RESUMO
Objetivo: Analisar as frequências de expressão dos antígenos de complexo principal de histocompatibilidade classe I (MHC-I) e células CD4 e CD8 no músculo esquelético na polimiosite (PM) e dermatomiosite (DM). Métodos: Estudo retrospectivo de 34 casos de PM, oito casos de DM e 29 controles com miopatias não inflamatórias. Resultados: Os antígenos MHC-I expressaram-se no sarcolema e/ou sarcoplasma em 79,4% dos casos de PM, 62,5% dos casos de DM e 27,6% dos controles (a expressão de CD4 foi observada em 76,5%, 75% e 13,8%, respectivamente). Quando os antígenos de MHC-I foram coexpressados com CD4, houve elevada suspeita de PM/DM (principalmente PM). Em 14,3% dos casos de PM/DM, observou-se a expressão isolada dos antígenos MHC-I, sem células inflamatórias. Conclusão: A expressão dos antígenos MHC-I e a positividade do CD4 podem aumentar a suspeita diagnóstica de PM/DM. Não foi observado infiltrado celular em 14,3% dos casos. .
Objective: To analyze the frequencies of the expression of major histocompatibility complex class I (MHC-I) antigens, and CD4 and CD8 cells in skeletal muscle in polymyositis (PM) and dermatomyositis (DM). Methods: This was a retrospective study of 34 PM cases, 8 DM cases, and 29 control patients with non-inflammatory myopathies. Results: MHC-I antigens were expressed in the sarcolemma and/or sarcoplasm in 79.4% of PM cases, 62.5% of DM cases, and 27.6% of controls (CD4 expression was observed in 76.5%, 75%, and 13.8%, respectively). There was a high suspicion of PM/DM (mainly PM) in participants in whom MHC-I antigens and CD4 were co-expressed. In 14.3% of PM/DM cases, we observed MHC-I antigens expression alone, without inflammatory cells. Conclusion: MHC-I antigens expression and CD4 positivity might add to strong diagnostic suspicion of PM/DM. No cellular infiltration was observed in approximately 14.3% of such cases. .
Assuntos
Humanos , Masculino , Feminino , Criança , Adolescente , Adulto , Pessoa de Meia-Idade , Idoso , Idoso de 80 Anos ou mais , Adulto Jovem , Antígenos CD4/biossíntese , Antígenos CD8/biossíntese , Dermatomiosite/metabolismo , Antígenos de Histocompatibilidade Classe I/biossíntese , Polimiosite/metabolismo , Antígenos CD4/análise , Antígenos CD8/análise , Dermatomiosite/imunologia , Antígenos de Histocompatibilidade Classe I/análise , Músculo Esquelético/química , Polimiosite/imunologia , Estudos RetrospectivosRESUMO
OBJECTIVE: To analyze the frequencies of the expression of major histocompatibility complex class I (MHC-I) antigens, and CD4 and CD8 cells in skeletal muscle in polymyositis (PM) and dermatomyositis (DM). METHODS: This was a retrospective study of 34 PM cases, 8 DM cases, and 29 control patients with non-inflammatory myopathies. RESULTS: MHC-I antigens were expressed in the sarcolemma and/or sarcoplasm in 79.4% of PM cases, 62.5% of DM cases, and 27.6% of controls (CD4 expression was observed in 76.5%, 75%, and 13.8%, respectively). There was a high suspicion of PM/DM (mainly PM) in patients in whom MHC-I antigens and CD4 were co-expressed. In 14.3% of PM/DM cases, we observed MHC-I antigens expression alone, without inflammatory cells. CONCLUSION: MCH-I antigens expression and CD4 positivity might add to strong diagnostic suspicion of PM/DM. No cellular infiltration was observed in 14.3% of such cases.
