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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.
Rev Assoc Med Bras (1992) ; 66(7): 904-907, 2020 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-32844923

RESUMO

Churg-Strauss syndrome, Eosinophilic granulomatosis with polyangiitis (EGPA), is a systemic vasculitis that affects small- to medium-sized vessels. It is rare and part of the Anti-neutrophil cytoplasm antibody-associated vasculitis (ANCA) group. We present a 37-year-old man, with a previous history of asthma, that was sent to the ED due to 2 weeks of productive cough, occasional dyspnea on exertion, fever (one week), asthenia, and anorexia. Upon physical examination, he was subfebrile and tachycardic. He had leukocytosis (17.00 x10^9/L) and eosinophilia of 20.0 % (3.4 X10^9/L), creatinine level of 1.5 mg/dL, subtle elevation on liver function tests and CRP of 10.82mg/dL. On Chest X-Ray, there was infiltrate on the right pulmonary base. Due to a strong suspicion of EGPA, he was started on 80mg of prednisolone from admission. ANCA MPO was positive, with the remaining auto-immune study negative. He underwent Thorax CT (under corticotherapy) without relevant changes, as well as bronchoalveolar lavage, without macroscopic signs of alveolar hemorrhage. Because of active urinary sediment, nephrotic proteinuria (6.5g/24h), and acute renal failure he underwent a renal biopsy, which revealed pauci-immune crescentic glomerulonephritis, with predominantly acute findings (in the context of ANCA-MPO Vasculitis - EGPA). After the biopsy, he received three 1g methylprednisolone pulses and was started on Cyclophosphamide. He remained asymptomatic and renal function was restored. This case highlights the importance of integrating all findings in one clinical scenario to prevent a more complex disease diagnosis, with a specific treatment, from being missed.


Assuntos
Asma , Síndrome de Churg-Strauss , Eosinofilia , Granulomatose com Poliangiite , Adulto , Síndrome de Churg-Strauss/diagnóstico , Síndrome de Churg-Strauss/tratamento farmacológico , Granulomatose com Poliangiite/diagnóstico , Granulomatose com Poliangiite/tratamento farmacológico , Humanos , Masculino , Metilprednisolona/uso terapêutico
3.
Cesk Patol ; 56(2): 68-73, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32493022

RESUMO

The most common group of systemic vasculitides in adulthood are anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). AAV represent autoimmune systemic vasculitides and include 3 clinical phenotypes: Granulomatosis with polyangiitis (GPA, formerly Wegener granulomatosis), Microscopic polyangiitis (MPA) and Eosinophilic granulomatosis with polyangiitis (EGPA, formerly Churg-Strauss syndrome). Histological features are similar to each other in all affected locations, and there are represented by necrotizing vascular inflammation of small and medium calibers, often venules, capillaries or arteriols, typically with fibrinoid vessel wall necrosis. The consequences of this condition are bleeding, as well as compromise of the lumen which may result in downstream tissue ischemia and necrosis. Typically affected locations in biopsy practice are: ENT, lung, skin, GIT, and kidney. The aim of this review is to provide a comprehensive overview of the important histopathological findings. ANCA positive vasculitis is a serious life-threatening disease and therefore requires a rapid diagnosis and appropriate therapy.


Assuntos
Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos , Síndrome de Churg-Strauss , Granulomatose com Poliangiite , Poliangiite Microscópica , Adulto , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/diagnóstico , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/patologia , Anticorpos Anticitoplasma de Neutrófilos , Síndrome de Churg-Strauss/diagnóstico , Síndrome de Churg-Strauss/patologia , Granulomatose com Poliangiite/diagnóstico , Granulomatose com Poliangiite/patologia , Humanos , Poliangiite Microscópica/diagnóstico , Poliangiite Microscópica/patologia
5.
Praxis (Bern 1994) ; 109(7): 509-512, 2020.
Artigo em Alemão | MEDLINE | ID: mdl-32456580

RESUMO

Mononeuritis Multiplex: A Diagnostic Challenge Abstract. Eosinophilic granulomatosis with polyangiitis (EGPA, formerly Churg-Strauss syndrome) is a multifaceted disease. Due to the variability in vascular and organ involvement, EPGA can manifest itself very differently. We report a case of a 60-year-old patient with a known bronchial asthma, pansinusitis and newly blood eosinophilia with a rapid-onset mononeuritis multiplex. The diagnosis was confirmed based on a histological examination. After initiation of therapy the patient can walk independently with a mobility aid.


