Your browser doesn't support javascript.
loading
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 1.470
Filtrar
1.
BMJ Case Rep ; 14(2)2021 Feb 01.
Artigo em Inglês | MEDLINE | ID: mdl-33526532

RESUMO

Polyangiitis overlap syndrome (POS) is a diagnostic term coined by Leavitt and Fauci that characterises patients with overlapping features of more than one vasculitis. Prior case studies of antineutrophil cytoplasmic antibodies (ANCA)-associated POS have only been published in patients with eosinophilic granulomatosis with polyangiitis (EGPA) and granulomatosis with polyangiitis alongside proteinase-3/cytoplasmic (C)-ANCA positivity. We present a case of a 60-year-old woman with dyspnoea, hemoptysis, positive perinuclear-ANCA and renal biopsy demonstrating evidence of microscopic polyangiitis. In addition, our patient also had asthma, mononeuritis multiplex, eosinophilia and migratory pulmonary infiltrates, thus fulfilling the criteria for EGPA. This novel case report suggests that POS is not limited to C-ANCA positivity and has variable presentations.


Assuntos
Síndrome de Churg-Strauss/diagnóstico , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Poliangiite Microscópica/diagnóstico , Mononeuropatias/fisiopatologia , Insuficiência Renal Crônica/fisiopatologia , Anticorpos Anticitoplasma de Neutrófilos/imunologia , Azatioprina/uso terapêutico , Síndrome de Churg-Strauss/tratamento farmacológico , Síndrome de Churg-Strauss/imunologia , Síndrome de Churg-Strauss/fisiopatologia , Ciclofosfamida/uso terapêutico , Feminino , Humanos , Imunossupressores/uso terapêutico , Poliangiite Microscópica/tratamento farmacológico , Poliangiite Microscópica/imunologia , Poliangiite Microscópica/fisiopatologia , Pessoa de Meia-Idade , Peroxidase/imunologia , Prednisona/uso terapêutico , Insuficiência Renal Crônica/patologia , Tomografia Computadorizada por Raios X
2.
Yonsei Med J ; 62(2): 149-158, 2021 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-33527794

RESUMO

PURPOSE: We investigated whether antineutrophil cytoplasmic antibody (ANCA) positivity is associated with vascular manifestations at diagnosis of Behçet's disease (BD) and poor outcomes during follow-up. MATERIALS AND METHODS: We retrospectively reviewed the medical records of 1060 patients with BD. Among them, 808 patients could be diagnosed with BD based on the revised version of the International Criteria for Behçet's Disease (ICBD) in 2014 (2014 ICBD criteria) and 588 patients could be diagnosed with BD based on the International Study Group (ISG) criteria proposed in 1990 (1990 ISG criteria). We examined the sites and patterns of vascular involvement in the BD patients at diagnosis and evaluated adverse outcomes during follow up, such as all-cause mortality, acute coronary syndrome, and deep vein thrombosis. RESULTS: Among the 808 patients with BD based on the 2014 ICBD criteria, the rate of ANCA positivity at diagnosis was 2.2%. ANCA-positive BD patients exhibited a higher frequency of overall vascular manifestations (22.2% vs. 6.1%) and higher frequencies of vascular involvement in the upper extremities and visceral arteries than ANCA-negative BD patients (5.6% vs. 0.1% and 5.6% vs. 0.1%). Among the 588 BD patients based on the 1990 ISG criteria, similarly, ANCA-positive BD patients exhibited a higher frequency of vascular manifestations than ANCA-negative BD patients. ANCA positivity, however, did not seem to be associated with poor outcomes in BD patients during follow up. CONCLUSION: ANCA positivity in BD patients was found to be associated with cross-sectional vascular involvement in the upper extremities and visceral arteries at diagnosis but was not predictive of poor outcomes during follow-up.


