Systemic scleroderma (SSD) is a rare immune-inflammatory systemic disease of connective tissue with a typical lesion of skin, blood vessels, musculoskeletal system and internal organs (lungs, heart, digestive tract, kidneys). The SSD pathogenesis is based on activation of a cascade of complex immune interactions that lead to vasculopathy. The presence of many pathophysiological links in the progression of the disease causes a variety of clinical manifestations in various patients with SSD. A full assessment of all stages of SSD development is still being carried out and every newly open element of the interaction of immunological subjects completes the overall picture of the disease. A number of studies show a correlation between level of several biomarkers and both disease prognosis and estimated therapy effectiveness. Recent data confirm importance of the biomarkers for formation of patterns of a particular disease phenotype in a specific patient. Depending on relation of the biomarkers to various biological processes, several of their categories are distinguished: biomarkers expressed in lung tissue, cellular units of immunity, nucleic acids, acute phase indicators, connective tissue growth factors, matrix proteinases and their inhibitors, chemokines and cytokines, as well as biomarkers of endothelial activation. Discovery of a novel set of the indicators can be decisive in determining the management tactics and forecasting the response to therapy of some groups of patients with SSD. By combining the most recent data on significant markers obtained in the framework of extensive studies, we have described the most significant biomarkers of SSD and their link to interstitial lung disease (ILD) that is formed in SSD.

Aim: to study risk factors of development of the post-COVID syndrome (PCS).

Material and methods. 210 patients with a history of new coronavirus infection (COVID-19) (47 men, 163 women aged 18–85 years) were examined by doctors of various specialties. Patients were divided into several groups depending on the presence of PCS, as well as the severity of the disease.

Results. The risk factors of the PCS development are moderate and severe course of the pathological process in acute period of COVID-19 disease (p < 0.001). In women, PCS is seen more often than in men (30 / 135 vs. 17 / 28, p < 0 / 001), other risk factors are age over 50 years (p < 0.05), polymorbidity (p < 0.01), treatment with glucocorticoids in acute disease period (76 / 165 vs. 4 / 45, p < 0.001). In cases of mild COVID-19 course, neither age nor polymorbidity increased the risk of PCS development (p > 0.05), however a dysfunction of cellular immunity was significant, specifically the proliferative activity of lymphocytes in response to mitogen: 50.6 ± 10.4 % vs. 54.0 ± 5.1 %, p < 0.05). In cases of severe COVID-19 course, the gender differences and dysfunction of the cellular immune system are not the determinants for the PCS development (p > 0.05), however the age (56.7 ± 13.1 years vs. 42.1 ± 15.4 years, p < 0.01) its linkage to somatic pathology (a cardiovascular disease) besides glucocorticoids threatment (64 / 89 vs. 3 / 9, p < 0.05) are important risk factors for PCS.

Conclusions. The main risk factor for PCS development is the moderate and severe course of the pathological process in the acute period of COVID-19 infection, female gender, age over 50 years, polymorbidity, treatment with glucocorticoids in the disease acute period. In case of mild COVID-19 course, neither age nor the polymorbidity increased the risk of PCS development, but the dysfunction of cellular immunity is significant. In case of severe COVID-19, the gender differences and dysfunction of the cellular immune system are not the determinants for the PCS development, however age, concomitant somatic pathology (a cardiovascular disease) and glucocorticoids treatment in acute period are important risk factors for the PCS development. The titer of protective IgG class antibodies to SARS-CoV-2 is not linked to risk of the PCS development.

An analysis of causes leading to pain recurrence in patients with polymyalgia rheumatica (PR) after tapering down the dose of glucocorticosteroids (GCS) is presented. True exacerbations resulting from the main disease recurrence and pseudo-exacerbations when the resumption of pain syndrome is not due inflammation but because of other causes – steroid myopathy or chronic adrenal insufficiency (AI) are identified. The experience of using tocilizumab, an inhibitor of interleukin-6 receptors, as steroid-sparing agent in patients with PR is considered. The factors contributing to pain syndrome development, which can mimic exacerbations of PR (associated myofascial pain, vitamin D deficiency, etc.) are indicated. The methodology for AI diagnosis in patients receiving GCS therapy is discussed in detail. Recommendations for the rational AI therapy including acute cases are given.

Objective: to describe a clinical case of polymorphic leukocytoclastic vasculitis (PLCV) associated with renal neoplasia.

Materials and methods. Patient K., a man, 67 y. o., was admitted to the surgical department No. 2 of the N. I. Pirogov State Clinical Hospital No. 1 with cardiac complaints and heart failure decompensation symptoms, severe respiratory insufficiency associated right lower lobe pneumonia. Besides cardiac function evaluation and pneumonia managing there was a conducting of differential diagnostic search to clarify the genesis of ulcerative necrotic rashs among systemic vasculitis, paraneoplastic syndrome including hematologic problems and sepsis.

