Pub Date : 2023-09-06DOI: 10.35465/31.2.2023.pp58-66
Sevdalina Lambova, G. Hadzhigeorgiev, G. Gelov, P. Uchilov
Abstract: SAPHO syndrome is characterized with a set of symptoms including skin lesions, osteoarticular manifestations like aseptic osteitis (of the anterior chest wall or other skeletal sites), peripheral synovitis. Due to the lack of randomized clinical trials and low prevalence of the condition, there are no established treatment guidelines for SAPHO syndrome. Here, we report a clinical case of SAPHO syndrome with osteitis of the sternum and palmoplantar pustulosis successfully treated with zoledronic acid and hydroxychloroquine.
{"title":"A case of SAPHO syndrome treated with zoledronic acid and hydroxychloroquine","authors":"Sevdalina Lambova, G. Hadzhigeorgiev, G. Gelov, P. Uchilov","doi":"10.35465/31.2.2023.pp58-66","DOIUrl":"https://doi.org/10.35465/31.2.2023.pp58-66","url":null,"abstract":"Abstract: SAPHO syndrome is characterized with a set of symptoms including skin lesions, osteoarticular manifestations like aseptic osteitis (of the anterior chest wall or other skeletal sites), peripheral synovitis. Due to the lack of randomized clinical trials and low prevalence of the condition, there are no established treatment guidelines for SAPHO syndrome. Here, we report a clinical case of SAPHO syndrome with osteitis of the sternum and palmoplantar pustulosis successfully treated with zoledronic acid and hydroxychloroquine.","PeriodicalId":38954,"journal":{"name":"Revmatologiia (Bulgaria)","volume":"49 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-09-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135203933","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-09-06DOI: 10.35465/31.2.2023.pp67-76
Sevdalina Lambova, Tsv. Abadzhieva, Nikolay Stoilov, Vladimira Boyadzhieva
Abstract: Taxanes (paclitaxel, docetaxel) are antineoplastic agents used in advanced ovarian, breast, lung, head and neck cancer. Diverse cutaneous adverse reactions associated with taxane administration have been reported such as bullous eruption, onycholysis, acral erythema, erythema multiforme, pustular eruption, scleroderma-like skin changes of both upper and lower extremities. Here, we report a case of 48-year-old female patient, who presented for consultation with rheumatologist with complaints of hand and finger oedema and stiffness. Oedema and mild skin thickening of the fingers and hands were evident at physical examination. Inflammatory joint pain, synovitis, Raynaud’s phenomenon and trophic changes were not present. Standard laboratory tests i.e., complete blood count and biochemistry tests were within normal values. The patient was euthyroid. Immunological tests were negative (antinuclear antibodies; antibodies against extractable nuclear antigens – dsDNA, Sm, RNP, Scl-70; antiphospholipid antibodies – anticardiolipin, anti-beta-2-glycoprotein; anti-CCP antibody and rheumatoid factor). Capillaroscopic examination did not reveal signs of microangiopathy. Skin biopsy was perfomed in the area of a proximal phalanx and the histological examination revealed dermal oedema and superficial scant perivascular infiltrate containing lymphocytes and histiocytes. The patient had undergone an operation for breast cancer 13 years ago and subsequent second operation because of cancer recurrence 7 years ago. After the second operation chemotherapy was performed that included 1 cycle with paclitaxel and 4 cycles with docetaxel. The analysis of the case led to the conclusion that the scleroderma-like skin changes of the fingers and hands are induced by taxane administration in the past. The patient received corticosteroid treatment for 7 months with gradual resolution of symptoms.
