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3.
Rev. patol. respir ; 23(supl.1): S12-S21, feb. 2020. graf, ilus
Artigo em Espanhol | IBECS | ID: ibc-188030

RESUMO

En la última década, hemos asistido a la introducción de los anticuerpos monoclonales para el tratamiento del asma grave, lo que ha supuesto una auténtica revolución en la vida de muchos de nuestros pacientes. Existen algunas patologías que a menudo, vemos asociadas al asma grave y con las que debemos hacer un adecuado diagnóstico diferencial como son: granulomatosis eosinofílica con poliangeitis (GEPA), síndromes hipereosinofílicos (SHE), neumonías eosinófilas crónicas (NEC) y aspergilosis broncopulmonar alérgica (ABPA) en las que se han probado dichos tratamientos con buenos resultados en la práctica clínica, pero cuya indicación se encuentra actualmente fuera de ficha técnica. Estas patologías suelen precisar para su control de tratamiento con corticoides sistémicos y/o inmunosupresores durante un tiempo prolongado, con los importantes efectos secundarios que conllevan. Las nuevas terapias están permitiendo un importante ahorro de corticoides sistémicos e incluso su retirada en muchos casos. Hemos realizado una revisión de estas enfermedades y de los resultados clínicos de dichos tratamientos biológicos en cada una. Otra situación en la que se dispone de nueva evidencia, es la utilización de biológicos para el asma alérgico grave durante el periodo de embarazo


In the last decade we have attended to the introduction of monoclonal antibodies for the treatment of severe asthma, which have supposed a real revolution in the lives of many of our patients. There are some pathologies that we often see associated with severe asthma and with which we must make an adequate differential diagnosis such as: eosinophilic granulomatosis with polyangiitis (EGPA), hypereosinophilic syndromes (HES), chronic eosinophilic pneumonia (cep) and allergic bronchopulmonary aspergillosis (ABPA). These treatments have been tested with good results in clinical practice, but being used off label. These pathologies usually require treatment with systemic corticosteroids and/or immunosuppressants for a long time producing important side effects. New therapies are allowing significant dose reduction of systemic corticosteroids and even their withdrawal in many cases. We have carried out a review of these diseases and the clinical results of biological treatments in each one of them. Another situation in which new evidence is available, is the use of biologicals for severe allergic asthma during pregnancy


Assuntos
Humanos , Ficha Clínica , Asma/terapia , Diagnóstico Diferencial , Granulomatose com Poliangiite/complicações , Síndrome Hipereosinofílica/complicações , Eosinofilia Pulmonar/complicações , Aspergilose Broncopulmonar Alérgica/complicações , Corticosteroides/administração & dosagem , Imunossupressores/administração & dosagem , Tomografia Computadorizada por Raios X
5.
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
7.
Postgrad Med ; 131(7): 546-549, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31483199

RESUMO

Objectives: To identify the cognitive and functional deficits in a well-characterized group of patients with vasculitis of the nervous system. Methods: Sixty-seven patients diagnosed with Central Nervous System (CNS) or Peripheral nervous System (PNS) vasculitis over a 14-year period were retrospectively identified. Data on clinical presentation, laboratory, radiographic and tissue biopsy investigations, and treatment were collated. Cognitive, functional and quality of life evaluation assessments were performed in 31 patients who agreed to participate and included Addenbrooke's Cognitive Examination-revised (ACE-R), Nottingham Extended Activities of Daily Living (NEADL) and EQ-5D-3L quality of life questionnaires. Results: CNS vasculitis patients exhibited cognitive impairment, with a mean ACE-R score of 74/100 (standard deviation (SD) 16). NEADL and EQ-5D-3L scores were in the impaired range at 41/66 (SD 21) and 57/81 (SD 22), respectively. Patients with just PNS vasculitis exhibited fewer cognitive deficits with ACE-R and NEADL scores of 87 (SD 8) and 46 (SD 16) respectively. EQ-5D-3L score was in the impaired range of 65 (SD 22). Conclusions: Vasculitis of the nervous system and, in particular, CNS vasculitis causes cognitive impairment and deficits in functional ability. Such patients should be targeted for cognitive rehabilitation.


