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Immunoglobulins and/or therapeutic antibody preparations are associated with a high rate of false-positive (1,3)-ß-D-glucan (BDG) tests in onco-hematological patients routinely screened for fungal infections. The benefit of BDG monitoring shall be balanced against the risk of false-positive tests leading to unnecessary investigations and costs in this population.
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Glucanos , beta-Glucanas , Humanos , Imunoglobulinas , Proteoglicanas , Sensibilidade e EspecificidadeRESUMO
Our aim was to analyze severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2)-specific antibody level kinetics after coronavirus disease 2019 (COVID-19) infection and determine the efficiency of vaccination on SARS-CoV-2-specific antibody levels. The study included 50 SARS-CoV-2 infected and 70 uninfected cases. Levels of SARS-CoV-2-specific IgG nucleocapsid protein (IgG-NP), IgG spike protein (IgG-SP), IgM nucleocapsid protein (IgM-NP), and IgA spike protein (IgA-SP) antibodies were evaluated by an enzyme-linked immunosorbent assay in sera obtained at baseline, 1st, 3rd, and 6th month follow-up visits for infected cases and at postvaccination visits for all cases. In symptomatic cases (n = 50), IgG-SP levels were decreased in 6 months compared with baseline, while IgA-SP levels were significantly increased. IgG-NP levels were significantly decreased in symptomatic cases at the 6-month visit. After vaccination, IgG-SP levels were increased in symptomatic cases compared with prevaccination levels. Among subjects vaccinated with CoronaVac (the Sinovac COVID-19 vaccine), infected cases had approximately double the IgG-SP level of uninfected cases. SARS-CoV-2-specific antibody levels were higher at the baseline in symptomatic cases. Nevertheless, all infected cases showed significantly reduced IgG-SP levels at the 6th month. Vaccination effectively increased IgG-SP levels.
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Vacinas contra COVID-19 , COVID-19 , Imunidade Humoral , Anticorpos Antivirais , COVID-19/prevenção & controle , Vacinas contra COVID-19/imunologia , Humanos , Imunoglobulina A , Imunoglobulina G , Imunoglobulina M , Proteínas do Nucleocapsídeo , SARS-CoV-2 , Glicoproteína da Espícula de Coronavírus , VacinaçãoRESUMO
Rabies still causes about 60,000 human deaths per year, mainly in poor populations in Africa and Asia. However, since Louis Pasteur developed the first vaccine 130 years ago, prophylactic measures have been considerably improved and simplified. They now consist of the vaccine combined with purified rabies immunoglobulins of equine or human origin. In general, however, post-exposure prophylaxis protocols are long and expensive. Furthermore, the immunoglobulins used for associated serotherapy are costly and not widely available in developing countries. Approaches have been developed to deal with these two issues that offer hope for a paradigm shift for the benefit of exposed populations. Finally, mass rabies vaccination in dogs, which are the most cost-effective measure for preventing rabies in humans, are difficult to implement and sometimes have moderate effectiveness. The identification and analysis of the epidemiological drivers conditioning the circulation of the virus in dog populations allow a better understanding of the key control points that need to be associated with these campaigns for a better efficacy.
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In Europe, Hepatitis E virus (HEV) genotype 3 causes most human infections, and domestic pigs and wild boar represent the main reservoirs. Contact to these animals represents one source of human infection. However, interpretation of studies is challenged in the absence of a serological gold standard. Hence, this study compared results of different HEV immunoassays. Plasma samples from 139 individuals who had professional contact to pigs (veterinarians, meat inspectors, slaughterhouse workers; n = 114) or hunted regularly (n = 25) were tested with assays specific for HEV IgG, HEV IgM, HEV IgA, and total HEV immunoglobulin as well as for viral RNA. Furthermore, overall HEV IgG was defined (i.e., two of three IgG assays reveal the same result) to compare serological findings. Borderline results were always quoted as positive. For IgG, apparent prevalence was higher in Wantai (48.2%) compared to Euroimmun (11.5%) and Mikrogen assays (17.3%) (p = 0.0001). The overall IgG prevalence was estimated to be 18.7%. For total Ig, Wantai (40.3%) also yielded higher prevalence than Euroimmun (15.8%) (p = 0.0001). The HEV IgM prevalence ranged from 0% (Euroimmun) to 4.3% (Mikrogen). Four percent of individuals tested IgA positive, whilst none harboured HEV RNA. Our results support previous studies that the higher IgG prevalence estimated with the Wantai assay result from a higher sensitivity of this test. However, further studies are needed to verify specificity given the challenge of defining true negative samples. The high percentage of individuals with HEV IgG observed in this study underlines that direct contact to pigs represents a risk factor for HEV infection.
