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1.
Rev Med Chil ; 150(4): 505-511, 2022 Apr.
Artigo em Espanhol | MEDLINE | ID: mdl-36155760

RESUMO

This article reviews the pulmonary manifestations of anti-neutrophil cytoplasmic antibody associated vasculitis (AAV). Its frequency in the different phenotypes of the disease, clinical manifestations and updated therapeutic recommendations are reviewed, aiming to alert the medical community about the existence of these diseases. We pretend to stimulate a timely suspicion, diagnostic precision, and the implementation of effective therapies, to reduce the eventual sequelae derived from a diagnostic omission or an inappropriate treatment for the different clinical scenarios in which these diseases appear.


Assuntos
Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos , Anticorpos Anticitoplasma de Neutrófilos , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/complicações , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/diagnóstico , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/tratamento farmacológico , Humanos , Pulmão
2.
Artigo em Inglês | MEDLINE | ID: mdl-36078685

RESUMO

Sjögren's syndrome (SS) is a disease with autoimmune features that affects mainly women and compromises the health-related quality of Life (HRQoL); it is important to evaluate illness experience for a better understanding of the life situation of the patient. The aim of the study was to summarize the individual life experiences and determine the impact of HRQoL and oral health-related quality of life (OHRQoL) and their correlation with health self-assessment in women with SS. The life experiences evaluation employed a concept mapping design to structure qualitative content obtained from semi-structured interviews. Hierarchical cluster analysis was used to analyze the patient's experiences. EQ-5D-5L and OHIP-14Sp were used. The correlation between appreciation of the general health status and OHIP-14 was evaluated. The experience classification by patients were analyzed and a dendrogram was obtained, identifying 10 clusters of disease experiences of SS, being limitations, pain and difficulties, coping and attitudes towards treatment the most common. Pain/discomfort in EQ-5D-5L and physical pain and psychological discomfort in OHIP-14 were the most affected dimensions in the patients. The results support the theoretical perspective that the experience of illness is relevant to describing the main difficulties of patients with SS and how it affects their quality of life.


Assuntos
Qualidade de Vida , Síndrome de Sjogren , Adaptação Psicológica , Feminino , Nível de Saúde , Humanos , Dor , Qualidade de Vida/psicologia , Inquéritos e Questionários
4.
Rev. med. Chile ; 150(6): 720-726, jun. 2022. ilus, tab
Artigo em Espanhol | LILACS | ID: biblio-1424123

RESUMO

BACKGROUND: Giant cell Arteritis (GCA) is the most common systemic vasculitis in patients over 50 years. Diagnosis is based on clinical, laboratory, imaging and biopsy. Temporal artery biopsy (TAB) may be inconclusive in up to 40% of patients. AIM: To describe disease features of patients diagnosed with GCA. MATERIAL AND METHODS: Review of pathology reports of giant cell arteritis and clinical records of patients seen with the diagnosis between 2000 and 2019. Demographic, clinical, laboratory, histopathology, imaging, treatment and follow-up variables were analyzed. RESULTS: We fetched 32 patients with a median age at diagnosis of 70.5 years (range 57-90), 81% women. Twenty eight percent had polymyalgia. 72% had only cranial symptoms, 12% had extracranial involvement and 13% exclusive extracranial involvement. The median time from onset of symptoms to diagnosis was two months (range 0.5-8). All had elevated erythrocyte sedimentation rate and c reactive protein. A TAB was performed in 27 patients and in 17 (65.4%) it confirmed the diagnosis. Transmural inflam- mation was the most frequent finding. All patients received steroids. Follow-up information was available from 25 patients and 92% received a steroid-spa- ring agent, usually methotrexate (74%). Ninety two percent achieved clinical remission in the first year and 59% had minor relapses during steroid tapering. CONCLUSIONS: Our patients showed frequent extracranial involvement and TAB was a useful diagnostic tool.


