RESUMO
BACKGROUND AND OBJECTIVES: ILD patients can be positive to highly specific autoantibodies of connective tissue diseases (CTD). Among them stand out myositis-specific and associated autoantibodies (MSA/MAA). There is limited knowledge about treatment response and prognosis of ILD patients positive to MSA/MAA (MSA/MAA-ILD). Our aim was to describe clinical, radiological and pulmonary function (PF) of MSA/MAA-ILD Latin-American patients and risk factors associated to PF at onset and long term follow up. METHODS: Multicentric retrospective study of MSA/MAA-ILD patients evaluated between 2016 and 2018 in 3 ILD clinics in Latin America. Clinical, functional and tomographic variables were described. Variables associated with poor baseline PF and associated with functional improvement (FI) were analyzed in a multivariate logistic regression model. RESULTS: We included 211 patients, 77.4% female, mean age 57 years old. Most frequent MSA/MAA were Ro-52 and Jo-1. Poor baseline PF was associated to ILD as initial diagnosis and NSIP/OP HRCT pattern. 121 patients were included in the follow up PF analysis: 48.8% remained stable and 33% had a significant FI. In multivariate analysis, OP pattern on HRCT was associated with FI. Systemic symptoms from the beginning and the absence of sclerodactyly showed a trend to be associated with FI. CONCLUSIONS: Worse baseline PF could be related to the absence of extra-thoracic symptoms and "classic" antibodies in CTD (ANA), which causes delay in diagnosis and treatment. In contrast, FI could be related to the presence of extra-thoracic signs that allow timely diagnosis and therapy, and more acute and subacute forms of ILD, such as OP pattern.
Assuntos
Doenças do Tecido Conjuntivo , Doenças Pulmonares Intersticiais , Miosite , Autoanticorpos , Estudos de Coortes , Doenças do Tecido Conjuntivo/complicações , Feminino , Humanos , Doenças Pulmonares Intersticiais/complicações , Doenças Pulmonares Intersticiais/diagnóstico , Masculino , Pessoa de Meia-Idade , Miosite/complicações , Miosite/diagnóstico , Estudos Retrospectivos , Estados UnidosRESUMO
BACKGROUND AND OBJECTIVES: ILD patients can be positive to highly specific autoantibodies of connective tissue diseases (CTD). Among them stand out myositis-specific and associated autoantibodies (MSA/MAA). There is limited knowledge about treatment response and prognosis of ILD patients positive to MSA/MAA (MSA/MAA-ILD). Our aim was to describe clinical, radiological and pulmonary function (PF) of MSA/MAA-ILD Latin-American patients and risk factors associated to PF at onset and long term follow up. METHODS: Multicentric retrospective study of MSA/MAA-ILD patients evaluated between 2016 and 2018 in 3 ILD clinics in Latin America. Clinical, functional and tomographic variables were described. Variables associated with poor baseline PF and associated with functional improvement (FI) were analyzed in a multivariate logistic regression model. RESULTS: We included 211 patients, 77.4% female, mean age 57 years old. Most frequent MSA/MAA were Ro-52 and Jo-1. Poor baseline PF was associated to ILD as initial diagnosis and NSIP/OP HRCT pattern. 121 patients were included in the follow up PF analysis: 48.8% remained stable and 33% had a significant FI. In multivariate analysis, OP pattern on HRCT was associated with FI. Systemic symptoms from the beginning and the absence of sclerodactyly showed a trend to be associated with FI. CONCLUSIONS: Worse baseline PF could be related to the absence of extra-thoracic symptoms and "classic" antibodies in CTD (ANA), which causes delay in diagnosis and treatment. In contrast, FI could be related to the presence of extra-thoracic signs that allow timely diagnosis and therapy, and more acute and subacute forms of ILD, such as OP pattern.
RESUMO
BACKGROUND: The presence of multiple lymphadenopathies can be a diagnostic challenge. AIM: To describe the clinical, laboratory and imaging characteristics of 19 patients with lymphadenopathies of rheumatologic origin. MATERIAL AND METHODS: Review of medical records of 19 patients aged 16 to 72 years (68%) with lymphadenopathies presumably secondary to a rheumatic disease. RESULTS: Six patients had systemic lupus erythematosus, six had Sjogren's disease, three had sarcoidosis, two had rheumatoid arthritis, one had IgG4 related disease and one had mixed connective tissue disease. A lymph node biopsy was performed in 11 patients and in eight a lymphoid follicular hyperplasia was found. Systemic symptoms were reported by 68% of patients. Blood lactate dehydrogenase was elevated only in cases associated with hemolytic anemia. There was no specific or predictable localization of the lymphadenopathies in imaging studies, except in the cases of sarcoidosis. The average size of the lymphadenopathies was 13.5 mm in diameter in short axis and there was no presence of necrosis, calcification, or conglomerate formation. Only one case presented splenomegaly. All patients responded favorably to corticosteroids. CONCLUSIONS: Lymphadenopathies associated with rheumatologic diseases can occur in a wide variety of diseases, especially systemic lupus erythematosus and Sjögren's disease. The absence of LDH elevation and splenomegaly and the absence of imaging findings such as conglomerates can orient to a rheumatologic origin.
