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High- density lipoprotein function is abnormal in idiopathic inflammatory myopathies.

OBJECTIVE: Damage to the vascular endothelium is strongly implicated in the pathogenesis of idiopathic inflammatory myopathies (IIM). Normally, high-density lipoprotein (HDL) protects the vascular endothelium from damage from oxidized phospholipids, which accumulate under conditions of oxidative stress. The current work evaluated the antioxidant function of HDL in IIM patients. METHODS: HDL's antioxidant function was measured in IIM patients using a cell-free assay, which assesses the ability of isolated patient HDL to inhibit oxidation of low-density lipoproteins and is reported as the HDL i

Musculoskeletal Chronic Graft versus Host Disease-A Rare Complication to Allogeneic Hematopoietic Stem Cell Transplant: A Case-Based Report and Review of the Literature.

Musculoskeletal graft versus host disease (GVHD) is a rare manifestation of chronic GVHD (cGVHD) following allogeneic hematopoietic stem cell transplantation (allo-HSCT). Left untreated, the disease can cause extensive damage to muscle tissue and joints. We describe a 62-year-old male with musculoskeletal GVHD and generalized muscle pain and stiffness. In addition, we performed a systemic literature review based on published cases of musculoskeletal GVHD between 1983 and 2019. We identified 85 cases, 62% male and 38% female with an age of 4-69 years and median age of 39 years at diagnosis. The

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… Graft-versus-host disease-related polymyositis. Clin. Rheumatol. 2010;29:431–433. doi: 10.1007/s10067-009-1350-5. 19. George B., Danda D., Chandy M., Srivastava A., Mathews V. Polymyositis--an unusual manifestation of chronic graft-versus-host disease. Rheumatol. … Case report: Acute polymyositis in a patient with chronic graft vs. host disease. Med. Oncol. Tumor Pharmacother. 1992;9:149–150. doi: 10.1007/BF02987746. 23. Pier N., Dubowitz V. Chronic graft versus host disease presenting with polymyositis. Br. Med. J. (Clin. Res. …

Объединения между хулиганскими пемфигоидами и коморбидами: шведское общенациональное когортное исследование 5,738 Пациенты.

Бульварный пемфигоид (БП) связан с неврологическими и психиатрическими расстройствами, однако ни одно общенациональное исследование, основанное на народонаселении, не позволило в полной мере изучить связь различных коморбидов у пациентов BP в Швеции. ЦЕЛИ: Изучение связей между BP и различными совпадениями путем сопоставления этих условий до и после диагностирования BP с соответствующей контрольной группой. МЕТОДЫ: В Швеции было проведено общенациональное когортное исследование 2005 в адрес 2016, включая 5,738 Случаи BP и 17,167 :: соответствующие меры контроля в отношении возраста, пола и мес

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… In other cases, the sample size was insufficient to conduct a robust analysis, as seen in dermatomyositis, polymyositis, and rheumatoid arthritis ( Table 4 ). Table 4. … Several conditions, including dermatomyositis, polymyositis, and rheumatoid arthritis, lack sufficient data to determine any associations. …

BLyS and APRIL form biologically active heterotrimers that are expressed in patients with systemic immune-based rheumatic diseases.

BLyS and APRIL are two members of the TNF superfamily that are secreted by activated myeloid cells and have costimulatory activity on B cells. BLyS and APRIL share two receptors, TACI and BCMA, whereas a third receptor, BAFF-R, specifically binds BLyS. Both BLyS and APRIL have been described as homotrimeric molecules, a feature common to members of the TNF superfamily. In this study, we show that APRIL and BLyS can form active heterotrimeric molecules when coexpressed and that circulating heterotrimers are present in serum samples from patients with systemic immune-based rheumatic diseases. Th

Гематологические маньяки маскируются как ревматологические заболевания: серия дел и обзор.

