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1.
BMC Musculoskelet Disord ; 23(1): 425, 2022 May 06.
Artigo em Inglês | MEDLINE | ID: mdl-35524238

RESUMO

OBJECTIVE: Immune-mediated necrotising myopathy (IMNM) is a subset of idiopathic inflammatory myopathies (IIM) characterized by significantly elevated creatine kinase level, muscle weakness and predominant muscle fibre necrosis in muscle biopsy. This study aimed to investigate the clinical and pathological characteristics of patients with IMNM in a single-centre muscle biopsy cohort. METHODS: A total of 860 patients who had muscle biopsy reports in our centre from May 2008 to December 2017 were enrolled in this study. IMNM was diagnosed according to the 2018 European Neuromuscular Centre (ENMC) clinicopathological diagnostic criteria for IMNM. RESULTS: The muscle biopsy cohort consisted of 531 patients with IIM (61.7%), 253 patients with non-IIM (29.4%), and 76 undiagnosed patients (8.8%). IIM cases were classified as IMNM (68[7.9%]), dermatomyositis (346[40.2%]), anti-synthetase syndrome (82[9.5%]), polymyositis (32[3.7%]), and sporadic inclusion body myositis (3[0.3%]). Limb girdle muscular dystrophy (LGMD) 2B and lipid storage myopathy (LSM) are the two most common non-IIM disorders in our muscle biopsy cohort. IMNM patients had a higher onset age (41.57 ± 14.45 vs 21.66 ± 7.86 and 24.56 ± 10.78, p < .0001), shorter duration (21.79 ± 26.01 vs 66.69 ± 67.67 and 24.56 ± 10.78, p < .0001), and more frequent dysphagia (35.3% vs. 3.4 and 6.3%, p = .001) than LGMD 2B and LSM patients. Muscle biopsy from IMNM showed more frequent muscle fibre necrosis (95.6% vs 72.4 and 56.3%, p < .0001), overexpression of major histocompatibility complex-I on sarcolemma (83.8% vs 37.9 and 12.9%, p < .0001), and CD4+ T cell endomysia infiltration (89.7% vs 53.6 and 50%, p < .0001) compared with those from LGMD 2B and LSM patients. CONCLUSIONS: It is easy to distinguish IMNM from other IIM subtypes according to clinical symptoms and myositis specific antibodies profiles. However, distinguishing IMNM from disorders clinically similar to non-IIM needs combined clinical, serological and pathological features.


Assuntos
Doenças Autoimunes , Distrofia Muscular do Cíngulo dos Membros , Miosite , Autoanticorpos , Doenças Autoimunes/diagnóstico , Biópsia , Humanos , Erros Inatos do Metabolismo Lipídico , Músculo Esquelético/patologia , Distrofias Musculares , Miosite/diagnóstico , Miosite/patologia , Necrose/patologia
2.
Acta Myol ; 41(1): 41-47, 2022 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-35465344

RESUMO

We report the case of a young woman with CMV infection, high level of creatine kinase and myopathy. Electromyography showed a myopathic pattern. Muscle biopsy showed a marked increase of NADH enzymatic activity in the central area of almost all type I fibres, few degenerative and necrotic fibres and scattered mononuclear cell infiltrates. Ultrastructural analysis showed a marked disarrangement of sarcomeric structure and large inclusions of thin filaments in some fibres, while immunohistochemistry evidenced alteration in desmin, actin and αB-crystallin protein signals. PCR for CMV detection on muscle sections was negative. Histological, immunological and ultrastructural evaluations were compatible with a necrotic inflammatory myopathy. The correlations between CMV liver infection and the myopathic pattern are discussed. This case underscores the need to consider CMV infection in the differential diagnosis of myopathy with undetermined aetiology, quickly providing directions for a targeted muscle pharmacological intervention.


Assuntos
Doenças Autoimunes , Infecções por Citomegalovirus , Doenças Musculares , Miosite , Infecções por Citomegalovirus/complicações , Infecções por Citomegalovirus/diagnóstico , Infecções por Citomegalovirus/metabolismo , Feminino , Humanos , Músculo Esquelético/patologia , Doenças Musculares/metabolismo , Miosite/diagnóstico , Miosite/patologia
3.
BMJ Case Rep ; 15(4)2022 Apr 04.
Artigo em Inglês | MEDLINE | ID: mdl-35379682

RESUMO

A woman in her late 30s recently diagnosed with viral myopericarditis presented with 1 month of worsening fatigue, diffuse myalgias and chest pain radiating to her back. While undergoing work-up for chest wall myositis, she rapidly decompensated, developing heart failure and acute hypoxaemic respiratory failure. Her clinical course was complicated by cardiac arrest and severe cardiogenic shock requiring intra-aortic balloon pump support.


