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2.
Otol Neurotol ; 42(4): e380-e387, 2021 04 01.
Artigo em Inglês | MEDLINE | ID: mdl-33534386

RESUMO

OBJECTIVE: To perform a systematic review of the diagnosis, treatment, and management of patients with otologic manifestations of eosinophilic granulomatosis with polyangiitis. DATABASES REVIEWED: PubMed, Embase, Cochrane. METHODS: A systematic search for relevant published literature in PubMed, Cochrane Library, and EMBASE databases was done. Data was collected on demographics, otologic manifestations, specific diagnostic criteria fulfilled, common clinical, and imaging findings as well as medical and surgical treatments received. RESULTS: Fifteen articles encompassing 219 patients met inclusion criteria including 8 case reports and 7 case series. The mean age was 52.8 (range of 24-70). The most common otologic presenting symptoms were hearing loss (76%), otitis media with effusion (44%), vertigo (22%), tinnitus (21%), and chronic otitis media (20%). The most common diagnostic criteria observed in these patients were paranasal sinus abnormalities (75%), asthma (66%), and eosinophilia >10% (44%). The most common treatment was systemic steroids (66%), followed by surgery (52%), with myringotomy with tube insertion (M&T) (32%) and endoscopic sinus surgery (21%) being the most prevalent. Thirty-four percent of patients received an immunosuppressive agent. Successful treatments included systemic steroids (23%), surgical modalities (13%) with M&T being the most common (7%), topical nasal steroids (12%), and cyclophosphamide (10%). CONCLUSION: Hearing loss and a middle ear effusion are the most common presenting otologic manifestations of eosinophilic granulomatosis with polyangiitis. Sinonasal disease was more prevalent in patients with otologic manifestations. Primary treatment consists of systemic steroids and immunomodulation. M&T, endoscopic sinus surgery, and local steroid administration can be adjunctive measures to alleviate local disease.Level of Evidence: Level 1.


Assuntos
Síndrome de Churg-Strauss , Granulomatose com Poliangiite , Otite Média com Derrame , Otite Média , Granulomatose com Poliangiite/complicações , Humanos , Pessoa de Meia-Idade , Ventilação da Orelha Média , Otite Média com Derrame/etiologia , Otite Média com Derrame/cirurgia
3.
Medicine (Baltimore) ; 100(7): e24789, 2021 Feb 19.
Artigo em Inglês | MEDLINE | ID: mdl-33607834

RESUMO

RATIONALE: Granulomatosis with polyangiitis (GPA) is a rare systemic autoimmune disease of unknown etiology. GPA affects multiple ocular tissues, most commonly the orbit, conjunctiva, cornea, and sclera. Retinal and choroidal manifestations are rare in GPA, but they often include choroidal neovascularization (CNV). PATIENT CONCERNS: A 36-year-old man was diagnosed with GPA. He had been taking oral steroid treatment for 8 years. He experienced disease recurrence and the dose of oral prednisolone was increased after steroid pulse therapy. Fundus examination showed small retinal pigment epithelial detachment and serous retinal detachment (SRD). Optical coherence tomography (OCT) revealed a protruded lesion inside the SRD. Fluorescein angiography (FA) showed a small, dot-shaped fluorescein leakage in the SRD, and indocyanine green fluorescein fundus angiography showed choroidal vascular hyperpermeability that was consistent with the hyperfluorescence seen with FA. We had to determine whether the protruded lesion inside the SRD was CNV secondary to the inflammation due to GPA or whether it was central serous chorioretinopathy (CSC)-like condition caused by high-dose steroid treatment. DIAGNOSES: We confirmed that the SRD was due to CSC but not CNV because the protruded lesion examined by B-scan OCT angiography (OCTA) showed no blood flow. INTERVENTIONS: We decided to reduce the dose of steroid. OUTCOMES: Since the reduction of steroids, no sign of worsening in the protruded lesions with SRD has been observed. LESSONS: We therefore propose the effectiveness of this advanced function of OCTA for the examination of blood flow signal images to detect CNV.