Assuntos
Humanos , Masculino , Feminino , Pessoa de Meia-Idade , Idoso , Idoso de 80 Anos ou mais , Arterite de Células Gigantes/diagnóstico , Arterite de Células Gigantes/patologia , Arterite de Células Gigantes/tratamento farmacológico , Esteroides/uso terapêutico , Artérias Temporais , Biópsia , Proteína C-Reativa , Metotrexato/uso terapêutico , Estudos Retrospectivos
5.
Rev. med. Chile ; 150(6): 705-710, jun. 2022. tab, ilus
Artigo em Espanhol | LILACS | ID: biblio-1424121

RESUMO

BACKGROUND: IgG4-related disease (IgG4 RD) is an immune-mediated fibro-inflammatory disorder, with tissue infiltration of IgG4+ plasma cells. It causes pseudotumors, tumors, and a wide spectrum of clinical manifestations. AIM: To report the clinical, laboratory, histopathological and treatment characteristics of a group of Chilean patients with IgG4 RD. MATERIAL AND METHODS: Review of medical records of 52 patients aged 18 to 76 years with IgG4 RD seen at six medical centers. RESULTS: Elevated IgG4 serum levels (> 135 mg/dl) were found in 18 of 44 (41%) patients. There was histological confirmation of the disease in 46 patients. The most common sites of involvement were lungs, eyes and kidneys. Eighteen (35%) patients had only one organ involved, 34 (65%) patients had two organs and 13 (25%) patients had three or more organs. The involvement of two organs was significantly more common in men (p < 0.05). In patients with only one organ involvement, the most frequent location was orbital and meningeal. All patients with kidney or lung disease had multiorgan involvement. All patients received corticosteroid therapy, 67% synthetic immunosuppressants, and 16% rituximab. CONCLUSIONS: ER-IgG4 can affect any tissue. Multiorgan involvement was more common in this series, with preference for lungs, eyes and kidneys. An excellent response to steroids is characteristic of the disease, but with a high relapse rate that requires additional immunosuppression.


Assuntos
Humanos , Masculino , Doenças Autoimunes/tratamento farmacológico , Doença Relacionada a Imunoglobulina G4/diagnóstico , Doença Relacionada a Imunoglobulina G4/tratamento farmacológico , Imunoglobulina G , Rituximab/uso terapêutico , Imunossupressores/uso terapêutico , Rim/patologia
6.
Rev. méd. Chile ; 150(4): 505-511, abr. 2022. ilus, tab
Artigo em Espanhol | LILACS | ID: biblio-1409818

RESUMO

This article reviews the pulmonary manifestations of anti-neutrophil cytoplasmic antibody associated vasculitis (AAV). Its frequency in the different phenotypes of the disease, clinical manifestations and updated therapeutic recommendations are reviewed, aiming to alert the medical community about the existence of these diseases. We pretend to stimulate a timely suspicion, diagnostic precision, and the implementation of effective therapies, to reduce the eventual sequelae derived from a diagnostic omission or an inappropriate treatment for the different clinical scenarios in which these diseases appear.


Assuntos
Humanos , Anticorpos Anticitoplasma de Neutrófilos , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/complicações , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/diagnóstico , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/tratamento farmacológico , Pulmão
7.
Rev Med Chil ; 150(6): 705-710, 2022 Jun.
Artigo em Espanhol | MEDLINE | ID: mdl-37906904

RESUMO

BACKGROUND: IgG4-related disease (IgG4 RD) is an immune-mediated fibro-inflammatory disorder, with tissue infiltration of IgG4+ plasma cells. It causes pseudotumors, tumors, and a wide spectrum of clinical manifestations. AIM: To report the clinical, laboratory, histopathological and treatment characteristics of a group of Chilean patients with IgG4 RD. MATERIAL AND METHODS: Review of medical records of 52 patients aged 18 to 76 years with IgG4 RD seen at six medical centers. RESULTS: Elevated IgG4 serum levels (> 135 mg/dl) were found in 18 of 44 (41%) patients. There was histological confirmation of the disease in 46 patients. The most common sites of involvement were lungs, eyes and kidneys. Eighteen (35%) patients had only one organ involved, 34 (65%) patients had two organs and 13 (25%) patients had three or more organs. The involvement of two organs was significantly more common in men (p < 0.05). In patients with only one organ involvement, the most frequent location was orbital and meningeal. All patients with kidney or lung disease had multiorgan involvement. All patients received corticosteroid therapy, 67% synthetic immunosuppressants, and 16% rituximab. CONCLUSIONS: ER-IgG4 can affect any tissue. Multiorgan involvement was more common in this series, with preference for lungs, eyes and kidneys. An excellent response to steroids is characteristic of the disease, but with a high relapse rate that requires additional immunosuppression.