Assuntos
Asma , Síndrome de Churg-Strauss , Granulomatose com Poliangiite , Mononeuropatias , Asma/complicações , Síndrome de Churg-Strauss/diagnóstico , Granulomatose com Poliangiite/diagnóstico , Humanos , Pessoa de Meia-Idade , Mononeuropatias/diagnóstico
6.
Intern Med ; 59(7): 991-995, 2020 Apr 01.
Artigo em Inglês | MEDLINE | ID: mdl-31813915

RESUMO

Eosinophilic granulomatosis with polyangiitis (EGPA) is a vasculitis characterized by an abnormally high number of eosinophils in the peripheral blood and tissues. EGPA is an extremely rare disorder, with an incidence of 0.5 to 3.7 new cases per million people per year and an overall prevalence of 2.4 to 14 per million adults. There is little knowledge about the genetic factors that influence this disease. There are only two reports of familial EGPA: one in Japan and one in Turkey. We herein report a third case of familial EGPA in a brother and sister who were negative for myeloperoxidase-antineutrophil cytoplasmic antibodies.


Assuntos
Anticorpos Monoclonais Humanizados/uso terapêutico , Síndrome de Churg-Strauss/tratamento farmacológico , Síndrome de Churg-Strauss/genética , Eosinofilia/tratamento farmacológico , Granulomatose com Poliangiite/diagnóstico , Granulomatose com Poliangiite/tratamento farmacológico , Anticorpos Anticitoplasma de Neutrófilos/sangue , Síndrome de Churg-Strauss/diagnóstico , Eosinofilia/diagnóstico , Eosinofilia/genética , Feminino , Predisposição Genética para Doença , Granulomatose com Poliangiite/genética , Humanos , Japão , Masculino , Pessoa de Meia-Idade , Irmãos , Resultado do Tratamento , Turquia
7.
Intern Med ; 59(8): 1029-1033, 2020 Apr 15.
Artigo em Inglês | MEDLINE | ID: mdl-31875634

RESUMO

We herein report two cases of eosinophilic granulomatosis with polyangiitis (EGPA) initially diagnosed as eosinophilic gastroenteritis (EGE) based solely on endoscopic biopsy results. One year after the EGE diagnosis, one patient presented with multiple purpura, and skin biopsy findings resulted in a change of the diagnosis to EGPA. In another patient, multiple skin and colonic ulcerations emerged eight years after the diagnosis of EGE, at which time histological examinations of endoscopic biopsy specimens revealed vasculitis, and the diagnosis was changed to EGPA. Physicians should be aware of the possible existence of EGPA in cases diagnosed as EGE.


Assuntos
Eosinofilia/diagnóstico , Eosinofilia/patologia , Granulomatose com Poliangiite/diagnóstico , Granulomatose com Poliangiite/patologia , Adulto , Biópsia , Síndrome de Churg-Strauss/diagnóstico , Enterite/diagnóstico , Feminino , Gastrite/diagnóstico , Humanos , Pessoa de Meia-Idade , Pele/patologia
9.
BMC Neurol ; 19(1): 288, 2019 Nov 15.
Artigo em Inglês | MEDLINE | ID: mdl-31729970

RESUMO

BACKGROUND: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic vasculitis of unknown cause involving the brain and accompanied by prominent eosinophilia. Intracardiac thrombosis is a major cardiac complication of EGPA that may cause thromboembolism. CASE PRESENTATION: A 53-year-old man presenting with abulia (consciousness disturbance) and left upper limb paralysis was admitted to our hospital. His case was complicated by penetrating branches, small vessel infarcts, and endocardial thrombosis in the right and left ventricle. Cardiomyopathy was also observed. Sixteen days after admission, the patient died from intracranial hemorrhage. Brain autopsy revealed two major findings: 1) large hemorrhagic infarction caused by cardiac embolism; and 2) granuloma and eosinophil infiltration. Vasculitis was accompanied by eosinophil infiltration in the cortical blood vessels and granuloma. CONCLUSIONS: In this case study, we report autopsy findings of brain infarction in a patient with EGPA and endocardial thrombosis. The brain infarction was caused by the cardiac embolisms and vasculitis.