Assuntos
Anticorpos Anticitoplasma de Neutrófilos/imunologia , Síndrome de Behçet/imunologia , Vasos Sanguíneos/patologia , Adulto , Síndrome de Behçet/diagnóstico , Estudos Transversais , Feminino , Seguimentos , Humanos , Masculino , Avaliação de Resultados em Cuidados de Saúde , Estudos Retrospectivos
3.
BMJ Case Rep ; 14(1)2021 Jan 25.
Artigo em Inglês | MEDLINE | ID: mdl-33495169

RESUMO

Infective endocarditis is associated with a variety of clinical signs, but its association with multisystem vasculitis is rarely reported. A high index of suspicion is necessary to differentiate a primary autoimmune vasculitis from an infectious cause as the wrong treatment can lead to significant morbidity and mortality. We present a 71-year-old female patient with negative blood cultures, on antibiotics for recent bacteraemia, who presented with cutaneous and renal leucocytoclastic vasculitis. Workup revealed a vegetation adjacent to her right atrial pacemaker lead consistent with infective endocarditis and her vasculitis completely resolved with appropriate antibiotics.


Assuntos
Lesão Renal Aguda/diagnóstico , Endocardite Bacteriana/diagnóstico , Dermatopatias Vasculares/diagnóstico , Infecções Estafilocócicas/diagnóstico , Vasculite/diagnóstico , Lesão Renal Aguda/etiologia , Lesão Renal Aguda/imunologia , Lesão Renal Aguda/terapia , Idoso , Antibacterianos/uso terapêutico , Anticorpos Anticitoplasma de Neutrófilos/imunologia , Bacteriemia/complicações , Bacteriemia/tratamento farmacológico , Ceftriaxona/uso terapêutico , Endocardite Bacteriana/complicações , Endocardite Bacteriana/tratamento farmacológico , Feminino , Humanos , Edema Pulmonar/etiologia , Edema Pulmonar/terapia , Diálise Renal , Insuficiência Respiratória/etiologia , Insuficiência Respiratória/terapia , Rifampina/uso terapêutico , Dermatopatias Vasculares/etiologia , Dermatopatias Vasculares/imunologia , Dermatopatias Vasculares/patologia , Infecções Estafilocócicas/complicações , Infecções Estafilocócicas/tratamento farmacológico , Vasculite/etiologia , Vasculite/imunologia , Vasculite/patologia
5.
Scand J Immunol ; 92(5): e12958, 2020 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-32794199

RESUMO

ANCA-associated vasculitis (AAV) is a group of chronic inflammatory diseases of small- and medium-sized vessels, which are broadly subdivided based on organ manifestations and disease-specific autoantibodies. The so called anti-neutrophil cytoplasmic antibodies (ANCA) mostly target one of the enzymes, proteinase 3 (PR3) or myeloperoxidase (MPO). Accumulating genetic data demonstrates that these two autoantibodies discriminate two distinct disease entities, more so than the clinical subdivision which is mainly criteria-based. Treatment of AAV includes heavy immunosuppression and is guided by which organs that are involved. Generally, patients with PR3-ANCA display higher risk for disease relapse than patients with MPO-ANCA. In this review, we will focus on the autoimmune features of PR3+ AAV and our current understanding of its triggers and the potential translation into clinical practice.


Assuntos
Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/imunologia , Anticorpos Anticitoplasma de Neutrófilos/imunologia , Mieloblastina/imunologia , Peroxidase/imunologia , Animais , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/metabolismo , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/patologia , Cadeias beta de HLA-DP/imunologia , Cadeias beta de HLA-DP/metabolismo , Humanos , Inflamação/imunologia , Inflamação/metabolismo , Modelos Imunológicos , Mieloblastina/metabolismo , Peroxidase/metabolismo , Subpopulações de Linfócitos T/imunologia , Subpopulações de Linfócitos T/metabolismo
6.
PLoS One ; 15(7): e0236017, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32658915

