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2.
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
3.
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.
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
7.
Presse Med ; 49(3): 104038, 2020 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-32634467

RESUMO

There have been great advances in the management of ANCA associated vasculitis over the past decades. We have gone from an era where the disease was almost universally fatal to trying to prevent long-term side effects of treatment regimens. With the ability to use pulse cyclophosphamide or rituximab as alternates to oral cyclophosphamide for induction of remission, side effects of therapy have been greatly reduced. New approaches have drastically changed our approach to maintenance and we now favor much longer durations of maintenance therapy, as they are more successful in preventing relapse. Steroids have long been the bane of treatment as they are associated with a significant risk of infection and metabolic consequences. We are now in a steroid-sparing and looking ahead to a steroid-free era with new data being published showing lower doses of steroids being equally effective and several ongoing seminal trials looking at agents that could completely replace steroids very early on.


Assuntos
Corticosteroides/efeitos adversos , Efeitos Colaterais e Reações Adversas Relacionados a Medicamentos/prevenção & controle , Granulomatose com Poliangiite/terapia , Poliangiite Microscópica/terapia , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/epidemiologia , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/patologia , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/terapia , Azatioprina/uso terapêutico , Cardiologia/métodos , Cardiologia/tendências , Ciclofosfamida/uso terapêutico , Efeitos Colaterais e Reações Adversas Relacionados a Medicamentos/epidemiologia , Granulomatose com Poliangiite/epidemiologia , Granulomatose com Poliangiite/patologia , Hematologia/métodos , Hematologia/tendências , Humanos , Imunossupressores/uso terapêutico , Quimioterapia de Manutenção/efeitos adversos , Quimioterapia de Manutenção/métodos , Poliangiite Microscópica/epidemiologia , Poliangiite Microscópica/patologia , Troca Plasmática , Indução de Remissão , Rituximab/uso terapêutico
8.
Presse Med ; 49(3): 104036, 2020 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-32652104

RESUMO

Eosinophilic granulomatosis with polyangiitis (EGPA, formerly Churg-Strauss syndrome) is the least frequent antineutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV). Major advances of our knowledge on its pathophysiology have revealed features of both AAV and eosinophilic disorders. The development of targeted biotherapies for both diseases opened new possibilities for EGPA management. In this review, we highlight the rationale underlying the routine treatment strategy, which relies mainly on corticosteroids, with immunosuppressant adjunction for severe disease. However, novel therapies are still needed for refractory/relapsing disease and to alleviate the corticosteroid-dependence of asthma and chronic rhinosinusitis. At present, the most promising biotherapies target either eosinophil biology, like mepolizumab, an anti-interleukin-5, or the B-cell compartment, with rituximab. Recent clinical data on new treatment options are discussed and therapeutic strategies are proposed.


Assuntos
Síndrome de Churg-Strauss/terapia , Corticosteroides/uso terapêutico , Anticorpos Anticitoplasma de Neutrófilos/efeitos adversos , Anticorpos Monoclonais Humanizados/uso terapêutico , Terapia Biológica/métodos , Terapia Biológica/tendências , Síndrome de Churg-Strauss/patologia , Glucocorticoides/uso terapêutico , Granulomatose com Poliangiite/patologia , Granulomatose com Poliangiite/terapia , Humanos , Imunossupressores/uso terapêutico , Rituximab/uso terapêutico
9.
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
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
12.
Am J Pathol ; 190(7): 1438-1448, 2020 07.
Artigo em Inglês | MEDLINE | ID: mdl-32251643

RESUMO

The immunologic mechanisms promoting eosinophilic granulomatosis with polyangiitis (EGPA) are unclear. To characterize the mechanisms underlying pulmonary EGPA, we examined and compared EGPA paraffin-embedded lung biopsies with normal lung biopsies, using immunostaining, RNA sequencing, and RT-PCR. The results revealed novel type 2 as well as immuneregulatory features. These features included basophils and increased mast cell contents; increased immunostaining for tumor necrosis factor ligand superfamily member 14; sparse mast cell degranulation; numerous forkhead box protein P3 (FoxP3)+ regulatory T cells and IgG4 plasma cells; and abundant arachidonate 15-lipoxygenase and 25-hydroxyvitamin D-1 α hydroxylase, mitochondrial. Significantly decreased 15-hydroxyprostaglandin dehydrogenase [NAD(+)], which degrades eicosanoids, was observed in EGPA samples. In addition, there was significantly increased mRNA for chemokine (C-C motif) ligands 18 and 13 and major collagen genes, IgG4-rich immune complexes coating alveolar macrophages, and increased immunostaining for phosphorylated mothers against decapentaplegic homolog 2/SMAD2, suggesting transforming growth factor-ß activation. These findings suggest a novel self-promoting mechanism of activation of alveolar macrophages by arachidonate 15-lipoxygenase-derived eicosanoids to express chemokines that recruit a combined type 2/immunoregulatory immune response, which produces these eicosanoids. These results suggest that the pulmonary EGPA immune response resembles the immune response to a tissue-invasive parasite infection.