{"title":"Taxane-induced scleroderma-like skin changes – review of the literature and case report","authors":"Sevdalina Lambova, Tsv. Abadzhieva, Nikolay Stoilov, Vladimira Boyadzhieva","doi":"10.35465/31.2.2023.pp67-76","DOIUrl":"https://doi.org/10.35465/31.2.2023.pp67-76","url":null,"abstract":"Abstract: Taxanes (paclitaxel, docetaxel) are antineoplastic agents used in advanced ovarian, breast, lung, head and neck cancer. Diverse cutaneous adverse reactions associated with taxane administration have been reported such as bullous eruption, onycholysis, acral erythema, erythema multiforme, pustular eruption, scleroderma-like skin changes of both upper and lower extremities. Here, we report a case of 48-year-old female patient, who presented for consultation with rheumatologist with complaints of hand and finger oedema and stiffness. Oedema and mild skin thickening of the fingers and hands were evident at physical examination. Inflammatory joint pain, synovitis, Raynaud’s phenomenon and trophic changes were not present. Standard laboratory tests i.e., complete blood count and biochemistry tests were within normal values. The patient was euthyroid. Immunological tests were negative (antinuclear antibodies; antibodies against extractable nuclear antigens – dsDNA, Sm, RNP, Scl-70; antiphospholipid antibodies – anticardiolipin, anti-beta-2-glycoprotein; anti-CCP antibody and rheumatoid factor). Capillaroscopic examination did not reveal signs of microangiopathy. Skin biopsy was perfomed in the area of a proximal phalanx and the histological examination revealed dermal oedema and superficial scant perivascular infiltrate containing lymphocytes and histiocytes. The patient had undergone an operation for breast cancer 13 years ago and subsequent second operation because of cancer recurrence 7 years ago. After the second operation chemotherapy was performed that included 1 cycle with paclitaxel and 4 cycles with docetaxel. The analysis of the case led to the conclusion that the scleroderma-like skin changes of the fingers and hands are induced by taxane administration in the past. The patient received corticosteroid treatment for 7 months with gradual resolution of symptoms.","PeriodicalId":38954,"journal":{"name":"Revmatologiia (Bulgaria)","volume":"29 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-09-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135203932","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-09-01DOI: 10.35465/31.2.2023.pp81-88
Ирина Иванова Момчева, Ivan Kazmin, Nikolay Stoilov
Inflammatory myopathies are a heterogeneous group of diseases rarely encountered in clinical practice. They primarily involve the transverse striated muscles, and in some cases there is also extramuscular involvement of the skin, lungs, joints, esophagus, heart. Acute forms of myositis, which are the most common, are associated with viral diseases and usually resolve spontaneously. Chronic forms usually have a subacute onset and unknown etiology. The presence of different clinical phenotypes and courses is associated with diversity in autoantibody production. Historically, the treatment of refractory forms of polymyositis and dermatomyositis has undergone significant dynamics. Data from various studies have been published that show a significant reduction in the symptoms of inflammation, but unfortunately some of these disease-modifying treatments could not be established in rheumatology practice as sufficiently effective. Limiting factors are the retrospective nature of these studies, as well as different inclusion and exclusion criteria. However, in recent years, more and more data have emerged that present rituximab as one of the promising molecules for the treatment of patients refractory to conventional synthetic disease-modifying antirheumatic agents We present a clinical case of a 40-year-old Caucasian man with onset of the disease – fever, asthenodynamia, muscle pain and weakness, pericardial and pleural effusions. The patient was treated with glucocorticoids, methotrexate, pulse therapies with methylprednisolone and cyclophosphamide, as well as pulse therapy with intravenous immunoglobulins. Due to the temporary effect of the treatment and the relapse of the disease, the patient was started on mycophenolate mofetil therapy (instead of methotrexate). However, a new peak in disease activity was reported, necessitating resumption of pulse therapies with methylprednisolone and cyclophosphamide. Due to the refractory course of the disease, the patient was treated with rituximab after signed informed consent. A significant reduction in disease activity and a good clinical and therapeutic effect were reported.
{"title":"Efficacy of rituximab in difficult to treatment patients with polymyositis","authors":"Ирина Иванова Момчева, Ivan Kazmin, Nikolay Stoilov","doi":"10.35465/31.2.2023.pp81-88","DOIUrl":"https://doi.org/10.35465/31.2.2023.pp81-88","url":null,"abstract":"Inflammatory myopathies are a heterogeneous group of diseases rarely encountered in clinical practice. They primarily involve the transverse striated muscles, and in some cases there is also extramuscular involvement of the skin, lungs, joints, esophagus, heart. Acute forms of myositis, which are the most common, are associated with viral diseases and usually resolve spontaneously. Chronic forms usually have a subacute onset and unknown etiology. The presence of different clinical phenotypes and courses is associated with diversity in autoantibody production. Historically, the treatment of refractory forms of polymyositis and dermatomyositis has undergone significant dynamics. Data from various studies have been published that show a significant reduction in the symptoms of inflammation, but unfortunately some of these disease-modifying treatments could not be established in rheumatology practice as sufficiently effective. Limiting factors are the retrospective nature of these studies, as well as different inclusion and exclusion criteria. However, in recent years, more and more data have emerged that present rituximab as one of the promising molecules for the treatment of patients refractory to conventional synthetic disease-modifying antirheumatic agents We present a clinical case of a 40-year-old Caucasian man with onset of the disease – fever, asthenodynamia, muscle pain and weakness, pericardial and pleural effusions. The patient was treated with glucocorticoids, methotrexate, pulse therapies with methylprednisolone and cyclophosphamide, as well as pulse therapy with intravenous immunoglobulins. Due to the temporary effect of the treatment and the relapse of the disease, the patient was started on mycophenolate mofetil therapy (instead of methotrexate). However, a new peak in disease activity was reported, necessitating resumption of pulse therapies with methylprednisolone and cyclophosphamide. Due to the refractory course of the disease, the patient was treated with rituximab after signed informed consent. A significant reduction in disease activity and a good clinical and therapeutic effect were reported.","PeriodicalId":38954,"journal":{"name":"Revmatologiia (Bulgaria)","volume":"53 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135248715","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-08-31DOI: 10.35465/31.2.2023.pp3-9
Serdar Kaymaz, M. Kavas, A. Demiray, U. Karasu, V. Çob
Objective: Sarcoidosis is a systemic granulomatous disease with an unknown etiology characterized by noncaseating granuloma formation. It most often affects the lungs. Protein tyrosine phosphatase non-receptor 22 (PTPN22) is a gene that acts as a negative regulator of T-cell activation. Polymorphisms of this gene are associated with multiple human autoimmune diseases. The aim of this study was to assess the relationship between PTPN22 polymorphisms and lung sarcoidosis in a selected population.
