Rheumatology (Bulgaria) http://www.rheumatologybg.org/journal/index.php?journal=revmatologiia <p>Rheumatology (Bulgaria) is the platinum open-access peer-reviewed journal owned by the Bulgarian Rheumatology Society and published by the Central Medical Library - Bulgaria.</p> <p>Rheumatology (Bulgaria) focuses on all aspects of rheumatic diseases. Revmatologiia features Original Articles, Society Recommendations, Editorials, Invited Reviews, Clinical Rheumatology Cases or Case-Based Reviews, Letters to the Editor. Guidelines unique to Bulgarian and Balkan Rheumatology will also be published.</p> <p>Indexing and abstracting: <strong>Scopus</strong>, EMBASE, Excerpta Medica, <strong>Google Scholar</strong>,<strong> CrossRef</strong>, Central Medical Library - Bulgaria, Bulgarian Medical Literature Database, OUCI.</p> <p>Online ISSN&nbsp;2738-831X; Print ISSN&nbsp;1310-0505.</p> Bulgarian Rheumatology Society en-US Rheumatology (Bulgaria) 1310-0505 Upadacitinib in real clinical practice: new horizons in the treatment of axial spondyloarthritis http://www.rheumatologybg.org/journal/index.php?journal=revmatologiia&page=article&op=view&path%5B%5D=249 <p><em><strong>Introduction.</strong></em> Axial spondyloarthritis (axSpA) is an inflammatory disorder affecting the axial skeleton, accompanied by pain, restricted mobility, and other symptoms. The Ankylosing Spondylitis Disease Activity Score (ASDAS) is a metric expressing disease activity, assessing inflammation and symptoms. In clinical trials, Upadacitinib has demonstrated reduced axSpA activity, but real-world evidence remains limited.&nbsp;<em><strong>Objective.</strong></em> The objective of this study is to evaluate the therapeutic effectiveness of Upadacitinib in axSpA by analysing its impact on symptoms, activity, and inflammatory markers in real clinical practice. Additional goals include comparing outcomes between biologic-naive and biologic-experienced patients, as well as assessing the influence of radiographic stage on Upadacitinib's therapeutic response.&nbsp;<strong><em>Methods.</em></strong> Sixty-four patients with axSpA were enrolled in Upadacitinib treatment. Among them, 42 were evaluated after 6 months and 13 after 12 months of therapy. ASDAS was assessed at various time points and analyzed based on prior experience with biologic drugs and radiographic stage of sacroiliitis.&nbsp;<em><strong>Results.</strong></em>&nbsp;A notable reduction in mean ASDAS values was observed after 6 months of Upadacitinib treatment (3.5 vs. 1.9, p &lt; 0.001), with this reduction being sustained after 12 months (1.6 vs. 1.9, p &gt; 0.05). A substantial proportion of patients (80.9%) achieved ASDAS values below 2.1 after 6 months, and this achievement was maintained after 12 months (84.6%, p &gt; 0.05). No significant differences were found between biologic-naive and biologic-experienced patient subgroups (84.2% vs. 78.3%, p &gt; 0.05). Similar trends were observed in the analysis of other parameters such as BASDAI, fatigue, pain, CRP, haemoglobin, and ESR. Upadacitinib increased the number of patients with low disease activity regardless of radiographic stage after 6 and 12 months (23.7 vs. 83.3% and 5.7 vs. 85.3%, p &lt; 0.001). <em><strong>Conclusion.</strong></em> Upadacitinib proves to be effective in axSpA treatment. Regardless of demographic, clinical, and radiographic disease characteristics, as well as "biologic-naive/biofailure" status, Upadacitinib leads to ASDAS improvement after 6 and 12 months of treatment. This medication represents an effective tool in real-world clinical practice for controlling axSpA activity.</p> Tanya Shivacheva Zhaklin Rumenova Dimova Desislava Simeonova Svetoslav Dimitrov Tsvetoslav Georgiev Simona Bogdanova Rosina Moraliyska Svetlana Hristova Georgi Gerganov ##submission.copyrightStatement## http://creativecommons.org/licenses/by-nc-sa/4.0 2023-12-12 2023-12-12 31 3 3 18 10.35465/31.3.2023.pp3-18 Increased level of C3 in synovial fluid in patients with activated osteoarthritis as a diagnostic and therapeutic target – pilot group of patients from the Rheumatology Department at UMHAT Burgas http://www.rheumatologybg.org/journal/index.php?journal=revmatologiia&page=article&op=view&path%5B%5D=223 <p>The clinical course of osteoarthritis (OA) indicates the role of low-grade synovitis as the main driver of the degenerative process. Components of the extracellular