Assuntos
Parada Cardíaca , Miocardite , Miosite , Feminino , Parada Cardíaca/complicações , Parada Cardíaca/terapia , Humanos , Balão Intra-Aórtico/efeitos adversos , Miocardite/complicações , Miocardite/diagnóstico , Miosite/complicações , Choque Cardiogênico/diagnóstico , Choque Cardiogênico/etiologia , Choque Cardiogênico/terapia
4.
Int J Mol Sci ; 23(8)2022 Apr 13.
Artigo em Inglês | MEDLINE | ID: mdl-35457124

RESUMO

Idiopathic inflammatory myopathies (IIM), collectively known as myositis, are a composite group of rare autoimmune diseases affecting mostly skeletal muscle, although other organs or tissues may also be involved. The main clinical feature of myositis is subacute, progressive, symmetrical muscle weakness in the proximal arms and legs, whereas subtypes of myositis may also present with extramuscular features, such as skin involvement, arthritis or interstitial lung disease (ILD). Established subgroups of IIM include dermatomyositis (DM), immune-mediated necrotizing myopathy (IMNM), anti-synthetase syndrome (ASyS), overlap myositis (OM) and inclusion body myositis (IBM). Although these subgroups have overlapping clinical features, the widespread variation in the clinical manifestations of IIM suggests different pathophysiological mechanisms. Various components of the immune system are known to be important immunopathogenic pathways in IIM, although the exact pathophysiological mechanisms causing the muscle damage remain unknown. Current treatment, which consists of glucocorticoids and other immunosuppressive or immunomodulating agents, often fails to achieve a sustained beneficial response and is associated with various adverse effects. New therapeutic targets have been identified that may improve outcomes in patients with IIM. A better understanding of the overlapping and diverging pathophysiological mechanisms of the major subgroups of myositis is needed to optimize treatment. The aim of this review is to report on recent advancements regarding DM and IMNM.


Assuntos
Doenças Autoimunes , Dermatomiosite , Miosite de Corpos de Inclusão , Miosite , Doenças Autoimunes/patologia , Doenças Autoimunes/terapia , Dermatomiosite/patologia , Dermatomiosite/terapia , Humanos , Debilidade Muscular/patologia , Músculo Esquelético/patologia
5.
Pediatr Rheumatol Online J ; 20(1): 28, 2022 Apr 12.
Artigo em Inglês | MEDLINE | ID: mdl-35414090

RESUMO

BACKGROUND: Environmental exposures have been associated with the juvenile idiopathic inflammatory myopathies (JIIM). We undertook a questionnaire-based study to evaluate patient-reported exposures as possible risk factors for JIIM. FINDINGS: One-hundred-seven patients with JIIM were enrolled in a myositis natural history protocol and completed environmental questionnaires. Frequencies of exposures in clinical and myositis-specific autoantibody (MSA) groups were examined. Patients with juvenile dermatomyositis (JDM) and juvenile connective tissue myositis (JCTM) more frequently received an immunization within 1 year of diagnosis compared to juvenile polymyositis (57.5 and 71.4% vs 0.0%, p ≤ 0.017). JCTM patients were more often underweight at diagnosis relative to JDM patients (42.9% vs 7.0%, p = 0.002). MSA-negative patients more frequently had gastroenteritis within a year of diagnosis compared to patients with anti-MDA5 autoantibodies (28.6% vs 0.0%, p = 0.032). Heavy exercise was more frequent in MSA-negative and anti-MDA5 groups compared to the anti-TIF-1 autoantibody group (42.9 and 35.3% vs. 9.0%, p ≤ 0.047). Medications received within 1 year of diagnosis were more frequent in MSA-negative patients relative to those with anti-MDA5 autoantibodies (92.9% vs. 52.8% p = 0.045). Being breastfed > 6 months was more frequent in MSA-negative patients (88.9%) compared to anti-TIF-1 and anti-MDA5 autoantibody groups (41.2 and 28.6%, p ≤ 0.036). CONCLUSIONS: Certain environmental exposures prior to diagnosis differed among clinical and serologic subgroups of JIIM, suggesting additional exposures to be explored as possible risk factors for JIIM phenotypes.


Assuntos
Dermatomiosite , Miosite , Autoanticorpos , Dermatomiosite/epidemiologia , Dermatomiosite/etiologia , Humanos , Miosite/epidemiologia , Miosite/etiologia , Fenótipo , Fatores de Risco
6.
Oncologist ; 27(4): e353-e356, 2022 Apr 05.
Artigo em Inglês | MEDLINE | ID: mdl-35380721

RESUMO

Immune checkpoint inhibitors (ICIs) have revolutionized the treatment of advanced cancers. However, activation of the immune system can occasionally cause life-threatening toxicity involving critical organs. Induction of immune-mediated toxicity is a significant concern for patients with thymic epithelial tumors (TETs) due to defects in immune tolerance. An increased risk of skeletal and cardiac muscle inflammation following treatment with ICIs is well recognized in patients with advanced TETs. However, uncommon musculoskeletal and rheumatic complications can also occur. The cases presented in this report highlight the spectrum of presentation of immune-mediated, joint-predominant musculoskeletal adverse events in patients with advanced TETs treated with ICIs, including polymyalgia rheumatica-like illness and inflammatory arthritis.


