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1.
Oral Health Prev Dent ; 18(1): 929-943, 2020 10 27.
Artigo em Inglês | MEDLINE | ID: mdl-33215484

RESUMO

PURPOSE: To present an update the orofacial manifestations of granulomatosis with polyangiitis (GPA) and present a clinical case with the initial signs in the oral cavity. MATERIALS AND METHODS: A bibliographic search was performed on Pubmed with the keywords 'Wegener's granulomatosis', 'etiology', 'oral manifestations', 'oral cavity', 'gingiva'. The inclusion criteria were papers published in English in the last 10 years that made reference to clinical cases with in which the oral cavity was affected. The quality of the results was assessed with 'The 2013 Care Checklist'. RESULTS: Nineteen clinical cases were analysed. The average quality was 7.68/13 (range 5-10/13). 73.7% of patients were women, the most frequent area for the lesions was the gingiva and the most prevalent lesion was gingival hyperplasia. 68.4% of the patients had this lesion as a first sign, 21.1% as a progression and 10.5% as a recurrence. 68.4% of the lesions resolved once medical treatment was established. CONCLUSION: GPA is a multisystem disorder associated with considerable morbidity and mortality if not treated. Early diagnosis improves the prognosis. The first manifestation of the disease can be seen in the oral cavity. It is important that dentists recognise the oral manifestation in order to improve the prognosis. Key words: granulomatosis, polyangiiitis, Wegener's granulomatosis.


Assuntos
Hiperplasia Gengival , Granulomatose com Poliangiite , Feminino , Gengiva , Granulomatose com Poliangiite/diagnóstico , Granulomatose com Poliangiite/tratamento farmacológico , Humanos , Prognóstico
3.
Arkh Patol ; 82(5): 63-66, 2020.
Artigo em Russo | MEDLINE | ID: mdl-33054034

RESUMO

The paper describes an autopsy case of a 69-year-old woman with microscopic polyangiitis lasting about 1.5 months despite intensive treatment. She had generalized productive vasculitis, extracapillary productive glomerulonephritis, adult respiratory distress syndrome with focal pneumonia, isolated lung atelectasis, splenic infarction, and toe and finger gangrene. Morphological differences from those of granulomatosis with polyangiitis and Churg-Strauss syndrome are presented.


Assuntos
Síndrome de Churg-Strauss , Granulomatose com Poliangiite , Poliangiite Microscópica , Adulto , Idoso , Feminino , Granulomatose com Poliangiite/diagnóstico , Humanos , Poliangiite Microscópica/diagnóstico
4.
Am J Otolaryngol ; 41(6): 102661, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32810787

RESUMO

PURPOSE: Distinguishing the prodromal nasal polyposis of eosinophilic granulomatosis with polyangiitis (EGPA) from chronic rhinosinusitis with nasal polyps (CRSwNP) is a challenge for rhinologists and rheumatologists. It has recently been reported that angiogenesis and CD105 expressed on vascular endothelial cells could have a role in the pathogenesis and development of nasal polyps. This exploratory study examined the structured histopathology of nasal polyps in patients with EGPA and CRSwNP, comparing CD105 expression in their nasal tissue with that of a control group with no chronic sinonasal inflammation. METHODS: A structured histopathological study was performed on surgical specimens of nasal tissue from 32 adults (13 with EGPA, 14 with CRSwNP, 5 controls), considering CD105 as a marker to determine microvessel density (MVD). RESULTS: The mean eosinophil count was higher in EGPA patients with tissue inflammation (p = .002), and in CRSwNP patients with sub-epithelial edema (p = .009). Neutrophil infiltration was significantly associated with severe tissue inflammation in EGPA patients (p = .04), but with the absence of fibrosis in CRSwNP patients (p = .04). In the EGPA group, CD105-MVD correlated with tissue eosinophil count (p = .05). Mean CD105-MVD was significantly higher in EGPA patients with mucosal ulceration (p = .004). In the CRSwNP group, a CD105-MVD correlated positively and significantly with tissue eosinophil count (p = .01). CONCLUSION: Alongside the known abundance of eosinophils, other cells might contribute to inflammatory processes. Neutrophils may amplify inflammation, eosinophil recruitment and tissue damage. CD105 expression in CRSwNP and EGPA nasal polyps supports the hypothesized involvement of angiogenesis in the pathogenesis and development of nasal polyps.


Assuntos
Endoglina/análise , Granuloma Eosinófilo/diagnóstico , Granulomatose com Poliangiite/diagnóstico , Pólipos Nasais/diagnóstico , Adulto , Idoso , Biomarcadores/análise , Doença Crônica , Diagnóstico Diferencial , Granuloma Eosinófilo/patologia , Eosinófilos , Feminino , Granulomatose com Poliangiite/patologia , Humanos , Inflamação , Contagem de Leucócitos , Masculino , Pessoa de Meia-Idade , Pólipos Nasais/patologia , Rinite , Sinusite
5.
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
6.
Z Rheumatol ; 79(9): 902-905, 2020 Nov.
Artigo em Alemão | MEDLINE | ID: mdl-32681196

RESUMO

Auricular chondritis frequently occurs in relapsing polychondritis. In addition to the primary form of the disease up to 30% of cases of chondritis can be secondary, e.g. due to autoimmune diseases. We describe the case of a 62-year-old male patient with auricular chondritis as the first symptom of granulomatosis with polyangiitis. Anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis should always be considered in the differential diagnostics of relapsing polychondritis and antibody testing should be performed accordingly.


