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1.
Einstein (Sao Paulo) ; 18: eAO5105, 2020.
Artigo em Inglês, Português | MEDLINE | ID: mdl-32159607

RESUMO

OBJECTIVE: To evaluate the density of anti-galectin-3-immunostained cells, collagen percentage, mast cell density and presence of pathological processes in intestinal muscle biopsies of patients. METHODS: Thirty-five patients who underwent intestinal biopsy were selected from 1997 to 2015. Patients were divided into three groups: chagasic patients with mucosal lesion (n=13), chagasic patients with intact mucosa (n=12) and non-chagasic patients with no mucosal lesion (n=10). Histological processing of the biopsied fragments and immunohistochemistry for galectin-3 were performed. Additional sections were stained with hematoxylin and eosin to evaluate the general pathological processes, picrosirius for evaluation of collagen and toluidine blue to evaluate the mast cell density. RESULTS: Patients of mucosal lesion group had a significantly higher frequency of ganglionitis and myositis when compared to the chagasic patients with intact mucosa and non-chagasic group. The density of anti-galectin-3-immunostained cells was significantly higher in the chagasic patients with intact mucosa group when compared to the non-chagasic group. The group of chagasic patients with intact mucosa presented a higher percentage of collagen in relation to the patients with mucosal lesion and to the non-chagasic group, with a significant difference. There was no significant difference in mast cell density among the three groups. CONCLUSION: The higher density of anti-galectin-3-immunostained cells in patients in the chagasic patients with intact mucosa group suggested the need for greater attention in clinical evaluation of these patients, since this protein is associated with neoplastic transformation and progression.


Assuntos
Anticorpos Monoclonais/análise , Doença de Chagas/patologia , Colonoscopia/métodos , Galectina 3/análise , Mucosa Intestinal/patologia , Megacolo/patologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Análise de Variância , Biópsia , Estudos de Casos e Controles , Contagem de Células , Colágeno/análise , Feminino , Fibrose , Galectina 3/imunologia , Humanos , Imuno-Histoquímica , Masculino , Mastócitos/patologia , Pessoa de Meia-Idade , Miosite/patologia , Estudos Retrospectivos , Estatísticas não Paramétricas
2.
Rev Med Suisse ; 16(685): 487-491, 2020 Mar 11.
Artigo em Francês | MEDLINE | ID: mdl-32167250

RESUMO

Targeted therapies are nowadays commonly used in connective tissue diseases and vasculitis. Experts recommend the use of belimumab and rituximab in refractory and/or severe cases of lupus. Rituximab can be also considered in difficult to treat cases of Sjögren's disease or myositis. Nintedanib seems a very promising weapon in the management of systemic sclerosis-associated pulmonary fibrosis. Regarding vasculitis, rituximab has become the preferred treatment for granulomatosis with polyangiitis and microscopic polyangiitis. Finally, tocilizumab is recommended as a steroid-sparing agent in giant cell arteritis. Further clinical trials are warranted to study the efficacy of other targeted therapies in connective tissue diseases and vasculitis.


Assuntos
Anticorpos Monoclonais Humanizados/uso terapêutico , Doenças do Tecido Conjuntivo/tratamento farmacológico , Rituximab/uso terapêutico , Vasculite/tratamento farmacológico , Arterite de Células Gigantes/tratamento farmacológico , Humanos , Miosite/tratamento farmacológico , Síndrome de Sjogren/tratamento farmacológico
3.
Rev Med Suisse ; 16(685): 504-507, 2020 Mar 11.
Artigo em Francês | MEDLINE | ID: mdl-32167253

RESUMO

Cancer management has been revolutionised by immune checkpoint inhibitors. Their use and indications increase in parallel with the recognition of their various side effects. Arthritis, myositis, and vasculitis are among the most common rheumatologic immune-related adverse events (irAE) of immunotherapy. Rheumatological irAEs can be of late onset, occur even after the cessation of the culprit drug and persist into time. In this article we discuss the principles of cancer immunotherapy, clinical manifestations and management of the most common rheumatologic irAEs, and aspects of immune checkpoint inhibitors therapy in patients with pre-existing autoimmune disease.