Assuntos
Infarto Cerebral/etiologia , Síndrome de Churg-Strauss/complicações , Granulomatose com Poliangiite/complicações , Tromboembolia/etiologia , Autopsia , Síndrome de Churg-Strauss/diagnóstico , Granulomatose com Poliangiite/diagnóstico , Cardiopatias/etiologia , Humanos , Masculino , Pessoa de Meia-Idade
10.
J Pediatr Ophthalmol Strabismus ; 56(6): 373-377, 2019 Nov 01.
Artigo em Inglês | MEDLINE | ID: mdl-31743405

RESUMO

PURPOSE: To describe four pediatric cases in which isolated orbital pseudotumor preceded the development of a systemic inflammatory disease by months to years. METHODS: The medical records of all patients with the clinical diagnosis of orbital pseudotumor seen at the Ocular Oncology Service of Wills Eye Hospital and Northern Virginia Ophthalmology Associates from 2010 to 2015 were reviewed retrospectively, and those associated with systemic inflammatory disease were selected for further study. Data were retrospectively collected from medical record review regarding patient demographics and clinical features, time to development of systemic inflammatory disease, and medical management. RESULTS: In four pediatric patients, isolated orbital pseudotumor preceded the development of a systemic inflammatory disease, including pauciarticular juvenile idiopathic arthritis, eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome), granulomatosis with polyangiitis (Wegener's granulomatosis), and Crohn's disease. CONCLUSIONS: Orbital pseudotumor may be an antecedent to systemic inflammatory disease in children. Because this was a small case series, the authors are not ready to suggest that a full systemic work-up for systemic inflammatory disease is warranted for every patient with orbital pseudotumor. However, close observation and suspicion for systemic inflammatory conditions may be reasonable in children who present with idiopathic orbital pseudotumor. [J Pediatr Ophthalmol Strabismus. 2019;56(6):373-377.].


Assuntos
Síndrome de Churg-Strauss/etiologia , Doença de Crohn/etiologia , Gerenciamento Clínico , Granulomatose com Poliangiite/etiologia , Imunossupressores/uso terapêutico , Pseudotumor Orbitário/complicações , Adolescente , Biópsia , Criança , Pré-Escolar , Síndrome de Churg-Strauss/diagnóstico , Doença de Crohn/diagnóstico , Feminino , Seguimentos , Granulomatose com Poliangiite/diagnóstico , Humanos , Imagem por Ressonância Magnética , Masculino , Pseudotumor Orbitário/diagnóstico , Pseudotumor Orbitário/tratamento farmacológico , Prognóstico , Estudos Retrospectivos , Fatores de Tempo
11.
Praxis (Bern 1994) ; 108(11): 733-736, 2019 Sep.
Artigo em Alemão | MEDLINE | ID: mdl-31480961

RESUMO

Pulmonary Infiltrate - Not Always Due to Bacterial Infection Abstract. A 65-year-old female patient with a history of controlled asthma was diagnosed with community-acquired pneumonia (CAP). After two weeks of treatment on co-amoxicillin, she failed to respond and was referred for further investigations. Clinical symptoms and laboratory results were suggestive for eosinophilic granulomatosis with polyangiitis (EGPA). Diagnosis and treatment of this disease are still challenging, especially in cases with vital organ involvement.


Assuntos
Infecções Bacterianas , Síndrome de Churg-Strauss , Granulomatose com Poliangiite , Idoso , Asma , Infecções Bacterianas/diagnóstico , Síndrome de Churg-Strauss/complicações , Síndrome de Churg-Strauss/diagnóstico , Feminino , Granulomatose com Poliangiite/complicações , Granulomatose com Poliangiite/diagnóstico , Humanos , Pneumopatias/diagnóstico
12.
Adv Ther ; 36(10): 2558-2566, 2019 10.
Artigo em Inglês | MEDLINE | ID: mdl-31473972

RESUMO

This article, coauthored by a patient with eosinophilic granulomatosis with polyangiitis (EGPA) initially presenting as severe eosinophilic asthma and his physician-specialist, discusses the use and management of oral corticosteroid (OCS) treatment. It also considers the importance of early diagnosis of a rare disease and patient education. The patient describes his journey from progressive worsening of asthma and eventual diagnosis of EGPA to long-term OCS treatment and then participation in a clinical trial for this rare disease, involving the introduction of targeted biologic therapy with OCS tapering. The physician describes the importance of patient referral to obtain a correct diagnosis and optimal maintenance treatment, the balance between risk of adverse events associated with long-term OCS use and benefits of disease control, and various aspects of patient participation in clinical trials. Finally, the patient describes the role of continual patient education in the management of disease and OCS treatment. These considerations can apply to all chronic inflammatory diseases requiring maintenance OCS treatment.Funding: AstraZeneca.