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Testes Diagnósticos de Rotina/métodos , Anticorpos Anti-Hepatite/sangue , Vírus da Hepatite E/imunologia , Hepatite E/diagnóstico , Imunoensaio/métodos , Exposição Ocupacional , Adulto , Idoso , Animais , Feminino , Alemanha , Humanos , Imunoglobulina A/sangue , Imunoglobulina G/sangue , Imunoglobulina M/sangue , Masculino , Pessoa de Meia-Idade , RNA Viral/sangue , Sensibilidade e Especificidade , Suínos , Adulto JovemRESUMO
There are considerable public concerns about the relationship between mobile phone radiation and human health. The present study assesses the effect of electromagnetic field (EMF) emitted from a mobile phone on the immune system in rats and the possible protective role of vitamin D. Rats were randomly divided into six groups: Group I: control group; Group II: received vitamin D (1000 IU/kg/day) orally; Group III: exposed to EMF 1 h/day; Group IV: exposed to EMF 2 h/day; Group V: exposed to EMF 1 h/day and received vitamin D (1000 IU/kg/day); Group VI: exposed to EMF 2 h/day and received vitamin D (1000 IU/kg/day). After 30 days of exposure time, 1 h/day EMF exposure resulted in significant decrease in immunoglobulin levels (IgA, IgE, IgM, and IgG); total leukocyte, lymphocyte, eosinophil and basophil counts; and a significant increase in neutrophil and monocyte counts. These changes were more increased in the group exposed to 2 h/day EMF. Vitamin D supplementation in EMF-exposed rats reversed these results when compared with EMF-exposed groups. In contrast, 7, 14, and 21 days of EMF exposure produced nonsignificant differences in these parameters among all experimental groups. We concluded that exposure to mobile phone radiation compromises the immune system of rats, and vitamin D appears to have a protective effect.
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Telefone Celular , Radiação Eletromagnética , Sistema Imunitário/efeitos dos fármacos , Sistema Imunitário/efeitos da radiação , Vitamina D/farmacologia , Animais , Basófilos/citologia , Basófilos/efeitos dos fármacos , Eosinófilos/citologia , Eosinófilos/efeitos dos fármacos , Sistema Imunitário/citologia , Imunoglobulinas/sangue , Imunoglobulinas/efeitos da radiação , Contagem de Leucócitos , Leucócitos/citologia , Leucócitos/efeitos dos fármacos , Contagem de Linfócitos , Masculino , Monócitos/citologia , Monócitos/efeitos dos fármacos , Neutrófilos/citologia , Neutrófilos/efeitos dos fármacos , RatosRESUMO
IgA nephropathy (IgAN) is the most common primary glomerulonephritis worldwide. Lifelong mesangial deposition of IgA1 complexes subsist inflammation and nephron loss, but the complex pathogenesis in detail remains unclear. In regard to the heterogeneous course, classical immunosuppressive and specific therapeutic regimens adapted to the loss of renal function will here be discussed in addition to the essential common renal supportive therapy. Renal supportive therapy alleviates secondary, surrogate effects or sequelae on renal function and proteinuria of high intraglomerular pressure and subsequent nephrosclerosis by inhibition of the renin angiotensin system (RAASB). In patients with physiological (ΔGFR < 1·5 ml/min/year) or mild (ΔGFR 1·5-5 ml/min/year) decrease of renal function and proteinuric forms (> 1 g/day after RAASB), corticosteroids have shown a reduction of proteinuria and might protect further loss of renal function. In patients with progressive loss of renal function (ΔGFR > 3 ml/min within 3 months) or a rapidly progressive course with or without crescents in renal biopsy, cyclophosphamide with high-dose corticosteroids as induction therapy and azathioprine maintenance has proved effective in one randomized controlled study of a homogeneous cohort in loss of renal function (ΔGFR). Mycophenolic acid provided further maintenance in non-randomized trials. Differentiated, precise, larger, randomized, placebo-controlled studies focused on the loss of renal function in the heterogeneous forms of IgAN are still lacking. Prospectively, fewer toxic agents will be necessary in the treatment of IgAN.