Methods: The study included 64 patients with lung sarcoidosis and 30 healthy controls. Patients were genotyped to determine two single nucleotide polymorphisms (SNPs) using the polymerase chain reaction (PCR) method. Hardy–Weinberg equilibrium (HWE), an important tool for detecting genotyping errors, was tested. Direct counting was used to estimate genotype frequencies. Logistic regression analysis was performed to compare allele and genotype frequencies between the patient and control groups.
Results: The results of the study showed no correlation between lung sarcoidosis and the tested two SNPs of the PTPN22 gene (rs2488457, rs1310182) (p>0.05). The categorization analysis according to the clinical features, laboratory, and radiographic characteristics showed no correlation between the tested polymorphism of PTPN22 and these characteristics (p>0.05).
Conclusion: The present study demonstrated that the studied two SNPs of the PTPN22 gene (rs2488457, rs1310182) were not correlated with lung sarcoidosis, suggesting that it might be different from other classic autoimmune disorders. There is a need for more studies to verify these results concerning lung sarcoidosis in other ethnic origins.
{"title":"Investigation of the Relationship between PTPN22 Polymorphisms and Lung Sarcoidosis: A cross sectional study","authors":"Serdar Kaymaz, M. Kavas, A. Demiray, U. Karasu, V. Çob","doi":"10.35465/31.2.2023.pp3-9","DOIUrl":"https://doi.org/10.35465/31.2.2023.pp3-9","url":null,"abstract":"Objective: Sarcoidosis is a systemic granulomatous disease with an unknown etiology characterized by noncaseating granuloma formation. It most often affects the lungs. Protein tyrosine phosphatase non-receptor 22 (PTPN22) is a gene that acts as a negative regulator of T-cell activation. Polymorphisms of this gene are associated with multiple human autoimmune diseases. The aim of this study was to assess the relationship between PTPN22 polymorphisms and lung sarcoidosis in a selected population.
 Methods: The study included 64 patients with lung sarcoidosis and 30 healthy controls. Patients were genotyped to determine two single nucleotide polymorphisms (SNPs) using the polymerase chain reaction (PCR) method. Hardy–Weinberg equilibrium (HWE), an important tool for detecting genotyping errors, was tested. Direct counting was used to estimate genotype frequencies. Logistic regression analysis was performed to compare allele and genotype frequencies between the patient and control groups.
 Results: The results of the study showed no correlation between lung sarcoidosis and the tested two SNPs of the PTPN22 gene (rs2488457, rs1310182) (p>0.05). The categorization analysis according to the clinical features, laboratory, and radiographic characteristics showed no correlation between the tested polymorphism of PTPN22 and these characteristics (p>0.05). 