Assuntos
Coriorretinopatia Serosa Central/etiologia , Glucocorticoides/efeitos adversos , Granulomatose com Poliangiite/tratamento farmacológico , Prednisolona/efeitos adversos , Descolamento Retiniano/etiologia , Administração Oral , Adulto , Progressão da Doença , Angiofluoresceinografia , Granulomatose com Poliangiite/complicações , Humanos , Masculino , Prednisolona/administração & dosagem , Tomografia de Coerência Óptica
5.
Medicine (Baltimore) ; 100(3): e24028, 2021 Jan 22.
Artigo em Inglês | MEDLINE | ID: mdl-33546000

RESUMO

RATIONALE: Bronchial involvement alone is a rare initial manifestation of granulomatosis with polyangiitis (GPA). Herein, we report a case of refractory GPA with obstructive pneumonia caused by bronchial involvement. PATIENT CONCERNS: A 65-year-old man complained of a 2-week cough and fever. DIAGNOSES: Considering the presence of opacities and multiple consolidations in both lungs due to obstruction or stenosis on the bronchus, which did not respond to antibiotics, and proteinase-3-antineutrophil cytoplasmic autoantibody positivity, he was diagnosed with GPA. Positron emission tomography- computed tomography scan revealed no abnormal findings in the upper respiratory tract. INTERVENTIONS: He was treated with prednisolone (PSL, 50 mg/d) and intravenous cyclophosphamide. OUTCOMES: His general and respiratory symptoms improved. However, 8 weeks after PSL treatment at 20 mg/d, he developed a relapse of vasculitis along with sinusitis and hypertrophic pachymeningitis. Hence, PSL treatment was resumed to 50 mg/d, and weekly administration of rituximab was initiated. Consequently, the symptoms gradually mitigated. LESSONS: GPA with bronchial involvement is often intractable and requires careful follow-up, which should include upper respiratory tract and hypertrophic pachymeningitis assessment.


Assuntos
Obstrução das Vias Respiratórias/etiologia , Granulomatose com Poliangiite/complicações , Meningite/etiologia , Pneumonia/etiologia , Idoso , Brônquios/patologia , Progressão da Doença , Humanos , Hipertrofia , Masculino
7.
BMJ Case Rep ; 14(1)2021 Jan 20.
Artigo em Inglês | MEDLINE | ID: mdl-33472807

RESUMO

Infectious scleritis is a rare but important cause of scleral inflammation. It is usually associated with an underlying ocular (prior ocular surgery or trauma) or systemic risk factor. A 53-year-old apparently systemically healthy woman presenting with spontaneous-onset pain, redness and watering in the left eye for 10 days was diagnosed with culture-proven Pseudomonas aeruginosa anterior scleritis. However, she was non-responsive to organism-sensitive antibiotics and scleral graft was performed twice, which showed graft re-infection. On repeated extensive systemic evaluations, the patient was diagnosed with biopsy-proven granulomatosis with polyangiitis (GPA). The patient was started on mycophenolate mofetil for both induction and maintenance phases and showed dramatic improvement with no recurrence till 1 year follow-up. High index of suspicion for autoimmune disorders, especially GPA, must be maintained for unilateral relentless infective scleritis masquerading as autoimmune necrotising scleritis. Mycophenolate mofetil holds a promising role for inducing as well as maintaining disease remission in ocular GPA.