Assuntos
Síndrome de Churg-Strauss/imunologia , Granulomatose com Poliangiite/imunologia , Imunoglobulina G/imunologia , Plasmócitos/imunologia , Adulto , Síndrome de Churg-Strauss/patologia , Feminino , Granulomatose com Poliangiite/patologia , Humanos , Masculino
13.
J Oral Pathol Med ; 49(5): 443-449, 2020 May.
Artigo em Inglês | MEDLINE | ID: mdl-32133698

RESUMO

BACKGROUND: Reports of oral manifestations of granulomatosis with polyangiitis (GPA) usually refer to single-case reports; "strawberry gingivitis" has been increasingly reported. OBJECTIVE: To study the clinicopathological findings of four patients in which the diagnosis of GPA was suspected from the observation of their oral lesions and compare these to existent data. METHODS: Retrospective study of a case series. RESULTS: One patient presented typical "strawberry gingivitis" with localized disease and negative ANCA results. Two patients presented rapidly growing oral ulcers associated with systemic compromise and high ANCA levels. One patient presented with a chronic granulomatous lesion that leaded to palatal perforation. CONCLUSION: Oral manifestations of GPA may vary from rapidly evolving lesions in acutely ill patients to chronic and locally destructive lesions in slowing developing disease. These differences are also evident in the histopathological findings.


Assuntos
Gengivite/patologia , Granulomatose com Poliangiite/patologia , Palato/patologia , Anticorpos Anticitoplasma de Neutrófilos/sangue , Gengivite/etiologia , Granulomatose com Poliangiite/complicações , Humanos , Estudos Retrospectivos
15.
Am J Kidney Dis ; 75(1): 124-137, 2020 01.
Artigo em Inglês | MEDLINE | ID: mdl-31358311

RESUMO

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of disorders characterized by inflammation and destruction of small- and medium-sized blood vessels and the presence of circulating ANCA. Clinical disease phenotypes include granulomatosis with polyangiitis, microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis, and renal-limited vasculitis. Serologic classification of AAV into proteinase 3-ANCA disease and myeloperoxidase-ANCA disease correlates with a number of disease characteristics. AAV has a predilection for the kidney, with >75% of patients having renal involvement characterized by rapidly progressive glomerulonephritis. The cause and pathogenesis of AAV are multifactorial and influenced by genetics, environmental factors, and responses of the innate and adaptive immune system. Randomized controlled trials in the past 2 decades have refined the therapy of AAV and transformed AAV from a fatal disease to a chronic illness with relapsing course and associated morbidity. This article in AJKD's Core Curriculum in Nephrology series provides a detailed review of the epidemiology, pathogenesis, diagnosis, and advances in the management of AAV.


Assuntos
Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/terapia , Glomerulonefrite/terapia , Glucocorticoides/uso terapêutico , Imunossupressores/uso terapêutico , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/genética , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/imunologia , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/patologia , Anticorpos Anticitoplasma de Neutrófilos/imunologia , Síndrome de Churg-Strauss/genética , Síndrome de Churg-Strauss/imunologia , Síndrome de Churg-Strauss/patologia , Síndrome de Churg-Strauss/terapia , Ciclofosfamida/uso terapêutico , Progressão da Doença , Glomerulonefrite/genética , Glomerulonefrite/imunologia , Glomerulonefrite/patologia , Granulomatose com Poliangiite/genética , Granulomatose com Poliangiite/imunologia , Granulomatose com Poliangiite/patologia , Granulomatose com Poliangiite/terapia , Humanos , Transplante de Rim , Poliangiite Microscópica/genética , Poliangiite Microscópica/imunologia , Poliangiite Microscópica/patologia , Poliangiite Microscópica/terapia , Ácido Micofenólico/uso terapêutico , Mieloblastina/imunologia , Peroxidase/imunologia , Indução de Remissão , Diálise Renal , Rituximab/uso terapêutico
16.
Intern Med ; 59(8): 1029-1033, 2020 Apr 15.
Artigo em Inglês | MEDLINE | ID: mdl-31875634