 Conclusion: The present study demonstrated that the studied two SNPs of the PTPN22 gene (rs2488457, rs1310182) were not correlated with lung sarcoidosis, suggesting that it might be different from other classic autoimmune disorders. There is a need for more studies to verify these results concerning lung sarcoidosis in other ethnic origins.","PeriodicalId":38954,"journal":{"name":"Revmatologiia (Bulgaria)","volume":"3 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-08-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135988892","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-08-31DOI: 10.35465/31.2.2023.pp18-40
Vladimira Boyadzhieva, Jaklin Svetoslavova Doncheva - Dilova, Nikolay Stoilov
Raynaud's phenomenon (RP), which can be primary or secondary, is a common vascular clinical syndrome due to abnormal arteriolar vasospasm. Its treatment is most often conservative depending on the etiology. In recent years, there have been reports of RP as a syndrome after a previous SARS-CoV-2 infection, in patients of different genders and ages. As an etiological factor for the development of vascular pathology in these cases, two main mechanisms are assumed: an autoimmune process or the thrombosis of arterial vessels, leading to tissue ischemia, and the so-called necrotizing Raynaud's phenomenon. In the pathogenesis of Raynaud's phenomenon, the influence of local, neuronal and hormonal mediators is emphasized. Some studies prove the role of estrogens, which explains the higher incidence of RP among women. At present, there is no convincing evidence for "candidate genes" to be associated with Raynaud's phenomenon, despite studies by some authors (Susol et al., 2000; Pistorius et al., 2006). Vasospasm in digital ischemia may be further complicated by COVID-19 infection. Another potential component is hypercoagulation (further complicated by the presence of antiphospholipid antibodies in certain patients) and elevated levels of D-dimer. A state of hypercoagulation is caused by the so-called cytokine storm. This inflammatory state, as a result of endovascular damage, increased platelet activity and coagulation cascade, causes the so-called phenomenon of immunothrombosis. Overactivation of the coagulation pathway during cytokine storm results from increased activity of thrombin, which has an additional role in the inflammatory process through proteinase-activated receptors (PARs). Acrocyanosis due to excessive coagulation status has been described in critically ill patients with COVID-19. In these patients, gangrene may arise from impaired blood flow and insufficient healing of digital wounds, which is associated with elevated levels of C-reactive protein (CRP). Ischemic limb lesions, usually seen in older patients with severe clinical course of the disease, represent a dangerous, although rare, complication associated with COVID-19 and are due to arterial occlusions. They are extremely difficult to treat and often lead to amputations. In patients with antiphospholipid syndrome, arterial and venous thrombi are primarily caused by the formation of neutrophil extracellular traps (NET), which in turn activate platelets, and their excessive formation can lead to local thrombosis. In addition to platelet activation, neutrophils release tissue factor, which initiates the coagulation cascade. NETs bind coagulation factor XII and activate it, and also induce an inflammatory reaction in the vessel wall. According to the available knowledge to date, the hypothesis that digital necrosis in patients with COVID-19 is primarily related to the formation of NETs has been developed.
Necrotizing Raynaud's phenomenon (NRP) is a vascular clinical syndrome charac
雷诺氏现象(Raynaud's phenomenon, RP)是一种常见的由动脉血管异常痉挛引起的血管综合征，可为原发性或继发性。根据病因，其治疗通常是保守的。近年来，有报道称，在不同性别和年龄的患者中，RP是先前SARS-CoV-2感染后的一种综合征。作为这些病例中血管病理发展的病因因素，假设有两种主要机制:自身免疫过程或动脉血管血栓形成，导致组织缺血，以及所谓的坏死性雷诺现象。在雷诺现象的发病机制中，强调了局部、神经元和激素介质的影响。一些研究证实了雌激素的作用，这解释了RP在女性中发病率较高的原因。目前，尽管有一些作者(Susol et al.， 2000;Pistorius et al.， 2006)。手指缺血时血管痉挛可能会因COVID-19感染而进一步恶化。另一个潜在因素是高凝(在某些患者中抗磷脂抗体的存在进一步复杂化)和d -二聚体水平升高。高凝状态是由所谓的细胞因子风暴引起的。这种炎症状态，由于血管内损伤，血小板活性增加和凝血级联，导致所谓的免疫血栓形成现象。在细胞因子风暴中，凝血途径的过度激活是由凝血酶活性增加引起的，凝血酶通过蛋白酶激活受体(PARs)在炎症过程中起着额外的作用。在COVID-19危重患者中有过凝血状态过度引起的肢绀。在这些患者中，坏疽可能是由血流受损和手指伤口愈合不足引起的，这与c反应蛋白(CRP)水平升高有关。缺血性肢体病变通常见于临床病程严重的老年患者，是与COVID-19相关的一种危险但罕见的并发症，由动脉闭塞引起。它们极难治疗，经常导致截肢。在抗磷脂综合征患者中，动脉和静脉血栓主要是由中性粒细胞胞外陷阱(NET)的形成引起的，NET反过来激活血小板，它们的过度形成可导致局部血栓形成。除了活化血小板，中性粒细胞释放组织因子，启动凝血级联反应。NETs结合凝血因子XII并激活凝血因子，同时在血管壁上诱导炎症反应。根据目前掌握的知识，已经提出了COVID-19患者指状坏死主要与NETs形成有关的假设。
