral vasculitis, thrombo-embolic stroke, cortical venous sinus thrombosis (CVST), and intracranial hemorrhage. We conducted a retrospective survey of neuro-vascular complications of LSDs in a university hospital-based stroke service within Kentucky Appalachia. Methods: Consecutive hospital admissions and consultations were retrospectively surveyed from January 2012 to January 2017. Inpatient medical records were reviewed to accumulate laboratory data and neuro-imaging studies. Diagnoses of SLE and other LSDs conformed to Systemic Lupus International Collaborating Clinics criteria. Results: During this 5-year survey, 10,953 cases of ischemic or hemorrhagic stroke were admitted or consulted and 75 patients (0.7%) met criteria for LSD. Female:male ratio favored women over men (4:1) and the median age was 48 years (range 22 - 87). The most common LSD was SLE and/or APS (n = 61; 81.3% total). The most frequent neuro-vascular complication was acute thrombo-embolic stroke (n = 30; 40%). Among 42 (56%) ischemic stroke patients, 17 had multifocal infarcts, 11 had isolated cortical strokes, and 7 had lacunes in the subcortical structures or brainstem. The majority of ischemic stroke patients (n = 48; 64%) did well and were ultimately discharged to home; 19 (25.3%) were re-admitted with recurrent stroke. Four patients had vasculitis (5.3%) and three had moyamoya syndrome (4%). Intracranial hemorrhage affected four patients (5.3%); hemorrhagic cerebral micro-angiopathy occurred in three (4%). Two patients had CVST (2.7%). Conclusion: Neuro-vascular complications of LSDs are heterogeneous and affect youthful patients with female preponderance. The large proportion of thrombo-embolic stroke in our population may be attributed to immune-mediated arterial coagulopathy or cardiac structural disease. Most patients with LSD complicated by ischemic stroke had good outcome, but there was high risk of readmission due to recurrence.

Systematic review of physical exercise for patients with idiopathic inflammatory myopathies.

Idiopathic inflammatory myopathies (IIM) are systemic autoimmune connective tissue diseases. The safety and effectiveness of exercise for patients with idiopathic inflammatory myopathies remains unclear. This study aimed to systematically review the evidence for physical exercise among patients with idiopathic inflammatory myopathies. Relevant experimental studies were identified through searching the PubMed, Cochrane, Embase, Scopus, and CINAHL databases, and studies involving any type of physical exercise for ≥1 month were considered. The primary outcome was muscle strength, and the secondary outcomes included aerobic fitness, functional performance, health status, quality of life, activities of daily living, pain, and fatigue. Eight randomized controlled trials and thirteen nonrandomized uncontrolled trials were reviewed. Physical exercise appeared safe, with several positive effects. However, selection or allocation biases and small sample sizes affected the certainty of the evidence. While physical exercise appeared safe for patients with idiopathic inflammatory myopathies with several positive effects, studies of a higher methodological quality and involving patients with active disease are needed. Furthermore, to design optimal exercise programs, consistent and sensitive outcome measures are needed to facilitate comparisons of results from different studies.

Системная красная волчанка или волчаночноподобный синдром при ВИЧ-инфекции: обзор клинических случаев

Background. Due to multi-organ lesions and a wide range of clinical manifestations, human immunodeficiency virus (HIV infection) resembles systemic connective tissue diseases, particularly systemic lupus erythematosus (SLE). Similarity of immune disorders in patients with HIV infection and SLE may lead to misdiagnosis resulting in wrong tactics of treatment (prescribing immunosuppressive therapy instead of high-dose antiretroviral therapy). Aim. To report the HIV infection and SLE differential diagnosis challenges. Results. By January 2021, the analysis of national literature revealed two cases of HIV infection in young women, initially misdiagnosed with suspected SLE. In addition to two patients described in literature, one more female patient with SLE manifestations combined with advanced stage of HIV infection was examined. Conclusion. HIV and SLE differential diagnosis is the ultimate challenge for clinicians. Physicians should be especially apprehensive about HIV infection when making the diagnosis of SLE. SLE therapy in patients with HIV infection should be appointed according to strict indications.

РЕВМАТИЧЕСКИЕ ЗАБОЛЕВАНИЯ И БЕРЕМЕННОСТЬ

. The problem of pregnancy in rheumatic diseases does not lose its relevance. The most studied is the course of pregnancy in patients with rheumatoid arthritis, systemic lupus erythematosus and axial spondyloarthritis. Observations in other systemic connective tissue diseases are episodic and do not allow us to draw reliable conclusions. The course of the disease itself, as well as the therapy received by patients, can affect pregnancy, starting with the possibility of conception and ending with unfavorable pregnancy outcomes for the mother and fetus. The onset of pregnancy should be planned at the time of remission or minimal activity of the disease. Of particular interest is drug therapy in patients with rheumatic diseases during gestation. This issue has been and remains complex and has not been fully studied due to the fact that any conclusions about the effect of drugs on the fetus are made retrospectively and cannot be the result of a deliberately initiated study. Currently allowed, though with some reservations, during pregnancy drugs are glucocorticoids, non-steroidal anti-inflammatory drugs, hydroxychloroquine and sulfasalazine. The development of genetic engineering has raised the question of the possibility of using genetic engineering basic therapy during gestation. Currently, the largest number of observations is available for TNF-α inhibitors, the use of which is recognized as possible during pregnancy. The presence of rheumatic disease in itself is not an absolute indication for a caesarean section. The question of the method of delivery is decided individually. Pregnant patients with rheumatic diseases should be under regular joint supervision of an obstetrician-gynecologist, rheumatologist and neonatologist

Aortic regurgitation: epidemiologic, etiologic, and pathophysiologic characteristics

The presented review concerns aortic regurgitation which occupies a significant place in the structure of valvular heart disease. The detailed anatomic and physiologic description of the aortic valve is provided. The characteristics of sinotubular, ventricular-aortic junctions, and virtual aortic annulus are presented. There are data about prevalence of aortic regurgitation on the basis of results of population studies, indicating the increase in incidence of aortic regurgitation among individuals older 70–74 years. The detailed etiologic structure of this valvular pathology is described with specifying of the most common causes of both aortic disease and aortic cusps alterations. In particular, there are some aortic diseases, resulting in acute aortic regurgitation, including acute aortic dissection and paravalvular regurgitation in incompetence of the prosthetic aortic valve; in chronic one – idiopathic dilation of the aortic root, inherited connective tissue dysplasias (Ehlers–Danlos, Marfan, and Loeys–Dietz syndromes), bicuspid aortic valve, aortitis of various origin, seronegative arthropathies (reactive, psoriatic arthritis, ankylosing spondylitis) etc. Infective endocarditis and traumatic exposure are commonly responsible for development of acute regurgitation due to aortic cusps abnormalities. Chronic aortic regurgitation as a consequence valve defects occurs in rheumatic heart disease, degenerative changes, congenital anomalies, systemic connective tissue diseases (systemic lupus erythematosus, rheumatoid arthritis), non-specific aortoarteritis, etc. The special attention is paid to pathophysiologic features of acute and chronic aortic regurgitation in the review. Acute aortic regurgitation is characterized by sudden increase in end-diastolic volume and due to the noncompliant left ventricle of normal size, it undergoes abrupt exposure a significant pre-load and after-load which results in decrease of left ventricle systolic function and stroke volume despite on relative preservation of contractile function of myocardium. In contrast to acute aortic regurgitation it is remarkable in its chronic form slow, progressive influence by increased overload of the left ventricle with possibility to adapt driven by its gradual dilation and hypertrophy.