Assuntos
Imunoterapia/efeitos adversos , Neoplasias/tratamento farmacológico , Neoplasias/imunologia , Doenças Reumáticas/induzido quimicamente , Artrite/induzido quimicamente , Artrite/complicações , Artrite/terapia , Humanos , Miosite/induzido quimicamente , Miosite/complicações , Miosite/terapia , Neoplasias/complicações , Doenças Reumáticas/complicações , Doenças Reumáticas/terapia
4.
Nat Rev Rheumatol ; 16(4): 187, 2020 04.
Artigo em Inglês | MEDLINE | ID: mdl-32051593
5.
Clin Exp Rheumatol ; 38(1): 1-10, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32041680

RESUMO

The idiopathic inflammatory myopathies (IIMs) are a rare group of immune, systemic diseases characterised by muscle inflammation and frequently by extramuscular involvement. IIMs are heterogeneous with generally a chronic or subacute onset, which vary from less severe to more serious manifestations, not always easy to diagnose and even less to manage. In the past year, many studies have been published in order to clarify disease pathogenesis and improve patient management and treatment.The purpose of this review article is to provide an overview of the new insights in pathogenesis, serological findings, clinical manifestations and treatment of IIMs, summarising the most relevant studies published over the last year.


Assuntos
Miosite , Humanos , Miosite/diagnóstico , Miosite/patologia , Miosite/terapia
6.
Autoimmun Rev ; 19(4): 102498, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-32062029

RESUMO

Myastenia-Inflammatory Myopathy (MG-IM) association has been described in less than 50 cases, as isolated reports or in few case series. In most cases, MG and IM onset occur simultaneously even if the overlapping clinical manifestations could lead to delay the diagnosis in the early stage of disease. In these cases, thymic pathology is present in more than 50% of cases. Pathological findings can be consistent of polymyositis (63%), dermatomyositis (25%) or granulomatosis (12%). Accurate clinical manifestations and severity of IM in MG, including muscle specific antibodies (MSA) and muscle MRI, have not been systematically investigated and focal or mild subclinical myositis have not been reported. We observed that focal myositis or asymptomatic CK elevation can also occur in MG. In this review we have also retrospectively re-analyzed the clinical, serological, pathological and muscle imaging data from 13 patients with MG- IM from our cohort of 441 MG patients (2,9%). Clinical onset occurred simultaneously in 10/13 patients, whereas in 2 patients the IM appeared later in MG disease course (range 10-14 years) and conversely in 1 patient MG symptoms occurred later in IM disease course (4 years). Median age at disease onset was 51 year (range 24-73 years) regardless of clinical onset (MG or IM). Median clinical follow-up was 88 months (range 31-237 months). IM was suspected by CK elevation in all patients (ranging 800-3000 UI/L at first detection) and non-fatigable muscle weakness unresponsive to acetylcholinesterase inhibitors. All the patients presented mild to moderate MG symptoms. Three main categories of muscle involvement, sometimes overlapping, were recognizable: distal, proximal and subclinical myositits, leading to three main clinical groups (A,B,C) and two overlapping subgroups (A/B and B/C). Thymus pathology was present in 10/13 patients. Anti-AChR was detected in al all patients associated with anti-Titin and -RyR1 in those patients with thymoma. No MSA, nor MAA antibodies were detected. Muscle biopsy confirmed IM in all patients. In conclusion we redefined the clinical spectrum of muscle involvement in MG-IM association, which represent a continuum among 3 main clinical groups: distal, proximal and subclinical muscle involvement. Minimal muscle involvement and focal myositis could be underestimated among myasthenic patients and early aggressive immunotherapy could be required in focal group.


Assuntos
Debilidade Muscular/complicações , Debilidade Muscular/fisiopatologia , Miastenia Gravis/complicações , Miastenia Gravis/fisiopatologia , Humanos , Miosite/complicações , Miosite/fisiopatologia , Estudos Retrospectivos , Timoma/complicações
8.
Autoimmun Rev ; 19(2): 102455, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31838162