Assuntos
Disfunção Cognitiva/psicologia , Doenças do Sistema Nervoso Periférico/psicologia , Vasculite do Sistema Nervoso Central/psicologia , Atividades Cotidianas , Adulto , Idoso , Idoso de 80 Anos ou mais , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/complicações , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/tratamento farmacológico , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/fisiopatologia , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/psicologia , Disfunção Cognitiva/etiologia , Disfunção Cognitiva/fisiopatologia , Estudos de Coortes , Estudos Transversais , Feminino , Arterite de Células Gigantes/complicações , Arterite de Células Gigantes/tratamento farmacológico , Arterite de Células Gigantes/fisiopatologia , Arterite de Células Gigantes/psicologia , Granulomatose com Poliangiite/complicações , Granulomatose com Poliangiite/tratamento farmacológico , Granulomatose com Poliangiite/fisiopatologia , Granulomatose com Poliangiite/psicologia , Nível de Saúde , Humanos , Imunossupressores/uso terapêutico , Masculino , Pessoa de Meia-Idade , Doenças do Sistema Nervoso Periférico/complicações , Doenças do Sistema Nervoso Periférico/tratamento farmacológico , Doenças do Sistema Nervoso Periférico/fisiopatologia , Qualidade de Vida , Estudos Retrospectivos , Vasculite/complicações , Vasculite/tratamento farmacológico , Vasculite/fisiopatologia , Vasculite/psicologia , Vasculite do Sistema Nervoso Central/complicações , Vasculite do Sistema Nervoso Central/tratamento farmacológico , Vasculite do Sistema Nervoso Central/fisiopatologia , Adulto Jovem
8.
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
10.
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
11.
BMJ Case Rep ; 12(8)2019 Aug 15.
Artigo em Inglês | MEDLINE | ID: mdl-31420437

RESUMO

A 60-year-old woman was admitted to the hospital with worsening dyspnoea, cough and chest pain. This was on a background of weight loss, decreased appetite, mononeuritis multiplex, chronic eosinophilia and a single episode of a non-blanching rash. Investigations demonstrated a raised troponin and ischaemic changes on ECG, and she was therefore initially treated for a presumed myocardial infarction. However, her symptoms failed to improve with treatment for the acute coronary syndrome. A coronary angiogram revealed no significant flow-limiting disease, and further investigations yielded confirmation of raised eosinophils and a positive perinuclear antineutrophil cytoplasmic antibody test. An echocardiogram demonstrated a pericardial effusion, and subsequent cardiac magnetic resonance features were compatible with myopericarditis. In light of these findings, the patient was diagnosed with eosinophilic granulomatous with polyangiitis and commenced on high-dose intravenous methylprednisolone and cyclophosphamide. She made an excellent recovery and remains in remission on azathioprine and a tapering dose of corticosteroids.


Assuntos
Granuloma Eosinófilo/complicações , Granulomatose com Poliangiite/complicações , Miocardite/etiologia , Pericardite/etiologia , Anti-Inflamatórios/administração & dosagem , Ciclofosfamida/administração & dosagem , Granuloma Eosinófilo/tratamento farmacológico , Feminino , Granulomatose com Poliangiite/tratamento farmacológico , Humanos , Imunossupressores/administração & dosagem , Metilprednisolona/administração & dosagem , Pessoa de Meia-Idade , Miocardite/tratamento farmacológico , Pericardite/tratamento farmacológico
12.
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
13.
Immunol Med ; 42(2): 94-98, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-31469613

RESUMO

Giant cell arteritis (GCA) is a type of large vessel vasculitis, and it involves the aorta, large vessels and terminal branches of the external carotid artery, especially the temporal artery. Temporal artery biopsy is a simple tool for the diagnosis of vasculitis, however, the histopathological findings do not always differentiate between the small-vessel vasculitis and GCA. We report the case of 72-year-old male who initially had a clinical diagnosis of GCA, then in the course of treatment, diagnostic histopathological approach revealed the necrotizing vasculitis with bronchocentric granulomatosis in the inflammatory nodule of the lung. The manifestations of patients with systemic vasculitis represent the disorders of multiple organ systems thus are diverse and may vary through the course of the disease. Presentation of unexpected features such as insufficient response to antibiotics, sinusitis, runny nose, discomfort of frontal region or pachymeningitis which anticipates re-evaluation of systemic vasculitis that may lead us to an appropriate diagnosis and the treatment.