RATALE: Гематологическая недостаточность может имитировать ревматологические заболевания, создавая серьезную диагностическую проблему из-за перекрывающихся клинических особенностей. В настоящем исследовании подчеркивается 5 - случаи гематологических маньяков, представляющих собой ревматологические расстройства, и обсуждаются связанные с этим диагностические сложности. БОЛЕЕ ТРУДЯЩИЕСЯ: 64 в адрес 78, с различными рематологическими симптомами, включая полиартрит, васкулит, явление Рейно, и такими систематическими симптомами, как потеря веса, усталость и ночной пот. В ходе первоначальной эксперт

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… Peripheral T-cell lymphoma with hypercalcemic crisis as a primary symptom accompanied by polymyositis: a case report and review of the literature. Oncol Lett. 2015;9:231–4. [17] Stockton D, Doherty VR, Brewster DH. … Risk of cancer in patients with dermatomyositis or polymyositis, and follow-up implications: a Scottish population-based cohort study. Br J Cancer. 2001;85:41–5. [18] Padhan P, Thakur B, Singh P, Mohanty I, Sahoo SR. …

Autoimmune Neuromuscular Disorders at a Molecular Crossroad: Linking Pathogenesis to Targeted Immunotherapy.

Myasthenia gravis, chronic inflammatory demyelinating polyneuropathy, and idiopathic inflammatory myopathies are among the most widely recognized autoimmune neuromuscular disorders. Although they differ in clinical presentation, shared immunopathogenic mechanisms place them at a molecular crossroads. Evidence of overlapping pathways has led to the development of targeted strategies including complement inhibition, FcRn antagonism, B-cell depletion, and the CAR-T cell approach. In this review, we analyze current knowledge regarding pathogenic mechanisms and their link to immunotherapy, extensiv

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… Polymyositis (PM) Clinical Features and Subtypes Polymyositis (PM) is now recognized as a rare phenotype. It is primarily a diagnosis of exclusion after other idiopathic inflammatory myopathies are considered [ 39 ]. … A defining feature of IBM is the infiltration of non-necrotic myofibers by cytotoxic CD8+ T cells, which differentiates it from polymyositis. …

Targeted Therapy for Idiopathic Inflammatory Myopathy.

BACKGROUND: Idiopathic inflammatory myopathies (IIM) are a heterogeneous group of autoimmune disorders characterized by chronic muscle inflammation and significant extramuscular involvement. A substantial proportion of patients exhibit refractory or relapsing disease despite conventional immunosuppressive therapies, necessitating the development of novel targeted treatments. Recent immunological advances have identified novel therapeutic targets-including B cells, T cells, cytokines and intracellular signalling pathways-paving the way for personalized treatment. Targeted therapies represent pr

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… Isaacs , “ Alemtuzumab (Campath‐1H) for Treatment of Refractory Polymyositis ,” Journal of Rheumatology 35 , no. 10 ( 2008 ): 2080 – 2082 . 18843768 43 T. W. Bunch , J. W. Worthington , J. J. Combs , D. M. Ilstrup , and A. G. Engel , “ Azathioprine With Prednisone for Polymyositis. … Kohsaka , “ Interleukin‐1 and Tumor Necrosis Factor α Blockade Treatment of Experimental Polymyositis in Mice ,” Arthritis and Rheumatism 64 , no. 8 ( 2012 ): 2655 – 2662 . 22392010 10.1002/art.34465 63 M. Zong , C. Dorph , M. …

ATOH8: a novel marker in human muscle fiber regeneration.

Regenerating muscle fibers emerge from quiescent satellite cells, which differentiate into mature multinuclear myofibers upon activation. It has recently been found that ATOH8, a bHLH transcription factor, is regulated during myogenic differentiation. In this study, expression and localization of ATOH8, the other well-described regeneration markers, vimentin, nestin and neonatal myosin, and the satellite cell marker Pax7 were analyzed on protein level in human myopathy samples by immunofluorescence studies. On mRNA level, expression levels of ATOH8 and vimentin were studied by quantitative rea

Identification of a SmD3 epitope with a single symmetrical dimethylation of an arginine residue as a specific target of a subpopulation of anti-Sm antibodies.