Assuntos
Doenças Autoimunes/diagnóstico , Diagnóstico Diferencial , Granulomatose com Poliangiite/diagnóstico , Infecções por Pseudomonas/diagnóstico , Esclerite/diagnóstico , Antibacterianos , Cefazolina/uso terapêutico , Feminino , Glucocorticoides/uso terapêutico , Granulomatose com Poliangiite/complicações , Granulomatose com Poliangiite/tratamento farmacológico , Granulomatose com Poliangiite/patologia , Humanos , Imunossupressores/uso terapêutico , Pessoa de Meia-Idade , Ácido Micofenólico , Polimixina B/uso terapêutico , Infecções por Pseudomonas/tratamento farmacológico , Infecções por Pseudomonas/etiologia , Infecções por Pseudomonas/patologia , Pseudomonas aeruginosa , Esclerite/tratamento farmacológico , Esclerite/etiologia , Esclerite/patologia , Tropanos/uso terapêutico
8.
Nihon Shokakibyo Gakkai Zasshi ; 118(1): 70-77, 2021.
Artigo em Japonês | MEDLINE | ID: mdl-33431752

RESUMO

A 22-year-old woman who was diagnosed with Crohn's disease experienced diarrhea and bloody stool. She was suspected of have aggravated Crohn's disease and was transferred to our hospital. Upper gastrointestinal endoscopy revealed multiple esophageal ulcers and erosive gastritis, while colonoscopy revealed multiple ulcers in the rectum to the sigmoid colon. Initially, the evidence suggested that the Crohn's disease had worsened, and consequently, prednisolone (PSL) therapy was initiated. However, the patient's condition was determined to be atypical inflammatory bowel disease, which was indicated by endoscopic findings and skin symptoms and because various test results did not meet the diagnostic criteria for Crohn's disease. As a result, her diagnosis was changed to granulomatosis with polyangiitis. Here, we report a case of granulomatosis with polyangiitis with gastrointestinal symptoms similar to Crohn's disease, both of which have been suggested to involve Th1/Th17 cells.


Assuntos
Doença de Crohn , Granulomatose com Poliangiite , Adulto , Colonoscopia , Doença de Crohn/complicações , Doença de Crohn/tratamento farmacológico , Diarreia , Feminino , Hemorragia Gastrointestinal , Granulomatose com Poliangiite/complicações , Granulomatose com Poliangiite/tratamento farmacológico , Humanos , Adulto Jovem
9.
BMJ Case Rep ; 14(1)2021 Jan 28.
Artigo em Inglês | MEDLINE | ID: mdl-33509867

RESUMO

A man in his early 60s with a medical history of granulomatosis with polyangiitis (GPA) in remission for two decades without maintenance therapy presented with non-specific complaints of profound fatigue and 40-pound weight loss. He was seronegative for antinuclear antibodies and cytoplasmic antineutrophilic antibodies, but erythrocyte sedimentation rate and C reactive protein levels were elevated. Endocrinological testing revealed adrenal insufficiency, hypogonadism, hypothyroidism and diabetes insipidus. An MRI of the head revealed extensive sinonasal inflammation eroding through the floor of the sella turcica and into the pituitary gland and stalk. Biopsy of the sinonasal tissues was inconclusive. On review of his case, a multidisciplinary team diagnosed him with panhypopituitarism secondary to a recurrence of GPA. He responded well to glucocorticoids and methotrexate with marked reduction of pituitary enhancement on imaging and resolution of diabetes insipidus. He will require lifelong testosterone, levothyroxine and glucocorticoids for hormone replacement therapy.


Assuntos
Insuficiência Adrenal/diagnóstico , Diabetes Insípido/diagnóstico , Granulomatose com Poliangiite/diagnóstico por imagem , Hipogonadismo/diagnóstico , Hipopituitarismo/diagnóstico , Hipotireoidismo/diagnóstico , Rinite/diagnóstico por imagem , Sinusite/diagnóstico por imagem , Insuficiência Adrenal/tratamento farmacológico , Insuficiência Adrenal/etiologia , Androgênios/uso terapêutico , Diabetes Insípido/etiologia , Fadiga/etiologia , Glucocorticoides/uso terapêutico , Granulomatose com Poliangiite/complicações , Granulomatose com Poliangiite/tratamento farmacológico , Granulomatose com Poliangiite/patologia , Terapia de Reposição Hormonal , Humanos , Hipogonadismo/tratamento farmacológico , Hipogonadismo/etiologia , Hipopituitarismo/tratamento farmacológico , Hipopituitarismo/etiologia , Hipotireoidismo/tratamento farmacológico , Hipotireoidismo/etiologia , Imunossupressores/uso terapêutico , Imagem por Ressonância Magnética , Masculino , Metotrexato/uso terapêutico , Pessoa de Meia-Idade , Doenças da Hipófise , Hipófise/diagnóstico por imagem , Recidiva , Rinite/patologia , Sela Túrcica/diagnóstico por imagem , Sinusite/patologia , Testosterona/uso terapêutico , Tiroxina/uso terapêutico , Perda de Peso
10.
BMJ Case Rep ; 14(1)2021 Jan 28.
Artigo em Inglês | MEDLINE | ID: mdl-33509869