Assuntos
Arterite de Células Gigantes/diagnóstico , Granulomatose com Poliangiite/diagnóstico , Idoso , Biópsia , Diagnóstico Diferencial , Imagem de Difusão por Ressonância Magnética , Arterite de Células Gigantes/complicações , Arterite de Células Gigantes/patologia , Arterite de Células Gigantes/terapia , Granulomatose com Poliangiite/complicações , Granulomatose com Poliangiite/patologia , Granulomatose com Poliangiite/terapia , Humanos , Pulmão/diagnóstico por imagem , Pulmão/patologia , Masculino , Radiografia Torácica , Rinite/etiologia , Sinusite/etiologia , Artérias Temporais/patologia , Tomografia Computadorizada por Raios X
14.
BMC Pulm Med ; 19(1): 154, 2019 Aug 19.
Artigo em Inglês | MEDLINE | ID: mdl-31426769

RESUMO

BACKGROUND: Atypical manifestations, such as elevated serum immunoglobulin-G4 (IgG4) and extra-pulmonary IgG4 positive plasmacyte infiltration, have been described in patients with eosinophilic granulomatosis with polyangiitis (EGPA), such complicated situation might not be readily differentiated from IgG4-related disease. CASE PRESENTATION: Here, we report an interesting and rare case of EGPA in a 41 year-old male with negative anti-neutrophil cytoplasmic antibodies (ANCAs), which showed abundant pulmonary IgG4-positive plasma cells infiltration and markedly elevated serum IgG4 levels without extra-pulmonary lesions of IgG4-related disease. The clinical characteristics hesitated us whether the diagnosis as EGPA overlapping with IgG4-related lung disease should be concluded because of the absence of storiform fibrosis and obliterative phlebitis with lymphoplasmacytic infiltration. The patient's systemic symptoms, pulmonary lesions, blood eosinophils count / percentage, and serum IgG4 levels were significantly improved with immunosuppressive therapy. CONCLUSIONS: We consider that the overlapping pathogenesis exists in the disease course of EGPA and IgG4-related disease, which may lead to interaction during the course of the diseases. And the utilization of diagnostic criteria for IgG4-related lung disease with the careful differentiation is needed in such cases.


Assuntos
Doenças Autoimunes/complicações , Eosinófilos/patologia , Granulomatose com Poliangiite/complicações , Imunoglobulina G/sangue , Pulmão/patologia , Adulto , Diagnóstico Diferencial , Granulomatose com Poliangiite/tratamento farmacológico , Humanos , Pulmão/diagnóstico por imagem , Masculino , Metilprednisolona/administração & dosagem , Sinusite/complicações , Tomografia Computadorizada por Raios X
15.
BMJ Case Rep ; 12(8)2019 Aug 10.
Artigo em Inglês | MEDLINE | ID: mdl-31401575

RESUMO

Intestinal perforation is a rare and life-threatening complication in granulomatosis with polyangiitis (GPA). A 55-year-old Japanese man who had been undergoing maintenance haemodialysis was diagnosed with GPA via a pathologically proven nasal granuloma and proteinase 3-antineutrophil cytoplasmic antibody-positive status. He was started on 60 mg prednisolone. Haematochezia was observed due to the colon ulcer after treatment initiation. Two doses of intravenous cyclophosphamide were administered every 2 weeks. Despite the treatment, there were two episodes of severe abdominal pain with peritonitis within a week. Perforation of descending colon and severe ischaemia of the ascending colon was observed during each emergency laparotomy, and ileostomy was performed. Vasculitis in the small vessels was confirmed. In conclusion, patient with severe intestinal involvement and who was unresponsive to conventional therapy was treated with surgery followed by rituximab administration, and remission was achieved.


Assuntos
Granulomatose com Poliangiite/tratamento farmacológico , Fatores Imunológicos/uso terapêutico , Perfuração Intestinal/etiologia , Rituximab/uso terapêutico , Colo Descendente/cirurgia , Granulomatose com Poliangiite/complicações , Granulomatose com Poliangiite/diagnóstico , Humanos , Perfuração Intestinal/cirurgia , Laparotomia , Masculino , Pessoa de Meia-Idade , Indução de Remissão
16.
Intern Med ; 58(21): 3129-3132, 2019 Nov 01.
Artigo em Inglês | MEDLINE | ID: mdl-31292396

RESUMO

The safety and efficacy of anti-programmed cell death-1 (PD-1) antibodies in patients with granulomatosis with polyangiitis (GPA) still remain unclear. An 83-year-old man with GPA that was well controlled with immunosuppressive therapy was diagnosed with a postoperative recurrence of non-small cell lung cancer (NSCLC). Because the programmed cell death ligand 1 (PD-L1) tumor proportion score was 90%, pembrolizumab was administered. After 10 cycles, immune-related adverse events or GPA flare was not observed, and the patient showed an antitumor response. Anti-PD-1 antibody should therefore be considered a treatment option for PD-L1-high-expressing NSCLC patients with well-controlled GPA.