Assuntos
Doenças Autoimunes , Doença Relacionada a Imunoglobulina G4 , Masculino , Humanos , Doença Relacionada a Imunoglobulina G4/diagnóstico , Doença Relacionada a Imunoglobulina G4/tratamento farmacológico , Imunossupressores/uso terapêutico , Imunoglobulina G , Rituximab/uso terapêutico , Rim/patologia , Doenças Autoimunes/tratamento farmacológico
8.
Rev Med Chil ; 150(6): 720-726, 2022 Jun.
Artigo em Espanhol | MEDLINE | ID: mdl-37906906

RESUMO

BACKGROUND: Giant cell Arteritis (GCA) is the most common systemic vasculitis in patients over 50 years. Diagnosis is based on clinical, laboratory, imaging and biopsy. Temporal artery biopsy (TAB) may be inconclusive in up to 40% of patients. AIM: To describe disease features of patients diagnosed with GCA. MATERIAL AND METHODS: Review of pathology reports of giant cell arteritis and clinical records of patients seen with the diagnosis between 2000 and 2019. Demographic, clinical, laboratory, histopathology, imaging, treatment and follow-up variables were analyzed. RESULTS: We fetched 32 patients with a median age at diagnosis of 70.5 years (range 57-90), 81% women. Twenty eight percent had polymyalgia. 72% had only cranial symptoms, 12% had extracranial involvement and 13% exclusive extracranial involvement. The median time from onset of symptoms to diagnosis was two months (range 0.5-8). All had elevated erythrocyte sedimentation rate and c reactive protein. A TAB was performed in 27 patients and in 17 (65.4%) it confirmed the diagnosis. Transmural inflam- mation was the most frequent finding. All patients received steroids. Follow-up information was available from 25 patients and 92% received a steroid-spa- ring agent, usually methotrexate (74%). Ninety two percent achieved clinical remission in the first year and 59% had minor relapses during steroid tapering. CONCLUSIONS: Our patients showed frequent extracranial involvement and TAB was a useful diagnostic tool.


Assuntos
Arterite de Células Gigantes , Humanos , Feminino , Pessoa de Meia-Idade , Idoso , Idoso de 80 Anos ou mais , Masculino , Arterite de Células Gigantes/diagnóstico , Arterite de Células Gigantes/tratamento farmacológico , Arterite de Células Gigantes/patologia , Artérias Temporais , Metotrexato/uso terapêutico , Proteína C-Reativa , Biópsia , Esteroides/uso terapêutico , Estudos Retrospectivos
9.
Am J Case Rep ; 22: e932751, 2021 Sep 10.
Artigo em Inglês | MEDLINE | ID: mdl-34504052

RESUMO

BACKGROUND Manifestations of Coronavirus disease 2019 (COVID-19), caused by the SARS-CoV-2 virus, are highly variable among healthy populations. In connective tissue disease patients, the spectrum of clinical manifestations is even broader. In mild COVID-19 patients, diffuse lymphadenopathy (DL) has not been described as a late manifestation, and only severe COVID-19 has been associated with lupus flare-ups. Herein, we report 3 cases of connective tissue disease patients that presented with DL after diagnosis and complete resolution of mild COVID-19 disease. CASE REPORT Case 1. A 28-year-old man with inactive lupus, mixed connective tissue disease (MCTD), and a history of lung and cutaneous involvement. He presented with fever, polyarthralgia, and multiple lymphadenopathies 3 weeks after COVID-19 disease resolution. After evaluation, immunosuppressive treatment was initiated, with rapid response. Case 2. A 25-year-old woman with inactive lupus with a history of articular, hematologic, and cutaneous involvement. Four weeks after resolution of COVID-19 disease, she presented with malaise and cervical lymphadenopathies. After laboratory testing and imaging, she was treated for lupus flare-up, with rapid response. Case 3. A 68-year-old woman with inactive lupus with a history of articular and cutaneous involvement. Four weeks after COVID-19 resolution, she presented with malaise and cervical and axillary lymphadenopathies. After extensive evaluation, immunosuppressive treatment resulted in a rapid response. CONCLUSIONS After 3 to 4 weeks of mild, outpatient-treated COVID-19 and complete resolution of symptoms, 3 patients with connective tissue disease presented diffuse lymphadenopathy associated with inflammatory and constitutional symptoms. Infectious and neoplastic causes were thoroughly ruled out. All patients responded to reintroduction of or an increase in immunosuppressive therapy. We recommend considering the diffuse lymphadenopathy as a possible post-acute COVID-19 syndrome (PACS) manifestation in these patients, mainly when they are in the inactive phase.