RESUMO

Granulomatosis with polyangiitis (GPA) is characterised by systemic necrotising vasculitis of small arteries and veins with multitude of organ involvement, with the most common being the upper and lower respiratory tract and renal system. Gastrointestinal involvement is a rare late manifestation with a high mortality rate and usually results in intestinal perforation. Our patient presented with gastrointestinal bleeding secondary to jejunal artery vasculitis. Gastrointestinal bleeding as initial presentation of GPA is very rarely documented. CT mesenteric angiogram is helpful for the localisation of bleed in these cases. In case of refractory bleeding, surgical excision is required.


Assuntos
Hemorragia Gastrointestinal/etiologia , Granulomatose com Poliangiite/complicações , Jejuno/irrigação sanguínea , Pneumopatias/etiologia , Angiografia , Embolização Terapêutica , Feminino , Glucocorticoides/uso terapêutico , Granulomatose com Poliangiite/diagnóstico , Granulomatose com Poliangiite/terapia , Hemorragia/etiologia , Humanos , Pneumopatias/diagnóstico por imagem , Pessoa de Meia-Idade , Plasmaferese , Pulsoterapia , Recidiva , Insuficiência Respiratória/etiologia , Circulação Esplâncnica
11.
BMJ Case Rep ; 14(1)2021 Jan 11.
Artigo em Inglês | MEDLINE | ID: mdl-33431530

RESUMO

Granulomatosis with polyangiitis (GPA) is a necrotising vasculitis of unknown cause that has several systemic manifestations. The disease is characterised by the classical triad involving acute inflammation of the upper and lower respiratory tracts with renal involvement. However, the disease pathology can involve the central nervous system. This case report presents a case of GPA with facial nerve palsy as the first manifestation of the disease, which has been rarely reported in the medical literature.


Assuntos
Paralisia Facial/etiologia , Granulomatose com Poliangiite/diagnóstico , Perfuração do Septo Nasal/etiologia , Convulsões/etiologia , Adolescente , Anticorpos Anticitoplasma de Neutrófilos/sangue , Encéfalo/diagnóstico por imagem , Ciclofosfamida/administração & dosagem , Paralisia Facial/sangue , Paralisia Facial/diagnóstico , Paralisia Facial/terapia , Feminino , Granulomatose com Poliangiite/sangue , Granulomatose com Poliangiite/complicações , Granulomatose com Poliangiite/terapia , Humanos , Imagem por Ressonância Magnética , Metilprednisolona/administração & dosagem , Perfuração do Septo Nasal/diagnóstico , Septo Nasal/diagnóstico por imagem , Seios Paranasais/diagnóstico por imagem , Plasmaferese , Pulsoterapia , Convulsões/sangue , Convulsões/diagnóstico , Convulsões/terapia , Tomografia Computadorizada por Raios X
12.
BMJ Case Rep ; 14(1)2021 Jan 08.
Artigo em Inglês | MEDLINE | ID: mdl-33419755