matrix, fibronectin isoforms, and fragments of hyaluronic acid are presumed ligands for DAMPs (particularly TLR). Complement fractions bind to the corresponding receptors on the cell membranes of chondrocytes and synoviocytes through TLR. Does the complement cascade play a role only as a clearing system and/or a leading pathogenetic factor in activated OA? The aim of this study is to answer this question. The study included 50 patients with activated OA of the knee joint. We examined the levels of C3 and C4 complement fractions in the blood plasma and synovial fluid. We found that the values of these proteins in synovial fluid were on average 34.90% for C3 and 30.97% for C4 of their values in blood plasma, with a generally accepted norm of 10% for complement levels in a healthy joint. The observation regarding the strength of correlation between the above results and the radiological stage confirmed a stronger correlation of the obtained results in the earlier stages of the disease when the activity of the repair processes is more pronounced. In this way, we objectified the pathogenetic role of complement in the arthritic process, as well as its role in clearing the joint space from degradation products. The question arises whether pharmacological intervention to balance complement activation could represent a future therapeutic strategy in the treatment of OA and prevent its progression.</p> Irina Ivanova Momcheva ##submission.copyrightStatement## http://creativecommons.org/licenses/by-nc-sa/4.0 2023-12-12 2023-12-12 31 3 19 29 10.35465/31.3.2023.pp19-29 Acceptance, tolerability, and effect of COVID-19 vaccination on disease activity in a cohort of Egyptian systemic lupus erythematosus patients: Real-life experience http://www.rheumatologybg.org/journal/index.php?journal=revmatologiia&page=article&op=view&path%5B%5D=247 <p><strong>Abstract</strong></p> <p><strong>Objective</strong>: To assess the tolerance and effects of COVID-19 vaccination in Egyptian patients with systemic lupus erythematosus (SLE), such as incident flare risk, based on the patient’s perspective.</p> <p><strong>Methods:</strong> SLE patients were included in this multicenter cross-sectional questionnaire-based analysis. Data included demographic and clinical features, prior COVID-19 infection, vaccine acceptability, type of vaccination, disease status before and after vaccination, and related side effects.</p> <p><strong>Results:</strong> The total number of patients included was 230. Ages ranged from 18 to 64 years, disease duration from 1 to 15 years, and 92.6% were females. Only 31.7% of the study population were COVID-infected; most (70.7%) were treated at home, while 1.2% needed ICU. In contrast, 53% of our patients were vaccinated, and 47% were not vaccinated, primarily due to fear of side effects or disease flare. After the first dose of vaccination, 44.1% reported vaccine-related side effects, of which fatigue, fever, myalgia, as well as injection site reaction were the prevalent complications. The highest incidence of side effects was in AstraZeneca (78%), while the least was in Sinopharm (29.6%). 16.3% of vaccinated patients were COVID infected after vaccination, and 88.2% were treated at home. Only 14.6% of the vaccinated population experienced a lupus flare, primarily after the first dose, with no significant difference between the type of vaccine and lupus flares.</p> <p><strong>Conclusion:</strong> The acceptance of COVID-19 vaccination among our lupus patients was neutral. The observed adverse effects were comparable to those reported by healthy individuals. Post-vaccination lupus flares were infrequent and unrelated to the vaccine type but rather to the pre-vaccination disease activity state.</p> <p><strong>Keywords:</strong> Lupus, COVID, Vaccine, Flare</p> Mohamed Hassan Abu-Zaid Hany M. Aly Abdelhfeez Moshrif Rasha Abdel Noor Nehal El-Ghobashy ##submission.copyrightStatement## http://creativecommons.org/licenses/by-nc-sa/4.0 2023-12-12 2023-12-12 31 3 30 37 10.35465/31.3.2023.pp30-37 Clinical disparity in rheumatoid arthritis patients with history of spontaneous abortion: Potential link to metabolic syndrome and under treatment http://www.rheumatologybg.org/journal/index.php?journal=revmatologiia&page=article&op=view&path%5B%5D=244 <p><em>Objective:</em> The aim of the work was to describe the characteristics of married rheumatoid arthritis (RA) female patients with history of spontaneous abortion and compare them to those without. <em>Patients and methods:</em> Three hundred and four female RA with history of abortion compared with another 304 RA married patients of matched age without history of abortion. Patients were subjected to full history taking and clinical examination. The routine laboratory investigations were done in addition to rheumatoid factor (RF), anti-cyclic citrullinated peptide (anti-CCP), antinuclear antibody. Also, plain x-ray hands were performed to detect erosions. Disease activity score (DAS28) and health assessment questionnaire (HAQ) were assessed.