Assuntos
Complexo Relacionado com a AIDS , COVID-19 , Lúpus Eritematoso Sistêmico , Doença Mista do Tecido Conjuntivo , Adulto , Idoso , Feminino , Humanos , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/diagnóstico , Masculino , Doença Mista do Tecido Conjuntivo/complicações , SARS-CoV-2 , Exacerbação dos Sintomas
10.
Arthritis Care Res (Hoboken) ; 73(8): 1210-1218, 2021 08.
Artigo em Inglês | MEDLINE | ID: mdl-32407572

RESUMO

OBJECTIVE: Sjögren's syndrome (SS) challenges everyday functioning and well-being. The aim of this study was to structure and summarize the life experiences of Chilean women with SS in an integrated model. METHODS: Interviews from a previous study yielded 75 experiences of living with SS. A sample of 30 women with SS sorted these experiences by content and rated their level of agreement with each experience. A hierarchical cluster analysis was used to structure the experiences of the participants with SS in a comprehensive overview. A team-based consensus analysis was used to define the number of clusters. The level of agreement was examined with Wilcoxon's signed rank test. RESULTS: Ten clusters were identified and grouped into 6 main categories: symptoms (clusters: mucosal dryness and related symptoms), social environment, emotion management (clusters: fears and sadness), information (clusters: uncertainty and lack of knowledge), coping strategy (clusters: resilience and self-care), and health staff relationship. The clusters that describe the more common experiences among patients were resilience, self-care, uncertainty, lack of knowledge, health staff relationship, and mucosal dryness. CONCLUSION: This study provided an integrated and structured overview of disease experiences comprising both biomedical and psychosocial aspects as being of vital importance for the health of patients with SS. The overview can be used to get a quick impression of disease experiences that are important for an individual patient, in a therapeutic goal setting, and in the construction and evaluation of medical and nonmedical interventions or education.


Assuntos
Efeitos Psicossociais da Doença , Acontecimentos que Mudam a Vida , Síndrome de Sjogren/complicações , Saúde da Mulher , Adaptação Psicológica , Adulto , Idoso , Atitude do Pessoal de Saúde , Lista de Checagem , Chile , Análise por Conglomerados , Feminino , Conhecimentos, Atitudes e Prática em Saúde , Nível de Saúde , Humanos , Entrevistas como Assunto , Saúde Mental , Pessoa de Meia-Idade , Educação de Pacientes como Assunto , Relações Profissional-Paciente , Qualidade de Vida , Síndrome de Sjogren/diagnóstico , Síndrome de Sjogren/fisiopatologia , Síndrome de Sjogren/psicologia
11.
Artigo em Inglês | MEDLINE | ID: mdl-33093767

RESUMO

OBJECTIVE: To describe the clinical and serological patients characteristics with Microscopic Polyangiitis (MPA) and Interstitial lung disease (ILD). METHODS: Of all the patients with AAV diagnosed between 2007-2017 at the Hospital Clinico Universidad de Chile, those with MPA and ILD were selected and studied retrospectively. RESULTS: All patients were Hispanic; median age at diagnosis 65 years (32-84). 59% were female. All were positive for p-ANCA, 16 patients for MPO. Most common manifestations were constitutional symptoms, weight loss and fever. CT-Scans patterns were Usual Interstitial Pneumonia (UIP) in 10 patients, Nonspecific Interstitial Pneumonia (NSIP) in 6 and fibrosis not UIP or NSIP pattern in 1. In 6 cases, ILD was diagnosed 0.5-14 years before MPA and concomitantly in 11. CONCLUSIONS: Although infrequent, Microscopic Polyangiitis should be suspected in patients with ILD particularly if extra-pulmonary manifestations that rise the possibility of a systemic illness are present, regardless of the time elapsed between the latter and the diagnosis of this type of lung involvement. (Sarcoidosis Vasc Diffuse Lung Dis 2020; 37 (1): 37-42).