RESUMO

BACKGROUND: Rapidly progressive glomerulonephritis (RPGN) has been known to have a poor prognosis. Although evidence across adult RPGN cases has accumulated over many years, the number of case series in adolescents and young adults has been limited, requiring further studies. METHODS: A total of 1,766 cases from 1989 to 2007 were included in this nationwide questionnaire survey, led by Intractable (former name, Progressive) Renal Diseases Research, Research on intractable disease, from the Ministry of Health, Labour and Welfare of Japan. To elucidate age-related differences in 2-year patient and renal survival rates, the cases were divided into the following four groups: children (0-18 years), young adults (19-39 years), the middle-aged (40-64 years), and the elderly (over 65 years). RESULTS: Of the 1,766 total RPGN cases, antineutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis comprised 1,128 cases (63.9% of all RPGN cases), showing a tendency to increase with age. Two-year patient survival for RPGN was 93.9% among children, 92.6% in young adults, 83.2% in the middle-aged, and 68.8% in the elderly. The younger group (children plus young adults) showed a clearly higher survival rate compared to the older group (middle-aged plus elderly) (p<0.05). ANCA-associated glomerulonephritis also showed similar age-related results with all RPGN cases. The comparison of renal prognosis showed no statistically significant differences both in RPGN and in ANCA-associated GN. CONCLUSION: The present study described the age-dependent characteristics of the classification of RPGN, especially focusing on a better prognosis of the younger group in patient survival both in RPGN and in ANCA-associated GN.


Assuntos
Anticorpos Anticitoplasma de Neutrófilos/imunologia , Glomerulonefrite/mortalidade , Adolescente , Adulto , Idoso , Criança , Pré-Escolar , Progressão da Doença , Feminino , Glomerulonefrite/epidemiologia , Glomerulonefrite/imunologia , Humanos , Lactente , Recém-Nascido , Japão/epidemiologia , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Retrospectivos , Inquéritos e Questionários , Taxa de Sobrevida , Adulto Jovem
7.
Sci Rep ; 10(1): 9466, 2020 06 11.
Artigo em Inglês | MEDLINE | ID: mdl-32528054

RESUMO

Microbial involvement in the pathogenesis have been suggested in both antineutrophil cytoplasmic antibody-associated vasculitis (AAV) and sarcoidosis, both of which have lung involvement. However, exhaustive research to assess the bacteria in the lung in AAV and in sarcoidosis have not been performed. We sought to elucidate the distinct dysbiotic lung microbiota between AAV and sarcoidosis. We used 16S rRNA gene high-throughput sequencing to obtain the bacterial community composition of bronchoalveolar lavage fluid (BALF) in patients with AAV (n = 16) compared to patients with sarcoidosis (n = 21). The patients had not undergone therapy with immunosuppressive medication when their BALF was acquired. No difference was observed in α-diversity between patients with AAV and patients with sarcoidosis when using all the detected taxa. We defined the taxa of the oral cavity by using the data of oral microbiota of healthy individuals from the Human Microbiome Project (HMP). The analysis using only oral taxa made the difference in α-diversity between AAV and sarcoidosis clearer compared with those using all the detected taxa. Besides, the analysis using detected taxa except for oral taxa also made the difference in α-diversity between AAV and sarcoidosis clearer compared with those using all the detected taxa. A linear negative relationship between the α-diversity and Birmingham vasculitis activity score (BVAS) was detected in the AAV group. The observed p-value for the effect of the disease groups on the ß-diversity was small while the effect of other factors including sex and smoking status did not have small p-values. By excluding oral taxa from all the detected taxa, we found a cluster mainly consisted of sarcoidosis patients which was characterized with microbial community monopolized by Erythrobacteraceae family. Our results suggested the importance of considering the influence of oral microbiota in evaluating lung microbiota.