Assuntos
Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos , Granulomatose com Poliangiite , Policondrite Recidivante , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/complicações , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/diagnóstico , Anticorpos Anticitoplasma de Neutrófilos , Diagnóstico Diferencial , Granulomatose com Poliangiite/diagnóstico , Humanos , Masculino , Pessoa de Meia-Idade , Policondrite Recidivante/diagnóstico
10.
Rinsho Shinkeigaku ; 60(7): 500-503, 2020 Jul 31.
Artigo em Japonês | MEDLINE | ID: mdl-32536662

RESUMO

A 66-year-old woman presented with dysesthesia over the right side of her face, hypoglossal nerve dysfunction, dysphagia, and dysgeusia of the right side. A MRI scan of the brain revealed cerebral dural thickening on the right side of the skull base, and histopathological examination revealed granulomatous inflammation of the dura. Based on paranasal sinusitis, bronchodilatation, laboratory tests showing weakly positive MPO-ANCA, intact renal function, and the patient's favorable response to steroids, we diagnosed the patient with limited granulomatosis with polyangiitis (GPA). Reportedly, autoimmune disease might occur in patients with exacerbation of monoclonal gammopathy of undetermined significance, which was observed in this case. This suggests the utility of immunoelectrophoresis.


Assuntos
Anticorpos Anticitoplasma de Neutrófilos , Granulomatose com Poliangiite/complicações , Granulomatose com Poliangiite/diagnóstico , Meningite/diagnóstico , Meningite/etiologia , Gamopatia Monoclonal de Significância Indeterminada/complicações , Gamopatia Monoclonal de Significância Indeterminada/diagnóstico , Peroxidase/imunologia , Idoso , Encéfalo/diagnóstico por imagem , Encéfalo/patologia , Progressão da Doença , Feminino , Granulomatose com Poliangiite/tratamento farmacológico , Granulomatose com Poliangiite/patologia , Humanos , Hipertrofia , Meningite/tratamento farmacológico , Meningite/patologia , Metilprednisolona/administração & dosagem , Gamopatia Monoclonal de Significância Indeterminada/tratamento farmacológico , Gamopatia Monoclonal de Significância Indeterminada/patologia , Prednisolona/administração & dosagem , Resultado do Tratamento
11.
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
12.
J Med Internet Res ; 22(7): e17231, 2020 07 20.
Artigo em Inglês | MEDLINE | ID: mdl-32459634

RESUMO

BACKGROUND: Utilizing the traditional centers of excellence approach to conduct clinical trials involving rare diseases remains challenging. Patient-based registries have been shown to be both feasible and valid in several other diseases. OBJECTIVE: This report outlines the clinical characteristics of a large internet registry cohort of participants with a self-reported diagnosis of granulomatosis with polyangiitis or microscopic polyangiitis. METHODS: Patients with a self-reported diagnosis of granulomatosis with polyangiitis or microscopic polyangiitis in an internet-based prospective longitudinal cohort (from the Vasculitis Patient-Powered Research Network) were included. Data on symptoms, diagnostic testing, and treatment were collected using standardized questionnaires. RESULTS: The study compared patients with granulomatosis with polyangiitis (n=762) and patients with microscopic polyangiitis (n=164). Of the cohort, 97.7% (904/925) reported the diagnosis had been confirmed by a physician. Compared to microscopic polyangiitis, patients with granulomatosis with polyangiitis reported significantly more ear, nose, and throat manifestations (granulomatosis with polyangiitis: 641/723, 88.7%; microscopic polyangiitis: 89/164, 54.3%; z=10.42, P<.001), fevers (granulomatosis with polyangiitis: 325/588, 55.3%; microscopic polyangiitis: 64/139, 46.0%; z=1.96, P=.05), joint involvement (granulomatosis with polyangiitis: 549/688, 79.8%; microscopic polyangiitis: 106/154, 68.8%; z=2.96, P=.003), and pulmonary involvement (granulomatosis with polyangiitis: 523/734, 71.3%; microscopic polyangiitis: 90/154, 58.4%; z=3.13, P=.002). Compared to microscopic polyangiitis, patients with granulomatosis with polyangiitis reported significantly less renal involvement (granulomatosis with polyangiitis: 457/743, 61.5%; microscopic polyangiitis: 135/163, 82.8%; z=-5.18, P<.001) and renal transplantation (granulomatosis with polyangiitis: 10/721, 1.4%; microscopic polyangiitis: 7/164, 4.3%; z=-2.43, P=.02). Antineutrophil cytoplasmic antibody positivity was reported in 94.2% (652/692) of patients with granulomatosis with polyangiitis and 96.1% (147/153) of patients with microscopic polyangiitis. A biopsy showing vasculitis was reported in 77.0% (562/730) of patients with granulomatosis with polyangiitis and 81.9% (131/160) of patients with microscopic polyangiitis. CONCLUSIONS: In this large, internet-based cohort of patients with a self-reported diagnosis of granulomatosis with polyangiitis or microscopic polyangiitis, disease manifestations were consistent with expectations for each type of vasculitis. Given the rarity of these and other vasculitides, conducting some types of research through internet-based registries may provide an efficient alternative to inperson, center-of-excellence clinical trials.