坏死性雷诺现象(Necrotizing Raynaud's phenomenon, NRP)是一种以低温或焦虑、应激状态下远端阻力血管收缩为特征的血管临床综合征。第一个症状是疼痛，由于缺氧，导致组织缺血。严重急性呼吸综合征冠状病毒2 (SARS-CoV-2)可引起内皮病变，伴微血管和大血管血栓事件。COVID-19引起高凝、血栓形成、内皮损伤和炎症，导致血管炎。在COVID-19中，凝血因子活性增加、保护性糖杯功能丧失和一氧化氮水平降低会加剧凝血功能障碍、炎症和血栓形成。COVID-19对RP患者的影响仍在研究中。
本文综述了一系列与既往COVID-19感染或接种疫苗继发的雷诺现象(坏死、新发、加重或作为另一系统性结缔组织疾病的一部分)相关的临床病例。
{"title":"Necrotizing Raynaud's phenomenon after recurrent COVID-19 infection","authors":"Vladimira Boyadzhieva, Jaklin Svetoslavova Doncheva - Dilova, Nikolay Stoilov","doi":"10.35465/31.2.2023.pp18-40","DOIUrl":"https://doi.org/10.35465/31.2.2023.pp18-40","url":null,"abstract":"Raynaud's phenomenon (RP), which can be primary or secondary, is a common vascular clinical syndrome due to abnormal arteriolar vasospasm. Its treatment is most often conservative depending on the etiology. In recent years, there have been reports of RP as a syndrome after a previous SARS-CoV-2 infection, in patients of different genders and ages. As an etiological factor for the development of vascular pathology in these cases, two main mechanisms are assumed: an autoimmune process or the thrombosis of arterial vessels, leading to tissue ischemia, and the so-called necrotizing Raynaud's phenomenon. In the pathogenesis of Raynaud's phenomenon, the influence of local, neuronal and hormonal mediators is emphasized. Some studies prove the role of estrogens, which explains the higher incidence of RP among women. At present, there is no convincing evidence for \"candidate genes\" to be associated with Raynaud's phenomenon, despite studies by some authors (Susol et al., 2000; Pistorius et al., 2006). Vasospasm in digital ischemia may be further complicated by COVID-19 infection. Another potential component is hypercoagulation (further complicated by the presence of antiphospholipid antibodies in certain patients) and elevated levels of D-dimer. A state of hypercoagulation is caused by the so-called cytokine storm. This inflammatory state, as a result of endovascular damage, increased platelet activity and coagulation cascade, causes the so-called phenomenon of immunothrombosis. Overactivation of the coagulation pathway during cytokine storm results from increased activity of thrombin, which has an additional role in the inflammatory process through proteinase-activated receptors (PARs). Acrocyanosis due to excessive coagulation status has been described in critically ill patients with COVID-19. In these patients, gangrene may arise from impaired blood flow and insufficient healing of digital wounds, which is associated with elevated levels of C-reactive protein (CRP). Ischemic limb lesions, usually seen in older patients with severe clinical course of the disease, represent a dangerous, although rare, complication associated with COVID-19 and are due to arterial occlusions. They are extremely difficult to treat and often lead to amputations. In patients with antiphospholipid syndrome, arterial and venous thrombi are primarily caused by the formation of neutrophil extracellular traps (NET), which in turn activate platelets, and their excessive formation can lead to local thrombosis. In addition to platelet activation, neutrophils release tissue factor, which initiates the coagulation cascade. NETs bind coagulation factor XII and activate it, and also induce an inflammatory reaction in the vessel wall. According to the available knowledge to date, the hypothesis that digital necrosis in patients with COVID-19 is primarily related to the formation of NETs has been developed.
 Necrotizing Raynaud's phenomenon (NRP) is a vascular clinical syndrome charac","PeriodicalId":38954,"journal":{"name":"Revmatologiia (Bulgaria)","volume":"4 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-08-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135988312","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
The infectious disease COVID-19, caused by the SARS-CoV-2 virus, is characterized by a wide range of clinical manifestations (pulmonary and extrapulmonary) and in some cases with post-infectious manifestations in persons who survived the infection. The vascular tropism of SARS-CoV-2 is the basis for the interest in using nailfold capillaroscopy to assess the microcirculation in children and adults who have recovered from COVID-19. Most often, non-specific changes are observed, without certain manifestations of microangiopathy and without a specific combination of microvascular findings, on the basis of which to define a specific capillaroscopic picture of the "COVID - 19" type. The possibility that capillaroscopy in combination with other findings can be used for personalized approach and determination of prognosis in patients with post-COVID syndrome needs further studies.
