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1.
Rheum Dis Clin North Am ; 47(4): 781-796, 2021 11.
Artigo em Inglês | MEDLINE | ID: mdl-34635304

RESUMO

This article provides an overview of the clinical presentation and diagnosis of select pediatric primary systemic vasculitides. Important advances in understanding the pathogenesis of these rare diseases also are discussed and efforts to harmonize treatment through consensus-based guidelines and multicenter and international collaborations highlighted.


Assuntos
Vasculite , Criança , Humanos , Estudos Multicêntricos como Assunto , Vasculite/diagnóstico , Vasculite/terapia
2.
Int J Mol Sci ; 22(14)2021 Jul 14.
Artigo em Inglês | MEDLINE | ID: mdl-34299162

RESUMO

IgA, previously called Henoch-Schönlein vasculitis, is an essential immune component that drives the host immune response to the external environment. As IgA has the unique characteristic of a flexible response to broad types of microorganisms, it sometimes causes an autoreactive response in the host human body. IgA vasculitis and related organ dysfunction are representative IgA-mediated autoimmune diseases; bacterial and viral infections often trigger IgA vasculitis. Recent drug developments and the presence of COVID-19 have revealed that these agents can also trigger IgA vasculitis. These findings provide a novel understanding of the pathogenesis of IgA vasculitis. In this review, we focus on the characteristics of IgA and symptoms of IgA vasculitis and other organ dysfunction. We also mention the therapeutic approach, biomarkers, novel triggers for IgA vasculitis, and epigenetic modifications in patients with IgA vasculitis.


Assuntos
Biomarcadores/análise , Epigênese Genética , Imunoglobulina A/metabolismo , Vasculite/terapia , Animais , Humanos , Vasculite/diagnóstico , Vasculite/etiologia , Vasculite/metabolismo
3.
Arthritis rheumatol. (Malden. Online) ; 73(8): 1366-1383, 20210708.
Artigo em Inglês | BIGG - guias GRADE | ID: biblio-1292449

RESUMO

To provide evidence-based recommendations and expert guidance for the management of antineutrophil cytoplasmic antibody-associated vasculitis (AAV), including granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). Clinical questions regarding the treatment and management of AAV were developed in the population, intervention, comparator, and outcome (PICO) format (47 for GPA/MPA, 34 for EGPA). Systematic literature reviews were conducted for each PICO question. The Grading of Recommendations Assessment, Development and Evaluation methodology was used to assess the quality of evidence and formulate recommendations. Each recommendation required ≥70% consensus among the Voting Panel. We present 26 recommendations and 5 ungraded position statements for GPA/MPA, and 15 recommendations and 5 ungraded position statements for EGPA. This guideline provides recommendations for remission induction and maintenance therapy as well as adjunctive treatment strategies in GPA, MPA, and EGPA. These recommendations include the use of rituximab for remission induction and maintenance in severe GPA and MPA and the use of mepolizumab in nonsevere EGPA. All recommendations are conditional due in part to the lack of multiple randomized controlled trials and/or low-quality evidence supporting the recommendations. This guideline presents the first recommendations endorsed by the American College of Rheumatology and the Vasculitis Foundation for the management of AAV and provides guidance to health care professionals on how to treat these diseases.


Assuntos
Humanos , Vasculite/prevenção & controle , Anticorpos Anticitoplasma de Neutrófilos/efeitos adversos , Vasculite/diagnóstico , Vasculite/terapia , Indução de Remissão
4.
Viruses ; 13(6)2021 05 30.
Artigo em Inglês | MEDLINE | ID: mdl-34070832

RESUMO

Hepatitis B virus (HBV) chronic infection causes progressive liver damage, although about 20% of patients develop extrahepatic manifestations such as cryoglobulinemic vasculitis (CV). Clinical manifestations range from mild to moderate (purpura, asthenia, arthralgia) to severe (leg ulcers, peripheral neuropathy, glomerulonephritis, non-Hodgkin lymphoma). A comprehensive review of therapeutic options for HBV-related CV is lacking. Nucleos(t)ide analogues (NA) suppress HBV replication in 90-100% of cases and induce clinical response in most patients with mild-to-moderate CV. Plasma exchange can be performed in patients with severe CV and should be considered in severe or life-threatening cases combined with high doses of corticosteroids and antiviral treatment. A cautious use of rituximab can be considered only in association with NA treatment in refractory cases. A review of the literature and an analysis of data collected by six centers of the Italian Group for the Study of Cryoglobulinemia on 18 HBV-CV nucleotide/nucleoside analogues (NAs)-treated patients were carried out.