RESUMO

BACKGROUND AND OBJECTIVE: Drug-induced myopathy is among the most common causes of muscle disease. An association has recently been described between programmed death-1 (PD-1)/PD-1 ligand (PD-L1) inhibitors and immune-related adverse events (irAE) affecting the muscle. Here, we report the clinical and pathological findings of nine unrelated patients with PD-1 and PD-L1 inhibitors-associated myopathy. METHODS: We retrospectively analyzed 317 muscle biopsies performed for diagnostic purposes from January 2017 to June 2019. Patients were attended in two tertiary centers and muscle biopsies were performed and analyzed by two myology experts. Muscle biopsies were frozen in cooled isopenthane, cryostat sectioned and stained. Immunohistochemistry studies were also performed as a routine procedure in our lab. RESULTS: We identified 9 patients receiving anti-PD-1 or PD-L1 inhibitors consulting for either muscle weakness, asthenia, myasthenic-like syndrome or other muscle related-symptoms, along with biopsy-proven inflammatory myopathy. One had concomitant myocarditis. In most of the cases muscle biopsy showed a marked phenomenon of necrosis, macrophagy and muscle regeneration with perivascular inflammatory infiltrates with a large component of macrophagic cells. A tendency to perifascicular atrophy was also noticed. The expression of MHC class I antigens predominated in the perifascicular zones. Raised muscle enzymes were detected in 7 patients. CONCLUSION: A characteristic clinic-pathological pattern, including a myasthenia gravis-like syndrome plus myositis was found in patients receiving PD-1 and PD-1 L inhibitors. A large component of macrophages resembling granulomas seems to be the pathological hallmark of the syndrome. Further information is required to understand the wide spectrum of immune-related adverse events involving the muscle during or after treatment with anti-PD-1 inhibitors, but the pathological picture seems to be characteristic.


Assuntos
Ligantes , Receptor de Morte Celular Programada 1/antagonistas & inibidores , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Miastenia Gravis/induzido quimicamente , Miastenia Gravis/metabolismo , Miastenia Gravis/patologia , Miosite/induzido quimicamente , Miosite/metabolismo , Miosite/patologia , Estudos Retrospectivos
9.
Rheumatology (Oxford) ; 59(1): 112-119, 2020 01 01.
Artigo em Inglês | MEDLINE | ID: mdl-31382295

RESUMO

OBJECTIVE: To clarify the incidence, risk factors, and impact of malignancy in patients with PM/DM-associated interstitial lung disease (ILD). METHODS: This study used data from 497 patients with PM/DM-associated ILD enrolled in a multicentre, retrospective and prospective cohort of incident cases. Cancer-associated myositis (CAM) was defined as malignancy diagnosed within 3 years before or after PM/DM diagnosis. Demographic and clinical information was recorded at the time of diagnosis, and data about the occurrence of mortality and malignancy was collected. RESULTS: CAM was identified in 32 patients with PM/DM-associated ILD (6.4%). Patients with CAM were older (64 vs 55 years, P < 0.001), presented with arthritis less frequently (24% vs 49%, P = 0.01), and showed a lower level of serum Krebs von den Lungen-6 (687 vs 820 IU/l, P = 0.03) than those without CAM. The distribution of myositis-specific autoantibodies, including anti-melanoma differentiation-associated gene 5, anti-aminoacyl tRNA synthetase, and anti-transcriptional intermediary factor 1-γ antibodies, did not differ between the groups. Survival analysis demonstrated that CAM patients had a poorer survival than non-CAM patients (P = 0.006), primarily due to excess deaths by concomitant malignancy, while mortality due to ILD-related respiratory failure was similar between the groups (P = 0.51). CONCLUSION: Concomitant malignancy can occur in patients with PM/DM-associated ILD, and has significant impact on mortality. Older age, lack of arthritis, and a lower level of serum Krebs von den Lungen-6 at diagnosis are predictors of concomitant malignancy.


Assuntos
Doenças Pulmonares Intersticiais/mortalidade , Miosite/mortalidade , Neoplasias/mortalidade , Fatores Etários , Aminoacil-tRNA Sintetases/imunologia , Autoanticorpos/sangue , Feminino , Humanos , Incidência , Japão/epidemiologia , Doenças Pulmonares Intersticiais/sangue , Doenças Pulmonares Intersticiais/etiologia , Masculino , Pessoa de Meia-Idade , Mucina-1/sangue , Miosite/sangue , Miosite/etiologia , Neoplasias/sangue , Neoplasias/complicações , Estudos Prospectivos , Estudos Retrospectivos , Fatores de Risco
10.
Rheumatology (Oxford) ; 59(1): 194-204, 2020 01 01.
Artigo em Inglês | MEDLINE | ID: mdl-31292651