RESUMO

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare disease with an estimated annual incidence of 0.5-6.8 per million. It is characterised by necrotising vasculitis with multiorgan eosinophilic infiltration. Pulmonary manifestations are the most common presentation of EGPA, and cardiac complications are the most common cause of death. Anaesthetic management of EGPA is challenging due to perioperative pulmonary complications, multiorgan involvement and greater risk of cholinesterase enzyme deficiency. We are reporting the anaesthetic management of a 58-year-old woman, diagnosed with EGPA 3 years ago, who underwent urgent intramedullary nail insertion for a femur fracture. The anaesthetic technique comprised femoral nerve block and spinal anaesthesia, thereby avoiding (1) the need for upper airway manipulation, (2) potential adverse effects of anticholinesterase drugs (for reversal of neuromuscular blockade) and (3) histamine release associated with morphine administration perioperatively. Surgery and anaesthesia were uneventful.


Assuntos
Raquianestesia , Eosinofilia/complicações , Fraturas do Fêmur/cirurgia , Fixação Intramedular de Fraturas , Granulomatose com Poliangiite/complicações , Bloqueio Nervoso , Feminino , Fraturas do Fêmur/complicações , Humanos , Pessoa de Meia-Idade
14.
Eur J Intern Med ; 85: 68-79, 2021 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-33358337

RESUMO

BACKGROUND: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic vasculitis. Cardiac specific involvement (CSI) is caused by coronary artery vasculitis, but also by myocardial eosinophilic infiltration. To date, the prevalence of CSI associated with EGPA is unresolved. Aim of this study was to systematically assess the prevalence and clinical impact of CSI in a consecutive outpatient EGPA population. METHODS: Between October 2018 and July 2019, we prospectively enrolled 52 consecutive EGPA patients. All underwent comprehensive evaluation including a standardized questionnaire, physical examination, 12-lead-ECG, echocardiography. Cardiac magnetic resonance and 24 h-Holter were performed as deemed clinically appropriate. Cardiac abnormalities were defined as CSI based on the likelihood of their relation to EGPA vasculitis, after exclusion of alternative diagnoses. RESULTS: 52 enrolled patients, mean age 59±1 years. Thirteen of the 52 patients (25%) were classified as CSI+. CSI was characterized by myocarditis in four patients, non-scar-related regional wall motions abnormalities (RWMA) in three, apical thrombosis in two (one also had RWMA), pericarditis in three and non-atherosclerotic coronary disease (Prinzmetal angina and coronaritis) in 2. Five (38%) of the 13 CSI+ patients, presented an apical aneurysm. Peak eosinophil count at diagnosis was higher in CSI+: 8000 /µl vs CSI-: 3000 /µl, p = 0.017. Overall, 2 patients had severe LV dysfunction, 5 required urgent hospitalization and 8 required long-term cardioactive therapy. CONCLUSIONS: CSI was present in one-quarter of patients, often associated with high peak eosinophils. Myocarditis, RWMA and apical aneurysms were the most common manifestations. Although rarely severe and life-threatening, CSI often required long-term cardioactive treatment.


Assuntos
Síndrome de Churg-Strauss , Granulomatose com Poliangiite , Síndrome de Churg-Strauss/complicações , Síndrome de Churg-Strauss/diagnóstico , Síndrome de Churg-Strauss/epidemiologia , Granulomatose com Poliangiite/complicações , Granulomatose com Poliangiite/epidemiologia , Coração , Humanos , Pessoa de Meia-Idade , Estudos Prospectivos , Centros de Atenção Terciária
15.
J Stroke Cerebrovasc Dis ; 30(3): 105539, 2021 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-33333478

RESUMO

BACKGROUND: Eosinophilic Granulomatosis with Polyangiitis (EGPA) is a group of systemic disorders characterized by inflammation of blood vessels and eosinophilia. Simultaneous brain and splenic infarcts are extremely rare in patients with EGPA. CASE DESCRIPTION: We report a case of a 61-year-old male with a history of asthma and sinusitis who presented with paresthesia and purpura in the lower extremities. Eosinophilia and positive Myeloperoxidase-anti-neutrophil cytoplasmic antibody were present and the diagnosis of EGPA was confirmed. Multiple bilateral cerebral and cerebellar infarcts and splenic infarction were detected. Although there was evidence of myocarditis, no cardiac thrombus was detected. Immunosuppressive and anticoagulation therapy were provided. The patient was fully recovered. CONCLUSIONS: EGPA can present as splenic infarction and ischemic stroke. Prompt diagnosis and treatment with anticoagulant and immunosuppressive agents may lead to good prognosis.