Assuntos
Linfadenopatia , Doenças Reumáticas , Adolescente , Adulto , Idoso , Humanos , Pessoa de Meia-Idade , Adulto JovemRESUMO
Background: The presence of multiple lymphadenopathies can be a diagnostic challenge. Aim: To describe the clinical, laboratory and imaging characteristics of 19 patients with lymphadenopathies of rheumatologic origin. Material and Methods: Review of medical records of 19 patients aged 16 to 72 years (68%) with lymphadenopathies presumably secondary to a rheumatic disease. Results: Six patients had systemic lupus erythematosus, six had Sjogren's disease, three had sarcoidosis, two had rheumatoid arthritis, one had IgG4 related disease and one had mixed connective tissue disease. A lymph node biopsy was performed in 11 patients and in eight a lymphoid follicular hyperplasia was found. Systemic symptoms were reported by 68% of patients. Blood lactate dehydrogenase was elevated only in cases associated with hemolytic anemia. There was no specific or predictable localization of the lymphadenopathies in imaging studies, except in the cases of sarcoidosis. The average size of the lymphadenopathies was 13.5 mm in diameter in short axis and there was no presence of necrosis, calcification, or conglomerate formation. Only one case presented splenomegaly. All patients responded favorably to corticosteroids. Conclusions: Lymphadenopathies associated with rheumatologic diseases can occur in a wide variety of diseases, especially systemic lupus erythematosus and Sjögren's disease. The absence of LDH elevation and splenomegaly and the absence of imaging findings such as conglomerates can orient to a rheumatologic origin.
Assuntos
Humanos , Adolescente , Adulto , Pessoa de Meia-Idade , Idoso , Adulto Jovem , Doenças Reumáticas , LinfadenopatiaRESUMO
Interstitial lung diseases are a broad, diverse, challenging group of diseases, most of them chronic whose prognosis is not good. In the last two decades there have been considerable advances in the knowledge of the epidemiology, pathological and genetic bases and treatment of several of these diseases. This article summarizes and presents updated information about their classification, new knowledge on genetics and treatments in idiopathic pulmonary fibrosis, advances in the diagnosis and management of hypersensitivity pneumonitis and a review of the broad spectrum of interstitial diseases associated with connective tissue diseases. Several clinical trials are currently underway whose results will be available in the coming years and will provide more information and tools to improve the treatment of these patients.
Assuntos
Doenças Pulmonares Intersticiais/classificação , Doenças Pulmonares Intersticiais/diagnóstico , Prognóstico , Doenças do Tecido Conjuntivo/diagnóstico , Diagnóstico Diferencial , Fibrose Pulmonar Idiopática/diagnóstico , Alveolite Alérgica Extrínseca/diagnósticoRESUMO
Interstitial lung diseases are a broad, diverse, challenging group of diseases, most of them chronic whose prognosis is not good. In the last two decades there have been considerable advances in the knowledge of the epidemiology, pathological and genetic bases and treatment of several of these diseases. This article summarizes and presents updated information about their classification, new knowledge on genetics and treatments in idiopathic pulmonary fibrosis, advances in the diagnosis and management of hypersensitivity pneumonitis and a review of the broad spectrum of interstitial diseases associated with connective tissue diseases. Several clinical trials are currently underway whose results will be available in the coming years and will provide more information and tools to improve the treatment of these patients.
Assuntos
Doenças Pulmonares Intersticiais/classificação , Doenças Pulmonares Intersticiais/diagnóstico , Alveolite Alérgica Extrínseca/diagnóstico , Doenças do Tecido Conjuntivo/diagnóstico , Diagnóstico Diferencial , Fibrose Pulmonar Idiopática/diagnóstico , PrognósticoRESUMO
Pulmonary artery sarcoma is an uncommon entity with high mortality. Its clinical presentation is usually indistinguishable from pulmonary embolism, which leads to a significant delay in diagnosis. Hughes-Stovin syndrome is characterized by venous thrombosis and aneurysms of the pulmonary or bronchial artery. We report a 59 year-old female with a history of recurrent pulmonary embolism. In the last thromboembolic episode a pulmonary artery aneurysm was found on a CT scan. The patient was operated performing a left inferior lobectomy. The patient died five days after surgery and the pathological examination of the surgical piece revealed a pulmonary artery sarcoma.
Assuntos
Feminino , Humanos , Pessoa de Meia-Idade , Aneurisma/diagnóstico , Artéria Pulmonar , Sarcoma/diagnóstico , Neoplasias Vasculares/diagnóstico , Trombose Venosa/diagnóstico , Diagnóstico Diferencial , Evolução Fatal , SíndromeRESUMO
Pulmonary artery sarcoma is an uncommon entity with high mortality. Its clinical presentation is usually indistinguishable from pulmonary embolism, which leads to a significant delay in diagnosis. Hughes-Stovin syndrome is characterized by venous thrombosis and aneurysms of the pulmonary or bronchial artery. We report a 59 year-old female with a history of recurrent pulmonary embolism. In the last thromboembolic episode a pulmonary artery aneurysm was found on a CT scan. The patient was operated performing a left inferior lobectomy. The patient died five days after surgery and the pathological examination of the surgical piece revealed a pulmonary artery sarcoma.
Assuntos
Aneurisma/diagnóstico , Artéria Pulmonar , Sarcoma/diagnóstico , Neoplasias Vasculares/diagnóstico , Trombose Venosa/diagnóstico , Diagnóstico Diferencial , Evolução Fatal , Feminino , Humanos , Pessoa de Meia-Idade , SíndromeRESUMO
Three main lines of investigation are discussed in this paper: (1) the comparison between the anatomical arrangement of the language areas and the large-scale neurocognitive cortical networks partly involved in active or working memory; (2) the relations between hemispheric specialization and the development of interhemispheric communication; and (3) the analysis of individual differences in brain organization for language. The hypothesis and evidence presented stem from work being performed in our laboratories