RESUMO

OBJECTIVE: The inflammatory idiopathic myopathies (IIM) are a group of rare autoimmune diseases defined by muscle weakness and characterized by pro-inflammatory infiltrates in muscle. Little is known about the immunological profile in peripheral blood of these patients and how this relates to IIM subtypes. This study aimed to stratify adult and juvenile-onset IIM patients according to immune cell profile. METHODS: Peripheral blood mononuclear cells from 44 patients with adult myositis (AM), 15 adolescent-onset juvenile dermatomyositis (a-JDM), and 40 age-matched healthy controls were analysed by flow cytometry to quantify 33 immune cell subsets. Adult myositis patients were grouped according to myositis subtype; DM and polymyositis; and also autoantibody specificity. Disease activity was determined by the myositis disease activity assessment tool and clinicians' decision on treatment. RESULTS: Unique immune signatures were identified for DM, polymyositis and a-JDM compared with healthy controls. DM patients had a T-cell signature comprising increased CD4+ and TH17 cell frequencies and increased immune cell expression of IL-6. Polymyositis patients had a B-cell signature with reduced memory B cells. A-JDM had decreased naïve B cells and increased CD4+T cells. All patient groups had decreased CD8+central memory T-cell frequencies. The distinct immune signatures were also seen when adult myositis patients were stratified according to auto-antibody expression; patients with anti-synthetase-antibodies had reduced memory B cells and patients with autoimmune rheumatic disease overlap had an elevated Th17 profile. CONCLUSION: Unique immune signatures were associated with adult vs juvenile disease. The Th17 signature in DM patients supports the potential use of IL-17 inhibitors in treatment of IIMs.


Assuntos
Autoanticorpos/sangue , Imunidade Celular , Leucócitos Mononucleares/imunologia , Miosite/imunologia , Adolescente , Adulto , Linfócitos B/imunologia , Biomarcadores/sangue , Feminino , Citometria de Fluxo , Humanos , Interleucina-6/sangue , Leucócitos Mononucleares/patologia , Masculino , Pessoa de Meia-Idade , Miosite/sangue , Linfócitos T/imunologia , Adulto Jovem
11.
Autoimmun Rev ; 19(1): 102426, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31734407

RESUMO

MGD and CG were responsible for the study's conception and design. VP and DM contributed to data acquisition. VP and DB planned and performed the statistical analyses. All Authors contributed to data interpretation. MGD, CG, VP and DM drafted the manuscript. AG and DB revised the manuscript critically for intellectual content. All authors gave their final approval of the version of the manuscript to be published.


Assuntos
Imunoglobulinas/uso terapêutico , Miosite/terapia , Creatina Quinase/sangue , Humanos , Força Muscular , Estudos Prospectivos
12.
F1000Res ; 82019.
Artigo em Inglês | MEDLINE | ID: mdl-31824645

RESUMO

Dermatomyositis (DM) is an inflammatory myopathy with characteristic skin manifestations, the pathologies of which are considered autoimmune diseases. DM is a heterogeneous disorder with various phenotypes, including myositis, dermatitis, and interstitial lung disease (ILD). Recently identified myositis-specific autoantibodies have been associated with distinct clinical features. For example, anti-melanoma differentiation-associated protein 5 antibodies have a high specificity for clinically amyopathic DM presenting rapidly progressive ILD. Furthermore, anti-transcriptional intermediary factor 1γ antibodies found in patients with juvenile and adult DM are closely correlated with malignancies, especially in elderly patients. Finally, patients with anti-aminoacyl-transfer RNA synthetase antibodies share characteristic clinical symptoms, including myositis, ILD, arthritis/arthralgia, Raynaud's phenomenon, and fever; thus, the term "anti-synthetase syndrome" is also used. With a focus on the characteristic cutaneous manifestations in each subgroup classified according to myositis-specific autoantibodies, we introduce the findings of previous reports, including our recent analysis indicating that skin eruptions can be histopathologically classified into myositis-specific autoantibody-associated subgroups and used to determine the systemic pathologies of the different types of antibody-associated DM.


