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1.
BMJ Case Rep ; 14(3)2021 Mar 18.
Artigo em Inglês | MEDLINE | ID: mdl-33737283

RESUMO

COVID-19 and granulomatosis with polyangiitis share many clinical and radiological features, making it challenging for clinicians to distinguish between the two. In this case report, we describe a patient who was diagnosed with COVID-19 in October 2020. One month later, she presented with persistent fatigue, shortness of breath and anaemia with worsening renal functions, found to have elevated antineutrophil cytoplasmic antibodies and antiproteinase 3 antibodies, and diagnosed with granulomatosis with polyangiitis.


Assuntos
/complicações , Granulomatose com Poliangiite/diagnóstico , Granulomatose com Poliangiite/etiologia , Anti-Inflamatórios/uso terapêutico , Biópsia , Diagnóstico Diferencial , Feminino , Granulomatose com Poliangiite/tratamento farmacológico , Humanos , Fatores Imunológicos/uso terapêutico , Rim/patologia , Hemissuccinato de Metilprednisolona/uso terapêutico , Pessoa de Meia-Idade , Rituximab/uso terapêutico
2.
BMJ Case Rep ; 14(2)2021 Feb 04.
Artigo em Inglês | MEDLINE | ID: mdl-33542003

RESUMO

A 69-year-old male patient presented to the retina clinic with a sudden decrease in vision in his right eye since 1 day. He was a known case of granulomatosis with polyangiitis and was on systemic immunosuppression for the past 3 years. The best-corrected visual acuity (BCVA) in his right eye was 20/60 and he has no perception of light in the left eye. Fundus examination revealed the presence of retinitis lesions in the right eye and total optic atrophy in the left eye. A vitreous biopsy was done and the PCR was found to be positive for cytomegalovirus (CMV). He was treated with intravitreal ganciclovir injections. Subsequently, the retinitis lesions regressed and BCVA in the right eye improved to 20/40.This case report elaborates on the risks of the development of opportunistic ocular infections in patients receiving long-term systemic immunosuppressants and the need for regular ocular examinations in such cases.


Assuntos
Antivirais/uso terapêutico , Retinite por Citomegalovirus , Ganciclovir/uso terapêutico , Granulomatose com Poliangiite/tratamento farmacológico , Imunossupressores/uso terapêutico , Idoso , Retinite por Citomegalovirus/diagnóstico , Retinite por Citomegalovirus/tratamento farmacológico , Humanos , Injeções Intravítreas , Masculino , Infecções Oportunistas , Atrofia Óptica/etiologia
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
4.
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
6.
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
7.
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.
Autoimmun Rev ; 20(2): 102737, 2021 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-33340770

RESUMO

OBJECTIVE: We investigated the effectiveness of rituximab (an anti-CD20 monoclonal antibody) in patients with eosinophilic granulomatosis with polyangiitis (EGPA). METHODS: We performed a systematic literature review from the inception dates until July 20, 2020 for articles reporting rituximab administration to treat EGPA. RESULTS: We identified a total of 171 patients; most of the patients had refractory or relapsing disease, whereas 14 patients were newly diagnosed with EGPA. Rituximab was used for induction therapy in all patients and administered as four infusions of 375 mg/m2/week, or two infusions of 1000 mg, given 2 weeks apart. The observation period was 6-36 months after rituximab initiation. The remission rates (defined as a Birmingham Vasculitis Activity Score of 0 along with low dose glucocorticoid) were 36 to 100%. Anti-neutrophil cytoplasmic antibody (ANCA)-positive patients tended to respond better to rituximab than ANCA-negative patients. All studies reported the successful reduction of glucocorticoid dose after rituximab treatment. The median glucocorticoid dose at rituximab initiation was 12.5-60 mg/day, which was successfully reduced to 0-8.5 mg/day after rituximab treatment. Scheduled rituximab maintenance treatment significantly reduced the relapse rates as compared to rituximab administered on demand. No new safety signal was reported. CONCLUSION: Rituximab effectively induced and sustained remission and reduced glucocorticoid dose in patients with newly diagnosed or relapsing and refractory EGPA; it also showed potentially greater benefit in ANCA-positive patients than in ANCA-negative patients.