Assuntos
Crioglobulinemia/etiologia , Crioglobulinemia/terapia , Vírus da Hepatite B , Hepatite B/complicações , Vasculite/etiologia , Vasculite/terapia , Idoso , Terapia Combinada , Crioglobulinemia/diagnóstico , Gerenciamento Clínico , Suscetibilidade a Doenças , Feminino , Seguimentos , Hepatite B/virologia , Humanos , Itália , Masculino , Pessoa de Meia-Idade , Nucleosídeos/análogos & derivados , Nucleosídeos/uso terapêutico , Fenótipo , Resultado do Tratamento , Vasculite/diagnóstico
9.
Methods Mol Biol ; 2225: 107-123, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33108660

RESUMO

Viruses have devised highly effective approaches that modulate the host immune response, blocking immune responses that are designed to eradicate viral infections. Over millions of years of evolution, virus-derived immune-modulating proteins have become extraordinarily potent, in some cases working at picomolar concentrations when expressed into surrounding tissues and effectively blocking host defenses against viral invasion and replication. The marked efficiency of these immune-modulating proteins is postulated to be due to viral engineering of host immune modulators as well as design and development of new strategies (i.e., some derived from host proteins and some entirely unique). Two key characteristics of viral immune modulators confer both adaptive advantages and desirable functions for therapeutic translation. First, many virus-derived immune modulators have evolved structures that are not readily recognized or regulated by mammalian immune pathways, ensuring little to no neutralizing antibody responses or proteasome-mediated degradation. Second, these immune modulators tend to target early steps in central immune responses, producing a powerful downstream inhibitory "domino effect" which may alter cell activation and gene expression.We have proposed that peptide metabolites of these immune-modulating proteins can enhance and extend protein function. Active immunomodulating peptides have been derived from both mammalian and viral proteins. We previously demonstrated that peptides derived from computationally predicted cleavage sites in the reactive center loop (RCL) of a viral serine proteinase inhibitor (serpin ) from myxoma virus, Serp-1 , can modify immune response activation. We have also demonstrated modulation of host gut microbiota produced by Serp-1 and RCL-derived peptide , S7, in a vascular inflammation model. Of interest, generation of derived peptides that maintain therapeutic function from a serpin can act by a different mechanism. Whereas Serp-1 has canonical serpin-like function to inhibit serine proteases, S7 instead targets mammalian serpins. Here we describe the derivation of active Serp- RCL peptides and their testing in inflammatory vasculitis models.


Assuntos
Fatores Imunológicos/imunologia , Myxoma virus/genética , Peptídeos/imunologia , Serpinas/imunologia , Transplante Homólogo/métodos , Vasculite/terapia , Proteínas Virais/imunologia , Animais , Aorta Torácica , Modelos Animais de Doenças , Feminino , Expressão Gênica , Fatores Imunológicos/genética , Fatores Imunológicos/farmacologia , Masculino , Camundongos , Camundongos Endogâmicos BALB C , Camundongos Endogâmicos C57BL , Camundongos Knockout , Peptídeos/genética , Peptídeos/farmacologia , Receptores de Interferon/deficiência , Receptores de Interferon/genética , Serpinas/genética , Serpinas/farmacologia , Vasculite/imunologia , Vasculite/patologia , Proteínas Virais/genética , Proteínas Virais/farmacologia
10.
Kidney Blood Press Res ; 46(1): 114-120, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33326976