Assuntos
Corticosteroides/uso terapêutico , Síndrome de Churg-Strauss/tratamento farmacológico , Síndrome de Churg-Strauss/psicologia , Administração Oral , Asma/complicações , Doença Crônica , Síndrome de Churg-Strauss/diagnóstico , Síndrome de Churg-Strauss/etiologia , Diagnóstico Diferencial , Progressão da Doença , Diagnóstico Precoce , Humanos , Educação de Pacientes como Assunto , Encaminhamento e Consulta
13.
J Ayub Med Coll Abbottabad ; 31(3): 478-479, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31535536

RESUMO

Granulomatosis with Polyangitis (GPA) is an uncommon immunologically mediated necrotizing vasculitis affecting the small and medium sized systemic blood vessels. We previously reported our experience with this condition and herein, we document our study findings and compare them to the clinical and radiological findings of various studies from around the world. By doing so we hope to further create awareness of this condition afflicting not only our part of the population but is part of a larger global phenomenon.


Assuntos
Síndrome de Churg-Strauss/diagnóstico , Granulomatose com Poliangiite/complicações , Granulomatose com Poliangiite/diagnóstico , Sarcoidose/diagnóstico , Diagnóstico Diferencial , Humanos , Nefropatias/etiologia , Otorrinolaringopatias/etiologia , Paquistão , Doenças Respiratórias/etiologia , Centros de Atenção Terciária
14.
Intern Med ; 58(24): 3583-3587, 2019 Dec 15.
Artigo em Inglês | MEDLINE | ID: mdl-31391393

RESUMO

We herein report the first pediatric case (a 13-year-old girl) of relapsing eosinophilic granulomatosis with polyangiitis (EGPA) successfully treated with mepolizumab (anti-interleukin-5). She was classified as having EGPA based on the presence of asthma, eosinophilia, pulmonary infiltrates, and extravascular eosinophil infiltration confirmed by a biopsy. She achieved remission after initial oral prednisolone (PSL) therapy, but EGPA relapsed during PSL tapering. Subsequent combined therapy with PSL and tacrolimus did not improve the recurrent disease. Intravenous methylprednisolone pulse therapy was started, followed by oral PSL. During PSL tapering, mepolizumab was added to the treatment, which resulted in sustained remission and successful PSL tapering.


Assuntos
Anticorpos Monoclonais Humanizados/uso terapêutico , Síndrome de Churg-Strauss/tratamento farmacológico , Interleucina-5/antagonistas & inibidores , Adolescente , Asma , Biópsia , Síndrome de Churg-Strauss/diagnóstico , Eosinofilia , Feminino , Glucocorticoides/uso terapêutico , Humanos , Pulmão/diagnóstico por imagem , Prednisolona/uso terapêutico , Radiografia
15.
Arerugi ; 68(7): 857-868, 2019.
Artigo em Japonês | MEDLINE | ID: mdl-31406082

RESUMO

BACKGROUND AND AIMS: It is difficult to evaluate neurological signs of multiple mononeuritis (MM) in patients with eosinophilic granulomatosis with polyangiitis (EGPA). We created a new questionnaire about motor and sensory disturbances in EGPA and investigated whether the questionnaire would be a useful tool in the management of MM in EGPA patients. METHODS: We classified 40 EGPA patients attending Hiratsuka City Hospital into two groups, namely 30 who were treated with intravenous immunoglobulin (IVIG) and 10 who achieved remission by conventional treatment without IVIG. We created a questionnaire for the evaluation of motor and sensory disturbance in EGPA (ANCA related.com). In patients who received IVIG, we evaluated motor and sensory disturbance scores at disease onset, before IVIG, 1 week and 1 month after the end of IVIG. In patients treated without IVIG, we evaluated these scores at disease onset and at the time of the latest examination. RESULTS: The total motor disturbance score at disease onset was significantly lower in EGPA patients who received IVIG than in those who did not receive it. Disease duration was significantly inversely correlated with the change in the sensory disturbance ratio, but not with the motor disturbance ratio. The motor disturbance ratio was significantly correlated with the manual muscle test improvement ratio. In patients who received IVIG, the total motor disturbance score increased significantly, and the total sensory disturbance score decreased significantly, 1 month after IVIG. CONCLUSION: By using the questionnaire we could evaluate changes in motor and sensory disturbance after IVIG treatment in patients with EGPA. The questionnaire should be useful in the management of MM in EGPA patients.