Assuntos
Granulomatose com Poliangiite/diagnóstico , Internet/normas , Poliangiite Microscópica/diagnóstico , Medidas de Resultados Relatados pelo Paciente , Estudos de Coortes , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos
14.
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
15.
Zhonghua Nei Ke Za Zhi ; 59(5): 360-365, 2020 May 01.
Artigo em Chinês | MEDLINE | ID: mdl-32370464

RESUMO

Objective: To analyze the clinical and prognostic characteristics in patients with eosinophilic granulomatosis with polyangitis (EGPA). Methods: The clinical data of 146 EGPA patients hospitalized in Peking Union Medical College Hospital from 2000 to 2019 were analyzed retrospectively, including clinical manifestations, laboratory results, treatment, complications and outcome at discharge. Birmingham Vasculitis activity score-V3 (BVAS-V3) was used to evaluate disease activity. Results: The ratio of male to female was 1.8∶1 with average age (41.7±16.1) year-old. The median time from disease onset to diagnosis was 18(6, 60) months (0.5~450). The most common clinical manifestations were lung [121(82.9%)] and nose/paranasal sinuses [119(81.5%)] involvement. The positive rate of anti-neutrophil cytoplasmic antibody (ANCA) was 24.7%, mainly peripheral (P)-ANCA/myeloperoxidase (MPO)-ANCA. Compared with ANCA-negative patients, the ANCA-positive patients had a higher incidence of renal involvement and nervous system involvement (66.7% vs. 20.9%, 80.6% vs. 51.8%, P<0.001), fever and optic neuropathy (66.7% vs. 40.9%,8.3% vs. 0, P<0.05), more active disease [median BVAS-V3 25(18,30)vs. 19(14,24),P=0.001] and more elevated erythrocyte sedimentation rate [40.5(20.5,82.8)mm/1h vs. 25.0(13.3,50.8)mm/1h,P=0.006] and C-reactive protein [37.1(11.8,72.9)mg/L vs.13.5(3.4,66.1)mg/L,P=0.036]. More ANCA-negative patients had pleural effusion (20.9% vs. 5.6%, P<0.04) compared with ANCA-negative patients. Pulmonary infection was the most common complication. A total of 12 EGPA patients (8.2%)achieved remission and 6 patients (4.1%)died or discharged themselves from the hospital. Conclusion: EGPA is a rare small vessel vasculitis. The clinical manifestations and outcomes are heterogenous. The mortality rate of EGPA is high.


Assuntos
Anticorpos Anticitoplasma de Neutrófilos/sangue , Granulomatose com Poliangiite/diagnóstico , Adulto , Eosinófilos , Feminino , Granulomatose com Poliangiite/mortalidade , Humanos , Masculino , Pessoa de Meia-Idade , Peroxidase , Derrame Pleural , Prognóstico , Estudos Retrospectivos
20.
Cardiovasc J Afr ; 31(4): e1-e4, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-31995113

RESUMO

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare form of systemic vasculitis in which cardiac involvement is relatively common and accounts for half of EGPA-related deaths. Cardiac involvement is more frequent in patients with an absence of anti-neutrophil cytoplasmic antibody and those with higher eosinophil counts. Clinical manifestations are various, including myocarditis, pericarditis, pericardial effusion, heart failure, arrhythmias, valvular insufficiencies and intra-cardiac thrombus formation. The pathology of cardiac involvement in EGPA is usually endomyocardial and pericardial eosinophilic infiltration. Considering the potentially adverse outcomes associated with cardiac involvement in EGPA, early detection is important. We experienced a rare case of EGPA with cardiac involvement presenting with non-infectious vegetations.


Assuntos
Granulomatose com Poliangiite/complicações , Cardiopatias/etiologia , Ventrículos do Coração/patologia , Miocárdio/patologia , Adulto , Procedimentos Cirúrgicos Cardíacos , Glucocorticoides/uso terapêutico , Granulomatose com Poliangiite/diagnóstico , Granulomatose com Poliangiite/tratamento farmacológico , Cardiopatias/diagnóstico por imagem , Cardiopatias/patologia , Cardiopatias/cirurgia , Ventrículos do Coração/diagnóstico por imagem , Ventrículos do Coração/cirurgia , Humanos , Masculino , Resultado do Tratamento
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