RESUMO

INTRODUCTION: Onconephrology is an emerging medical subspecialization that focuses on the numberless interrelations between cancer and kidney diseases. Tumor cells evade immune surveillance through activation of immune checkpoint pathways that suppress antitumor immune responses. By blocking checkpoints, new anticancer agents disrupt immune homeostasis but potentially induce immune-mediated diseases. Nephrologists and nephroimmunologists should be able to treat the nephrotoxic sequelae of cancer therapy and ensure continuation of the life-saving treatment. METHODS: Thirty-seven renal biopsies have been carried out over 42 months in oncologic patients, that is, 5.2% of the total native renal biopsies were carried out in the same period. The commonest diagnoses (>6 cases) were interstitial tubular nephritis, membranous glomerulopathy, IgA nephropathy, vasculitis, and focal and segmental glomerulosclerosis. CASE PRESENTATION: Three example cases, including focusing on key questions which could involve the nephrologists are reported in detail. They include a cancer-related Goodpasture Syndrome, the peculiar toxic effects of pemetrexed on tubular cells, and the intriguing relationship between bevacizumab and cryoglobulinemic glomerulonephritis. CONCLUSION: As shown by these 3 example cases, nephrologists need to be open-minded with regard to kidney biopsy in order to get a timely diagnosis. Nephrologists also need to improve their knowledge of cancer biology and therapy in order to prevent kidney problems, manage therapy-related immune-mediated disorders, and improve patient life expectancy.


Assuntos
Rim/patologia , Neoplasias/complicações , Nefrite/complicações , Idoso , Gerenciamento Clínico , Feminino , Glomerulonefrite por IGA/complicações , Glomerulonefrite por IGA/patologia , Glomerulonefrite por IGA/terapia , Glomerulonefrite Membranosa/complicações , Glomerulonefrite Membranosa/patologia , Glomerulonefrite Membranosa/terapia , Glomerulosclerose Segmentar e Focal/complicações , Glomerulosclerose Segmentar e Focal/patologia , Glomerulosclerose Segmentar e Focal/terapia , Humanos , Masculino , Pessoa de Meia-Idade , Neoplasias/terapia , Nefrite/patologia , Nefrite/terapia , Vasculite/complicações , Vasculite/patologia , Vasculite/terapia
12.
Clin Dermatol ; 38(6): 613-628, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-33341196

RESUMO

Drug-induced vasculitis and anticoagulant-related skin reactions are commonly encountered in the inpatient and outpatient settings. The spectrum of clinical presentation is broad and ranges from focal, skin-limited disease, to more extensive cutaneous and soft tissue necrosis, to potentially fatal systemic involvement. The prompt recognition of these adverse events can have a significant impact on patient morbidity and mortality. We highlight the key features of the clinical presentation with an emphasis on primary lesion morphology, distribution, and epidemiology of purpuric drug reactions. The proposed pathophysiology, histologic findings, and therapeutic interventions of these potentially life-threatening diseases are discussed.


Assuntos
Púrpura/induzido quimicamente , Púrpura/diagnóstico , Dermatopatias Vasculares/induzido quimicamente , Dermatopatias Vasculares/diagnóstico , Vasculite/induzido quimicamente , Vasculite/diagnóstico , Anti-Infecciosos/efeitos adversos , Anti-Inflamatórios não Esteroides/efeitos adversos , Anticonvulsivantes/efeitos adversos , Antidiuréticos/efeitos adversos , Antipsicóticos/efeitos adversos , Fatores Biológicos/efeitos adversos , Fármacos Cardiovasculares/efeitos adversos , Inibidores do Fator Xa/efeitos adversos , Humanos , Púrpura/patologia , Púrpura/terapia , Dermatopatias Vasculares/patologia , Dermatopatias Vasculares/terapia , Vasculite/patologia , Vasculite/terapia
14.
Chest ; 158(5): e225-e227, 2020 11.
Artigo em Inglês | MEDLINE | ID: mdl-33160541

RESUMO

Diffuse alveolar hemorrhage often presents as dyspnea, cough, or hemoptysis, and it is mediated by both immune and nonimmune processes. Isolated pauci-immune capillaritis (IPPC) is a rare diagnosis in which capillaritis, small-vessel vasculitis of the lung, is found on biopsy in the absence of an underlying systemic disorder. Traditionally, IPPC has been treated similarly to anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis with cyclophosphamide and glucocorticoids. However, few cases describing management options are available in the literature, especially among pediatric patients. Our report of successful induction of remission in an adolescent girl suggests that the combination of IV rituximab and pulse methylprednisolone may be a viable option for disease control in pediatric patients with IPPC.