Anti-Sm antibodies, identified in 1966 by Tan and Kunkel, are highly specific serological markers for systemic lupus erythrematosus (SLE). Anti-Sm reactivity is found in 5-30% of SLE patients, depending on the autoantibody detection system and the racial background of the SLE population. The Sm autoantigen complex comprises at least nine different polypeptides. All of these core proteins can serve as targets of the anti-Sm B-cell response, but most frequently the B and D polypeptides are involved. Because the BB'Sm proteins share cross-reactive epitopes (PPPGMRPP) with U1 specific ribonucleopr

Резюме научных материалов, представленных ГКОМ 2019 : Берлин, Германия. 27 - 30 Март 2019

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… The subgroups of the patients were: polymyositis 40.7%, dermatomyositis 38.2%, clinically amyopathic dermatomyositis patients 21.1%. MSAs were detected in 59.3% of the patients with anti-Jo-1 antibody being the commonest (13.2%). … : Polymyositis and dermatomyositis-associated interstitial lung disease (PM/DM-ILD) have variable courses with different myositis specific autoantibodies (MSAs) and different histopathological and radiological types. …

Ten-year experience with endomyocardial biopsy in myocarditis presenting with congestive heart failure: frequency, pathologic characteristics, treatment and follow-up.

The present study summarizes our ten-year (1985-1995) experience with endomyocardial biopsy (EMB) in patients with idiopathic congestive hea … small vessel and capillaries; in 7 cases, there were myocyte changes similar to those described in polymyositis; in 1 case, we observed subplasmalemmal buddings, but no viral particles; in 6 cases, there was extensive myocyte damage with myofibrillar lysis and focal adipous metaplasia; the remaining 6 cases showed myocyte damage of differing extent and severi …

Targeting B Cells for the Treatment of Idiopathic Inflammatory Myopathy.

Idiopathic inflammatory myopathy (IIM) is a group of heterogeneous diseases that can affect multiple systems. Currently, it is classified into different subtypes based on myositis-specific antibodies and muscle tissue pathology. These subtypes vary in treatment response and clinical prognosis, with poor treatment outcomes observed in cases of rapidly progressive interstitial lung disease and severe muscle involvement. Despite conventional treatments, there remains a high rate of mortality and disability. The production of B cells and autoantibodies plays a crucial role in the pathogenesis of I

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… of refractory adult and juvenile dermatomyositis and adult polymyositis: a randomized, placebo-phase trial. … , Bregoli L, O’Connor KC, Amato AA (2005) Plasma cells in muscle in inclusion body myositis and polymyositis. …

Заболеваемая беременностью гипертензия и связь с будущими аутоиммунными заболеваниями.

ЦЕЛЬ: изучение связей между гипертоническими расстройствами беременности и последующим развитием аутоиммунных заболеваний. МЕТОДЫ: В этом ретроспективном когортном исследовании использовался TriNetX - федеративная сеть данных реального мира. Используя данные Глобальной сети сотрудничества, мы собрали электронные медицинские записи 102 организаций здравоохранения, 131 million записи пациентов из 2006 в адрес 2020. Исследование позволило оценить риск аутоиммунных заболеваний у женщин пожилого возраста. 16-45 Годы. Two Были сопоставлены следующие категории: обусловленная беременностью когорта гип

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… These diseases included SLE, multiple sclerosis, Addison disease, antiphospholipid syndrome, inflammatory bowel disease, mixed connective tissue disease, RA, Sjögren syndrome, polymyositis, dermatomyositis, Behçet disease, and polyarteritis nodosa. … However, the risks of other autoimmune diseases such as Sjogren syndrome, polymyositis, dermatomyositis, Behçet disease, and polyarteritis nodosa were not significantly elevated (Table 2 ). Table 2. …

Усовершенствованные испытания самообслуживания в системном склерозе

Системный склероз — это системное аутоиммунное ревматическое заболевание, характеризующееся иммунными аномалиями, приводящее к васкулопатии и фиброзу. Тестирование самодисциплины становится все более важной частью диагностики и прогнозирования. Клинические врачи были ограничены антиядерной антителной (АНА), антитопоизомеразой I (также известной как anti-Scl-70) антитела и антисентромерные антитела. Многие врачи в настоящее время улучшили доступ к расширенному профилю автоантиорганических тестов. В этой статье мы анализируем эпидемиологию, клинические ассоциации и предсказанную ценность совреме