Assuntos
Miosite , Neoplasias Epiteliais e Glandulares , Neoplasias , Polimialgia Reumática , Neoplasias do Timo , Humanos , Inibidores de Checkpoint Imunológico , Imunoterapia/efeitos adversos , Miosite/induzido quimicamente , Neoplasias/tratamento farmacológico , Neoplasias Epiteliais e Glandulares/tratamento farmacológico , Polimialgia Reumática/tratamento farmacológico , Polimialgia Reumática/etiologia , Neoplasias do Timo/tratamento farmacológico
7.
J Med Case Rep ; 16(1): 132, 2022 Apr 04.
Artigo em Inglês | MEDLINE | ID: mdl-35369881

RESUMO

BACKGROUND: Overlap syndromes account for about 25% of autoimmune diseases. They are many possible combinations of known autoimmune diseases increasingly diagnosed with the identification of of a large number of autoantibodies. In this case report, we present a patient with rare overlapping rheumatoid arthritis-antisynthetase syndrome with associated secondary Sjögren's syndrome atypically presenting without interstitial lung disease. CASE PRESENTATION: A 52-year-old Sinhalese female, a known patient with type 2 diabetes mellitus, presented with a history of symmetrical inflammatory-type polyarthritis with significant morning stiffness, proximal muscle weakness, pain, and roughening of the fingertips with associated sicca symptoms of 5 months duration. Examination revealed features of active joint inflammation, mechanic's hand, xerostomia, and left-sided breast lump. Investigations confirmed the presence of rheumatoid arthritis with strongly positive rheumatoid factor (202 U/ml) and anti-cyclic citrullinated peptide antibody (717 U/ml). Antisynthetase syndrome was also diagnosed with borderline-positive anti-aminoacyl-tRNA antibodies but without interstitial lung disease. Sjögren's syndrome was confirmed by the clinical history and histology and considered a secondary disorder. As her breast lump proved to be benign, no further interventions were done. She was started on sulfasalazine and methotrexate with steroid bridging therapy and achieved remission and had good control of the disease without any joint deformity or flare-up on 6-month clinic follow-up. DISCUSSION: Overlapping rheumatoid arthritis-antisynthetase syndrome is a very rare disease with disabling complications. Early identification of the atypical presentations of the overlap syndromes, by thorough investigations, helps physicians to prescribe proper disease-modifying antirheumatoid drugs and biological drugs. It also helps predict the prognosis of the patients before they develop complications.


Assuntos
Artrite Reumatoide , Diabetes Mellitus Tipo 2 , Miosite , Síndrome de Sjogren , Artrite Reumatoide/complicações , Artrite Reumatoide/diagnóstico , Artrite Reumatoide/tratamento farmacológico , Diabetes Mellitus Tipo 2/complicações , Feminino , Humanos , Pessoa de Meia-Idade , Miosite/complicações , Miosite/diagnóstico , Miosite/tratamento farmacológico , Síndrome de Sjogren/complicações , Síndrome de Sjogren/diagnóstico , Síndrome de Sjogren/tratamento farmacológico
8.
Front Immunol ; 13: 756018, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35371068

RESUMO

The idiopathic inflammatory myopathies (IIM) are a rare clinically heterogeneous group of conditions affecting the skin, muscle, joint, and lung in various combinations. While myositis specific autoantibodies are well described, we postulate that broader immune endotypes exist in IIM spanning B cell, T cell, and monocyte compartments. This study aims to identify immune endotypes through detailed immunophenotyping of peripheral blood mononuclear cells (PBMCs) in IIM patients compared to healthy controls. We collected PBMCs from 17 patients with a clinical diagnosis of inflammatory myositis and characterized the B, T, and myeloid cell subsets using mass cytometry by time of flight (CyTOF). Data were analyzed using a combination of the dimensionality reduction algorithm t-distributed stochastic neighbor embedding (t-SNE), cluster identification, characterization, and regression (CITRUS), and marker enrichment modeling (MEM); supervised biaxial gating validated populations identified by these methods to be differentially abundant between groups. Using these approaches, we identified shared immunologic features across all IIM patients, despite different clinical features, as well as two distinct immune endotypes. All IIM patients had decreased surface expression of RP105/CD180 on B cells and a reduction in circulating CD3+CXCR3+ subsets relative to healthy controls. One IIM endotype featured CXCR4 upregulation across all cellular compartments. The second endotype was hallmarked by an increased frequency of CD19+CD21loCD11c+ and CD3+CD4+PD1+ subsets. The experimental and analytical methods we describe here are broadly applicable to studying other immune-mediated diseases (e.g., autoimmunity, immunodeficiency) or protective immune responses (e.g., infection, vaccination).


Assuntos
Leucócitos Mononucleares , Miosite , Autoanticorpos , Humanos , Imunofenotipagem , Monócitos
9.
Arch. argent. pediatr ; 120(2): e93-e97, abril 2022. ilus
Artigo em Espanhol | LILACS, BINACIS | ID: biblio-1363988

