Vol 23, No 2 (2026)

BACKGROUND: Due to the successful use of immunoglobulins, non-infectious complications have become the main cause of disability and mortality in patients with primary immunodeficiencies, necessitating the development of treatment protocols for complications of immune dysregulation. The development of lymphocytic infiltration of organs, granulomatous disease, autoimmune manifestations, and cancer exacerbates the course of the underlying disease and determines the patient’s life prognosis. Rituximab is one of the most commonly used medications. No large randomized controlled studies have been conducted on the use of rituximab in patients with common variable immune deficiency with signs of immune dysregulation.

AIM: Evaluation of the effectiveness of rituximab therapy for the treatment of immune dysregulation complications in patients with common variable immune deficiency over the age of 18.

METHODS: The study included 31 patients aged 19 to 62 years with a general variable immune deficiency, manifested by symptoms of immune dysregulation without an identified molecular genetic defect. Rituximab was included in the complex therapy of the disease for all patients.

RESULTS: 31 case histories of patients with common variable immune deficiency aged 19 to 62 years were analyzed. The average age of the total sample was 38.13 ± 2.20 years. Of these, 18 (58.06 %) were women and 13 (41.94 %) were men. All patients showed symptoms of immune dysregulation: granulomatous-lymphocytic interstitial lung disease in 28 (90.32 %) patients, including 16 (88.88 %) women and 12 (92.30 %) men. Splenomegaly was observed in 26 (83.87%) of all patients. Splenectomy was performed in 5 (16.13 %) patients (all patients were male). Lymphadenopathy in 30 (96.77 %) patients, 12 (92.31 %) men and 18 (100 %) women. Thrombocytopenia in 22 (70.97 %) patients, 10 (76.92 %) men and 12 (66.67 %) women. Autoimmune hemolytic anemia in 5 (16.13 %) patients in the total sample, in 3 (23.07 %) men, 2 (11.11 %) women. Еighteen patients (58,06 %) in the anamnesis received other lines of therapy without effect or with a short-term effect. All patients received therapy with rituximab. Treatment regimen: 4-fold administration of rituximab at a dose of 375 mg/m² at 7-day intervals, followed by 3 administrations every 3 months. After one year of treatment, 89.28 % of patients with granulomatous-lymphocytic interstitial lung disease experienced complete regression of symptoms and changes in instrumental examinations. Additionally, 63.33 % of patients reported a reduction in the size of their spleen and lymph nodes. Normalization of blood parameters (thrombocytopenia) in 59,09 % of patients. No adverse events were observed during the year-long use of rituximab.

CONCLUSION: The efficacy and safety of rituximab in adult patients with common variable immune deficiency with immune regulation manifestations have been proven.

BACKGROUND: There is no data on the nature of medical care provided to patients with chronic spontaneous urticaria from the patient’s perspective.

AIM: To analyze the clinical and social aspects of chronic spontaneous urticaria and the patterns of healthcare delivery using data from an online survey of patients from 63 cities across Russia.

METHODS: A structured online survey was performed among respondents with chronic spontaneous urticaria over the age of 18. The survey was conducted between April and June 2025 and included 40 questions covering demographic characteristics, disease severity, comorbidities, patient routing, medical visits, and treatment of chronic spontaneous urticaria. The data were analyzed quantitatively, with the calculation of the proportions of responses for each question from patients in 63 cities across Russia. IBM SPSS Statistics 21 was used for statistical analysis.

RESULTS: The study involved 1,061 patients with chronic spontaneous urticaria. Most of them of working age; more than 60 % did not achieve control according to the chronic spontaneous urticaria test. Common comorbidities were sleep disorders and anxiety disorders. Patients consulted several specialists before diagnosis; an allergist-immunologist played a leading role in the management of chronic spontaneous urticaria. Satisfaction with standard-dose antihistamines was low, and the availability of second-line therapy was limited.

CONCLUSION: The results highlight the need to improve patient referral pathways, increase physicians’ awareness of the principles of diagnosis and management of chronic spontaneous urticaria, enhance access to modern therapeutic options, and promote a multidisciplinary approach to chronic spontaneous urticariamanagement. Overall, the findings are consistent with international studies while also providing insight into the specific features of chronic spontaneous urticariamanagement in the Russian healthcare setting.

BACKGROUND: Continuous monitoring studies of the regional spore and pollen aerospectrum for the southern part of the Tyumen Region have not been carried out.

AIM: To identify regional characteristics of the spore and pollen spectrum in the air environment in Tyumen based on the results of five years of airborne pollen monitoring.

METHODS: A volumetric pollen trap was used to collect pollen and spores from the city air during the spring and autumn of 2018–2022. Subsequent identification and counting of pollen grains and spores was carried out under a light microscope at × 400 magnification.

RESULTS: Pollen from at least 13 anemophilous plants from different taxonomic groups was detected in the air mass of Tyumen. Spores from at least two fungal taxa were also detected. Cladosporium was the most prevalent. The number of tree pollen taxa was almost four times higher than the number of grass pollen taxa. Birch pollen was found to be the most productive in the city’s air spectrum, peaking during the first wave in May. Nettle pollen is the second most common allergen in terms of total pollen count. Its peak occurs during the second wave. The absence of ragweed pollen was demonstrated. The dynamics of pollen and spore frequency during the specified observation period were analysed. A regional pollen calendar was compiled.

CONCLUSION: The spore-pollen spectrum is dominated by birch, pine, and nettle pollen, as well as Cladosporium fungus spores, but ragweed pollen is not present.