Assuntos
Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/microbiologia , Anticorpos Anticitoplasma de Neutrófilos/imunologia , Bactérias/imunologia , Pulmão/imunologia , Pulmão/microbiologia , Microbiota/imunologia , Sarcoidose/microbiologia , Idoso , Idoso de 80 Anos ou mais , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/imunologia , Bactérias/genética , Líquido da Lavagem Broncoalveolar/imunologia , Líquido da Lavagem Broncoalveolar/microbiologia , Feminino , Humanos , Masculino , Microbiota/genética , Pessoa de Meia-Idade , RNA Ribossômico 16S/genética , Sarcoidose/imunologia
10.
Neurology ; 94(16): e1726-e1737, 2020 04 21.
Artigo em Inglês | MEDLINE | ID: mdl-32217776

RESUMO

OBJECTIVE: To investigate the clinicopathologic features of eosinophilic granulomatosis with polyangiitis (EGPA)-associated neuropathy with a focus on the presence or absence of anti-neutrophil cytoplasmic antibodies (ANCAs). METHODS: We examined the clinical features and pathologic findings of sural nerve biopsy specimens from 82 patients with EGPA-associated neuropathy. Of these patients, 32.9% were myeloperoxidase (MPO)-ANCA positive, and 67.1% were MPO-ANCA negative. PR3-ANCA was negative in all of 78 examined patients. RESULTS: Upper limb symptoms were more frequently reported as initial neuropathic manifestations in the MPO-ANCA-positive group than in the MPO-ANCA-negative group (44.4% vs 14.6%, p < 0.01). The serum levels of C-reactive protein were significantly higher in the MPO-ANCA-positive group than in the MPO-ANCA-negative group (p < 0.05). Sural nerve biopsy specimens showed findings suggestive of vasculitis (i.e., destruction of vascular structures) in epineurial vessels; these results were seen more frequently in the MPO-ANCA-positive group than in the MPO-ANCA-negative group (p < 0.0001). Conversely, the numbers of eosinophils in the lumen of the epineurial vessels (p < 0.01) and epineurial vessels occluded by intraluminal eosinophils (p < 0.05) were higher in the MPO-ANCA-negative group than in the MPO-ANCA-positive group. Furthermore, the incidence of eosinophil infiltration in the endoneurium was higher in the MPO-ANCA-negative group than in the MPO-ANCA-positive group (p < 0.01). CONCLUSIONS: This study suggests that the pathogenesis of EGPA comprises at least 2 distinct mechanisms: ANCA-associated vasculitis resulting in ischemic effects and inflammation, which is prominent in MPO-ANCA-positive patients, and eosinophil-associated vascular occlusion leading to ischemia and eosinophil-associated tissue damage, which is conspicuous in MPO-ANCA-negative patients.


Assuntos
Anticorpos Anticitoplasma de Neutrófilos/imunologia , Síndrome de Churg-Strauss/fisiopatologia , Debilidade Muscular/fisiopatologia , Nervos Periféricos/irrigação sanguínea , Doenças do Sistema Nervoso Periférico/fisiopatologia , Distúrbios Somatossensoriais/fisiopatologia , Idoso , Asma/etiologia , Síndrome de Churg-Strauss/complicações , Síndrome de Churg-Strauss/imunologia , Eletrodiagnóstico , Feminino , Humanos , Nefropatias/etiologia , Extremidade Inferior/inervação , Pneumopatias/etiologia , Imagem por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Debilidade Muscular/etiologia , Mieloblastina/imunologia , Condução Nervosa , Otorrinolaringopatias/genética , Nervos Periféricos/patologia , Doenças do Sistema Nervoso Periférico/etiologia , Doenças do Sistema Nervoso Periférico/imunologia , Doenças do Sistema Nervoso Periférico/patologia , Peroxidase/imunologia , Dermatopatias Vasculares/etiologia , Distúrbios Somatossensoriais/etiologia , Nervo Sural/patologia , Tomografia Computadorizada por Raios X , Extremidade Superior/inervação
12.
Int J Mol Sci ; 21(4)2020 Feb 18.
Artigo em Inglês | MEDLINE | ID: mdl-32085664