RESUMO

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


Assuntos
Eosinofilia/diagnóstico , Eosinofilia/patologia , Granulomatose com Poliangiite/diagnóstico , Granulomatose com Poliangiite/patologia , Adulto , Biópsia , Síndrome de Churg-Strauss/diagnóstico , Enterite/diagnóstico , Feminino , Gastrite/diagnóstico , Humanos , Pessoa de Meia-Idade , Pele/patologia
17.
Scand J Rheumatol ; 49(1): 57-67, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31610684

RESUMO

Objectives: Neutrophil apoptosis is mandatory for resolving inflammation and is regulated by expression of pro- and anti-apoptotic genes. We studied neutrophils isolated from patients with granulomatosis with polyangiitis (GPA) to investigate apoptosis alterations and to identify transcriptional and circulating factors affecting this process.Method: We enrolled 36 patients (18 in active stage, 18 in remission) and 18 healthy controls. Circulating levels of tumour necrosis factor-α (TNF-α), granulocyte-macrophage colony-stimulating factor (GM-CSF), macrophage migration inhibitory factor, plasminogen activator inhibitor-1, interferon-γ, vascular cell adhesion molecule-1, intercellular adhesion molecule-1, platelet endothelial cell adhesion molecule-1, soluble Fas (sFas), sFas ligand, survivin, and pentraxin-3 (PTX3) were evaluated by enzyme-linked immunosorbent assay/Luminex; circulating apoptotic neutrophils by flow cytometry; and apoptosis-related gene transcripts by real-time polymerase chain reaction.Results: Patients had decreased fractions of circulating apoptotic neutrophils and delayed neutrophil apoptosis was present in vitro. Circulating levels of TNF-α, GM-CSF, sFas, and PTX3 were higher in GPA. Delayed neutrophil apoptosis was accompanied by decreased mRNA of pro-apoptotic genes and transcription factors (DIABLO, PMAIP1, BAX, CASP3, CASP7, RUNX3, E2F1, TP53) and increased anti-apoptotic CFLAR and BCL2A1 mRNA. TNF-α and sFas levels correlated with circulating apoptotic neutrophils and expression of apoptosis genes. Stimulation with TNF-α of neutrophils from controls significantly down-regulated E2F1 and CASP3 expression.Conclusions: Circulating neutrophils in GPA have anti-apoptotic phenotype involving both intrinsic and extrinsic pathways of apoptosis. This is accompanied by increased levels of circulating pro-survival factors (GM-CSF, TNF-α, sFas), independent of disease activity. Anti-apoptotic phenotype of neutrophils in GPA is reproduced by exposure to low concentrations of TNF-α.


Assuntos
Proteínas Reguladoras de Apoptose/genética , Apoptose , Regulação da Expressão Gênica , Granulomatose com Poliangiite/genética , Neutrófilos/patologia , Proteínas Reguladoras de Apoptose/sangue , Células Cultivadas , Feminino , Citometria de Fluxo , Granulomatose com Poliangiite/sangue , Granulomatose com Poliangiite/patologia , Humanos , Masculino , Pessoa de Meia-Idade , Fenótipo
18.
Medicine (Baltimore) ; 98(51): e18139, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31860960