Assuntos
Dermatomiosite , Doenças Pulmonares Intersticiais , Miosite , Aminoacil-tRNA Sintetases , Autoanticorpos , Humanos
13.
Beijing Da Xue Xue Bao Yi Xue Ban ; 51(6): 989-995, 2019 Dec 18.
Artigo em Chinês | MEDLINE | ID: mdl-31848492

RESUMO

OBJECTIVE: To investigate the clinical and pathological features of immune-mediated necrotic myopathies (IMNM) with different myositis-specific antibodies (MSAs). METHODS: In the study, 104 IMNM patients who met any of the following three criteria were selected from idiopathic inflammatory myopathy patients who had MSAs results and underwent muscle biopsy from 2008 to 2018 in China-Japan Friendship Hospital: (1) Anti-signal recognition particle (SRP) antibody positive; (2) Anti-3-hydroxy-3-methylglutaryl coenzyme A reductase (HMGCR) antibody positive; (3) MSAs negative and consistent with the pathological diagnostic criteria of IMNM defined by the European Neuromuscular Centre in 2004. The clinical, laboratory and muscle pathological information of the IMNM patients were retrospectively collected and compared in anti-SRP, anti-HMGCR and MSAs negative groups. RESULTS: Of 104 IMNM patients, 47 patients (45.2%) were positive for anti-SRP antibody, 23 (22.1%) were positive for anti-HMGCR antibody, and 34 (32.7%) were negative for MSAs. The common symptoms of IMNM patients were muscle weakness (92.3%), elevated serum creatine kinase level (92.3%), dysphagia (33.7%) and interstitial lung diseases (ILD) (49.5%). The anti-HMGCR-positive patients were more frequent to have "V" sign (30.4% vs. 4.3% and 5.9%, P<0.01) as compared with the anti-SRP-positive and MSAs-negative patients. The incidence of ILD in the anti-SRP-positive patients was higher than that in the anti-HMGCR-positive and MSAs negative patients (64.4% vs. 34.8% and 29.0%, P<0.01). The prevalence of the patients combined with other connective tissue diseases in MSAs-negative IMNM was higher than that in the other two groups (32.4% vs. 8.5% and 4.3%, P<0.01). 93.3% of the anti-SRP-positive patients were found with antinuclear antibody positivity, higher than those of the anti-HMGCR-positive and MSAs-negative patients (93.3% vs. 36.4% and 58.8%, P<0.001). The common pathological features of IMNM were muscle fibre necrosis (94.2%), regeneration (67.3%) and phagocytosis (65.4%), overexpression of major histocompatibility complex1 on sarcolemma (78.8%), infiltration of CD4+ T cells (81.7%) and CD68+ macrophage (79.8%) and expression of membrane attack complex (MAC) (77.8%). The endomysial infiltration of CD4+ T cells and CD68+ macrophage and MAC expression on sarcolemma in the MSAs-negative group were more common than that in the anti-SRP and anti-HMGCR groups (88.2% vs. 57.4% and 60.9%, 91.2% vs. 59.1% and 38.1%, 76.5% vs. 45.5% and 42.9%, respectively, P<0.01). CONCLUSION: There is heterogeneity in anti-SRP-positive, anti-HMGCR-positive or MSAs-negative patients. The detection of MSAs and performing of muscle biopsy are useful for distinguishing different types of IMNM.


Assuntos
Miosite , Autoanticorpos , China , Humanos , Músculo Esquelético , Estudos Retrospectivos
15.
Indian J Dent Res ; 30(4): 634-638, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31745065

RESUMO

Masseter traumatic myositis chondro-ossificans (TMCO) is a rare pathological condition that causes severe mandibular function restriction. The aim of the present study is to report a TMCO case after direct masseter muscle injury and correlate it to bone and cartilage biomarkers up-regulation. Caucasian male patient, 38 years old, seeks treatment nine days after trauma with severe mouth opening limitation. Physical examination revealed a circumscribed hardened area connected to masseter muscle on the left side. Cone beam tomography and ultrasonography of masseter region were requested. There was incomplete fracture between the posterior board of inferior jaw and coronoid process as well as calcification within masseter muscle. The proposed treatment was excisional biopsy of calcification, coronoid process removal to enhance mouth opening as well as incomplete condyle fracture monitoring. Material removed was sent for histological analysis in order to confirm diagnosis. Immuhistochemistry was conducted and it was found that chondro-ossification biomarkers such as TGF-b1, Indian Hegdehog (IHH), BMP2, osteopontin (OP) and osteocalcin (OC) were up-regulated. One-year follow-up showed that the patient is stable with increased mouth opening and satisfactory jaw movements. Pathologists and maxillofacial surgeons must be aware of differential diagnosis of TMCO. Understanding cellular mechanisms of muscle tissue after trauma is also important once cellular pathway modifications leads to clinical features that differ from previously described in literature.