Assuntos
Granulomatose com Poliangiite/complicações , Infarto do Baço/etiologia , Anticorpos Anticitoplasma de Neutrófilos/sangue , Anticoagulantes/uso terapêutico , Biomarcadores/sangue , Granulomatose com Poliangiite/diagnóstico , Granulomatose com Poliangiite/tratamento farmacológico , Humanos , Imunossupressores/uso terapêutico , /tratamento farmacológico , Masculino , Pessoa de Meia-Idade , Infarto do Baço/diagnóstico por imagem , Infarto do Baço/tratamento farmacológico , Resultado do Tratamento
18.
Front Immunol ; 11: 2086, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32983161

RESUMO

Immunosuppressive therapies increase the susceptibility of patients to infections. The current pandemic with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) compels clinicians to develop recommendations for successful clinical management and surveillance of immunocompromised patients at high risk for severe disease progression. With only few case studies published on SARS-CoV-2 infection in patients with rheumatic diseases, we report a 25-year-old male who developed moderate coronavirus disease 2019 (COVID-19) with fever, mild dyspnea, and no major complications despite having received high-dose prednisolone, cyclophosphamide, and rituximab for the treatment of highly active, life-threatening eosinophilic granulomatosis with polyangiitis (EGPA).


Assuntos
Betacoronavirus/genética , Síndrome de Churg-Strauss/complicações , Síndrome de Churg-Strauss/tratamento farmacológico , Infecções por Coronavirus/complicações , Ciclofosfamida/uso terapêutico , Granulomatose com Poliangiite/complicações , Granulomatose com Poliangiite/tratamento farmacológico , Imunossupressores/uso terapêutico , Pneumonia Viral/complicações , Adulto , Antivirais/uso terapêutico , Infecções por Coronavirus/tratamento farmacológico , Infecções por Coronavirus/virologia , Quimioterapia Combinada , Glucocorticoides/uso terapêutico , Humanos , Hospedeiro Imunocomprometido , Masculino , Pandemias , Pneumonia Viral/tratamento farmacológico , Pneumonia Viral/virologia , Prednisolona/uso terapêutico , RNA Viral/genética , Reação em Cadeia da Polimerase Via Transcriptase Reversa , Rituximab/uso terapêutico , Resultado do Tratamento
19.
RMD Open ; 6(2)2020 09.
Artigo em Inglês | MEDLINE | ID: mdl-32878994

RESUMO

OBJECTIVES: Patients with inflammatory rheumatic diseases (IRD) infected with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) may be at risk to develop a severe course of COVID-19. The influence of immunomodulating drugs on the course of COVID-19 is unknown. To gather knowledge about SARS-CoV-2 infections in patients with IRD, we established a registry shortly after the beginning of the pandemic in Germany. METHODS: Using an online questionnaire (www.COVID19-rheuma.de), a nationwide database was launched on 30 March 2020, with appropriate ethical and data protection approval to collect data of patients with IRD infected with SARS-CoV-2. In this registry, key clinical and epidemiological parameters-for example, diagnosis of IRD, antirheumatic therapies, comorbidities and course of the infection-are documented. RESULTS: Until 25 April 2020, data from 104 patients with IRD infected with SARS-CoV-2 were reported (40 males; 63 females; 1 diverse). Most of them (45%) were diagnosed with rheumatoid arthritis, 59% had one or more comorbidities and 42% were treated with biological disease-modifying antirheumatic drugs. Hospitalisation was reported in 32% of the patients. Two-thirds of the patients already recovered. Unfortunately, 6 patients had a fatal course. CONCLUSIONS: In a short time, a national registry for SARS-CoV2-infected patients with IRD was established. Within 4 weeks, 104 cases were documented. The registry enables to generate data rapidly in this emerging situation and to gain a better understanding of the course of SARS-CoV2-infection in patients with IRD, with a distinct focus on their immunomodulatory therapies. This knowledge is valuable for timely information of physicians and patients with IRD, and shall also serve for the development of guidance for the management of patients with IRD during this pandemic.