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… Anti-Ku Antibodies Initially discovered in individuals with scleroderma–polymyositis overlap syndrome, anti-Ku antibodies were first reported in 1981 by Mimori et al. … Myositis-specific antibodies are predominantly found in patients with polymyositis or dermatomyositis, while myositis-associated antibodies are usually found in patients with overlapping features of myositis and other SARDs [ 78 ]. 3.1. …

Систематический обзор и метаанализ посттравматического стрессового расстройства как фактора риска множественных аутоиммунных заболеваний.

Посттравматический стрессовый расстройство (ПТСР) является распространенным и изнурительным психическим заболеванием, связанным с плохим состоянием здоровья и повышенным риском возникновения хронических заболеваний, включая множественные аутоиммунные заболевания, такие, как системный лейкоцит (SLE), инфламматический болезнь Боул (IBD), ревматоидный артрит (RA) и множественный склероз (MS). АИМ: Этот метаанализ позволяет оценить эпидемиологические исследования в этой области и кратко изучить гипотетические нейробиологические и иммунологические механизмы, которые могут лежать в основе связи межд

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… between PTSD and the development of autoimmune diseases as a whole appears relatively consistent across cohort studies, the strength of association and significance of the association of PTSD with specific autoimmune conditions, particularly Rheumatoid Arthritis (RA), IBD, and uncommon conditions such as Polymyositis … For example, only 2 studies examined polymyositis or dermatologic conditions, limiting their statistical power. Analysis was also limited by the data reported in the included studies, complicating statistical analysis. …

Diffuse alveolar hemorrhage in rheumatic diseases: Clinical features, diagnosis, and management.

Diffuse alveolar hemorrhage (DAH) is a catastrophic pulmonary manifestation of systemic autoimmune and rheumatic diseases, particularly systemic lupus erythematosus (SLE), ANCA-associated vasculitis (AAV), and antiphospholipid syndrome (APS). It arises from immune-mediated injury to the pulmonary microcirculation, leading to intra-alveolar bleeding, respiratory failure, and high mortality. Despite advances in immunosuppression, DAH continues to pose major diagnostic and therapeutic challenges, with limited consensus on optimal management. We conducted an updated and comprehensive review of the

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… Inflammatory Myopathies Polymyositis (PM) and dermatomyositis (DM) are rare but recognized causes of DAH, with only a few documented cases in the literature. [ 24 , 25 , 26 ] These inflammatory myopathies typically present with symptoms such as muscle pain, proximal muscle weakness, elevated muscle … A primary manifestation of polymyositis Am J Respir Crit Care Med. 1995 151 2037 – 2040 7767555 10.1164/ajrccm.151.6.7767555 [40] Malik A Ismail M Chaudhary M Abbas S Taylor J. 1170: A case of diffuse alveolar hemorrhage leading to the diagnosis of rheumatoid arthritis Critical Care Medicine 2025 53 …

Clinical applications of immunoglobulin: update.

Human immunoglobulin is the most used blood product in the clinical practice. Immunoglobulin applications have increased quickly since the elucidation of its immunomodulatory and antiinflammatory properties which turned this blood product into a precious tool in the treatment of numerous diseases that present with humoral immune deficiency or that cause immune system dysfunction. Currently, the approved indications for Ig are: primary immunodeficiencies, secondary immunodeficiencies (multiple myeloma or chronic lymphoid leukemia), Kawasaki syndrome, immune thrombocytopenic purpura, Guillain Ba

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… Arch Dis Child . 1995 ; 72 ( 1 ): 25 - 8 7717731 10.1136/adc.72.1.25 PMC1510958 83 Cherin P Piette JC Wechsler B Bletry O Ziza JM Laraki R et al Intravenous gamma globulin as first line therapy in polymyositis and dermatomyositis: an open study in 11 adult patients . … J Rheumatol . 1994 ; 21 ( 6 ): 1092 - 7 7932419 84 Cherin P Pelletier S Teixeira A Laforet P Genereau T Simon A et al Results and long-term followup of intravenous immunoglobulin infusions in chronic, refractory polymyositis: an open study with thirty-five adult patients . …

Ассоциация аутоиммунных заболеваний с раком поджелудочной железы: общенациональное исследование из Швеции.