Assuntos
Síndrome de Churg-Strauss , Granulomatose com Poliangiite , Anticorpos Anticitoplasma de Neutrófilos , Granulomatose com Poliangiite/diagnóstico , Granulomatose com Poliangiite/tratamento farmacológico , Humanos , Indução de Remissão , Rituximab/uso terapêutico
10.
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
12.
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
14.
Cochrane Database Syst Rev ; 9: CD008333, 2020 09 29.
Artigo em Inglês | MEDLINE | ID: mdl-32990324

RESUMO

BACKGROUND: Anti-neutrophilic cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) are a group of rare auto-inflammatory diseases that affects mainly small vessels. AAV includes: granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA). Anti-cytokine targeted therapy uses biological agents capable of specifically targeting and neutralising cytokine mediators of the inflammatory response. OBJECTIVES: To assess the benefits and harms of anti-cytokine targeted therapy for adults with AAV. SEARCH METHODS: We searched the Cochrane Central Register of Controlled Trials (2019, Issue 7), MEDLINE and Embase up to 16 August 2019. We also examined reference lists of articles, clinical trial registries, websites of regulatory agencies and contacted manufacturers. SELECTION CRITERIA: Randomised controlled trials (RCTs) or controlled clinical trials of targeted anti-cytokine therapy in adults (18 years or older) with AAV compared with placebo, standard therapy or another modality and anti-cytokine therapy of different type or dose. DATA COLLECTION AND ANALYSIS: We used standard methodological procedures expected by Cochrane. MAIN RESULTS: We included four RCTs with a total of 440 participants (mean age 48 to 56 years). We analysed the studies in three groups: 1) mepolizumab (300 mg; three separate injections every four weeks for 52 weeks) versus placebo in participants with relapsing or refractory EGPA; 2) belimumab (10 mg/kg on days 0, 14, 28 and every 28 days thereafter until 12 months after the last participant was randomised) or etanercept (25 mg twice a week) with standard therapy (median 25 months) versus placebo with standard therapy (median 19 months) in participants with GPA/MPA; and 3) infliximab (3 mg/kg on days 1 and 14, before the response assessment on day 42) versus rituximab (0.375g/m2 on days 1, 8, 15 and 22) in participants with refractory GPA for up to 12 months. None of the studies were assessed as low risk of bias in all domains: one study did not report randomisation or blinding methods clearly. Three studies were at high risk and one study was at unclear risk of bias for selective outcome reporting. One trial with 136 participants with relapsing or refractory EGPA compared mepolizumab with placebo during 52 weeks of follow-up and observed one death in the mepolizumab group (1/68, 1.5%) and none in the placebo group (0/68, 0%) (Peto odds ratio (OR) 7.39, 95% confidence interval (CI) 0.15 to 372.38; low-certainty evidence). Low-certainty evidence suggests that more participants in the mepolizumab group had ≥ 24 weeks of accrued remission over 52 weeks compared to placebo (27.9% versus 2.9%; risk ratio (RR) 9.5, 95% CI 2.30 to 39.21), and durable remission within the first 24 weeks sustained until week 52 (19.1% mepolizumab versus 1.5% placebo; RR 13.0, 95% CI 1.75 to 96.63; number