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Glomerulonefrite por IGA/terapia , Terapia de Imunossupressão , Imunossupressores/uso terapêutico , Biomarcadores , Gerenciamento Clínico , Progressão da Doença , Glomerulonefrite por IGA/diagnóstico , Glomerulonefrite por IGA/etiologia , Humanos , Terapia de Imunossupressão/efeitos adversos , Terapia de Imunossupressão/métodos , Imunossupressores/administração & dosagem , Imunossupressores/efeitos adversos , Terapia de Alvo Molecular , Prognóstico , Fatores de Tempo , Resultado do TratamentoRESUMO
INTRODUCTION: Edematous dermatomyositis is a rare entity with localized or generalized subcutaneous edema and only 21 cases have been reported in the literature. It is considered to be a severe form of dermatomyositis which needs quick therapeutic decision. We report 2 cases with difficult therapeutic decisions. OBSERVATIONS: Two patients aged 23 and 80 years were admitted in hospital for DM with typical cutaneous and muscular involvement without any sign of gravity and which have been treated by steroids: methylprednisolone bolus and prednisone. They both then developed severe edema of the upper limbs as well as worsening of the cutaneous and muscular symptoms with dysphagia. The addition of mycophenolate mofetil and intravenous immunoglobulin has permitted in the case of the first patient the disappearance of the cutaneous symptoms in particular the edema with restitution of the muscular force and withdrawal of the dysphagia and swallowing symptoms. The therapeutic failure for the second patient was due to a refusal of the treatment and a probable paraneoplastic context. CONCLUSION: Subcutaneous edema localized or generalized must not be confused with periorbital erythematous edema, classically observed in DM, nor with DM with mucinosis. Potential marker of gravity, it is often associated to important muscular weakness and dysphagia. In this case, an aggressive treatment associating corticosteroids, immunosuppressive therapy and intravenous immunoglobulin is necessary.
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Dermatomiosite/tratamento farmacológico , Edema/tratamento farmacológico , Idoso de 80 Anos ou mais , Dermatomiosite/complicações , Edema/complicações , Feminino , Humanos , Imunoglobulinas Intravenosas , Índice de Gravidade de Doença , Adulto JovemRESUMO
BACKGROUND: Papular mucinosis is characterised by primary mucin deposition in the dermis. The classification distinguishes between the localised form and the systemic form, which alone can result in complications, but this classification occasionally proves to be inadequate. Herein we report the progression of papular mucinosis, initially atypical due to the absence of cutaneous sclerosis and of misleading granulomatous histological features, which subsequently developed into characteristic scleromyxedema. PATIENTS AND METHODS: A 55-year-old male developed a rash comprising countless acral papules. Several biopsies were necessary before a diagnosis of papular mucinosis was made, due to the initial granulomatous appearance at histology. Tests showed monoclonal immunoglobulin of indeterminate significance, but, due to the absence of cutaneous sclerosis, we were able to conclude on typical localised papular mucinosis. Two years later, extensive sclerotic induration of the skin appeared and the diagnosis was modified to one of scleromyxedema. Treatment with intravenous immunoglobulins was given and proved efficacious, but relapse occurred on discontinuation of the therapy. DISCUSSION: Papular mucinosis is a rare disease of unknown physiopathology. The disease classification distinguishes between the localised and systemic forms, but it occasionally proves to be inadequate. Our case suggests a continuum between the localised and systemic forms of the disease. Further, the initial biopsies of acral papules in our patient had a misleading granulomatous appearance, as has been reported numerous times for the systemic forms. This granulomatous histological variant thus appears to constitute a diagnostic criterion for scleromyxedema.