RESUMO

La miositis de origen vírico o bacteriano es frecuente en la edad pediátrica. Causa dolor muscular y debilidad, con fiebre y malestar general. Una causa es la infección por Bartonella henselae, bacteria implicada en la enfermedad por arañazo de gato que, a veces, causa afectación multisistémica. Se presenta el caso de una adolescente que acudió al servicio de urgencias por mialgia intensa, malestar, adelgazamiento y esplenomegalia. En el labortorio se observaron parámetros inflamatorios elevados. Refería contacto con un gato. Entre los estudios realizados, la resonancia magnética (RM) de miembros inferiores mostró una imagen compatible con miositis inflamatoria bilateral. En la RM abdominal, se observaron tres lesiones esplénicas no detectadas previamente y el fondo de ojo mostraba una lesión compatible con oclusión arterial retiniana o vasculitis. Se indicó tratamiento antibiótico por vía intravenosa durante 21 días con cefotaxima y cloxacilina, tras los cuales desaparecieron los signos y síntomas, aunque los reactantes inflamatorios persistieron elevados. Con base en el cuadro clínico (miositis + coriorretinitis + absceso esplénico) se pensó en una posible infección por B. henselae y se inició tratamiento oral con azitromicina y rifampicina durante 14 días. Luego del tratamiento, los valores de laboratorio fueron normales, así como la RM de control, y se constató una IgG positiva para la bacteria


Infectious myositis, whether viral or bacterial, is frequent in pediatric age. It causes muscle pain and weakness, associated with fever and general malaise. One cause is Bartonella henselae, responsible for cat scratch disease, which sometimes causes systemic symptoms. We report the case of an adolescent who came to the emergency room with intense myalgia, malaise, weight loss and splenomegaly. Blood tests showed high inflammatory markers. She had been in touch with a cat. Studies were carried out including: lower limbs MRI suggestive of bilateral inflammatory myositis, abdominal MRI with three previously undetected splenic lesions and dilated fundus examination that showed possible retinal arterial occlusion or vasculitis. After 21 days of intravenous antibiotic therapy (cefotaxime + cloxaciline), she became asymptomatic, but inflammatory markers remained high. Suspecting Bartonella henselaeinfection (myositis + chorioretinitis + splenic abscess), oral azithromycin and rifampicin were prescribed for 14 days. Blood tests and control MRI became normal, and IgG was positive.


Assuntos
Humanos , Feminino , Adolescente , Esplenopatias/complicações , Esplenopatias/microbiologia , Vasculite , Doença da Arranhadura de Gato/complicações , Doença da Arranhadura de Gato/diagnóstico , Doença da Arranhadura de Gato/tratamento farmacológico , Bartonella henselae , Miosite/diagnóstico , Miosite/etiologia
10.
Rheum Dis Clin North Am ; 48(2): 411-428, 2022 May.
Artigo em Inglês | MEDLINE | ID: mdl-35400368

RESUMO

Immune checkpoint inhibitors activate the immune system to combat cancer. In doing so, however, they can cause immune-related adverse events (irAEs), including rheumatic syndromes, such as inflammatory arthritis, polymyalgia rheumatica, and myositis. This article reviews rheumatic irAEs that may be encountered in the general medicine practice and provides guidance to support prompt recognition, referral, and treatment of these patients.


Assuntos
Miosite , Neoplasias , Polimialgia Reumática , Doenças Reumáticas , Humanos , Inibidores de Checkpoint Imunológico/efeitos adversos , Miosite/induzido quimicamente , Neoplasias/tratamento farmacológico , Polimialgia Reumática/induzido quimicamente , Polimialgia Reumática/tratamento farmacológico , Doenças Reumáticas/terapia
11.
Front Immunol ; 13: 855408, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35401576

RESUMO

Dermatomyositis (DM) is an idiopathic inflammatory myopathy primarily involving skin and muscles. Clinically amyopathic dermatomyositis (CADM), a subset of DM, presents with characteristic cutaneous manifestations without clinical evidence of myositis. Although rare, vesiculobullous eruptions could develop in DM patients. Such "bullous DM" is commonly considered a sign of internal malignancy. However, some cases with similar presentations were diagnosed as autoimmune blistering disease eventually. Herein, we reported two cases of CADM with autoimmune blisters formed. Case 1 presented with vesicles and was diagnosed with CADM initially. However, this patient developed blisters again years later and was diagnosed with "pemphigus foliaceous" (PF) accordingly. Case 2, with a history of nasopharyngeal carcinoma and CADM, developed bullous pemphigoid several days after using a heat patch on her abdomen. The association between disease occurrence and local skin damage might provide more evidence to support the "epitope spreading" hypothesis. Moreover, we reviewed related literature and discussed the differences between the two disease entities in clinical presentations, pathogenesis, therapy, and the risk of complications.


Assuntos
Doenças Autoimunes , Dermatomiosite , Miosite , Doenças Autoimunes/complicações , Doenças Autoimunes/diagnóstico , Doenças Autoimunes/patologia , Vesícula/complicações , Vesícula/diagnóstico , Dermatomiosite/complicações , Dermatomiosite/diagnóstico , Dermatomiosite/tratamento farmacológico , Feminino , Humanos , Miosite/patologia , Pele/patologia
12.
Biochem Biophys Res Commun ; 603: 29-34, 2022 05 07.
Artigo em Inglês | MEDLINE | ID: mdl-35276460