Assuntos
Síndrome de Churg-Strauss/diagnóstico , Granulomatose com Poliangiite/diagnóstico , Inquéritos e Questionários , Síndrome de Churg-Strauss/complicações , Granulomatose com Poliangiite/complicações , Humanos , Imunoglobulinas Intravenosas/uso terapêutico
17.
BMJ Case Rep ; 12(7)2019 Jul 10.
Artigo em Inglês | MEDLINE | ID: mdl-31296634

RESUMO

Eosinophilic granulomatosis with polyangiitis (EGPA) is a multisystemic small-vessel vasculitic disease that can present with positive MPO-P-ANCA (myeloperoxidase-perinuclear-anti-neutrophil cytoplasmic antibody). It is a rare condition that is difficult to diagnose. We present the case of a 64-year-old man with late-onset adult asthma and treated nasopharyngeal carcinoma who initially presented to us with proximal myopathy. Thereafter, he developed a constellation of fleeting symptoms which included rhinosinusitis, mononeuritis multiplex, skin vasculitis and arthritis. Blood investigations showed that he had eosinophilia, and skin biopsy demonstrated dermal vasculitis with eosinophils. He was found to be MPO-C-ANCA positive, and although initially thought to have granulomatosis with polyangiitis, the diagnosis was later revised to EGPA. This case highlights the diagnostic challenges with atypical presentations of EGPA and also presents a rare case of positive MPO-C-ANCA that has never been described in EGPA before.


Assuntos
Anticorpos Anticitoplasma de Neutrófilos/sangue , Asma/complicações , Síndrome de Churg-Strauss/complicações , Síndrome de Churg-Strauss/diagnóstico , Granulomatose com Poliangiite , Peroxidase/sangue , Síndrome de Churg-Strauss/sangue , Diagnóstico Diferencial , Humanos , Masculino , Pessoa de Meia-Idade
18.
Arerugi ; 68(6): 696-700, 2019.
Artigo em Japonês | MEDLINE | ID: mdl-31308336

RESUMO

Atopic dermatitis and bronchial asthma are common diseases in children. We report the development of eosinophilic polyangiitis granulomatosis (EGPA) in a young girl being treated for both atopic dermatitis, diagnosed at 1 year of age, and bronchial asthma, diagnosed at 4 years of age. Her eruption did not result in lichenification and was not fully responsive to corticosteroid ointment. Asthma lightened by treatment of inhalational steroids. Hypereosinophilia was detected at 5 years of age, at least 20% of white blood cells, and 44% at 8 years of age. At 10 years of age, she was diagnosed with anti-neutrophil cytoplasmic antibody-negative EGPA. The diagnosis was based on findings of eosinophil-infiltrating granulomatous vasculitis of the skin accompanied by notable peripheral blood eosinophilia, sinusitis, and pulmonary nodules on radiographic evaluation. Asymptomatic myocardial involvement was also detected utilizing dual perfusion and metabolic scintigraphy with 201Tl/123I-BMIPP, which was relieved by 1-year treatment of glucocorticoid combined with immunosuppressive drugs. EGPA is an extremely rare vasculitis that develops several years after preceding allergic disorders. Pediatric-onset EGPA has a poorer prognosis than adult-onset EGPA, which can be attributed to a high prevalence of cardiac involvement. Therefore, accurate diagnosis is critical for improving prognosis. EGPA should be considered when atypical findings are noted in management of atopic dermatitis and bronchial asthma.