needed to treat for an additional beneficial outcome (NNTB) 6, 95% Cl 4 to 13). Mepolizumab probably decreases risk of relapse (55.8% versus 82.4%; RR 0.68, 95% CI 0.53 to 0.86; NNTB 4, 95% CI 3 to 9; moderate-certainty evidence). There was low-certainty evidence regarding similar frequency of adverse events (AEs): total AEs (96.9% versus 94.1%; RR 1.03, 95% CI 0.96 to 1.11), serious AEs (17.7% versus 26.5%; RR 0.67, 95% CI 0.35 to 1.28) and withdrawals due to AEs (2.9% versus 1.5%; RR 2.00, 95% CI 0.19 to 21.54). Disease flares were not measured. Based on two trials with different follow-up periods (mean of 27 months for etanercept study; up to four years for belimumab study) including people with GPA (n = 263) and a small group of participants with MPA (n = 22) analysed together, we found low-certainty evidence suggesting that adding an active drug (etanercept or belimumab) to standard therapy does not increase or reduce mortality (3.4% versus 1.4%; Peto OR 2.45, 95% CI 0.55 to 10.97). Etanercept may have little or no effect on remission (92.3% versus 89.5%; RR 0.97, 95% CI 0.89 to 1.07), durable remission (70% versus 75.3%; RR 0.93, 95% CI 0.77 to 1.11; low-certainty evidence) and disease flares (56% versus 57.1%; RR 0.98, 95% CI 0.76 to 1.27; moderate-certainty evidence). Low-certainty evidence suggests that belimumab does not increase or reduce major relapse (1.9% versus 0%; RR 2.94, 95% CI 0.12 to 70.67) or any AE (92.5% versus 82.7%; RR 1.12, 95% CI 0.97 to 1.29). Low-certainty evidence suggests a similar frequency of serious or severe AEs (47.6% versus 47.6%; RR 1.00, 95% CI 0.80 to 1.27), but more frequent withdrawals due to AEs in the active drug group (11.2%) compared to the placebo group (4.2%), RR 2.66, 95% CI 1.07 to 6.59). One trial involving 17 participants with refractory GPA compared infliximab versus rituximab added to steroids and cytotoxic agents for 12 months. One participant died in each group (Peto OR 0.88, 95% CI, 0.05 to 15.51; 11% versus 12.5%). We have very low-certainty evidence for remission (22% versus 50%, RR 0.44, 95% Cl 0.11 to 1.81) and durable remission (11% versus 50%, RR 0.22, 95% CI 0.03 to 1.60), any severe AE (22.3% versus 12.5%; RR 1.78, 95% CI 0.2 to 16.1) and withdrawals due to AEs (0% versus 0%; RR 2.70, 95% CI 0.13 to 58.24). Disease flare/relapse and the frequency of any AE were not reported. AUTHORS' CONCLUSIONS: We found four studies but concerns about risk of bias and small sample sizes preclude firm conclusions. We found moderate-certainty evidence that in patients with relapsing or refractory EGPA, mepolizumab compared to placebo probably decreases disease relapse and low-certainty evidence that mepolizumab may increase the probability of accruing at least 24 weeks of disease remission. There were similar frequencies of total and serious AEs in both groups, but the study was too small to reliably assess these outcomes. Mepolizumab may result in little to no difference in mortality. However, there were very few events. In participants with GPA (and a small subgroup of participants with MPA), etanercept or belimumab may increase the probability of withdrawal due to AEs and may have little to no impact on serious AEs. Etanercept may have little or no impact on durable remission and probably does not reduce disease flare.