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Escleromixedema/patologia , Biópsia , Humanos , Masculino , Pessoa de Meia-Idade , Pele/patologiaRESUMO
Chronic idiopathic demyelinating polyradiculoneuropathy (CIDP) is a rare disease, the most frequent one within the spectrum of the so-called "chronic immune-mediated neuropathies". Challenges in the treatment of CIDP firstly concern its diagnosis, which may be difficult, mainly for the atypical forms. Secondly, challenges encompass the choice of the first-line treatment, such as corticosteroids, intravenous immunoglobulins (IVIg), and plasma exchanges (PE) that have been proven as efficacious by several randomized controlled trials (RCT). Recent reports have focused on both different regimens of corticosteroids, and the occurrence of relapses following treatment with either corticosteroids or IVIg. These data may be helpful for the choice of the first-line treatment and may result in changing the guidelines for treatment of CIDP in clinical practice. The third and more difficult challenge is to manage long-term treatment for CIDP, since no immunomodulatory treatment has to date been proven as efficacious in this situation. Lastly, challenges in the treatment concern the choice of the best outcome measure for CIDP in RCT and clinical practice. The aim of this article is to overview the results of the more recently reported published trials for CIDP, and to give some insights for the current and future management of CIDP.
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Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/terapia , Ensaios Clínicos como Assunto , Humanos , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/diagnósticoRESUMO
BACKGROUND: Dermato-neuro syndrome is a specific neurological complication of scleromyxedema presenting with fever, coma, seizures and flu-like syndrome. To our knowledge, it has only been reported about twenty times in the literature. Its outcome is uncertain. We describe the case of a patient in whom a favorable outcome was achieved using a combination of plasmapheresis and intravenous immunoglobulin (IVIG). PATIENTS AND METHODS: A 57-year-old woman was diagnosed 14 years ago with scleromyxedema resistant to multiple lines of treatment. In November 2011, she presented an initial episode of epileptic seizure followed by post-seizure coma, and later, confusional state with visual hallucinations. She recovered spontaneously within a few days. CT scan, MRI, EEG and screening for infection were perfectly normal, resulting in suspicion of neurological involvement associated with her scleromyxedema. In December 2012 and August 2013, she presented two further episodes of status epilepticus, followed once more by a confusional state, with etiological explorations again proving unfruitful. On this occasion, her confusional state persisted for two months until the initiation of plasmapheresis and IVIG. This combination therapy led to rapid regression of all neurological symptoms and an improvement in her general condition. DISCUSSION: The dermato-neuro syndrome is a rare neurological complication of scleromyxedema. Its pathophysiology is unknown. The monoclonal gammopathy induced by the scleromyxedema could account for the patient's hypercoagulable state and for the formation of neutrophilic aggregates leading to impaired microcirculation. Treatment is empirical and poorly codified. The course of the disease is unpredictable and may be lethal.
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Imunoglobulinas Intravenosas/uso terapêutico , Síndromes Neurocutâneas/etiologia , Síndromes Neurocutâneas/terapia , Plasmaferese , Escleromixedema/complicações , Terapia Combinada , Feminino , Humanos , Pessoa de Meia-Idade , Indução de RemissãoRESUMO
Blood samples were collected from 30 women with age ranged from 27-70 years after 3 cycles of chemotherapy. Sera were used for IgA, IgG, IgM, C3, C4, IL-6 and TNF-α estimation. After 3 cycles of chemotherapy, all the immunological parameters reduced except TNF-α. Patients who developed disease reoccurrence after chemotherapy exhibit a significantly higher IgA, C3, IL-6 and TNF-α levels after 3 cycles of chemotherapy than patients who did not (p < 0.05). Therefore, serum IgA, C3, IL-6 and TNF-α can be used as predictors for breast cancer reoccurrence.