Assuntos
Anticorpos Anticitoplasma de Neutrófilos/sangue , Doenças Pulmonares Intersticiais/sangue , Poliangiite Microscópica/sangue , Peroxidase/imunologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Biomarcadores/sangue , Chile , Feminino , Humanos , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/imunologia , Masculino , Poliangiite Microscópica/diagnóstico , Poliangiite Microscópica/imunologia , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Prognóstico , Estudos Retrospectivos , Testes Sorológicos , Tomografia Computadorizada por Raios X
12.
Rev. méd. Chile ; 148(6): 755-761, jun. 2020. tab, graf
Artigo em Espanhol | LILACS | ID: biblio-1139368

RESUMO

Background: Cumulative survival in patients with anti-neutrophil cytoplasmic antibodies (ANCA) associated vasculitis (VAA) is 88 and 78% at 1 and 5 years, respectively. Despite this, mortality continues to be 2.7 times higher than the general population. Differences in the clinical profile of VAA in different ethnicities have been observed. Aim: To identify factors at the time of diagnosis, associated with mortality at one year of follow-up and to describe the clinical characteristics of these patients. Material and Methods: We identified in local databases and reviewed clinical records of patients with VAA with at least one year of follow up in a clinical hospital. Demographic and laboratory parameters and clinical activity scores were analyzed. Results: Of 103 patients with VAA identified, 65 met the inclusion criteria and were analyzed. Their age ranged from 45 to 63 years and 56% were women. Thirty-five patients (54%) were diagnosed as granulomatosis with Polyangiitis (GPA) and 30 patients (46%) with Microscopic Polyangiitis (MPA). The frequency of renal disease was 53% and pulmonary involvement occurred in 72%. At one year of follow-up 11 patients died resulting in a mortality of 17%. Seven patients died within three months after diagnosis. MPO ANCA were more common than PR3 ANCA. In the multivariate analysis, the presence of ophthalmological involvement, lung kidney syndrome and a Five Factor Score (FFS) of 1 or more were independent factors associated with mortality at one year. Conclusions: In these patients, pulmonary manifestations predominate. Lung kidney syndrome, ophthalmological involvement and a FFS score ≥ 1 were associated with mortality.


Assuntos
Humanos , Masculino , Feminino , Pessoa de Meia-Idade , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos , Granulomatose com Poliangiite , Estudos Retrospectivos , Peroxidase , Anticorpos Anticitoplasma de Neutrófilos , Mieloblastina
13.
Rev Med Chil ; 148(6): 755-761, 2020 Jun.
Artigo em Espanhol | MEDLINE | ID: mdl-33480373

RESUMO

BACKGROUND: Cumulative survival in patients with anti-neutrophil cytoplasmic antibodies (ANCA) associated vasculitis (VAA) is 88 and 78% at 1 and 5 years, respectively. Despite this, mortality continues to be 2.7 times higher than the general population. Differences in the clinical profile of VAA in different ethnicities have been observed. AIM: To identify factors at the time of diagnosis, associated with mortality at one year of follow-up and to describe the clinical characteristics of these patients. MATERIAL AND METHODS: We identified in local databases and reviewed clinical records of patients with VAA with at least one year of follow up in a clinical hospital. Demographic and laboratory parameters and clinical activity scores were analyzed. RESULTS: Of 103 patients with VAA identified, 65 met the inclusion criteria and were analyzed. Their age ranged from 45 to 63 years and 56% were women. Thirty-five patients (54%) were diagnosed as granulomatosis with Polyangiitis (GPA) and 30 patients (46%) with Microscopic Polyangiitis (MPA). The frequency of renal disease was 53% and pulmonary involvement occurred in 72%. At one year of follow-up 11 patients died resulting in a mortality of 17%. Seven patients died within three months after diagnosis. MPO ANCA were more common than PR3 ANCA. In the multivariate analysis, the presence of ophthalmological involvement, lung kidney syndrome and a Five Factor Score (FFS) of 1 or more were independent factors associated with mortality at one year. CONCLUSIONS: In these patients, pulmonary manifestations predominate. Lung kidney syndrome, ophthalmological involvement and a FFS score ≥ 1 were associated with mortality.