{"title":"Capillaroscopy in COVID-19","authors":"Jaklin Svetoslavova Doncheva - Dilova, Vladimira Boyadzhieva, Nikolay Stoilov, Sevdalina Lambova","doi":"10.35465/31.2.2023.pp41-57","DOIUrl":"https://doi.org/10.35465/31.2.2023.pp41-57","url":null,"abstract":"The infectious disease COVID-19, caused by the SARS-CoV-2 virus, is characterized by a wide range of clinical manifestations (pulmonary and extrapulmonary) and in some cases with post-infectious manifestations in persons who survived the infection. The vascular tropism of SARS-CoV-2 is the basis for the interest in using nailfold capillaroscopy to assess the microcirculation in children and adults who have recovered from COVID-19. Most often, non-specific changes are observed, without certain manifestations of microangiopathy and without a specific combination of microvascular findings, on the basis of which to define a specific capillaroscopic picture of the \"COVID - 19\" type. The possibility that capillaroscopy in combination with other findings can be used for personalized approach and determination of prognosis in patients with post-COVID syndrome needs further studies.","PeriodicalId":38954,"journal":{"name":"Revmatologiia (Bulgaria)","volume":"8 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-08-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135988315","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-08-31DOI: 10.35465/31.2.2023.pp77-80
M. Krstevski, Olivera Gjeorgjieva Janev, L. Krstic
Thrombotic thrombocytopenic purpura (TTP) is a rare, potentially fatal blood disorder that can be challenging for clinicians to identify in the setting of autoimmune diseases such as systemic lupus erythematosus (SLE). SLE can present with thrombotic microangiopathy (TMA) and differentiation between the two diseases can be quite difficult.
In this case report we present a 62-year-old female with a history of SLE who was hospitalized with thrombocytopenia, dysarthria, dizziness, paresthesia and altered mental status. The laboratory results revealed direct Coombs negative hemolytic anemia, severe thrombocytopenia, significant elevation of lactate dehydrogenase, leukocytosis and presence of few schistocytes in the peripheral film. In addition, we also present evidence for circulating anti-ADAMTS13 antibody/ADAMTS13 antigen. Based on clinical, hematological, and biochemical findings, we concluded that it was a case of TTP-like microangiopathy associated with SLE and indicated treatment with methylprednisolone pulses and plasmapheresis. Later on, she developed psychosis and made plasmapheresis difficult to perform. We continued corticosteroids (dose 1mg/kg), RhoGAM, Chloroquine and Azathioprine, obtaining a satisfactory response after one week of treatment.
{"title":"Overlapping features of thrombotic thrombocytopenic purpura and systemic lupus erythematosus, a diagnostic and a management challenge","authors":"M. Krstevski, Olivera Gjeorgjieva Janev, L. Krstic","doi":"10.35465/31.2.2023.pp77-80","DOIUrl":"https://doi.org/10.35465/31.2.2023.pp77-80","url":null,"abstract":"Thrombotic thrombocytopenic purpura (TTP) is a rare, potentially fatal blood disorder that can be challenging for clinicians to identify in the setting of autoimmune diseases such as systemic lupus erythematosus (SLE). SLE can present with thrombotic microangiopathy (TMA) and differentiation between the two diseases can be quite difficult.
 In this case report we present a 62-year-old female with a history of SLE who was hospitalized with thrombocytopenia, dysarthria, dizziness, paresthesia and altered mental status. The laboratory results revealed direct Coombs negative hemolytic anemia, severe thrombocytopenia, significant elevation of lactate dehydrogenase, leukocytosis and presence of few schistocytes in the peripheral film. In addition, we also present evidence for circulating anti-ADAMTS13 antibody/ADAMTS13 antigen. Based on clinical, hematological, and biochemical findings, we concluded that it was a case of TTP-like microangiopathy associated with SLE and indicated treatment with methylprednisolone pulses and plasmapheresis. Later on, she developed psychosis and made plasmapheresis difficult to perform. We continued corticosteroids (dose 1mg/kg), RhoGAM, Chloroquine and Azathioprine, obtaining a satisfactory response after one week of treatment.