RESUMO

Autoantibodies encountered in patients with systemic rheumatic diseases bear clinical significance as a biomarker to help or predict diagnosis, clinical phenotypes, prognosis, and treatment decision-making. Furthermore, evidence has accumulated regarding the active involvement of disease-specific or disease-associated autoantibodies in the pathogenic process beyond simple association with the disease, and such knowledge has become essential for us to better understand the clinical value of autoantibodies as a biomarker. This review will focus on the current update on the autoantibodies of four rheumatic diseases (rheumatoid arthritis, myositis, systemic sclerosis, and anti-neutrophil cytoplasmic antibody associated vasculitis) where there has been a tremendous progress in our understanding on their biological effects and clinical use.


Assuntos
Autoanticorpos/imunologia , Biomarcadores/metabolismo , Doenças Reumáticas/imunologia , Anticorpos Anticitoplasma de Neutrófilos/imunologia , Humanos
13.
Medicine (Baltimore) ; 99(5): e18857, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-32000387

RESUMO

RATIONALE: IgG4-related disease (IgG4-RD) is a slowly progressing inflammatory disease that can involve multiple organ systems. There is considerable overlap between IgG4-RDs and anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). Herein, we present an unusual case of IgG4-associated tubulointerstitial nephritis (IgG4-TIN) and ANCA-associated glomerulonephritis (ANCA-GN) co-occurring with C3 glomerulonephritis (C3GN). PATIENT CONCERNS: A 72-year-old male was admitted to hospital because of fever and fatigue. He was diagnosed with elevated serum creatinine and IgG4 levels, and was positive for ANCA. DIAGNOSIS: Initially, the pathology supported a diagnosis of IgG4-TIN and ANCA-GN; however, further examination revealed he also had C3GN. INTERVENTIONS: The patient was treated with methylprednisolone and cyclophosphamide and received regular follow-up care. OUTCOMES: After treatment, the patient no longer exhibited fever or fatigue and had no complications. The seven-month follow-up showed downward trends in IgG4 and MPO-ANCA levels and stable 24-hour urine protein, serum creatinine levels. LESSONS: Anti-neutrophil cytoplasmic antibody-associated glomerulonephritis and IgG4-associated tubulointerstitial nephritis with C3glomerulonephritis rarely occur simultaneously. Laboratory analysis and pathology are both needed to ensure diagnostic accuracy. However, in this case, the three diseases overlapped to such a large extent that achieving a definitive diagnosis was particularly challenging. Timely and accurate diagnosis is crucial for selecting the best treatment course and optimizing patient outcome.


Assuntos
Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/diagnóstico , Glomerulonefrite/diagnóstico , Nefrite Intersticial/diagnóstico , Idoso , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/tratamento farmacológico , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/imunologia , Anticorpos Anticitoplasma de Neutrófilos/imunologia , Complemento C3/imunologia , Ciclofosfamida/uso terapêutico , Glomerulonefrite/tratamento farmacológico , Glomerulonefrite/imunologia , Glucocorticoides/uso terapêutico , Humanos , Imunoglobulina G/imunologia , Imunossupressores/uso terapêutico , Masculino , Metilprednisolona/uso terapêutico , Nefrite Intersticial/tratamento farmacológico , Nefrite Intersticial/imunologia
15.
J Am Soc Nephrol ; 31(2): 297-307, 2020 02.
Artigo em Inglês | MEDLINE | ID: mdl-31772138