RESUMO

Idiopathic inflammatory myopathy (IIM) is an autoimmune disease that invades skeletal muscle; however, the etiology of IIM is still poorly understood. Toll-like receptor (TLR) 4 has been widely reported to take part in the autoimmune inflammation of IIMs. The mammalian target of rapamycin, mTOR, is a key central substance which mediates immune responses and metabolic changes, and also has been confirmed to be involved in the pathogenesis of IIMs. However, the interconnectedness between TLR4 and mTOR in IIM inflammation has not been fully elucidated. We hypothesized that TLR4 may play an important role in IIM inflammatory muscle injury by regulating mTOR. Mice were divided into four groups: a normal control group, IIM animal model (experimental autoimmune myositis, EAM) group, TAK242 intervention group and rapamycin (RAPA) intervention group. The results of EAM mice showed that TLR4, mTOR, nuclear factor-kappa B (NF-κB) and inflammatory factors interleukin-17A (IL-17A) and interferon γ (IFN-γ) mRNA levels were significantly upregulated. These factors were positively correlated with the degree of muscle inflammatory injury. When EAM mice were given the antagonist TAK242 to inhibit the TLR4 pathway, the results demonstrated that both mTOR and NF-κB were downregulated in the muscle of the mice. Muscle staining showed that the inflammatory injury was alleviated and the EAM mouse muscle strength was improved. Then, RAPA was used to inhibit the mTOR pathway, and the inflammatory factors IL-17A and IFN-γ were downregulated in EAM mouse muscle and serum. Consistently, muscle inflammatory injury was significantly reduced, and muscle strength was significantly improved. Our results suggest that TLR4 may regulate inflammatory muscle injury in EAM by activating the mTOR and NF-κB pathways, which provides both an experimental complement for the pathological mechanism of IIM and an encouraging target for the selection of effective treatments.


Assuntos
Miosite , Doença Autoimune do Sistema Nervoso Experimental , Serina-Treonina Quinases TOR , Receptor 4 Toll-Like , Animais , Interferon gama/metabolismo , Interleucina-17/metabolismo , Mamíferos/metabolismo , Camundongos , Músculo Esquelético/metabolismo , Miosite/metabolismo , NF-kappa B/metabolismo , Serina-Treonina Quinases TOR/metabolismo , Receptor 4 Toll-Like/metabolismo
13.
Front Immunol ; 13: 845988, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35320936

RESUMO

Background: Interstitial lung disease (ILD) is frequently observed in anti-melanoma differentiation-associated protein 5 (MDA5) antibody positive dermatomyositis (DM) and anti-synthetase syndrome (ASS), where they often develop a rapidly progressive ILD (RP-ILD) leading to poor prognosis. Objective: The aim of this study was to construct multivariable prediction risk factors for rapid progressive ILD (RP-ILD) in anti-MDA5 positive DM (MDA5+DM) and ASS. Methods: 333 idiopathic inflammatory myopathy (IIM) associated ILD patients were studied retrospectively. Risk factors for RP-ILD in MDA5+DM and ASS patients were identified by univariate and multivariable logistic regression analysis. The mortality was assessed using Kaplan-Meier analysis. Results: RP-ILD was more prevalent in MDA5+DM patients than ASS patients. MDA5+DM patients with RP-ILD had significantly lower survival rates than those in ASS patients. The independent risk factors for RP-ILD in MDA5+DM patients were fever (OR 3.67, 95% CI:1.79-7.52), lymphopenia (OR 2.14, 95% CI:1.01-4.53), especially decreased levels of CD3+T cells (OR 2.56, 95% CI:1.17-5.61), decreased levels of CD3+CD4+ T cells (OR 2.80, 95% CI:1.37-5.73), CD3+CD8+T cells (OR 2.18, 95% CI:1.05-4.50), elevated CD5-CD19+ B cells (OR 3.17, 95% CI:1.41-7.13), elevated ALT (OR 2.36, 95% CI:1.15-4.81), high lactate dehydrogenase (LDH) (OR 3.08, 95% CI:1.52-6.27), hyper-ferritin (OR 4.97, 95% CI:1.97-12.50), elevated CEA (OR 2.28, 95% CI:1.13-4.59), and elevated CA153 (OR 3.31, 95% CI:1.50-7.27). While the independent risk factors for RP-ILD in ASS patients were elevated CEA (OR 5.25, 95% CI: 1.73-15.93), CA125 (OR 2.79, 95% CI: 1.10-7.11) and NSE (OR 4.86, 95% CI: 1.44-16.37). Importantly, serum ferritin>2200ng/ml predicted patient's death within half a year in MDA5+DM patients with RP-ILD, but not in ASS patients. Conclusions: There were significant different mortality and multivariable risk factors for RP-ILD in MDA5+DM patients and ASS patients. Potential clinical benefits of using these different risk factors deserve assessment of severity and prognosis in IIM patients.