Assuntos
Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos , Anticorpos Anticitoplasma de Neutrófilos , Feminino , Granulomatose com Poliangiite , Humanos , Masculino , Pessoa de Meia-Idade , Mieloblastina , Peroxidase , Estudos Retrospectivos
14.
Musculoskeletal Care ; 14(4): 233-242, 2016 12.
Artigo em Inglês | MEDLINE | ID: mdl-27061842

RESUMO

BACKGROUND: Sjögren's syndrome and the associated dryness can have multiple consequences. The aim of the present qualitative study was to give an in-depth account of the life experiences of women with primary Sjögren's syndrome (pSS) and health-related behaviours, and to summarize these experiences in an integrated model. METHODS: Twelve women diagnosed with pSS who regularly attended the Hospital of the University of Chile participated in detailed interviews. The data were analysed using qualitative methods based on the principles of grounded theory. RESULTS: Selective coding identified three categories: illness experience, social interaction and psychological response. An integrated model was developed connecting these dynamic aspects and suggesting how they could lead to a life cycle crisis in cases of maladjustment. We found that problem-solving strategies, reconstruction of identity, acceptance and a social support may prevent this life cycle crisis. DISCUSSION: Xerostomia and other consequences of pSS can have a profound influence on daily life. However, the severity of the consequences depends on individual experiences with the illness, social influences and the psychological responses of the patient. Physicians, dentists and other healthcare professionals can help the patient by listening to their problems and exploring solutions based on a psychological approach.


Assuntos
Síndrome de Sjogren/psicologia , Adaptação Psicológica , Idoso , Feminino , Humanos , Pessoa de Meia-Idade , Síndrome de Sjogren/fisiopatologia , Ajustamento Social
15.
Rev Med Chil ; 142(7): 924-9, 2014 Jul.
Artigo em Espanhol | MEDLINE | ID: mdl-25378014

RESUMO

Aortitis is a nonspecific term that describes an inflammation of the aortic wall caused by inflammatory, infectious, paraneoplastic and idiopathic diseases. The symptoms are variable and nonspecific; therefore a high level of clinical suspicion is required to diagnose it. It is often an incidental finding while looking for other diagnoses and it is confirmed mainly through imaging studies. We report three cases of aortitis: A 29-year-old woman presenting with alopecia, oral and nasal ulcers and positive antinuclear antibodies. A CAT scan showed a segmental thickening of thoracic aorta, with dilated and stenotic areas. She was successfully treated with steroids, hydroxychloroquine, cyclophosphamide and azathioprine. A 41-year-old male presenting with dorsal pain and cough. The CAT scan showed an extra-intimal thickening of the descending aorta and stenosis of the celiac artery. The final diagnosis was a polyangiitis and was treated with steroids, cyclophosphamide and azathioprine. A 28-year-old woman presenting with pain in the left upper abdomen. Imaging studies showed a thickening of the aortic arch and subclavian artery. The final diagnosis was sarcoidosis and the patient was treated with prednisone.


Assuntos
Aortite/diagnóstico por imagem , Adulto , Aortite/etiologia , Feminino , Humanos , Masculino , Tomografia Computadorizada por Raios X
16.
Rev. méd. Chile ; 142(7): 924-929, jul. 2014. ilus
Artigo em Espanhol | LILACS | ID: lil-726182

RESUMO

Aortitis is a nonspecific term that describes an inflammation of the aortic wall caused by inflammatory, infectious, paraneoplastic and idiopathic diseases. The symptoms are variable and nonspecific; therefore a high level of clinical suspicion is required to diagnose it. It is often an incidental finding while looking for other diagnoses and it is confirmed mainly through imaging studies. We report three cases of aortitis: A 29-year-old woman presenting with alopecia, oral and nasal ulcers and positive antinuclear antibodies. A CAT scan showed a segmental thickening of thoracic aorta, with dilated and stenotic areas. She was successfully treated with steroids, hydroxychloroquine, cyclophosphamide and azathioprine. A 41-year-old male presenting with dorsal pain and cough. The CAT scan showed an extra-intimal thickening of the descending aorta and stenosis of the celiac artery. The final diagnosis was a polyangiitis and was treated with steroids, cyclophosphamide and azathioprine. A 28-year-old woman presenting with pain in the left upper abdomen. Imaging studies showed a thickening of the aortic arch and subclavian artery. The final diagnosis was sarcoidosis and the patient was treated with prednisone.