Assuntos
Anticorpos Monoclonais Humanizados/uso terapêutico , Antígeno B7-H1/antagonistas & inibidores , Carcinoma Pulmonar de Células não Pequenas/tratamento farmacológico , Granulomatose com Poliangiite/complicações , Neoplasias Pulmonares/tratamento farmacológico , Idoso , Carcinoma Pulmonar de Células não Pequenas/complicações , Carcinoma Pulmonar de Células não Pequenas/diagnóstico por imagem , Carcinoma Pulmonar de Células não Pequenas/secundário , Glucocorticoides/uso terapêutico , Granulomatose com Poliangiite/tratamento farmacológico , Humanos , Neoplasias Pulmonares/complicações , Neoplasias Pulmonares/patologia , Masculino , Recidiva Local de Neoplasia/tratamento farmacológico , Tomografia por Emissão de Pósitrons , Prednisolona/uso terapêutico , Parede Torácica/diagnóstico por imagem , Tomografia Computadorizada por Raios X
17.
BMC Pulm Med ; 19(1): 122, 2019 Jul 08.
Artigo em Inglês | MEDLINE | ID: mdl-31286925

RESUMO

BACKGROUND: Granulomatosis with polyangiitis (GPA) is characterised by the main violation of the upper and lower respiratory tract and kidney. GPA is considered a systemic vasculitis of medium-sized and small blood vessels where aortic involvement is extremely rare. CASE PRESENTATION: A 28-year-old male was admitted to the hospital due to 4 h of chest pain. Computed tomography scan of the aorta showed a thickened aortic wall, pulmonary lesions, bilateral pleural effusion and pericardial effusion. The aortic dissection should be considered. An emergency operation was performed on the patient. Surgical biopsies obtained from the aortic wall showed destructive changes, visible necrosis, granulation tissue hyperplasia and a large number of acute and chronic inflammatory cells. Nearly a year later, the patient was re-examined for significant pulmonary lesions. His laboratory studies were significantly positive for anti-neutrophilic antibody directed against proteinase 3. Finally, the diagnosis of GPA was obviously established. CONCLUSIONS: Although GPA rarely involves the aorta, we did not ignore the fact that GPA may involve large blood vessels. In addition, GPA should be included in the systemic vasculitis that can give rise to aortitis and even aortic dissection.


Assuntos
Aneurisma Dissecante/diagnóstico por imagem , Aortite/diagnóstico por imagem , Granulomatose com Poliangiite/diagnóstico , Pulmão/patologia , Adulto , Anticorpos Anticitoplasma de Neutrófilos/sangue , Dor no Peito/etiologia , Ecocardiografia , Granulomatose com Poliangiite/complicações , Granulomatose com Poliangiite/fisiopatologia , Humanos , Masculino , Mieloblastina/imunologia , Tomografia Computadorizada por Raios X
18.
BMJ Case Rep ; 12(6)2019 Jun 21.
Artigo em Inglês | MEDLINE | ID: mdl-31229976

RESUMO

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare antineutrophil cytoplasmic antibody-associated vasculitis that can affect any organ system. It is most often characterised by chronic airway inflammation along with prominent peripheral blood eosinophilia, although the disease can affect the cardiovascular, gastrointestinal, renal or central nervous systems. Ocular manifestations are uncommon and when they do occur, are varied in their clinical presentations. To the best of our knowledge, this is the first case of corneal melt secondary to EGPA to have been reported.