RESUMO

BACKGROUND: Myeloperoxidase-specific ANCA (MPO-ANCA) are implicated in the pathogenesis of vasculitis and GN. Kinins play a major role during acute inflammation by regulating vasodilatation and vascular permeability and by modulating adhesion and migration of leukocytes. Kinin system activation occurs in patients with ANCA vasculitis. Previous studies in animal models of GN and sclerosing kidney diseases have demonstrated protective effects of bradykinin receptor 1 (B1R) blockade via interference with myeloid cell trafficking. METHODS: To investigate the role of B1R in a murine model of MPO-ANCA GN, we evaluated effects of B1R genetic ablation and pharmacologic blockade. We used bone marrow chimeric mice to determine the role of B1R in bone marrow-derived cells (leukocytes) versus nonbone marrow-derived cells. We elucidated mechanisms of B1R effects using in vitro assays for MPO-ANCA-induced neutrophil activation, endothelial adherence, endothelial transmigration, and neutrophil adhesion molecule surface display. RESULTS: B1R deficiency or blockade prevented or markedly reduced ANCA-induced glomerular crescents, necrosis, and leukocyte influx in mice. B1R was not required for in vitro MPO-ANCA-induced neutrophil activation. Leukocyte B1R deficiency, but not endothelial B1R deficiency, decreased glomerular neutrophil infiltration induced by MPO-ANCA in vivo. B1R enhanced ANCA-induced neutrophil endothelial adhesion and transmigration in vitro. ANCA-activated neutrophils exhibited changes in Mac-1 and LFA-1, important regulators of neutrophil endothelial adhesion and transmigration: ANCA-activated neutrophils increased surface expression of Mac-1 and increased shedding of LFA-1, whereas B1R blockade reduced these effects. CONCLUSIONS: The leukocyte B1R plays a critical role in the pathogenesis of MPO-ANCA-induced GN in a mouse model by modulating neutrophil-endothelial interaction. B1R blockade may have potential as a therapy for ANCA GN and vasculitis.


Assuntos
Anticorpos Anticitoplasma de Neutrófilos/imunologia , Glomerulonefrite/etiologia , Peroxidase/imunologia , Receptor B1 da Bradicinina/fisiologia , Animais , Antagonistas de Receptor B1 da Bradicinina/uso terapêutico , Adesão Celular , Modelos Animais de Doenças , Células Endoteliais/fisiologia , Glomerulonefrite/tratamento farmacológico , Camundongos , Camundongos Endogâmicos C57BL , Neutrófilos/fisiologia
17.
Rheumatology (Oxford) ; 59(4): 905-907, 2020 04 01.
Artigo em Inglês | MEDLINE | ID: mdl-31598716

Assuntos
Doenças Pulmonares Intersticiais/diagnóstico , Proteínas de Membrana/genética , Dermatopatias Vasculares/diagnóstico , Doenças Vasculares/diagnóstico , Idade de Início , Anemia , Anticorpos Anticitoplasma de Neutrófilos/imunologia , Anticorpos Antinucleares/imunologia , Artralgia/tratamento farmacológico , Artralgia/genética , Artralgia/imunologia , Sedimentação Sanguínea , Bronquiectasia/diagnóstico , Bronquiectasia/tratamento farmacológico , Bronquiectasia/genética , Bronquiectasia/imunologia , Proteína C-Reativa/imunologia , Pré-Escolar , Tosse , Insuficiência de Crescimento , Feminino , Febre/tratamento farmacológico , Febre/genética , Febre/imunologia , Sequenciamento de Nucleotídeos em Larga Escala , Humanos , Imunoglobulina G/imunologia , Imunossupressores/uso terapêutico , Lactente , Interferon Tipo I , Doenças Pulmonares Intersticiais/tratamento farmacológico , Doenças Pulmonares Intersticiais/genética , Doenças Pulmonares Intersticiais/imunologia , Nódulos Pulmonares Múltiplos/diagnóstico , Nódulos Pulmonares Múltiplos/tratamento farmacológico , Nódulos Pulmonares Múltiplos/genética , Nódulos Pulmonares Múltiplos/imunologia , Osteoartropatia Hipertrófica Primária , Análise de Sequência de DNA , Proteína Amiloide A Sérica , Dermatopatias Vasculares/tratamento farmacológico , Dermatopatias Vasculares/genética , Dermatopatias Vasculares/imunologia , Doenças Vasculares/tratamento farmacológico , Doenças Vasculares/genética , Doenças Vasculares/imunologia
18.
J Clin Rheumatol ; 26(1): 24-32, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-30273264