Assuntos
Miosite Ossificante , Miosite , Adulto , Proteína Morfogenética Óssea 2 , Humanos , Masculino , Músculo Masseter , Osteocalcina , Osteopontina , Fator de Crescimento Transformador beta1 , Regulação para Cima
16.
Z Rheumatol ; 78(9): 810-812, 2019 11.
Artigo em Alemão | MEDLINE | ID: mdl-31686205
17.
Clin Nucl Med ; 44(12): 961-963, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31689277

RESUMO

A 57-year-old woman with a history of uterine endometrial carcinoma underwent PET/CT examinations for initial staging and posttreatment survey. Multiple patchy accumulations were noted in the muscles, particularly in both thighs. These accumulations resolved spontaneously 6 months after the follow-up examination. However, 3.5 years after the surgery, the multiple patchy accumulations reappeared in the muscle of the upper and lower extremities showing an increase in signal intensity from previous examination. A biopsy of the right thigh revealed epithelioid cell granuloma without necrosis. We therefore consider that this case might be "idiopathic" granulomatous myositis.


Assuntos
Fluordesoxiglucose F18 , Granuloma/complicações , Miosite/complicações , Miosite/diagnóstico por imagem , Tomografia Computadorizada com Tomografia por Emissão de Pósitrons , Neoplasias do Endométrio/complicações , Neoplasias do Endométrio/diagnóstico por imagem , Neoplasias do Endométrio/patologia , Neoplasias do Endométrio/terapia , Feminino , Humanos , Pessoa de Meia-Idade , Miosite/patologia , Estadiamento de Neoplasias
18.
Zhonghua Nei Ke Za Zhi ; 58(12): 899-904, 2019 Dec 01.
Artigo em Chinês | MEDLINE | ID: mdl-31775453

RESUMO

Objective: To analyze the diagnostic value of skeletal muscle biopsy in patients with rhabdomyolysis. Methods: Clinical and pathological data of 26 patients with rhabdomyolysis from January 2002 to December 2018 undergoing muscle biopsy were collected. Results: Eighteen males and 8 females were finally recruited with median age of 6-73 (37.3±19.6) years. The average time from onset to biopsy was 44 days (median course was 30 days). All patients had acute manifestations with muscle pain and/or weakness. Serum creatine kinase was between 1 648-92 660 U/L. Muscle biopsies showed nonspecific changes in 12 cases (a few with type 2 muscle fiber atrophy, slight deposition of lipid droplets), 10 cases with necrotizing myopathy (muscle fiber necrosis and regeneration). Toxic neurogenic damages were seen in 2 cases (type 1 and type 2 angular atrophic muscle fibers with group change), lipid storage disease in 1 case (lipid droplets deposit significantly) and idiopathic inflammatory myopathy in 1 case (muscle fiber necrosis and regeneration, with lymphocyte infiltration). The etiology of non-specific pathological changes included short-term strenuous exercise in 6 patients, poisoning in two, chronic kidney disease in one, viral infection in one, hypothyroidism in one and unknown reason in one. As to patients with necrotizing myopathy, seven were poisoning or drug-related, one with hyperthyroidism, two with unknown reason. Conclusions: Among the numerous causes of rhabdomyolysis, exercise usually links nonspecific skeletal muscle changes and poisoning or drug-related disorders are commonly associated with necrotic myopathy. Rhabdomyolysis induced by primary myopathy is rare.