Assuntos
Antígenos CD4/biossíntese , Antígenos CD8/biossíntese , Dermatomiosite/metabolismo , Antígenos de Histocompatibilidade Classe I/biossíntese , Polimiosite/metabolismo , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Antígenos CD4/análise , Antígenos CD8/análise , Criança , Dermatomiosite/imunologia , Feminino , Antígenos de Histocompatibilidade Classe I/análise , Humanos , Masculino , Pessoa de Meia-Idade , Músculo Esquelético/química , Polimiosite/imunologia , Estudos Retrospectivos , Adulto JovemRESUMO
OBJECTIVE: To assess MHC I and II expressions in muscle fibres of juvenile dermatomyositis (JDM) and compare with the expression in polymyositis (PM), dermatomyositis (DM) and dystrophy. PATIENTS AND METHODS: Forty-eight JDM patients and 17 controls (8 PM, 5 DM and 4 dystrophy) were studied. The mean age at disease onset was 7.1+/-3.0 years and the mean duration of weakness before biopsy was 9.4+/-12.9 months. Routinehistochemistry and immunohistochemistry (StreptABComplex/HRP) for MHC I and II (Dakopatts) were performed on serial frozen muscle sections in all patients. Mann-Whitney, Kruskal Wallis, chi-square and Fisher's exact statistical methods were used. RESULTS: MHC I expression was positive in 47 (97.9%) JDM cases. This expression was observed independent of time of disease, corticotherapy previous to muscle biopsy and to the grading of inflammation observed in clinical, laboratorial and histological parameters. The expression of MHC I was similar on JDM, PM and DM, and lower in dystrophy. On the other hand, MHC II expression was positive in just 28.2% of JDM cases and was correlated to histological features as inflammatory infiltrate, increased connective tissue and VAS for global degree of abnormality (p<0.05). MHC II expression was similar in DM/PM and lower in JDM and dystrophy, and it was based on the frequency of positive staining rather than to the degree of the MCH II expression. CONCLUSIONS: MHC I expression in muscle fibres is a premature and late marker of JDM patient independent to corticotherapy, and MHC II expression was lower in JDM than in PM and DM.
Assuntos
Dermatomiosite/metabolismo , Antígenos HLA/metabolismo , Músculo Esquelético/metabolismo , Biomarcadores/metabolismo , Biópsia , Criança , Pré-Escolar , Estudos Transversais , Dermatomiosite/diagnóstico , Dermatomiosite/patologia , Diagnóstico Diferencial , Regulação da Expressão Gênica , Genes MHC Classe I/genética , Genes MHC da Classe II/genética , Humanos , Músculo Esquelético/patologia , Distrofias Musculares/diagnóstico , Distrofias Musculares/metabolismo , Distrofias Musculares/patologia , Polimiosite/diagnóstico , Polimiosite/metabolismo , Polimiosite/patologiaRESUMO
To assess the differential expression of adhesion molecules ICAM-1 and VCAM-1 in vessels and muscle fibers in acquired inflammatory myopathy, a series comprising thirty-seven muscle biopsy specimens from patients with JDM, fifteen with DM, fifteen with PM and seven with IBM was studied. Histochemical and immunohistochemical tests (StreptABCcomplex/HRP) for ICAM-1 and VCAM-1 (Dakopatts) were performed in serial frozen sections. ICAM-1 expression in vessels was significantly (p<0.0001) more present in JDM than PM, DM or IBM. However, in muscle fibers, ICAM-1 expression was absent in both JDM and IBM, but present in 33.4% and 40% in PM and DM respectively (p<0.0001). VCAM-1 expression in vessels was significantly more present in PM and DM than JDM and IBM (p<0.0001) while VCAM-1 expression in muscle fibers was almost absent in the four groups (p=0.2632). These findings emphasize the importance of adhesion molecules in the pathophysiology of the inflammatory myopathies, mainly the marked ICAM-1 expression in vessels in JDM, corroborating the microvascular involvement in this disease. In contrast, VCAM-1 seems not to play a major role in JDM, as previously described in PM, DM and IBM. Adhesion molecule expression in JDM presents a differential characteristic when compared to PM, DM and IBM.
Assuntos
Molécula 1 de Adesão Intercelular/metabolismo , Miosite/metabolismo , Molécula 1 de Adesão de Célula Vascular/metabolismo , Adulto , Vasos Sanguíneos/química , Vasos Sanguíneos/metabolismo , Vasos Sanguíneos/patologia , Criança , Dermatomiosite/metabolismo , Dermatomiosite/patologia , Humanos , Molécula 1 de Adesão Intercelular/análise , Fibras Musculares Esqueléticas/química , Fibras Musculares Esqueléticas/metabolismo , Fibras Musculares Esqueléticas/patologia , Miosite/patologia , Miosite de Corpos de Inclusão/metabolismo , Miosite de Corpos de Inclusão/patologia , Polimiosite/metabolismo , Polimiosite/patologia , Molécula 1 de Adesão de Célula Vascular/análiseRESUMO
We describe a 47-year-old woman who developed chronic subluxing arthropathy associated with polymyositis (PM) and positive anti-Jo1 antibodies. After a followup of 3 years, PM did not recur and polyarthritis dominated her clinical picture, leading to deformities and periarticular calcifications in her hands, shoulders, and feet. Patients with myositis, anti-Jo1 antibodies, and periarticular calcifications are at risk of developing deforming arthritis unresponsive to conventional therapy.