Sarcoidosis is a multi-organ granulomatosis of unknown origin. Modern diagnostic methods allow detecting this disease at an early stage. The absence of specific markers requires a comprehensive approach to diagnosis based on comparison of radiation, clinical, morphological and functional data. The course of sarcoidosis without damage to the respiratory system presents significant difficulties. It is extremely important to understand the time and means of starting Sarcoidosis’s treatment to avoid the early initiation of hormones and cytostatics and, on the other hand, to timely respond to progression and threatening conditions. Methods. Clinical recommendations are based on the analysis of Russian and English publications of the latest sarcoidosis research. The target audience of these clinical guidelines are therapists, general practitioners, pulmonologists, TB doctors, rheumatologists, dermatologists, radiation diagnosticians, immunologists, and clinical pharmacologists. Each thesis-recommendation for diagnosis and treatment is evaluated on an 1 to 5 scale of levels of evidence and an A, B, C scale of the grades of recommendations. The clinical guidelines also contain comments and explanations for the theses-recommendations, diagnostic algorithms, treatment strategies, reference materials on the use of recommended drugs. Conclusion. Current information on epidemiology, clinical manifestations, diagnosis and management strategies for patients with sarcoidosis are covered in the presented clinical guidelines. Approved by the decision of the Scientific and Practical Council of the Ministry of Health of the Russian Federation (2022).

Увеличивается ли тонус сосудов системы микроциркуляции при артериальной гипертонии?

Хронические фиброзирующие интерстициальные заболевания легких с прогрессирующим фенотипом

The aim of this study was to determine the effectiveness of pirfenidone in patients with idiopathic pulmonary fibrosis (IPF) seen in clinical practice. Fifty-five adults with IPF were enrolled in this multicenter, open-label, non-randomized, non-controlled, interventional clinical study. All patients received pirfenidone 2403 mg/day (three 267 mg capsules three times daily) for 26 weeks. After 26 weeks of treatment, the mean change in absolute forced vital capacity (FVC) was 128.8 mL (95% confidence interval [CI] −26.8, 284.4) and the mean change in relative predicted FVC was −0.10% (95% CI −3.18, 2.99). Stable disease (defined as improvement of ≥0% or a decline of <10% to 0% of the corresponding FVC value) was observed in most patients (relative FVC, 90.9%; absolute FVC, 83.6%). There was no statistically significant change in the mean high-resolution computed tomography fibrosis score or lung opacity score at week 26 compared with baseline. Treatment-emergent adverse events were reported in 80% of patients during the treatment period; most of them were mild or moderate in severity. No serious pirfenidone-related adverse events were observed during the study period. Pirfenidone was generally safe and effective for controlling functional decline and stabilizing disease in patients with IPF encountered in clinical practice in Russia.

Sarcoidosis is a systemic inflammatory disease of unknown etiology characterized by the formation of noncaseating granulomas in various organs and tissues and the activation of T-cells at the site of granulomatous inflammation with the release of various chemokines and cytokines [1]. The incidence on average ranges from 10 to 20 per 100,000 population [2]. Most often in patients with sarcoidosis, lesions of the lungs and intrathoracic lymph nodes are detected. Significantly less often (in about 5–20% of patients) damage to the nervous system is noted [6,7,9]. In 9-18% of patients with neurosarcoidosis, involvement of the pituitary gland, pituitary infundibulum and hypothalamus is found, which is manifested by a variety of clinical symptoms [8,10]. We observed a patient with sarcoidosis whose disease debuted with clinical symptoms of hypogonadism, followed by the development of signs of secondary hypothyroidism, adrenal insufficiency, and diabetes insipidus, which was initially regarded as panhypopituitarism against the background of a hypothalamic lesion of unknown origin. Later, additional examination revealed signs of intrathoracic lymphadenopathy and focal changes in the lung parenchyma on CT, as well as skin lesions. Despite the biochemical compensation of hypopituitarism, the clinical efficacy of hormonal therapy with cabergoline, testosterone, hydrocortisone and levothyroxine sodium was insufficient, and the patient’s condition improved after the addition of immunosuppressive and anti-inflammatory therapy with methotrexate and methylprednisolone.

Cushing’s disease is a rare severe neuroendocrine disorder caused by chronic overproduction of adrenocorticotropic hormone by a pituitary tumor. Supraphysiological concentrations of cortisol in endogenous hypercortisolism have an immunosuppressive and anti-inflammatory effect similar to therapy with systemic glucocorticosteroids. This may reduce the activity of the patient’s concomitant autoimmune inflammatory diseases. On the other hand, a decrease in cortisol levels during treatment for Cushing’s disease may be associated with a reactivation of the immune system that pose a risk of onset or recurrence of an autoimmune disorder. We present our own clinical case demonstrating the development of sarcoidosis after surgical treatment of Cushing’s disease.