RESUMO

BACKGROUND/OBJECTIVE: Immunostimulatory drugs including immune checkpoint inhibitors and levamisole can induce inflammatory disease including vasculitis, rashes, tissue necrosis, and arthritis. METHODS: This prospective cohort study determined the 5-year outcomes of cocaine-levamisole-induced inflammatory disease as to outcomes and survival. Thirty-one consecutive cocaine-levamisole autoimmune patients and 45 primary vasculitis patients were characterized as to clinical differentiating features, antineutrophil cytoplasmic antibody (ANCA) status, treatment, the presence of acute and chronic arthritis, and 5-year outcome. RESULTS: Seventy-one percent (22/31) of cocaine-levamisole vasculopathy cases were ANCA positive (86% p-ANCA and 14% c-ANCA), whereas 53% (23/45) of the primary vasculitis were ANCA positive (p = 0.04). The ANCA-positive cocaine-levamisole cohort at onset were characterized by younger age (45 ± 12 vs 53 ± 14 years, p = 0.04), superficial skin necrosis (82% vs 54%, p = 0.036), depressed complement C3 (27% vs 4%, p = 0.33), antiphospholipid antibodies (50% vs 4%, p < 0.001), neutropenia (18% vs 0%, p = 0.044), and elevated antimyeloperoxidase (MPO) antibody levels (100% vs 67%, p < 0.001). Chronic cocaine-levamisole disease was characterized by severe cicatrical deformities of the face and extremities (45.5% vs 8.3%, p = 0.005). Arthralgias (71% vs 82%, p = 0.19) and acute arthritis (33% vs 32%, p = 0.25) were similar between the 2 groups. However, a substantial proportion cocaine-levamisole-induced autoimmune patients (18% vs 0%, p = 0.045) developed a chronic deforming inflammatory arthritis that was rheumatoid factor, anti-cyclic-citrillinated antibody antibody, and HLA-B27 negative, but p-ANCA-and MPO antibody positive. CONCLUSIONS: Patients exposed to cocaine-levamisole may develop serious chronic sequelae including cicatrical cutaneous and facial deformities and an atypical seronegative, p-ANCA and MPO antibody-positive, HLA-B27-negative chronic deforming inflammatory arthritis.


Assuntos
Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/imunologia , Anticorpos Anticitoplasma de Neutrófilos/imunologia , Artrite/induzido quimicamente , Cocaína/efeitos adversos , Levamisol/efeitos adversos , Adulto , Fatores Etários , Idoso , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/epidemiologia , Artralgia/induzido quimicamente , Artralgia/epidemiologia , Artralgia/fisiopatologia , Artrite/imunologia , Doença Crônica , Estudos de Coortes , Feminino , Deformidades Adquiridas da Mão/diagnóstico , Deformidades Adquiridas da Mão/epidemiologia , Hospitais Universitários , Humanos , Masculino , Pessoa de Meia-Idade , New Mexico , Prevalência , Prognóstico , Estudos Prospectivos , Medição de Risco , Índice de Gravidade de Doença , Fatores Sexuais
19.
Am J Kidney Dis ; 75(1): 124-137, 2020 01.
Artigo em Inglês | MEDLINE | ID: mdl-31358311

RESUMO

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of disorders characterized by inflammation and destruction of small- and medium-sized blood vessels and the presence of circulating ANCA. Clinical disease phenotypes include granulomatosis with polyangiitis, microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis, and renal-limited vasculitis. Serologic classification of AAV into proteinase 3-ANCA disease and myeloperoxidase-ANCA disease correlates with a number of disease characteristics. AAV has a predilection for the kidney, with >75% of patients having renal involvement characterized by rapidly progressive glomerulonephritis. The cause and pathogenesis of AAV are multifactorial and influenced by genetics, environmental factors, and responses of the innate and adaptive immune system. Randomized controlled trials in the past 2 decades have refined the therapy of AAV and transformed AAV from a fatal disease to a chronic illness with relapsing course and associated morbidity. This article in AJKD's Core Curriculum in Nephrology series provides a detailed review of the epidemiology, pathogenesis, diagnosis, and advances in the management of AAV.