","PeriodicalId":38954,"journal":{"name":"Revmatologiia (Bulgaria)","volume":"133 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-08-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135988564","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-08-31DOI: 10.35465/31.2.2023.pp10-17
Ahmed Elsaman, Asmaa Khalifa, Sohir Mahrous, Medhat Ibraheem, Fatma Ali, Ahmed Radwan
Background: The value of steroid and lidocaine injection of the sacroiliac joint in axial spondyloarthritis patients on magnetic resonance imaging scores and clinical disease outcome measures is not yet well assessed.
Patient and methods: Participants were randomly assigned into two groups; Group I received unilateral ultrasound-guided sacroiliac joint injection with .5% lidocaine hydrochloride mixed with triamcinolone. At the same time, group II received subcutaneous saline injection. All participants fulfilled the ASAS criteria for axial SpA and all had bone marrow edema of at least one sacroiliac joint at baseline. Outcomes measures were: Visual Analogue Scale (VAS), Ankylosing Spondylitis Disease Activity Score (ASDAS), Bath Ankylosing Spondylitis Functional Index (BASFI), and Spondyloarthritis Research Consortium of Canada (SPARCC) scores. Participants were assessed at baseline (before and after sacroiliac joint injection) and after three months. This clinical trial was registered on clinicaltrials.gov under the number NCT04895228.
Results: In this study, a total of 61 participants were registered. There was a significant difference between both groups regarding pain, spine mobility, SPARCC, and ASDAS scores in favor of group I. Spine mobility showed the earliest improvement, followed by pain whilst SPARCC, and ASDAS scores showed improvement after three months. Higher disease activity, younger age, and shorter disease duration were all associated with better outcomes. Bilateral hip involvement was a predictor of poor response.
Conclusion: Sacroiliac joint injection of lidocaine and triamcinolone in axial SpA patients is a cost-effective and practical technique for controlling pain, improving function, disease activity scores, and bone marrow edema with acceptable complications and relatively sustained effect.
{"title":"Impact of steroid and lidocaine injection in the sacroiliac joint in axial spondyloarthritis patients: a randomized controlled trial","authors":"Ahmed Elsaman, Asmaa Khalifa, Sohir Mahrous, Medhat Ibraheem, Fatma Ali, Ahmed Radwan","doi":"10.35465/31.2.2023.pp10-17","DOIUrl":"https://doi.org/10.35465/31.2.2023.pp10-17","url":null,"abstract":"Background: The value of steroid and lidocaine injection of the sacroiliac joint in axial spondyloarthritis patients on magnetic resonance imaging scores and clinical disease outcome measures is not yet well assessed.
 Patient and methods: Participants were randomly assigned into two groups; Group I received unilateral ultrasound-guided sacroiliac joint injection with .5% lidocaine hydrochloride mixed with triamcinolone. At the same time, group II received subcutaneous saline injection. All participants fulfilled the ASAS criteria for axial SpA and all had bone marrow edema of at least one sacroiliac joint at baseline. Outcomes measures were: Visual Analogue Scale (VAS), Ankylosing Spondylitis Disease Activity Score (ASDAS), Bath Ankylosing Spondylitis Functional Index (BASFI), and Spondyloarthritis Research Consortium of Canada (SPARCC) scores. Participants were assessed at baseline (before and after sacroiliac joint injection) and after three months. This clinical trial was registered on clinicaltrials.gov under the number NCT04895228.
 Results: In this study, a total of 61 participants were registered. There was a significant difference between both groups regarding pain, spine mobility, SPARCC, and ASDAS scores in favor of group I. Spine mobility showed the earliest improvement, followed by pain whilst SPARCC, and ASDAS scores showed improvement after three months. Higher disease activity, younger age, and shorter disease duration were all associated with better outcomes. Bilateral hip involvement was a predictor of poor response.
 Conclusion: Sacroiliac joint injection of lidocaine and triamcinolone in axial SpA patients is a cost-effective and practical technique for controlling pain, improving function, disease activity scores, and bone marrow edema with acceptable complications and relatively sustained effect.
","PeriodicalId":38954,"journal":{"name":"Revmatologiia (Bulgaria)","volume":"4 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-08-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135988316","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-05-25DOI: 10.35465/31.1.2023.pp108-119
Polina Georgieva Marinova
Abstract
Tophaceouse gout is a rare condition and chronic complication of gout due to storage of derivates of uric acid – crystals of monosodium urate, accompanied by lipids, mucopolisaharides matrix and proteins. Rheumatology meets the surgery in cases of rupture, ulceration and inflammation of tophi with complication with severe soft tissue infection, sepsis, large swelling, destruction of affected joints and periphery neuropathy chronic pain. Spontaneous or traumatic perforation and ulceration of tophi are rare conditions, that’s why there no any rheumatology or surgical guideline for management of that condition. We present a case report of male patient with posttraumatic perforated gout tophus and soft tissue secondary infection of left foot. He was treated with standard surgical debridement and local ozone therapy with stabilized ozonides over wound surface. That article presents a pioneer experience in the treatment of complicated tophaceouse gout with topical administration of ozonated olive oil with vitamin E and shows the effect of that treatment in progress.