Assuntos
Asma/complicações , Síndrome de Churg-Strauss/diagnóstico , Dermatite Atópica/complicações , Eczema/complicações , Corticosteroides/uso terapêutico , Asma/diagnóstico , Criança , Pré-Escolar , Síndrome de Churg-Strauss/complicações , Dermatite Atópica/diagnóstico , Eczema/diagnóstico , Feminino , Humanos
19.
Clin Rheumatol ; 38(12): 3493-3499, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31317422

RESUMO

To investigate the relevance of RF in patients with EGPA, we reviewed consecutive patients who were newly diagnosed with EGPA from August 1998 to February 2019 in Keio University Hospital with RF titer at diagnosis available. We divided the patients according to the median level of RF titer of 75 IU/mL and compared clinical features between the two groups. Among 16 patients identified, 8 patients were in the RF high group and the other 8 patients were in the RF low group. All patients in the high RF group were negative for MPO-ANCA, whereas all in the low RF group was positive for MPO-ANCA with a mean titer of 103 IU/mL. The eosinophil count at diagnosis was significantly higher in the RF high group than the RF low group (20001/µL vs 5144/µL, p < 0.01). Gastrointestinal lesion was significantly more frequent in the RF high group, and parenchymal organ lesions, such as heart and renal organ involvement, were frequent in the RF low group. With principal component analysis, RF high and low groups were clearly divided by the combination of eosinophil count, MPO-ANCA titer, gastrointestinal lesions, musculoskeletal symptoms, and disease activity score. Those results suggest EGPA can be divided into two groups in association with RF.Key Points• Our study showed that patients with EGPA can be separated into two groups according to RF titer.• The two subtypes reflect different underlying pathogenesis in EGPA, and the optimal treatment for them may be different.


Assuntos
Síndrome de Churg-Strauss/classificação , Fator Reumatoide/sangue , Adulto , Idoso , Idoso de 80 Anos ou mais , Síndrome de Churg-Strauss/sangue , Síndrome de Churg-Strauss/diagnóstico , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Análise de Componente Principal , Estudos Retrospectivos
20.
Rheumatol Int ; 39(11): 1899-1905, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31302739

RESUMO

In this study, we investigated whether hypereosinophilia (peripheral eosinophil ≥ 1500/mm3) at diagnosis could estimate the increased current activity and predict the poor prognosis during follow-up in patients with eosinophilic granulomatosis with polyangiitis (EGPA). We retrospectively reviewed the medical records of 42 patients with EGPA and finally included 30 systemic immunosuppressive drug-naïve patients. We obtained clinical and laboratory data including clinical manifestations, Birmingham vasculitis activity score (BVAS), five-factor score (FFS) (2009), and routine laboratory results. Hypereosinophilia was defined as peripheral eosinophil ≥ 1500/mm3. We divided EGPA patients based on hypereosinophilia and compared variables between the two groups. The cumulative relapse-free survival rates were compared by the Kaplan-Meier survival analysis. Patients with hypereosinophilia more commonly exhibited cutaneous manifestation than those without (50.0% vs. 14.3%, P = 0.038), but there were no significant differences in BVAS and FFS (2009) at diagnosis. Patients with hypereosinophilia showed the higher median WBC (14,200.0/mm3 vs. 7940.0/mm3) and CRP (17.6 mg/L vs. 2.0 mg/L) at diagnosis than those without. During follow-up, patients with hypereosinophilia at diagnosis exhibited the similar cumulative relapse-free survival rate to those without (P = 0.393). Whereas, patients with FFS (2009) at diagnosis ≥ 2, which was a well-known predictor of the poor prognosis of EGPA, exhibited the lower cumulative relapse-free survival rate than those with FFS (2009) < 2 (P = 0.030). Hypereosinophilia at diagnosis could neither estimate the current activity nor predict relapse in systemic immunosuppressive drug-naïve patients with EGPA unlike theoretical assumption.


Assuntos
Síndrome de Churg-Strauss/diagnóstico , Eosinofilia/diagnóstico , Imunossupressores/uso terapêutico , Adolescente , Adulto , Idoso , Síndrome de Churg-Strauss/tratamento farmacológico , Síndrome de Churg-Strauss/mortalidade , Eosinofilia/tratamento farmacológico , Eosinofilia/mortalidade , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Recidiva , Estudos Retrospectivos , Índice de Gravidade de Doença , Taxa de Sobrevida , Adulto Jovem
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