<em> Results: </em>In this study 3.5 % of patients with history of abortion out of a large RA cohort (n = 8750), had a mean age of 42.8±11.4 years and disease duration of 6.2 ±5.4 years. 14 (4.6 %) were smokers. There was a significant increase in the frequency of metabolic syndrome in those with abortion (17.4%) than in those without (10.9%) (p=0.008). Methotrexate (MTX) and hydroxychloroquine (HCQ) were less frequently used among patients with a history of spontaneous abortion (67.8% and 68.8%) compared to those without (82.6% and 78%) (p =0.005 and p =0.037 respectively). Disease activity and the functional status were comparable between those with and without abortion. <em>Conclusion:</em> Spontaneous abortion in RA females was higher in those with metabolic syndrome. Reluctance and/or incompliance in using the basic DMARD treatment with MTX or HCQ in controlling the disease may add to the possibility of abortion.</p> S. S. El-Adle E. F. Mohamed N. Samy H. Taha R. Fawzy F. Ismail S. Tharwat M. Nassr S. Senara H. El-Saadany H. M. Fathi D. Mosad S. Sayed M. A. Amer S. El-Zokm A. Elhefny K. El Hadidi T. S. El Hadidi Tamer A Gheita S. Elwan ##submission.copyrightStatement## http://creativecommons.org/licenses/by-nc-sa/4.0 2023-12-12 2023-12-12 31 3 38 43 10.35465/31.3.2023.pp38-43 Capillaroscopic findings in undifferentiated connective tissue disease with Raynaud’s phenomenon http://www.rheumatologybg.org/journal/index.php?journal=revmatologiia&page=article&op=view&path%5B%5D=270 <p>Background: Undifferentiated connective tissue disease (UCTD) is characterized with presence of clinical signs and immunological findings suggestive of connective autoimmune disease, but the criteria for a definite rheumatic disease are not fulfilled. Raynaud’s phenomenon (RP) could be found in approximately 50% of cases with UCTD and about half of patients with UCTD and RP exhibit “scleroderma-like” pattern.</p> <p>The aim of the study: To assess the characteristics of capillaroscopic changes in early UCTD.</p> <p>Patients and methods: Inclusion criterion for the study was newly diagnosed by rheumatologist, early UCTD in patients with RP. 26 patients were included in the study – 24 females and 2 males, mean age 38±14 years (range 19 – 66 years). Capillaroscopic examination was performed in all patients using USB microscope Dino-Lite. Follow-up was perfomed for a period between 1 and 3.5 years.</p> <p>Results and discussion: At the time of the initial diagnosis, “scleroderma-like” pattern, “early” phase (giant capillaries, presence of hemorrhages in some cases, preserved distribution, normal capillary density) was found in 17 patients (65%). More advanced capillaroscopic changes including devascularization and derangement were not observed. In 4 patients nonspecific capillaroscopic findings were present (dilated capillaries, hemorrhages, increased tortuousity, elongated capillaries,) and in 5 cases – normal capillaroscopic picture was found. During the follow-up in one patient the diagnosis was revised to systemic lupus erythematous due to newly appeared clinical, laboratory and immunological findings. The normal capillaroscopic pattern was changed to nonspecific findings in this case. Two patients fulfilled the criteria for prescleroderma during the follow-up without skin and visceral involvement. In the rest patients clinical diagnosis and capillaroscopic findings remained unchanged.</p> <p>Conclusion: In conclusion, “scleroderma” type microangiopathy, “early” phase is a common finding in UCTD with RP, while more advanced microvascular pathology is not usually observed. Stable capillaroscopic pattern during the follow-up correlates with the stable clinical course. Capillaroscopy is a key technique for assessment of RP patients in rheumatology and for early diagnosis of UCTD with peripheral vascular syndrome.