Assuntos
Dermatomiosite , Doenças Pulmonares Intersticiais , Miosite , Autoanticorpos , Antígeno Carcinoembrionário , Progressão da Doença , Ferritinas , Humanos , Ligases , Doenças Pulmonares Intersticiais/etiologia , Miosite/complicações , Estudos Retrospectivos , Fatores de Risco , Síndrome
14.
Tohoku J Exp Med ; 256(4): 303-308, 2022 Apr 20.
Artigo em Inglês | MEDLINE | ID: mdl-35296571

RESUMO

Myositis-specific autoantibodies are relevant factors that define the disease phenotype of dermatomyositis (DM). Anti-Mi-2 antibody-positive DM patients may present with the typical skin lesions and prominent myositis. On the other hand, adult DM patients with anti-TIF-γ antibody seem to be associated with internal malignancy. Here, we report a rare case of juvenile dermatomyositis (JDM) exhibiting anti-Mi-2 and anti-transcriptional intermediary factor-1 gamma (TIF1-γ) antibodies, with no internal malignancy. A 16-year-old female Japanese patient under treatment with a 2-year history of chronic eczematous lesions was admitted to our department with elevated levels of muscle enzymes. Characteristic skin changes, such as Gottron's papules of the hand, heliotrope rash of the eyelids, and poikiloderma-like legions and diffuse pigmentation on the back, were observed. Histologically, the patient's skin was characterized by the presence of lymphocytic vascular inflammation and endothelial swelling, which are consistent with DM. Severe symmetric proximal muscle weakness, elevated serum muscle enzymes and the presence of anti-TIF1-γ and Mi-2 antibodies were noted. The diagnosis of JDM was made according to the European League Against Rheumatism (EULAR) diagnostic criteria. A high dose of corticosteroids and following intravenous cyclophosphamide treatment (750 mg three times) resulted in an improvement in clinical manifestations and functional outcomes, and recurrence did not occur. Estimation of autoantibodies may serve as an ancillary tool in delineating and defining distinct clinical phenotypes in JDM.


Assuntos
Dermatomiosite , Eczema , Miosite , Neoplasias , Autoanticorpos , Dermatomiosite/complicações , Dermatomiosite/diagnóstico , Dermatomiosite/tratamento farmacológico , Eczema/complicações , Eczema/diagnóstico , Eczema/tratamento farmacológico , Feminino , Humanos , Miosite/complicações
15.
Immunotherapy ; 14(7): 511-520, 2022 May.
Artigo em Inglês | MEDLINE | ID: mdl-35321560

RESUMO

Immune checkpoint inhibitors have been incorporated into the treatment of various malignancies. An increasing body of literature is reporting rare but potentially fatal adverse events associated with these agents. In this case series, the authors report the clinical features and outcomes of seven patients who received immune checkpoint inhibitors for different solid organ malignancies and developed a tetrad of immune-related myocarditis, myositis, myasthenia gravis and transaminitis. Herein the authors review the literature and describe the current diagnostic and management approach for this overlapping syndrome. The authors' series highlights the importance of a high index of clinical suspicion, prompt comprehensive investigations, early multidisciplinary team involvement and initiation of immunosuppressive therapy when immune-related adverse events are suspected.


Cancer immunotherapy is used in the treatment of different cancer types. Immunotherapy activates the immune system to detect and attack cancer cells, but side effects may arise from the immune system inadvertently attacking normal tissues and organs. The increased use of immunotherapy has led to an increase in the reporting of rare but potentially life-threatening treatment-related side effects. In this case series, the authors report the clinical features and outcomes of seven patients who developed inflammation of the heart, muscles, nerve and muscle junctions and liver following treatment with immunotherapy. The authors review the scientific literature and discuss the current understanding of and management approach to this rare syndrome. The authors' report highlights the importance of a high degree of clinical suspicion, prompt comprehensive testing to confirm diagnosis, early involvement of experts from different specialties and early initiation of treatment in the management of this unique syndrome.


Assuntos
Miastenia Gravis , Miocardite , Miosite , Neoplasias , Humanos , Inibidores de Checkpoint Imunológico/efeitos adversos , Miastenia Gravis/induzido quimicamente , Miocardite/induzido quimicamente , Miocardite/diagnóstico , Miocardite/tratamento farmacológico , Miosite/induzido quimicamente , Miosite/diagnóstico , Neoplasias/tratamento farmacológico
16.
Rinsho Shinkeigaku ; 62(4): 267-271, 2022 Apr 27.
Artigo em Japonês | MEDLINE | ID: mdl-35354723

RESUMO

BACKGROUND: Intravenous immunoglobulin (IVIg) have been administrated for the long time in patients with several autoimmune neuromuscular diseases. Eczematous eruption has been described as IVIg-induced adverse effect. OBJECTIVE: The purpose of this study is to clarify the incidence and characteristic of IVIg-induced eczematous eruption in autoimmune neuromuscular disease. METHODS: We retrospectively collected the data from 92 patients with autoimmune neuromuscular diseases, including 35 patients with chronic inflammatory demyelinating polyneuropathy (CIDP), 8 patients with multifocal motor neuropathy (MMN), 25 patients with myositis, 15 patients with Guillain-Barré syndrome (GBS), and 9 patients with myasthenia gravis (MG), who have administrated IVIg in Yamaguchi University Hospital. RESULTS: There are 10 patients (6 CIDP/4 MMN), who had an eczematous skin reaction after IVIg infusion. The frequencies of IVIg-induced eczematous eruption were significantly higher in patients with multifocal acquired demyelinating sensory and motor (MADSAM) and MMN than in patients with GBS, myositis, and MG. In addition, corticosteroids or immunosuppressive drugs had been administrated before IVIg treatment more frequently in patients with myositis and MG than in those with MADSAM and MMN. CONCLUSION: MADSAM or MMN patients had more frequently IVIg-induced eczematous eruption than other autoimmune neuromuscular diseases. Pathophysiology of MADAM and MMN is considered to be cell-mediated immunity against the peripheral nerve and the accumulation of IgG in both epidermis and dermis of the hand after IVIg may induce the infiltration of inflammatory cells around the vessels in the skin, causing eczematous eruption in MADSAM and MMN.