Results. It was no data for systemic connective tissue diseases and systemic vasculitis during clinical examination and immunological diagnostics. On contrast-enhanced multispiral computed tomography of abdomen and retroperitoneal space there was latent malignant neoplasia of left kidney Т2аN1M0. PLCV was considered to paraneoplastic syndrome associated with malignancy. Due to the progression of vasculitis and the patient’s refusal of surgical treatment was prescribed GC 0.5 mg / kg / day as a result positive dynamics was noted.

Conclusion. This clinical case demonstrates necessity of providing examination aimed at malignancy excluding for early neoplasm’s detecting for patients with vasculitis.

The aim of the study was to describe a clinical case of noninsulinoma pancreatogenous hypoglycemia (NIPH).

Materials and methods. Patient R. 42 years old, woman, was admitted with complaints on spastic abdominal pain, heartburn, flatulence, bloating. The patient had a history of cramping pains in the upper abdomen, episodes of hypoglycemia up to once a day, periodically diarrhea with undigested food up to 3 times a day, and frequent weakness during last 9 years. In 2013, she was diagnosed with a neuroendocrine tumor of the pancreas, and therefore distal pancreatectomy was performed that year. According to histological and immuno-histochemical studies, foci of islet-cell hyperplasia (nesidioblastosis) were noted in the tail of the pancreas against the background of tissue fibrosis. Non-insulinoma pancreatogenous hypoglycemia of adults (NIPH) was diagnosed, enzyme replacement therapy and Octreotide-depo were prescribed. Relapses were noted twice. Two weeks before admission, the patient noted episodes of hypoglycemia. Upon admission, the patient had state of moderate severity, irregular stools up to 3–4 times a day. Antibacterial treatment was carried out, enzyme replacement therapy, octreotide was continued.

Results. On the 7th day of hospitalization, the patient was stabilized: the level of glycemia was 4.5–4.9 mmol / l, the frequency and consistency of stool normalized. No data for decompensation of the disease has been received. The patient was discharged in a satisfactory condition.

Conclusion. This clinical case demonstrates the influence of NIPH on the patient’s quality of life and the need for constant vigilance against the recurrence of hypoglycemic episodes, despite the treatment. This case can improve the awareness about this rare but important disease.

Introduction. One of the distinguishing features of systemic vasculitis is their manifestation under the guise of a lesion of one or another organ system, which is often multi-organ in nature with signs of systemic inflammation. The latter is interpreted primarily as part of an infectious or paraneoplastic process, which causes a delay in the diagnosis.

The aim of the study was to present the diversity of the clinical picture in vasculitis associated with antineutrophil cytoplasmic antibodies (ANCA), the speed and large volume of diagnostic measures with the effective cooperation of therapeutic and surgical specialists, radiologists on the way to verifying granulomatosis with polyangiitis in a young woman.

Materials and methods. Patient K., 46 y. o., was hospitalized in the Otolaryngology Department of the N.I. Pirogov City Clinical Hospital No. 1 of the Moscow Health Department with complaints of hearing loss, pain and stuffiness in the left ear, unproductive cough, hoarseness and fever up to 38.5 °C. According to the radiography (RG) of the chest organs, right-sided pneumonia was detected. Conducted antibiotic therapy without effect. As part of the differential diagnostic search, the following nosologies were excluded: infective endocarditis, sepsis, tuberculosis, primary multiple or central lung cancer complicated by paracancer pneumonia, metastatic lesion, infectious, brucellosis spondylodiscitis.

Results. In the blood test, attention was drawn to a decrease in the level of hemoglobin to 111 g / l, an increase in the rate of erythrocyte sedimentation to 45 mm / h and the level of C-reactive protein to 142 mg / l, microhematuria according to the general urine analysis. Instrumental research methods – RG of 16.02.22, MSCT of the chest organs on 17.02.22, 27.02.22, 10.03.22 showed progressive bilateral focal pneumonia with a focus of consolidation in the middle lobe, EchoCG, ultrasound of the abdominal cavity and small pelvis, RG of the temporal bone, bronchoscopy with bronchoalveolar lavage and microscopic analysis, for atypia and bacteriological culture. A gynecological examination and a smear from the cervical canal for microscopic analysis were performed, atypical cells, consulted by a phthisiatrician (no data for tuberculosis), consulted three times by a thoracic surgeon (exclusion of volumetric formation of the middle lobe of the right lung). Given the history and clinical presentation (female gender, young age, bilateral otitis media, hoarseness, and destructive nature of pneumonia), granulomatosis with polyangiitis was suspected, and tests for ANCA were prescribed. A transthoracic biopsy of the right lung was performed. A rheumatologist prescribed induction pulse therapy with corticosteroids, and after serological and histological confirmation (antibodies to Proteinase-3 Anti-PR3 > 200 IU / ml, productive pneumonitis, granulomas without signs of tuberculosis), immunosuppressive therapy with cyclophosphamide. Against the background of pathogenetic treatment, a pronounced clinical and laboratory effect was noted.

Conclusion. In this clinical situation, the simultaneous involvement of specialists of various profiles, the performance of a large number of laboratory and instrumental studies in dynamics, the absence of delay in histological verification made it possible to quickly exclude common diseases in the population and suspect systemic vasculitis, establishing a correct diagnosis within 5 weeks of the hospitalization period.