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INTRODUCTION: Kawasaki syndrome (KS) is a systemic vasculitis of unknown etiology that affects medium and small blood vessels. The aim of our study is to analyze coronary artery lesions in children with KS and their risk factors. MATERIAL AND METHODS: All children under the age of 15 years-old presenting KS and admitted in the pediatric department of three university hospital (Sahloul hospital, and Farhat Hached hospital of Sousse, Ibn El Jazzar hospital of Kairoun) from January 2000 to December 2018 were included. RESULTS: Sixty-five patients were included in our study. The mean age at diagnosis was of 29.9 months [2-120 months] and the sex ratio was of 1.7. Echocardiography was performed in all patients. It showed coronary dilation in 37% of patients with coronary artery diameter of 4.2 mm on average [3.2-7mm]. The coronary aneurysm was small in 19 cases and medium in 5 cases. No giant aneurysm has been identified. In univariate analysis, the predictors of coronary artery lesions were male sex, atypical form, fever duration more than 10 days, hepatic cytolysis, thrombocytosis and anemia. In multivariate analysis, only the last four parameters were the predictive factors of the coronary artery involvement. CONCLUSION: Several risk factors can be used to determine which children are predisposed to develop coronary dilations. In case of patient with risk factors, intravenous immunoglobulins should be initiated early to avoid these serious complications.
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Aneurisma Coronário , Doença da Artéria Coronariana , Síndrome de Linfonodos Mucocutâneos , Adolescente , Criança , Aneurisma Coronário/diagnóstico por imagem , Aneurisma Coronário/epidemiologia , Aneurisma Coronário/etiologia , Doença da Artéria Coronariana/complicações , Doença da Artéria Coronariana/etiologia , Vasos Coronários , Feminino , Humanos , Lactente , Masculino , Síndrome de Linfonodos Mucocutâneos/complicações , Síndrome de Linfonodos Mucocutâneos/diagnóstico , Estudos Retrospectivos , Fatores de RiscoRESUMO
Idiopathic pulmonary fibrosis (IPF) is a lethal respiratory disease characterized by the excessive deposition of extracellular matrix in the alveolar zones. The bronchiolar epithelium has been implicated in the development of this disease and is capable of secreting IgA into the airway lumen thanks to its expression of the polymeric immunoglobulin receptor. Several elements indicate a dysregulation of this system, such as raised serum IgA levels in IPF patients and the pro-fibrotic effect of IgA on several key cell types. Our work aims at studying the underlying mechanisms so as to better understand the role of IgA mucosal immunity in this disease.
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Fibrose Pulmonar Idiopática , Receptores de Imunoglobulina Polimérica , Humanos , Imunoglobulina A Secretora/metabolismo , Receptores de Imunoglobulina Polimérica/metabolismo , Sistema Respiratório/metabolismoRESUMO
Germinal center regulation pathways are often involved in lymphomagenesis and myelomagenesis. Most of the lymphomas (and multiple myeloma) derive from post-germinal center B-cells that have undergone somatic hypermutation and class switch recombination. Hence, B-cell clonal expansion can be responsible for the presence of a monoclonal component (immunoglobulin) of variable titer which, owing to physicochemical properties, can provoke pathologically defined entities of diseases. These diseases can affect any functional part of the kidney, by multiple mechanisms, either well known or not. The presence of renal deposition is influenced by germinal gene involved, immunoglobulin primary structure, post-translational modifications and microenvironmental interactions. The two ways immunoglobulin can cause kidney toxicity are (i) an excess of production (overcoming catabolism power by proximal tubule epithelial cells) with an excess of free light chains within the distal tubules and a subsequent risk of precipitation due to local physicochemical properties; (ii) by structural characteristics that predispose immunoglobulin to a renal disease (whatever their titer). The purpose of this manuscript is to review literature concerning the pathophysiology of renal toxicities of clonal immunoglobulin, from molecular B-cell expansion mechanisms to immunoglobulin renal toxicity.