The thymus is a lymphoepithelial organ located in the upper part of the anterior mediastinum and is both the central organ of the immune system and the endocrine gland. An increase in the volume and mass of the thymus above the age limits while maintaining normal histoarchitectonics is called thymomegaly. Thymomegaly occurs mainly in children and can be both idiopathic and secondary, occurring as a rebound phenomenon after exposure to various stress factors or accompanying other diseases and conditions. The pathogenetic basis for this is considered to be the dysfunction of the hypothalamic-pituitary system. Thymomegaly, especially severe degrees, is often accompanied by a violation of T-cell immunity, as well as a decrease in the size of the adrenal glands with suppression of their hormonal function, and therefore individuals with thymomegaly are at risk of developing prolonged, complicated, and fulminant forms of infectious diseases. Instrumental methods for thymomegaly detecting are chest X-ray and thymic ultrasound. Thymomegaly can also be detected by computed tomography/magnetic resonance imaging of the chest. Thymic T-lymphocyte development is assessed by detection of T-receptor excision circles in the blood using polymerase chain reaction. The complex of therapeutic and preventive measures for thymomegaly includes T-cell immunity disorder correction (if present), a personalized vaccination approach, preoperative preparation, and careful monitoring in the postoperative period. The need for thymectomy is determined individually. The prognosis in children is more often favorable, in adults it depends on the cause.

We present a clinical case of early-onset and severe hereditary angioedema type 1, caused by a pathogenic variant in the SERPING1 gene, in a two-year-old girl. Hereditary angioedema onset occurred at 18 months, which is extremely early for this disorder. The disease onset was characterized by severe clinical manifestations: frequent, debilitating episodes of angioedema of the face, upper and lower extremities (over 8 months, the child experienced 14 episodes of angioedema: 5 in the face and 9 involving peripheral sites). Due to such an early onset and severe course of hereditary angioedema, long-term prophylaxis with lanadelumab was initiated. After more than 5 months of therapy, the child has not experienced a single episode of angioedema, despite exposure to provoking factors. No side effects were observed.

In the literature, only five clinical cases with onset and a definitive diagnosis of hereditary angioedema in children under 24 months of age, with only one of the five requiring long-term prophylaxis. Thus, we present a unique clinical example of the successful use of lanadelumab as long-term prophylaxis in early childhood.

Atopic dermatitis is a chronic multifactorial inflammatory skin disease characterized by pruritus, a relapsing course, and characteristic morphological and topographical features that develops in genetically predisposed individuals against the background of a T2-immune response. Cytokines from T helper 2 and type 2 innate lymphoid cells — interleukins 4, 13, and 31 — play a key role in the pathogenesis of the disease, activating the JAK/STAT signaling pathways (primarily JAK1/STAT6). Despite the proven efficacy of selective Janus kinase inhibitors, including the selective Janus kinase inhibitor upadacitinib, in many inflammatory diseases, including atopic dermatitis, there remains a need to accumulate real-world clinical data, especially in patients with refractory atopic dermatitis and comorbid conditions.

Aim — to evaluate the long-term efficacy, safety, and dynamics of potential biomarkers during upadacitinib therapy in patients with severe atopic dermatitis and comorbid pathology.

This case series presents data from 5 adult patients (3 men, 2 women) with severe atopic dermatitis refractory to standard therapy. All patients received upadacitinib 30 mg once daily for 52 weeks. Efficacy was assessed by dynamics in Scoring Atopic Dermatitis Index, Eczema Area and Severity Index, Investigator’s Global Assessment scores, Numerical Rating Scale; Dermatology Life Quality Index. Safety was evaluated through monitoring of adverse events. Gene expression of cytokines (TARC/CCL17, MDC/CCL22, PARC/CCL18, CTACK/CCL27, eotaxin-3/CCL26, interleukins 4, 13, 17, 22, 25, 33, thymic stromal lymphopoietin) was analyzed using real-time polymerase chain reaction at baseline and at week 16 of therapy.

Control over atopic dermatitis symptoms was achieved in all patients: by week 40, 100 % (5/5) of patients had achieved an Eczema Area and Severity Index 90 response, which was maintained until the end of the 52-week observation period. The median Scoring Atopic Dermatitis Index decreased from 65.3 to 2.6 by week 40, and the median Eczema Area and Severity Index score decreased from 47.6 to 0.7 by week 28. Numerical Rating Scale score decreased from 5 to 1 (median) by week 16. During the observation period, 26 mild adverse events were recorded; no serious adverse events were reported. Complete drug-induced remission was observed in one female patient with comorbid alopecia areata. No statistically significant changes were detected in the profile of the studied biomarkers.

In this case series, upadacitinib demonstrated high efficacy and a favorable safety profile in patients with severe refractory atopic dermatitis over 52 weeks of therapy. The observed remission of comorbid alopecia areata expands understanding of the drug’s therapeutic potential. Larger prospective studies are needed to confirm these findings and to identify predictive biomarkers.

The article presents the consensus opinion of experts on the role of 5% dexpanthenol preparations, particularly the original 5% dexpanthenol cream, in the comprehensive management of patients with inflammatory dermatoses such as atopic dermatitis and eczema. The material was prepared based on the results of the Expert Council held on December 17, 2025. The article reviews current concepts of the pathogenesis of these diseases with an emphasis on the central role of epidermal barrier disruption. The main approaches to restoring the skin barrier within existing therapy are described. Current clinical guidelines are analyzed and problem areas related to algorithms for the use of preparations that restore skin barrier function are identified. An overview of the evidence base for the multifaceted action of 5 % dexpanthenol, which has regenerating, anti-inflammatory, and epidermal barrier-restoring effects, is presented. The place of 5 % dexpanthenol in the treatment of atopic dermatitis is defined: as monotherapy in mild forms, and as part of combination and sequential therapy in moderate-to-severe cases. The final part of the paper formulates practical recommendations for the inclusion of dexpanthenol medicinal products in clinical algorithms for patient management.