Assuntos
Doenças Autoimunes , Exantema , Síndrome de Guillain-Barré , Miosite , Doenças Neuromusculares , Polineuropatias , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica , Doenças Autoimunes/tratamento farmacológico , Exantema/tratamento farmacológico , Síndrome de Guillain-Barré/tratamento farmacológico , Humanos , Imunoglobulinas Intravenosas/efeitos adversos , Miosite/tratamento farmacológico , Doenças Neuromusculares/tratamento farmacológico , Polineuropatias/tratamento farmacológico , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/terapia , Estudos Retrospectivos
17.
J Investig Med High Impact Case Rep ; 10: 23247096221074589, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35264047

RESUMO

Idiopathic inflammatory myopathies (IIMs) are a rare, heterogeneous group of diseases with a characteristic clinical presentation consisting of muscle inflammation and weakness. They often present with accompanying extra-muscular findings, most notably in the skin, lungs, and joints. Inflammatory myopathies are also identified by their characteristic laboratory abnormalities, including a 10- to 50-fold increase in creatinine kinase, elevated liver enzymes, and characteristic electromyography and magnetic resonance imaging findings. Distinct autoimmune markers and clinical phenotypes have advanced our understanding of IIMs and have led to the recognition of 5 distinct entities, each with its unique pathophysiology, autoimmune markers, and clinical features. While autoimmune panels and muscle biopsies help clinicians distinguish one entity from the other, their sensitivity and specificity vary. Of the various inflammatory myopathies, polymyositis remains the most elusive. Often, the diagnosis is ultimately made by combining clinical findings and laboratory data. As our case report illustrates, clinicians must use this constellation of data to initiate treatment for suspected polymyositis despite negative autoimmune panels and negative muscle biopsy.


Assuntos
Transtornos de Deglutição , Miosite , Polimiosite , Rabdomiólise , Autoanticorpos , Transtornos de Deglutição/etiologia , Humanos , Miosite/complicações , Miosite/diagnóstico , Polimiosite/complicações , Polimiosite/diagnóstico , Rabdomiólise/complicações , Rabdomiólise/diagnóstico
18.
Front Immunol ; 13: 825799, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35281002

RESUMO

Inflammatory rheumatic diseases (IRD) and autoimmune liver diseases (AILD) share many similarities regarding epidemiology, genetics, immunology and therapeutic regimens, so it is not surprising that approximately 20% of patients with AILD are diagnosed with an IRD as well. Clinical features and biochemical hallmarks of IRD and AILD often intertwine and cross diagnostic criteria. Therefore, the real distinction of underlying disorders in a patient with these comorbidities may be challenging. The present report is the first report of simultaneously developed juvenile dermatomyositis (JDM) and autoimmune sclerosing cholangitis (ASC) with both entities fulfilling the latest guidelines for a definite diagnosis. Both of these diagnoses are difficult to definitely establish since ASC has a similar serologic profile as autoimmune hepatitis and liver histological analysis is frequently non-specific, whereas clinically amyopathic JDM diagnosis depends mostly on classical dermatological symptoms, while the rest of the diagnostic criteria, including the necessity for skin or muscle biopsy and the presence of myositis specific antibodies, are still not uniformed. In spite of these challenges, our patient clearly met European League Against Rheumatism/American College of Rheumatology classification criteria for CAJDM and The European Society for Pediatric Gastroenterology, Hepatology and Nutrition diagnostic criteria for ASC. Since elevated serum transaminases, the presence of serum antinuclear antibodies and hypergammaglobulinemia could be explained as a part of both JDM and ASC, the underlying pathophysiology remains debatable. Intriguingly, JDM and ASC share genetic predisposition including human leukocyte antigen allele DRB1*0301 and tumor necrosis factor α 308A allele. Furthermore, both humoral and cellular components of the adaptive immune system contribute to the pathogenesis of JDM and ASC. Moreover, recent findings indicate that the loss of the CD28 expression on T-cells plays a significant role in their pathogenesis along with the Th17 immune pathway. Despite these common features that suggest shared autoimmunity, AILD and autoimmune myositis are traditionally studied and managed independently. The lack of therapies that target the underlying cause results in a high rate of adverse events due to unspecific immunosuppressive therapy. Shared autoimmunity is an ideal area to develop new, targeted immunotherapy that would hopefully be beneficial for more than one disease.