</p> Sevdalina Lambova Nikolay Stoilov Vladimira Boyadzhieva ##submission.copyrightStatement## http://creativecommons.org/licenses/by-nc-sa/4.0 2023-12-13 2023-12-13 31 3 44 54 10.35465/31.3.2023.pp44-58 Clinical case of pulmonary cement embolism and adjacent fractures as a complication after vertebroplasty http://www.rheumatologybg.org/journal/index.php?journal=revmatologiia&page=article&op=view&path%5B%5D=222 <p>Introduction: Osteoporosis is a skeleton disease with reduction in bone mass and disruption of the bone microstructure. A rare secondary form is pregnancy-associated osteoporosis. The standard method for determining bone density is DEXA measurement (dual X-ray absorptiometry). With the radiofrequency echographic multi spectrometry (REMS) method this is done using ultrasound without radiation exposure. Osteoporosis often leads to pathological fractures of the vertebrae. In case of severe pain and immobilization vertebroplasty is considered standard procedure. However, it can have side effects such as cement leakage into surrounding tissue, pulmonary embolism and adjacent fractures.</p> <p>Case: We present a 27-year-old female patient with compression fractures of the Th8 and Th12 5 months after labour. DEXA measurements showed values of the T-score at hip neck -2,5 SD (total) and -3,5 SD (L1-L4) at spine. The fractures of the Th8 and Th12 were then treated with vertebroplasty. A post-operative CT showed cement leakage and extravasation, as well as bilateral cement embolism of the proximal branches of the pulmonary arteries and progression of the height loss of Th5, Th7 and L1.</p> <p>Discussion: Pulmonary cement embolism is a rare complication of vertebroplasty. Cement leakage is more frequent complication, although most of the time asymptomatic. These risks of complications make the alternative conservative treatment worth considering before proceeding to surgical interventions. Only after failure to achieve adequate pain management kyphoplasty or vertebroplasty should be considered, due to the risks of prolonged immobilization of the patient leading to further bone and muscle loss.</p> <p>Conclusion: Pregnancy associated osteoporosis is very rare.&nbsp; The radiation free REMS method is suitable for pregnant women. Vertebroplasty offers pain relief and rapid mobilization of the patient. However, it poses a risk of numerous complications. The osteoporotic fractures of the vertebrae are usually stable and a conservative treatment prior to surgical interventions should always be considered.</p> Nikola Kirilov Kirilov F. Bischoff M. Kovachev E. Simeonov H. Gigov S. Vladeva M. Nikolov N. Nikolov E. Bischoff ##submission.copyrightStatement## http://creativecommons.org/licenses/by-nc-sa/4.0 2023-12-12 2023-12-12 31 3 55 63 10.35465/31.3.2023.pp55-63 IgG4-related disease - a clinical case http://www.rheumatologybg.org/journal/index.php?journal=revmatologiia&page=article&op=view&path%5B%5D=258 <p>Immunoglobulin G4-related disease (IgG4-RD) is a systemic disease affecting one or more organs. Pathomorphologically, a dense infiltrate of lymphocytes and IgG4 plasmocytes, and subsequent fibrosis is detected. Despite the many hypotheses of genetic predisposition, molecular mimicry and autoimmune nature of the disease, the etiology still remains unclear. Both innate and acquired immunity are believed to play a key role in disease pathogenesis. The involvement of occupational risk factors is also discussed. The possible involvement of multiple organs and systems (most often lacrimal and salivary glands, kidneys, lungs, aorta, pancreas, hepatobiliary duct, lymph nodes, as well as retroperitoneal fibrosis) is the reason for the multidisciplinary approach in this disease. In pulmonary involvement, clinical manifestations are nonspecific (cough, dyspnea, chest or back pain, hemoptysis, low-grade fever, weight loss) or patients are asymptomatic and pulmonary changes are an incidental finding on imaging. Up to 60% of cases of autoimmune pancreatitis represent a pancreatic manifestation of IgG4-related disease, with the majority of patients being elderly men. The most common differential diagnosis is a malignant process due to the tumor-like changes of the affected organ. Treatment with corticosteroids, immunosuppressors and symptomatic agents in most cases