Assuntos
Antirreumáticos/uso terapêutico , Produtos Biológicos/uso terapêutico , Infecções por Coronavirus/fisiopatologia , Pneumonia Viral/fisiopatologia , Sistema de Registros , Doenças Reumáticas/tratamento farmacológico , Adulto , Idoso , Idoso de 80 Anos ou mais , Artrite Psoriásica/complicações , Artrite Psoriásica/tratamento farmacológico , Artrite Reumatoide/complicações , Artrite Reumatoide/tratamento farmacológico , Betacoronavirus , Infecções por Coronavirus/complicações , Infecções por Coronavirus/mortalidade , Feminino , Alemanha , Granulomatose com Poliangiite/complicações , Granulomatose com Poliangiite/tratamento farmacológico , Hospitalização , Humanos , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Masculino , Pessoa de Meia-Idade , Pandemias , Pneumonia Viral/complicações , Pneumonia Viral/mortalidade , Polimialgia Reumática/complicações , Polimialgia Reumática/tratamento farmacológico , Prognóstico , Doenças Reumáticas/complicações , Escleroderma Sistêmico/complicações , Escleroderma Sistêmico/tratamento farmacológico , Índice de Gravidade de Doença , Espondilite Anquilosante/complicações , Espondilite Anquilosante/tratamento farmacológico , Adulto Jovem
20.
Presse Med ; 49(3): 104039, 2020 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-32650042

RESUMO

Lung involvement is one of the most common clinical features in ANCA-associated vasculitides (AAV), including granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). In this review, we detail the five main presentations of pulmonary involvement in AAV: necrotizing granulomatous inflammation, tracheobronchial inflammation, pulmonary capillaritis, interstitial lung disease (ILD) and asthma with their clinical, radiological and therapeutic characteristics. The prevalence of these manifestations is variable according to the subtype of AAV, necrotizing granulomatous inflammation and tracheobronchial inflammation being defining features of GPA whereas ILD is primarily seen in patients with MPA, especially in association with ANCA directed against myeloperoxydase (MPO-ANCA), and asthma is characteristic of EGPA. Despite recent progresses in the diagnosis and management of these conditions, several questions remain and are discussed here, including local treatments for subglottic stenosis, the uncertain efficacy of plasma exchanges for alveolar hemorrhage, the potential role of antifibrotic agents in ILD associated with MPA, and the use of novel anti-IL-5 strategies in EGPA.


Assuntos
Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/complicações , Doenças Pulmonares Intersticiais/etiologia , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/patologia , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/terapia , Asma/etiologia , Asma/patologia , Asma/terapia , Síndrome de Churg-Strauss/complicações , Síndrome de Churg-Strauss/patologia , Síndrome de Churg-Strauss/terapia , Granuloma/etiologia , Granuloma/patologia , Granuloma/terapia , Granulomatose com Poliangiite/complicações , Granulomatose com Poliangiite/patologia , Granulomatose com Poliangiite/terapia , Humanos , Inflamação/etiologia , Inflamação/patologia , Inflamação/terapia , Doenças Pulmonares Intersticiais/patologia , Doenças Pulmonares Intersticiais/terapia , Poliangiite Microscópica/complicações , Poliangiite Microscópica/patologia , Poliangiite Microscópica/terapia , Necrose/etiologia , Necrose/patologia , Necrose/terapia
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