RESUMO

RATIONALE: Rituximab is recommended to induce remission of severe granulomatosis with polyangiitis (GPA). Plasma exchange (PE) may be considered in the setting of rapidly progressive glomerulonephritis (RPGN) with a serum creatinine increase of more than 5.6 mg/dl or diffuse alveolar hemorrhage (DAH). However, there are no sufficient studies on combination therapy with rituximab and PE in GPA. PATIENT CONCERNS: A 23-year-old woman was admitted with fever, abdominal pain, and diarrhea on suspicion of infectious colitis. Colonoscopy showed hemorrhagic colitis and antibiotic treatment was ineffective. Physical examination revealed episcleritis and skin lesions similar to Janeway lesions or Osler nodes on her palms and soles. Transesophageal echocardiogram (TEE) revealed mitral valve vegetation mimicking infective endocarditis. However, no pathogen was grown in the blood culture. Ten days after admission, blood-tinged sputum and respiratory distress developed. Imaging studies of lung, bronchoscopy, and bronchoalveolar lavage indicated DAH. Moreover, serum creatinine levels rapidly increased from 0.8 mg/dl to 6.1 mg/dl with proteinuria. DIAGNOSIS: The patient was diagnosed with GPA and non-infectious endocarditis, DAH, and RPGN, based on a biopsy which revealed pauci-immune crescentic glomerulonephritis with granuloma and leukocytoclastic vasculitis and antineutrophil cytoplasmic antibodies against proteinase 3- positivity. INTERVENTIONS: Initial methylprednisolone pulse therapy (1 g daily for 3 days) proved unsuccessful. After initiating PE, creatinine levels began to slowly decline, but DAH continued to deteriorate. Rituximab combined with PE therapy was considered. We performed PE every 2 to 3 days for 5 total treatments combined with rituximab (375 mg/m, once weekly for 4 weeks). OUTCOMES: After the combination treatment of rituximab and PE, alveolar hemorrhage stopped. Chest X-ray and laboratory data, including serum creatinine and hemoglobin, notably improved. Mitral valve vegetation was no longer observed in follow-up TEE. GPA remained stable with low dose prednisolone and immunosuppressants over a follow-up period of 5 years. LESSONS: This case suggests that the use of rituximab and concurrent PE may represent a promising combination for severe and refractory GPA.


Assuntos
Granulomatose com Poliangiite/patologia , Granulomatose com Poliangiite/terapia , Troca Plasmática/métodos , Rituximab/uso terapêutico , Anticorpos Monoclonais/uso terapêutico , Biópsia por Agulha , Colite/diagnóstico , Colite/etiologia , Colonoscopia/métodos , Terapia Combinada , Esquema de Medicação , Serviço Hospitalar de Emergência , Feminino , Seguimentos , Granulomatose com Poliangiite/diagnóstico , Humanos , Imuno-Histoquímica , Medição de Risco , Índice de Gravidade de Doença , Fatores de Tempo , Tomografia Computadorizada por Raios X/métodos , Resultado do Tratamento , Adulto Jovem
19.
Expert Rev Clin Immunol ; 15(12): 1273-1286, 2019 12.
Artigo em Inglês | MEDLINE | ID: mdl-31762340

RESUMO

Introduction: The majority of the patients with anti-neutrophil cytoplasmic autoantibody (ANCA) associated vasculitis (AAV) achieve remission with effective induction therapy. Therefore, prevention of relapses and avoiding long-term damage and treatment-related toxicity are major challenges.Areas covered: This review provides an update on maintenance therapy in AAV, emphasizing the available treatment options for granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA). Among the spectrum of all patients with AAV, those at higher risk of relapse have recently been identified. Clinical trials have yielded robust results about various options for maintenance of remission including common disease-modifying anti-rheumatic drugs (DMARDs, i.e. azathioprine, methotrexate, and mycophenolate mofetil) and rituximab (RTX). However, outcomes of these studies are not easy to compare.Expert opinion: Regardless of the treatment used, patients presenting with an anti-proteinase-3 ANCA, relapsing GPA have a substantially higher risk of relapse compared to patients with newly diagnosed MPA or positive anti-myeloperoxidase ANCA. While the efficacy of common DMARDs for remission maintenance is heterogeneous, the role of RTX seems particularly promising for the high-risk patients, although the most appropriate dose and timing of retreatment with RTX remain under controversial. Low-dose glucocorticoid use for remission maintenance versus complete discontinuation also remains under investigation.


Assuntos
Antirreumáticos/uso terapêutico , Granulomatose com Poliangiite/tratamento farmacológico , Quimioterapia de Manutenção , Poliangiite Microscópica/tratamento farmacológico , Ensaios Clínicos como Assunto , Granulomatose com Poliangiite/imunologia , Granulomatose com Poliangiite/patologia , Humanos , Poliangiite Microscópica/imunologia , Poliangiite Microscópica/patologia , Indução de Remissão
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