has a good effect, although relapses of the disease are also observed. We present a clinical case of a 72-year-old patient whose first complaints were nonspecific, for which a chest computed tomography was performed with visualization of four solid nodules in the right lung. After antibiotic therapy, without effect and deterioration of the patient's condition, video-assisted thoracoscopy, biopsy, histology and immunohistochemistry were performed. A diagnosis of IgG4-RD with pleural and lung involvement was confirmed. Anemic syndrome, increased values ​​of amylase, lipase, and acute phase parameters were found from the laboratory tests. Through endoscopic retrograde cholangio-pancreatography, autoimmune pancreatitis was diagnosed in the course of IgG4-RD. A plastic stent was placed on the common bile duct. Treatment with Prednisolone 40 mg/day was started with a subsequent dose reduction. Follow-up computed tomography of the lung after 9 months demonstrated a reduction of the described changes in the lungs, but with persistent lymphadenopathy and fibrotic changes. Azathioprine 100 mg daily was added to prednisone therapy. Against the background of the combined treatment, an improvement of the clinical condition and laboratory parameters was established.</p> Vladimira Boyadzhieva Jaklin Svetoslavova Doncheva - Dilova Rumen Stoilov V. Milanov Sevdalina Lambova Nikolay Stoilov ##submission.copyrightStatement## http://creativecommons.org/licenses/by-nc-sa/4.0 2023-12-14 2023-12-14 31 3 64 78 10.35465/31.3.2023.pp64-78 Successful Combination Therapeutic Strategy for Treatment of Digital Necrosis in Systemic Sclerosis http://www.rheumatologybg.org/journal/index.php?journal=revmatologiia&page=article&op=view&path%5B%5D=271 <p>Currently, there are no randomized trials that assess combination treatments of digital ulcers in systemic sclerosis (SSc). Here, we present a clinical case of successful treatment of digital necrosis in SSc patient with diffuse cutaneous involvement with complete recovery for a period of 2 months, using combination therapeutic strategy (felodipine, intravenous iloprost, sildenafil, anticoagulant, local treatment). Treatment of severe digital ischemia in SSc is a challenge in rheumatology. However, complete and quick recovery including in cases with digital necrosis with minimal tissue loss could be achieved with close monitoring of the patients, providing complex care with the use of combination pharmacological therapy and local treatment.</p> Sevdalina Lambova Nikolay Stoilov Vladimira Boyadzhieva ##submission.copyrightStatement## http://creativecommons.org/licenses/by-nc-sa/4.0 2023-12-14 2023-12-14 31 3 79 87 10.35465/31.3.2023.pp79-87 Clinical case of patient with Cryopyrin-associated Periodic Syndrome (Muckle-Wells syndrome) and systemic lupus erhythematodes http://www.rheumatologybg.org/journal/index.php?journal=revmatologiia&page=article&op=view&path%5B%5D=233 <p>Muckle-Wells syndrome is a cryopyrin-associated periodic syndrome with an autosomal dominant transmission pattern. The disease characterized by episodes of step move. We present a clinical case a woman of 34-years old with cryopyrin-associated periodic syndrome (Muckle-Wells syndrome) and systemic lupus erhythematodes (SLE). Patient is a heterozygous carrier of a 3-nucleotide duplication in exon 3 of the NLRP3&nbsp;gene (c.1315_1317dupGCG), inserting an alanine between amino acid positions 439 and 440 (p.Ala439dup). This mutation is a characteristic with Muckle-wells syndrome. From the clinical examination she has clinical symptoms of SLE with moderate activity from SLEDAI = 9 points (“malar rash”, arthritis, leucopenia with lymphopenia, elevated levels of ANA-antibodys and anti-dsDNA-antibodys). Patient was treatment with methylprednisolone 12 mg daily, methotrexate 15 mg weekly, colchicin 2 time of 0,5 mg daily. Over 3 months patient has partial improvement&nbsp; of symptoms without full reduction. On the moment patient lives in Germany and has treatment with Anakinra with excellent results and good quality of life. In the literature is not found other with connection between cryopyrin-associated periodic syndrome (Muckle-Wells syndrome) and connective tissue disease.</p> Valentina Simeonova Reshkova Simeon Monov ##submission.copyrightStatement## http://creativecommons.org/licenses/by-nc-sa/4.0 2023-12-14 2023-12-14 31 3 88 95 10.35465/31.3.2023.pp88-95