Assuntos
Adulto , Feminino , Humanos , Masculino , Aortite , Aortite/etiologia , Tomografia Computadorizada por Raios X
17.
Rheumatol Int ; 32(6): 1819-25, 2012 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-21769486

RESUMO

Citrullinated vimentin (cVIM) is one of the antigens specifically targeted by anti-citrullinated protein antibodies (ACPA) in rheumatoid arthritis (RA) patients. The association between ACPA and certain HLA-DRB1 alleles, those coding for the shared epitope (SE), suggests that this response could be T-cell mediated. HLA-DR9 alleles, which do not code for the SE, have recently been associated with ACPA (+) RA. The objective of this work was to study CD4+ T cell responses to cVIM in RA patients and healthy controls carrying HLA-DR9 alleles. Fourteen RA patients and ten healthy controls previously genotyped for HLA-DRB1 were studied for the presence of serum anti-cVIM antibodies by Western blot and ELISA. Peripheral blood mononuclear cells were stimulated with native vimentin and cVIM, and CD4+ T cells proliferation was assessed by flow cytometry. Citrulline-specific CD4+ T cells proliferation was found not only in RA patients but also in healthy controls. Although most patients carrying HLA-DR9 alleles present anti-cVIM antibodies, HLA-DR9 alleles were associated with weaker cVIM-driven CD4+ T-cell responses among RA patients. These results suggest that HLA-DR9 alleles could exert a protective effect on the recognition of cVIM epitopes by CD4+ T cells. In this context, other citrullinated proteins may break T and B cell tolerance, with cVIM only acting as a cross-reactive target for ACPA.


Assuntos
Artrite Reumatoide/genética , Artrite Reumatoide/imunologia , Linfócitos T CD4-Positivos/imunologia , Citrulina/imunologia , Subtipos Sorológicos de HLA-DR/genética , Vimentina/imunologia , Adulto , Idoso , Autoanticorpos/sangue , Western Blotting , Estudos de Casos e Controles , Proliferação de Células , Células Cultivadas , Chile , Ensaio de Imunoadsorção Enzimática , Feminino , Citometria de Fluxo , Genótipo , Humanos , Epitopos Imunodominantes , Ativação Linfocitária , Pessoa de Meia-Idade , Fenótipo
18.
Immunobiology ; 216(12): 1256-63, 2011 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-21840621

RESUMO

The aim of this work was to study the effect of anti-TNF treatment on CD4+ Th1, Th17 and regulatory T cells (Tregs), together with CD8+ T cells and NK cells from rheumatoid arthritis (RA) patients. For this purpose, 18 RA patients received adalimumab during 16weeks and their peripheral blood lymphocytes were assessed by flow cytometry at the beginning and at the end of the study. We found that the proportion of Th17 cells was directly correlated with Th1 cells, but inversely correlated with IFN-γ-producing NK cells. A decrease was observed in Th1, Th17 cells and IFN-γ-producing CD8+ T cells by anti-TNF therapy. Conversely, the proportion of Tregs increased, as did the percentage of IFN-γ-producing NK cells. We postulate that a rise in IFN-γ production due to recovery of NK cells' function, together with expanded Tregs, contribute to decrease the Th17 response in anti-TNF-treated RA patients.


Assuntos
Anticorpos Monoclonais Humanizados/administração & dosagem , Artrite Reumatoide/tratamento farmacológico , Artrite Reumatoide/imunologia , Imunoterapia , Fator de Necrose Tumoral alfa/imunologia , Adalimumab , Adulto , Anticorpos Monoclonais Humanizados/efeitos adversos , Contagem de Células , Separação Celular , Feminino , Citometria de Fluxo , Humanos , Imunofenotipagem , Interferon gama/metabolismo , Células Matadoras Naturais/imunologia , Células Matadoras Naturais/metabolismo , Células Matadoras Naturais/patologia , Pessoa de Meia-Idade , Subpopulações de Linfócitos T/imunologia , Subpopulações de Linfócitos T/metabolismo , Subpopulações de Linfócitos T/patologia , Linfócitos T Reguladores/imunologia , Linfócitos T Reguladores/metabolismo , Linfócitos T Reguladores/patologia , Células Th1/imunologia , Células Th1/metabolismo , Células Th1/patologia , Células Th17/imunologia , Células Th17/metabolismo , Células Th17/patologia
19.
Clin Rheumatol ; 30(3): 391-5, 2011 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-21234628