Assuntos
Hemoptise , Doenças Pulmonares Intersticiais , Metilprednisolona/administração & dosagem , Rituximab/administração & dosagem , Vasculite , Adolescente , Capilares/patologia , Quimioterapia Combinada , Dispneia/diagnóstico , Dispneia/etiologia , Feminino , Hemoptise/diagnóstico , Hemoptise/etiologia , Hemoptise/terapia , Humanos , Fatores Imunológicos/administração & dosagem , Pulmão/diagnóstico por imagem , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/etiologia , Doenças Pulmonares Intersticiais/fisiopatologia , Doenças Pulmonares Intersticiais/terapia , Pulsoterapia/métodos , Indução de Remissão/métodos , Tomografia Computadorizada por Raios X/métodos , Resultado do Tratamento , Vasculite/complicações , Vasculite/diagnóstico , Vasculite/fisiopatologia , Vasculite/terapia
16.
Front Immunol ; 11: 574738, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-33193364

RESUMO

Vasculitis can be a life-threatening complication associated with high mortality and morbidity among patients with primary immunodeficiencies (PIDs), including variants of severe and combined immunodeficiencies ((S)CID). Our understanding of vasculitis in partial defects in recombination activating gene (RAG) deficiency, a prototype of (S)CIDs, is limited with no published systematic evaluation of diagnostic and therapeutic modalities. In this report, we sought to establish the clinical, laboratory features, and treatment outcome of patients with vasculitis due to partial RAG deficiency. Vasculitis was a major complication in eight (13%) of 62 patients in our cohort with partial RAG deficiency with features of infections and immune dysregulation. Vasculitis occurred early in life, often as first sign of disease (50%) and was complicated by significant end organ damage. Viral infections often preceded the onset of predominately non-granulomatous-small vessel vasculitis. Autoantibodies against cytokines (IFN-α, -ω, and IL-12) were detected in a large fraction of the cases tested (80%), whereas the majority of patients were anti-neutrophil cytoplasmic antibodies (ANCA) negative (>80%). Genetic diagnosis of RAG deficiency was delayed up to 2 years from the onset of vasculitis. Clinical cases with sole skin manifestation responded well to first-line steroid treatment, whereas systemic vasculitis with severe end-organ complications required second-line immunosuppression and/or hematopoietic stem cell transplantation (HSCT) for definitive management. In conclusion, our data suggest that vasculitis in partial RAG deficiency is prevalent among patients with partial RAG deficiency and is associated with high morbidity. Therefore, partial RAG deficiency should be included in the differential diagnosis of patients with early-onset systemic vasculitis. Diagnostic serology may be misleading with ANCA negative findings, and search for conventional autoantibodies should be extended to include those targeting cytokines.


Assuntos
Proteínas de Ligação a DNA/genética , Proteínas de Homeodomínio/genética , Proteínas Nucleares/genética , Imunodeficiência Combinada Severa/imunologia , Vasculite/imunologia , Adolescente , Adulto , Autoanticorpos/sangue , Biomarcadores/sangue , Criança , Pré-Escolar , Proteínas de Ligação a DNA/deficiência , Bases de Dados Factuais , Feminino , Predisposição Genética para Doença , Humanos , Lactente , Interferon Tipo I/imunologia , Interferon-alfa/imunologia , Interleucina-12/imunologia , Masculino , Pessoa de Meia-Idade , Proteínas Nucleares/deficiência , Fenótipo , Prevalência , Prognóstico , Imunodeficiência Combinada Severa/epidemiologia , Imunodeficiência Combinada Severa/genética , Imunodeficiência Combinada Severa/terapia , Vasculite/epidemiologia , Vasculite/terapia , Adulto Jovem
17.
Mod Rheumatol Case Rep ; 4(1): 102-105, 2020 01.
Artigo em Inglês | MEDLINE | ID: mdl-33086972

RESUMO

Rituximab represents a milestone in the treatment of mixed cryoglobulinemic vasculitis. Despite usually well-tolerated, rituximab may induce different types of adverse drug reactions, including exacerbation of vasculitis. Rituximab biosimilar have been recently approved in Europe in the treatment of rheumatoid arthritis, but no data are available about effectiveness and safety of rituximab biosimilar in the treatment of mixed cryoglobulinemic vasculitis. We describe a severe skin vasculitis reactivation in a patient affected by rheumatoid arthritis and mixed cryoglobulinemic vasculitis treated with rituximab biosimilar. After 7 days from the first infusion, a severe purpuric rash at lower limbs appeared, that resolved in about 2 weeks with high dose-corticosteroid. Rituximab-induced vasculitis has also been described since 2001, but its pathophysiology is still controversial due to the anecdotical descriptions in literature and the variability of the time between the rituximab infusion and the onset of skin lesions. Up to date, this is the first report describing a vasculitic flare in a patient affected by mixed cryoglobulinemic vasculitis treated with rituximab biosimilar.