Assuntos
Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/tratamento farmacológico , Imunossupressores/administração & dosagem , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/mortalidade , Anticorpos Monoclonais Humanizados/administração & dosagem , Anticorpos Monoclonais Humanizados/efeitos adversos , Síndrome de Churg-Strauss/tratamento farmacológico , Etanercepte/administração & dosagem , Etanercepte/efeitos adversos , Granulomatose com Poliangiite/tratamento farmacológico , Humanos , Imunossupressores/efeitos adversos , Infliximab/administração & dosagem , Infliximab/efeitos adversos , Poliangiite Microscópica/tratamento farmacológico , Pessoa de Meia-Idade , Números Necessários para Tratar , Placebos/uso terapêutico , Ensaios Clínicos Controlados Aleatórios como Assunto , Rituximab/administração & dosagem , Rituximab/efeitos adversos , Prevenção Secundária , Esteroides/administração & dosagem
15.
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
16.
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
17.
Yonsei Med J ; 61(8): 712-719, 2020 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-32734735

RESUMO

PURPOSE: There has been no extensive study to compare the efficacy between rituximab originator (Mabthera®) and its biosimilar (Truxima®) for microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA). Here, we investigated the clinical effects of rituximab on poor outcomes of MPA and GPA in Korean patients, and compared those between Mabthera® and Truxima®. MATERIALS AND METHODS: We retrospectively reviewed the medical records of a total of 139 patients, including 97 MPA patients and 42 GPA patients. At diagnosis, antineutrophil cytoplasmic antibody positivity and comorbidities were assessed. During follow-up, all-cause mortality, relapse, end-stage renal disease, cerebrovascular accident and acute coronary syndrome were evaluated as poor outcomes. In this study, rituximab was used as either Mabthera® or Truxima®. RESULTS: The median age at diagnosis was 60.1 years and 46 patients were men (97 MPA and 42 GPA patients). Among poor outcomes, patients receiving rituximab exhibited a significantly lower cumulative relapse-free survival rate compared to those not receiving rituximab (p=0.002). Nevertheless, rituximab use did not make any difference in other poor outcomes of MPA and GPA except for relapse, which might be a rebuttal to the fact that rituximab use after relapse eventually led to better prognosis. There were no significant differences in variables at diagnosis and during follow-up between patients receiving Mabthera® and those receiving Truxima®. Patients receiving Truxima® exhibited a similar pattern of the cumulative survival rates of each poor outcome to those receiving Mabthera®. CONCLUSION: Truxima® prevents poor outcomes of MPA and GPA as effectively as does Mabthera®.


Assuntos
Medicamentos Biossimilares/uso terapêutico , Granulomatose com Poliangiite/tratamento farmacológico , Poliangiite Microscópica/tratamento farmacológico , Rituximab/uso terapêutico , Feminino , Granulomatose com Poliangiite/mortalidade , Humanos , Masculino , Poliangiite Microscópica/mortalidade , Pessoa de Meia-Idade , Prognóstico , Recidiva , Estudos Retrospectivos , Taxa de Sobrevida , Resultado do Tratamento
18.
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
19.
Reumatol. clín. (Barc.) ; 16(4): 294-297, jul.-ago. 2020. ilus
Artigo em Inglês | IBECS | ID: ibc-194957

RESUMO

Antineutrophil cytoplasmic antibodies (ANCA) associated vasculitides include granulomatosis with polyangiitis (GPA, previously called Wegener's), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA), previously called Churg-Strauss). In this report we used mycophenolate mofetil (MMF) and steroids to induce and maintain remission in two newly diagnosed cases with c-ANCA associated GPA. The two patients' maintained remission with no disease relapses during one year follow-up


Las vasculitis asociadas a anticuerpos anticitoplasma de neutrófilos (ANCA) incluyen granulomatosis con poliangeitis (GPA) anteriormente llamada de Wegener, poliangeítis microscópica (MPA) y granulomatosis eosinofílica con poliangeítis (EGPA) anteriormente llamada síndrome de Churg-Strauss. En este informe utilizamos micofenolato mofetilo (MMF) y esteroides para inducir y mantener la remisión en 2 casos recientemente diagnosticados con GPA asociado a c-ANCA. La remisión mantenida de los 2 pacientes sin enfermedad recae durante un año de duración del seguimiento


Assuntos
Humanos , Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Granulomatose com Poliangiite/tratamento farmacológico , Inibidores Enzimáticos/uso terapêutico , Ácido Micofenólico/uso terapêutico , Resultado do Tratamento , Seguimentos , Indução de Remissão
20.
Presse Med ; 49(3): 104031, 2020 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-32645418

RESUMO

Treatment of vasculitides associated with anti-neutrophil cytoplasm antibodies (ANCA) (AAVs) has evolved dramatically in recent years, particularly since the demonstration of rituximab efficacy as remission induction and maintenance therapy for granulomatosis with polyangiitis and microscopic polyangiitis. In 2013, the French Vasculitis Study Group (FVSG) published recommendations for its use by clinicians. Since then, new data have made it possible to better specify and codify prescription of rituximab to treat AAVs. Herein, the FVSG Recommendations Committee, an expert panel comprised of physicians with extensive experience in the treatment and management of vasculitides, presents its consensus guidelines based on literature analysis, the results of prospective therapeutic trials and personal experience.


Assuntos
Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/terapia , Terapia Biológica/normas , Cardiologia/normas , Imunossupressores/uso terapêutico , Quimioterapia de Manutenção/normas , Terapia Biológica/métodos , Cardiologia/organização & administração , França , Granulomatose com Poliangiite/tratamento farmacológico , Humanos , Quimioterapia de Manutenção/métodos , Guias de Prática Clínica como Assunto , Indução de Remissão , Sociedades Médicas/organização & administração , Sociedades Médicas/normas
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