Assuntos
Antibacterianos/uso terapêutico , Ciprofloxacino/uso terapêutico , Perfuração da Córnea/patologia , Úlcera da Córnea/patologia , Granulomatose com Poliangiite/complicações , Transtornos da Visão/etiologia , Idoso , Anticorpos Anticitoplasma de Neutrófilos , Perfuração da Córnea/microbiologia , Perfuração da Córnea/terapia , Úlcera da Córnea/microbiologia , Granulomatose com Poliangiite/fisiopatologia , Humanos , Masculino , Resultado do Tratamento , Transtornos da Visão/tratamento farmacológico , Transtornos da Visão/microbiologia , Transtornos da Visão/fisiopatologia
19.
Cesk Slov Oftalmol ; 74(5): 167-174, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31234629

RESUMO

Granulomatosis with polyangiitis (GPA), formerly known as Wegeners granulomatosis, is an autoimmune vasculitis of small vessels, presenting as necrotizing granulomatous inflammation especially of the upper and lower respiratory tract and necrotizing glomerulonephritis. GPA affects more often Caucasians in northern states, predominantly is affected the age-range group of 50 - 60 years. GPA may affect any organ; the eye symptoms are stated in the range of 16-78 %. The eye symptoms are very variable, and in up to 27 % they are the first sign of undiagnosed GPA. The etiology of GPA was not until now explained. Anti-neutrophil cytoplasmic antibodies (ANCA) play important role in the pathogenesis of this disease. GPA is ranked among ANCA associated vasculitis. The GPA is diagnosed on the basis of clinical signs and symptoms of systemic vasculitis, laboratory and histological tests and imaging studies. Immunomodulative therapy made a contribution to the improvement of GPA prognosis in the last decades; biological treatment reaches the prominence of the GPA treatment procedures. Good collaboration with other specialties is necessary for the early diagnosis and treatment of this life and vision threating disease. The ophthalmologist in the collaboration with specialists of other medical branches may take an important part in the GPA diagnostics, monitoring of the diseases course, or adverse affects of the medication. This paper pays attention to the eye symptoms of the GPA; the literature is supplemented with own photographs of GPA eye symptoms in patients followed up at the Department of Ophthalmology, First medical faculty, Charles University and General Faculty Hospital in Prague, Czech Republic, E.U. Key words: Granulomatosis with polyangiitis (GPA), orbit, scleritis, peripheral ulcerative keratitis (PUK), immunomodulation.


Assuntos
Oftalmopatias , Granulomatose com Poliangiite , Anticorpos Anticitoplasma de Neutrófilos , República Tcheca , Oftalmopatias/etiologia , Granulomatose com Poliangiite/complicações , Humanos , Pessoa de Meia-Idade , Órbita
20.
BMC Ophthalmol ; 19(1): 139, 2019 Jun 28.
Artigo em Inglês | MEDLINE | ID: mdl-31253129

RESUMO

BACKGROUND: Granulomatosis with polyangiitis (GPA) is an antineutrophil cytoplasmic antibodies (ANCA)-associated necrotizing granulomatous vasculitis that affects small to medium size vessels. While the classical form with renal and respiratory tract involvement is mainly seen, a limited form (i.e., with no renal disease) may also occur. We present an unusual case of GPA manifesting merely as a bilateral ocular involvement and complete heart block. CASE PRESENTATION: We report a case of a 60-year-old male patient with a limited form of GPA who initially presented with bilateral chronic conjunctivitis and complete atrioventricular block. His visual acuity subsequently declined due to progression to bilateral panuveitis with exudative retinal detachment. The laboratory investigation revealed the elevation of acute phase reactants and strongly positive cytoplasmic ANCA (c-ANCA). Despite negative conjunctival and musculocutaneous biopsy results, the positive c-ANCA, and the clinical manifestation, i.e., heart and ocular involvement, led to the diagnosis of GPA. The remission was achieved with cyclophosphamide and methylprednisolone systemic therapy. CONCLUSIONS: A limited form of GPA may be a diagnostic chameleon. Though rare, it is essential to consider even extremely uncommon findings. Our patient is the first case of such a unique demonstration of the limited GPA manifesting as a bilateral ocular involvement and complete heart block.


Assuntos
Conjuntivite/etiologia , Granulomatose com Poliangiite/complicações , Bloqueio Cardíaco/etiologia , Descolamento Retiniano/etiologia , Acuidade Visual , Anticorpos Anticitoplasma de Neutrófilos/imunologia , Biópsia , Doença Crônica , Conjuntivite/diagnóstico , Eletrocardiografia , Granulomatose com Poliangiite/diagnóstico , Granulomatose com Poliangiite/imunologia , Bloqueio Cardíaco/diagnóstico , Humanos , Masculino , Pessoa de Meia-Idade , Descolamento Retiniano/diagnóstico , Tomografia Computadorizada por Raios X , Ultrassonografia
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