{"title":"Ruptured ulcerated and inflammed gout tophi with deep soft tissue infection of left foot – when rheumatology meets surgery: a case report treated with local ozone therapy","authors":"Polina Georgieva Marinova","doi":"10.35465/31.1.2023.pp108-119","DOIUrl":"https://doi.org/10.35465/31.1.2023.pp108-119","url":null,"abstract":"Abstract
 Tophaceouse gout is a rare condition and chronic complication of gout due to storage of derivates of uric acid – crystals of monosodium urate, accompanied by lipids, mucopolisaharides matrix and proteins. Rheumatology meets the surgery in cases of rupture, ulceration and inflammation of tophi with complication with severe soft tissue infection, sepsis, large swelling, destruction of affected joints and periphery neuropathy chronic pain. Spontaneous or traumatic perforation and ulceration of tophi are rare conditions, that’s why there no any rheumatology or surgical guideline for management of that condition. We present a case report of male patient with posttraumatic perforated gout tophus and soft tissue secondary infection of left foot. He was treated with standard surgical debridement and local ozone therapy with stabilized ozonides over wound surface. That article presents a pioneer experience in the treatment of complicated tophaceouse gout with topical administration of ozonated olive oil with vitamin E and shows the effect of that treatment in progress.","PeriodicalId":38954,"journal":{"name":"Revmatologiia (Bulgaria)","volume":"12 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-05-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"136346196","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2021-07-26DOI: 10.35465/29.2.2021.pp40-51
T. Georgiev, P. Kabakchieva
The close link between osteoarthritis (OA) and metabolic disorders on the one hand and hormonal disorders on the other suggests a possible association between OA and endocrine-metabolic disorders, such as PCOS. The aim of this review is to analyze the relationship between PCOS and OA, to consider the common pathogenetic mechanisms between the two conditions, and to summarize the data accumulated so far in the literature. For the purposes of our narrative review, a comprehensive search was conducted within credible databases. Our literature search found that epidemiological studies have shown a higher incidence of knee and hip OA in women with PCOS. This can be partly explained by obesity, which is a common intersection between the two conditions. Potential mechanisms among OA, PCOS, and obesity were considered. Another common point between OA and PCOS is that both conditions can be considered as highly heterogeneous syndromes with various etiologies, the result of a combination of systemic (genetic, hormonal, and metabolic) and local factors. To date, hyperandrogenism and greater cartilage thickness in young women with PCOS remain unclear in terms of determining the risk of developing OA. Prospective longitudinal studies are needed to assess the “fate” of the weight-bearing joints in women with PCOS, who are more likely to suffer from knee joint complaints.
{"title":"Polycystic ovary syndrome metabolic and hormonal dysfunction in relation to osteoarthritis onset and development: innocent bystander or major risk factor?","authors":"T. Georgiev, P. Kabakchieva","doi":"10.35465/29.2.2021.pp40-51","DOIUrl":"https://doi.org/10.35465/29.2.2021.pp40-51","url":null,"abstract":"The close link between osteoarthritis (OA) and metabolic disorders on the one hand and hormonal disorders on the other suggests a possible association between OA and endocrine-metabolic disorders, such as PCOS. The aim of this review is to analyze the relationship between PCOS and OA, to consider the common pathogenetic mechanisms between the two conditions, and to summarize the data accumulated so far in the literature. For the purposes of our narrative review, a comprehensive search was conducted within credible databases. Our literature search found that epidemiological studies have shown a higher incidence of knee and hip OA in women with PCOS. This can be partly explained by obesity, which is a common intersection between the two conditions. Potential mechanisms among OA, PCOS, and obesity were considered. Another common point between OA and PCOS is that both conditions can be considered as highly heterogeneous syndromes with various etiologies, the result of a combination of systemic (genetic, hormonal, and metabolic) and local factors. To date, hyperandrogenism and greater cartilage thickness in young women with PCOS remain unclear in terms of determining the risk of developing OA. Prospective longitudinal studies are needed to assess the “fate” of the weight-bearing joints in women with PCOS, who are more likely to suffer from knee joint complaints.","PeriodicalId":38954,"journal":{"name":"Revmatologiia (Bulgaria)","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-07-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"43576594","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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