RESUMO

The introduction of antitumor necrosis factor (TNF) agents has improved the outcome for many patients with rheumatoid arthritis (RA). To date, the only replicated genetic predictor of anti-TNF response is the -308 G > A single-nucleotide polymorphism in the TNF promoter region. The presence of the -308 TNF G/G genotype appears to be a marker of good response to anti-TNF treatment. Anti-citrullinated protein antibodies (ACPA) have been linked with erosive disease, and have been established as the single most reliable prognostic factor in clinical practice. To test the hypothesis that the ACPA status may affect the -308 G/G patients rate of response to TNF blockade, we prospectively investigated a group of 52 RA patients with the -308 G/G genotype who were ACPA (+) or ACPA (-). All patients were treated with adalimumab, and the clinical response was studied using the Disease Activity Score in 28 joints (DAS28) at 24 weeks of treatment. Over 85% of patients were DAS28 responders in both groups. No significant differences were found between patients from both groups, according to the DAS28 criteria of response at week 24 (p = 0.79). In conclusion, our findings suggest that the ACPA status does not affect the clinical response to anti-TNF therapy in -308 TNF G/G patients.


Assuntos
Anticorpos Monoclonais/uso terapêutico , Artrite Reumatoide , Autoanticorpos/sangue , Peptídeos Cíclicos/sangue , Polimorfismo Genético , Regiões Promotoras Genéticas , Fator de Necrose Tumoral alfa/genética , Adalimumab , Adulto , Anticorpos Monoclonais Humanizados , Antirreumáticos/uso terapêutico , Artrite Reumatoide/tratamento farmacológico , Artrite Reumatoide/genética , Artrite Reumatoide/imunologia , Feminino , Humanos , Pessoa de Meia-Idade , Índice de Gravidade de Doença , Resultado do Tratamento
20.
Arthritis Res Ther ; 12(2): R68, 2010.
Artigo em Inglês | MEDLINE | ID: mdl-20398308

RESUMO

INTRODUCTION: Several molecules help preserve peripheral B cell tolerance, but when altered, they may predispose to autoimmunity. This work studied the expression of the costimulatory molecule CD86 and the inhibitory receptor for IgG immune complexes FcgammaRIIb (CD32b), on B cells from rheumatoid arthritis (RA) patients, and the influence of anti-tumor necrosis factor (TNF) therapy. METHODS: Peripheral B cells from 18 RA patients and 13 healthy donors were characterized using flow cytometry. Eleven patients who underwent a six-month adalimumab therapy were further assessed for phenotypic changes on their B cells. RESULTS: RA patients exhibited a high percentage of naïve and memory B cells expressing CD86. In contrast, expression of FcgammaRIIb was significantly reduced on RA memory B cells and plasmablasts as compared to healthy donors, probably due to downregulation of this receptor when differentiating from naïve to memory cells. These alterations on FcgammaRIIb were associated with high levels of anti-citrullinated vimentin autoantibodies. In addition, treatment with adalimumab normalized the expression of CD86 on memory B cells and reduced the expression of FcgammaRIIb, mainly on naïve B cells. CONCLUSIONS: Our findings show that peripheral B cells from RA patients have an altered expression of key molecules, such as CD86 and FcgammaRIIb. Because this latter receptor is required for feedback inhibition, a deficient expression might contribute to humoral autoimmune responses. Furthermore, these molecules are likely to be influenced by inflammatory factors, since they were modulated by TNF inhibition.


Assuntos
Anticorpos Monoclonais/uso terapêutico , Antirreumáticos/uso terapêutico , Artrite Reumatoide/tratamento farmacológico , Linfócitos B/efeitos dos fármacos , Antígeno B7-2/metabolismo , Receptores de IgG/metabolismo , Adalimumab , Anticorpos Monoclonais Humanizados , Artrite Reumatoide/imunologia , Artrite Reumatoide/metabolismo , Autoanticorpos/sangue , Linfócitos B/imunologia , Linfócitos B/metabolismo , Feminino , Humanos , Articulações/fisiopatologia , Pessoa de Meia-Idade , Peptídeos Cíclicos/imunologia , Índice de Gravidade de Doença , Vimentina/imunologia
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