Assuntos
Anticorpos Monoclonais Murinos/efeitos adversos , Crioglobulinemia/complicações , Rituximab/efeitos adversos , Vasculite/diagnóstico , Vasculite/etiologia , Corticosteroides/uso terapêutico , Anticorpos Monoclonais Murinos/uso terapêutico , Medicamentos Biossimilares/efeitos adversos , Medicamentos Biossimilares/uso terapêutico , Crioglobulinemia/diagnóstico , Crioglobulinemia/tratamento farmacológico , Humanos , Rituximab/uso terapêutico , Pele/patologia , Resultado do Tratamento , Vasculite/terapia
18.
Hautarzt ; 71(11): 870-879, 2020 Nov.
Artigo em Alemão | MEDLINE | ID: mdl-32997218

RESUMO

Vasculitis is a rare cause of skin ulceration. Depending on the size of the affected vessel, the patient's comorbidities and the pathophysiology present, different clinical morphologies can be seen, which can often give preliminary indications of the type of underlying vasculitis. There may be systemic or cutaneous manifestations; thus, a targeted diagnostic workup should be initiated at an early stage. Treatment should be interdisciplinary if there is systemic participation. Vasculopathies (e.g., livedoid vasculopathy), in which occlusion of the vascular lumen is the main pathophysiological feature, should be delimitated from vasculitis. If vasculitic or vasculopathic ulceration is present, stage-appropriate wound management is recommended.


Assuntos
Dermatopatias Vasculares , Úlcera Cutânea , Vasculite , Diagnóstico Diferencial , Humanos , Dermatopatias Vasculares/diagnóstico , Dermatopatias Vasculares/terapia , Úlcera Cutânea/diagnóstico , Úlcera Cutânea/terapia , Vasculite/diagnóstico , Vasculite/terapia
19.
Am J Case Rep ; 21: e925779, 2020 Aug 13.
Artigo em Inglês | MEDLINE | ID: mdl-32790652

RESUMO

BACKGROUND Coronavirus disease 2019 (COVID-19) infection commonly presents as fever, cough, and shortness of breath in adults. Children are thought to have milder respiratory symptoms and to recover more quickly. We describe a new presentation of COVID-19 infection in children consisting of multisystem inflammation with decreased left ventricular function and evidence of lung disease. CASE REPORT Three children presented with fever, conjunctivitis, dry and cracked lips, rash, and/or cervical lymphadenopathy for at least 5 days. Two of these children required mechanical ventilation, and 1 of the 2 needed extracorporeal membrane oxygenation (ECMO) to support cardiorespiratory function. All of these children had moderate to severe hyponatremia and lymphopenia, which is usually seen in COVID-19. They were treated with intravenous immunoglobulin and high-dose aspirin. All of the children recovered. CONCLUSIONS Early recognition of children with multisystem inflammation is important because they are at increased risk for deterioration. Treatment with intravenous immunoglobulin and aspirin was used because this regimen has been shown to be beneficial in vasculitis of Kawasaki disease. The development of shock due to cardiac involvement may require ECMO.


Assuntos
Betacoronavirus , Infecções por Coronavirus/diagnóstico , Pneumonia Viral/diagnóstico , Síndrome de Resposta Inflamatória Sistêmica/virologia , Antipiréticos/uso terapêutico , Aspirina/uso terapêutico , COVID-19 , Criança , Pré-Escolar , Conjuntivite/terapia , Conjuntivite/virologia , Infecções por Coronavirus/terapia , Exantema/terapia , Exantema/virologia , Oxigenação por Membrana Extracorpórea , Feminino , Febre/terapia , Febre/virologia , Insuficiência Cardíaca/terapia , Insuficiência Cardíaca/virologia , Humanos , Hiponatremia/terapia , Hiponatremia/virologia , Imunoglobulinas Intravenosas , Linfadenopatia/terapia , Linfadenopatia/virologia , Linfopenia/terapia , Linfopenia/virologia , Masculino , Pandemias , Pneumonia Viral/terapia , Respiração Artificial , SARS-CoV-2 , Síndrome de Resposta Inflamatória Sistêmica/diagnóstico , Síndrome de Resposta Inflamatória Sistêmica/terapia , Vasculite/terapia , Vasculite/virologia
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