ЦЕНТРАЛЬНАЯ ИНФОРМАЦИЯ: Во всем мире растет заболеваемость раком поджелудочной железы и аутоиммунными заболеваниями. Хотя некоторые связи между конкретными АД, такими, как панкреатит и ПК, уже давно хорошо подтверждены, ориентированные на население исследования, посвященные изучению более широкого спектра АД и их связи с риском, связанным с ПК, по-прежнему носят ограниченный характер. МЕТОДЫ: Мы провели когортное исследование на основе демографических данных для анализа взаимосвязей между АД и уязвимостью ПК. Диагностическая информация 43 АД была получена в шведском стационарном регистре (Swed

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… The highest SIRs were polymyositis (PM) (1.89), celiac disease (1.84), and discoid lupus erythematosus (DLE) (1.82). There were several differences between men and women: the 95% CI for men was 1.24 (95% CI 1.18–1.31), and that for women was 1.19 (95% CI 1.14–1.25). … 0.07 1.05 11 1.43 0.71 2.57 14 0.87 0.47 1.46 Pemphigoid 13 1.48 0.79 2.54 12 1.23 0.63 2.15 25 1.35 0.87 1.99 Pernicious anemia 55 1.70 1.28 2.22 56 1.57 1.19 2.04 111 1.63 1.34 1.97 Polymyalgia rheumatica 63 0.99 0.76 1.27 142 1.16 0.98 1.37 205 1.10 0.96 1.26 Polymyositis …

Tumors masquerading as neurological diseases: A caution for clinicians in planning diagnosis and treatment.

INTRODUCTION: Neurological diseases can be due to direct diseases of the central nervous system (CNS) or peripheral nervous system (PNS) or be a bystander syndrome of systemic diseases. Treatment options depend on the cause. Toxic, metabolic and nutritional, and immune-mediated consequences of clinically occult neoplasms produce a spectrum of neurological diseases, recognition of which has therapeutic and prognostic importance. PATIENTS AND METHODS: Children, as well as adults who presented to the authors in the last 5 years with neurological diseases and later their diseases could be diagnos

Пациенты с системным склерозом с синдромом перепадов и без него демонстрируют схожие микроваскулярные аномалии.

ВВЕДЕНИЕ: видеокаплироскопия с капиллярным покрытием (NVC) является полезным инструментом для измерения плотности капилляров (CD) и параметров капиллярной морфологии и используется главным образом в системном склерозе (SSc). ЦЕЛЬ: Мы стремились оценить распространенность SSc-образца и CD-диска в различных соединительных болезнях тканей (КТД). МЕТОДЫ: NVC был выполнен на 268 Пациенты с КТД. В состав контрольных групп входили: 104 добровольцы (ГТС) и 36 Первичные пациенты Рейно. МЕРОПРИЯТИЯ: Уменьшившийся компакт-диск был более распространен в SSc, системном волшебном эретематосе (SLE), подстрек

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… Nailfold capillaroscopic changes in dermatomyositis and polymyositis Clin. Rheumatol. 2014 34 279 284 10.1007/s10067-014-2795-8 25318613 28. Vaz J.L.P. Dancour M.A.A. Bottino D.A. Bouskela E. … Polymyositis and Dermatomyositis N. Engl. J. Med. 1975 292 344 347 10.1056/NEJM197502132920706 1090839 37. Petri M. Orbai A.-M. Alarcón G.S. Gordon C. Merrill J.T. Fortin P.R. Bruce I.N. Isenberg D. Wallace D.J. Nived O. …