Assuntos
Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/terapia , Glomerulonefrite/terapia , Glucocorticoides/uso terapêutico , Imunossupressores/uso terapêutico , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/genética , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/imunologia , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/patologia , Anticorpos Anticitoplasma de Neutrófilos/imunologia , Síndrome de Churg-Strauss/genética , Síndrome de Churg-Strauss/imunologia , Síndrome de Churg-Strauss/patologia , Síndrome de Churg-Strauss/terapia , Ciclofosfamida/uso terapêutico , Progressão da Doença , Glomerulonefrite/genética , Glomerulonefrite/imunologia , Glomerulonefrite/patologia , Granulomatose com Poliangiite/genética , Granulomatose com Poliangiite/imunologia , Granulomatose com Poliangiite/patologia , Granulomatose com Poliangiite/terapia , Humanos , Transplante de Rim , Poliangiite Microscópica/genética , Poliangiite Microscópica/imunologia , Poliangiite Microscópica/patologia , Poliangiite Microscópica/terapia , Ácido Micofenólico/uso terapêutico , Mieloblastina/imunologia , Peroxidase/imunologia , Indução de Remissão , Diálise Renal , Rituximab/uso terapêutico
20.
Rheumatology (Oxford) ; 59(5): 959-967, 2020 05 01.
Artigo em Inglês | MEDLINE | ID: mdl-31377800

RESUMO

OBJECTIVES: The therapeutic effects of IVIG in patients with ANCA-associated vasculitis (AAV) have not been established so far. This study aims to estimate the effects of IVIG on AAV by conducting a systematic review and meta-analysis. METHODS: A comprehensive systematic review was conducted in accordance with the guidelines of PRISMA (Preferred Reporting Items for Systematic Reviews And Meta-analyses). PubMed and Google Scholar were used to search for original studies on AAV and collect clinical data before and after IVIG treatment. A meta-analysis of each clinical parameter was performed, and standardized mean difference (SMD) and 95% CI were calculated using the random effects model. RESULTS: A total of 220 studies were identified, and nine met the selection criteria for the meta-analysis. IVIG was administered to active AAV patients as an immunomodulatory therapy in the nine studies selected. Significant reductions in BVAS (SMD -1.7; 95% CI [-2.66, -0.73]; P = 0.0006), ANCA (SMD -0.72; 95% CI [-1.13, -0.31]; P = 0.0006) and CRP (SMD -0.92; 95% CI [-1.49, -0.35]; P = 0.002) were noted within 6 months after administration of IVIG. Subgroup analysis in the unmodified immunotherapy population showed reductions in BVAS (SMD -1.39; 95% CI [-2.31, -0.48]; P = 0.003) and CRP (SMD -0.56; 95% CI [-0.93, -0.19]; P = 0.002) within half a month after IVIG treatment. CONCLUSION: IVIG was associated with rapid improvements in disease activity and the related biomarkers in patients with active AAV.


Assuntos
Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/diagnóstico , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/terapia , Imunoglobulinas Intravenosas/administração & dosagem , Imunomodulação/efeitos dos fármacos , Adulto , Fatores Etários , Idoso , Anticorpos Anticitoplasma de Neutrófilos/imunologia , Biomarcadores/sangue , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Medição de Risco , Fatores Sexuais , Resultado do Tratamento
SELEÇÃO DE REFERÊNCIAS
DETALHE DA PESQUISA
...