Assuntos
Músculo Esquelético/patologia , Rabdomiólise/patologia , Adolescente , Adulto , Idoso , Biópsia , Criança , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Doenças Musculares/diagnóstico , Miosite/patologia , Adulto Jovem
19.
Best Pract Res Clin Rheumatol ; 33(3): 101433, 2019 06.
Artigo em Inglês | MEDLINE | ID: mdl-31590993

RESUMO

Myalgia is a common symptom of various neuromuscular disorders: myalgia occurs in metabolic muscle diseases, inflammatory muscle diseases, dystrophic myopathies and myotonic muscle disorders. Myalgia leads to a significantly reduced quality of life. Other muscular symptoms that are present along with myalgia often provide the clue towards a diagnosis and include weakness, cramps and myotonia as well as the type of pain. In addition, extramuscular symptoms like an erythema in dermatomyositis can lead to the correct diagnosis. Basic diagnostic workup includes a detailed medical history, full neurologic assessment, laboratory tests, EMG and nerve conduction studies. Muscle imaging, genetic testing and muscle biopsy may be required to make a diagnosis. Whenever possible, treatment should aim to improve or correct the underlying cause for myalgia such as inflammation or hypothyroidism. Symptomatic therapy includes different avenues: Myotonia can be treated with mexiletine. Carbamazepine or phenytoin can be used in myotonic syndromes, particularly with muscle cramps. Pregabalin, gabapentin, or amitriptyline can be tried in conditions with myalgic pain. This review summarizes the symptoms, diagnostic strategies, and therapeutic approach in neuromuscular disorders that present with myalgia.


Assuntos
Doenças Musculares/complicações , Doenças Musculares/diagnóstico , Mialgia/etiologia , Miosite/complicações , Miosite/diagnóstico , Humanos , Qualidade de Vida
20.
Neurology ; 93(19): e1768-e1777, 2019 11 05.
Artigo em Inglês | MEDLINE | ID: mdl-31594859

RESUMO

OBJECTIVE: To define the clinical phenotype of dermatomyositis (DM) with anti-Mi2 autoantibodies. METHODS: In this longitudinal cohort study, the prevalence and severity of clinical features at disease onset and during follow-up in patients with anti-Mi2-positive DM were compared to patients with anti-Mi2-negative DM, antisynthetase syndrome (AS), and immune-mediated necrotizing myopathy (IMNM). Longitudinal anti-Mi2 autoantibody titers were assessed. RESULTS: A total of 58 patients with anti-Mi2-positive DM, 143 patients with anti-Mi2-negative DM, 162 patients with AS, and 170 patients with IMNM were included. Among patients with anti-Mi2-positive DM, muscle weakness was present in 60% at disease onset and occurred in 98% during longitudinal follow-up; fewer patients with anti-Mi2-negative DM developed weakness (85%; p = 0.008). Patients with anti-Mi2-positive DM were weaker and had higher creatine kinase (CK) levels than patients with anti-Mi2-negative DM or patients with AS. Muscle biopsies from patients with anti-Mi2-positive DM had prominent necrosis. Anti-Mi2 autoantibody levels correlated with CK levels and strength (p < 0.001). With treatment, most patients with anti-Mi2-positive DM had improved strength and CK levels; among 10 with multiple serum samples collected over 4 or more years, anti-Mi2 autoantibody titers declined in all and normalized in 3, 2 of whom stopped immunosuppressant treatment and never relapsed. Patients with anti-Mi2-positive DM had less calcinosis (9% vs 28%; p = 0.003), interstitial lung disease (5% vs 16%; p = 0.04), and fever (7% vs 21%; p = 0.02) than did patients with anti-Mi2-negative DM. CONCLUSIONS: Patients with anti-Mi2-positive DM have more severe muscle disease than patients with anti-Mi2-negative DM or patients with AS. Anti-Mi2 autoantibody levels correlate with disease severity and may normalize in patients who enter remission.


Assuntos
Autoanticorpos/imunologia , Calcinose/epidemiologia , Dermatomiosite/imunologia , Febre/epidemiologia , Doenças Pulmonares Intersticiais/epidemiologia , Complexo Mi-2 de Remodelação de Nucleossomo e Desacetilase/imunologia , Debilidade Muscular/epidemiologia , Adulto , Idoso , Calcinose/fisiopatologia , Estudos de Casos e Controles , Estudos de Coortes , Creatina Quinase/sangue , Dermatomiosite/sangue , Dermatomiosite/epidemiologia , Dermatomiosite/fisiopatologia , Feminino , Humanos , Estudos Longitudinais , Doenças Pulmonares Intersticiais/fisiopatologia , Masculino , Pessoa de Meia-Idade , Debilidade Muscular/fisiopatologia , Miosite/imunologia , Miosite/fisiopatologia , Necrose , Fenótipo , Prevalência , Índice de Gravidade de Doença
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