Assuntos
Colangite Esclerosante , Dermatomiosite , Hepatite Autoimune , Miosite , Criança , Colangite Esclerosante/diagnóstico , Dermatomiosite/diagnóstico , Cadeias HLA-DRB1/genética , Humanos
19.
Lupus Sci Med ; 9(1)2022 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-35351810

RESUMO

OBJECTIVE: The objectives of this study were to compare the clinical features of patients with SLE with and without myopathy and to describe the muscle biopsy features of patients with SLE myopathy. METHODS: This nested case-control study included all subjects enrolled in the Hopkins Lupus Cohort database from May 1987 to June 2016. Subjects with elevated creatine kinase along with evidence of muscle oedema on MRI, myopathic electromyography and/or myopathic muscle biopsy features were defined as having SLE myopathy. Demographic, serological and clinical features were compared between patients with SLE with and without myopathy. Muscle biopsies were histologically classified as polymyositis, dermatomyositis, necrotising myopathy or non-specific myositis. RESULTS: From among 2437 patients with SLE, 179 (7.3%) had myopathy. African American patients were more likely to develop myositis than Caucasian patients (p<0.0001). Compared with those without myopathy, patients with SLE myopathy were more likely to have malar rash (OR 1.67, 1.22-2.29), photosensitivity (OR 1.43, 1.04-1.96), arthritis (OR 1.81, 1.21-2.69), pleurisy (OR 1.77, 1.3-2.42), pericarditis (OR 1.49, 1.06-2.08), acute confusional state (OR 2.07, 1.09-3.94), lymphopaenia (OR 1.64, 1.2-2.24), anti-double-stranded DNA antibodies (OR 1.52, 1.09-2.13), lupus anticoagulant (OR 1.42, 1-2), cognitive impairment (OR 1.87, 1.12-3.13), cataract (OR 1.5, 1.04-2.18), pulmonary hypertension (OR 1.98, 1.13-3.47), pleural fibrosis (OR 2.01, 1.27-3.18), premature gonadal failure (OR 1.9, 1.05-3.43), diabetes (OR 1.92, 1.22-3.02) or hypertension (OR 1.45, 1.06-2). Among 16 muscle biopsies available for review, the most common histological classifications were necrotising myositis (50%) and dermatomyositis (38%). CONCLUSIONS: Patients with SLE myopathy have a higher prevalence of numerous SLE disease manifestations. Necrotising myopathy and dermatomyositis are the most prevalent histopathological features in SLE myopathy.


Assuntos
Dermatomiosite , Lúpus Eritematoso Sistêmico , Doenças Musculares , Miosite , Estudos de Casos e Controles , Dermatomiosite/complicações , Dermatomiosite/epidemiologia , Humanos , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/epidemiologia , Miosite/complicações , Miosite/epidemiologia , Miosite/patologia
20.
Arch Argent Pediatr ; 120(2): e93-e97, 2022 Apr.
Artigo em Espanhol | MEDLINE | ID: mdl-35338824

RESUMO

Infectious myositis, whether viral or bacterial, is frequent in pediatric age. It causes muscle pain and weakness, associated with fever and general malaise. One cause is Bartonella henselae, responsible for cat scratch disease, which sometimes causes systemic symptoms. We report the case of an adolescent who came to the emergency room with intense myalgia, malaise, weight loss and splenomegaly. Blood tests showed high inflammatory markers. She had been in touch with a cat. Studies were carried out including: lower limbs MRI suggestive of bilateral inflammatory myositis, abdominal MRI with three previously undetected splenic lesions and dilated fundus examination that showed possible retinal arterial occlusion or vasculitis. After 21 days of intravenous antibiotic therapy (cefotaxime + cloxaciline), she became asymptomatic, but inflammatory markers remained high. Suspecting Bartonella henselae infection (myositis + chorioretinitis + splenic abscess), oral azithromycin and rifampicin were prescribed for 14 days. Blood tests and control MRI became normal, and IgG was positive.


La miositis de origen vírico o bacteriano es frecuente en la edad pediátrica. Causa dolor muscular y debilidad, con fiebre y malestar general. Una causa es la infección por Bartonella henselae, bacteria implicada en la enfermedad por arañazo de gato que, a veces, causa afectación multisistémica. Se presenta el caso de una adolescente que acudió al servicio de urgencias por mialgia intensa, malestar, adelgazamiento y esplenomegalia. En el labortorio se observaron parámetros inflamatorios elevados. Refería contacto con un gato. Entre los estudios realizados, la resonancia magnética (RM) de miembros inferiores mostró una imagen compatible con miositis inflamatoria bilateral. En la RM abdominal, se observaron tres lesiones esplénicas no detectadas previamente y el fondo de ojo mostraba una lesión compatible con oclusión arterial retiniana o vasculitis. Se indicó tratamiento antibiótico por vía intravenosa durante 21 días con cefotaxima y cloxacilina, tras los cuales desaparecieron los signos y síntomas, aunque los reactantes inflamatorios persistieron elevados. Con base en el cuadro clínico (miositis + coriorretinitis + absceso esplénico) se pensó en una posible infección por B. henselae y se inició tratamiento oral con azitromicina y rifampicina durante 14 días. Luego del tratamiento, los valores de laboratorio fueron normales, así como la RM de control, y se constató una IgG positiva para la bacteria.


Assuntos
Bartonella henselae , Doença da Arranhadura de Gato , Miosite , Esplenopatias , Vasculite , Adolescente , Doença da Arranhadura de Gato/complicações , Doença da Arranhadura de Gato/diagnóstico , Doença da Arranhadura de Gato/tratamento farmacológico , Criança , Feminino , Humanos , Miosite/diagnóstico , Miosite/etiologia , Esplenopatias/complicações , Esplenopatias/microbiologia
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