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Imunoglobulinas , Nefropatias , Humanos , Imunoglobulinas/metabolismo , Nefropatias/induzido quimicamente , Nefropatias/metabolismo , Linfócitos B/metabolismo , Linfócitos B/patologia , Anticorpos Monoclonais , Rim/metabolismoRESUMO
Anti-TNF alpha monoclonal antibodies are used in the treatment of chronic inflammatory diseases, which commonly affect women of childbearing age. Due to their similar structure to native immunoglobulins, the evaluation of their placental transfer by a pharmacological approach of the mechanisms involved is an interesting source of information, useful to the risk-benefit assessment of drugs in pregnant women.
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Anticorpos Monoclonais , Troca Materno-Fetal , Inibidores do Fator de Necrose Tumoral , Anticorpos Monoclonais/farmacocinética , Feminino , Humanos , Placenta , Gravidez , Inibidores do Fator de Necrose Tumoral/farmacocinéticaRESUMO
Immune thrombocytopenic purpura (ITP) is a rare hematological disorder with an autoimmune-mediated, often dramatic reduction of platelets in peripheral blood. Thrombocytopenia results from a reduced life span of thrombocytes and an additionally decreased production in bone marrow. For decades, the first-line therapy for ITP has been corticosteroids. As significant thrombocytopenic bleedings occur, the use of additional medication may be needed. Recent updates on therapy guidelines recommend the shortest possible use of corticosteroids. Thrombopoietin-receptor agonists are often used second line. Today splenectomy, which was previously recommended after unsuccessful first-line therapy, is usually considered much later. Patients who do not respond even after multiple lines of therapy continue to pose a major challenge. New drugs for ITP treatment are now available after steroid failure and will be discussed. This review gives a short summary on actual therapy guidelines taking into account newly available therapy options. In addition, comparisons between selected published data and experience at our department are made.
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The first line treatment of immune thrombocytopenic purpura (ITP) is well established and based on short course of corticosteroids associated with intravenous immunoglobulins (IVIg) for the most severe forms. Predniso(lo)ne is the corticosteroid agent usually given but dexamethasone appears as an alternative. Some guidelines recommend to use dexamethasone as first line when a rapid increase of platelet count is required. Dexamethasone could be used rather than IVIg for moderate to severe but non life-threatening bleeding manifestations. Other therapeutic options such as anti FcRn monoclonal antibodies or recombinant FcγR currently in development for ITP could be an option in the future. In newly diagnosed ITP, we unfortunately lack robust predictive risk factors of severity and chronic outcome. Identifying such factors could be helpful for considering the early use of some treatments which are commonly used as second or third line.
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Púrpura Trombocitopênica Idiopática , Trombocitopenia , Corticosteroides/uso terapêutico , Adulto , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Contagem de Plaquetas , Púrpura Trombocitopênica Idiopática/diagnóstico , Púrpura Trombocitopênica Idiopática/tratamento farmacológicoRESUMO
BACKGROUND: The intrathoracic manifestations of IgG4-related disease include a range of conditions and severity, and can on occasion cause acute respiratory failure as reported in the case described here. OBSERVATION: A 69-year-old male former smoker, was admitted to our hospital with dyspnea, fever, cough, fatigue, and a 3-month history of weight loss. He received high flow oxygen therapy and non-invasive ventilation for severe respiratory failure. Chest computed tomography revealed multifocal condensations and ground glass opacities, accompanied by thickening of the perilymphatic interstitium, mediastinal lymphadenopathy and bilateral pleural effusion. Elevated serum concentrations of IgG4 suggested an IgG4-Related Disease. He developed renal failure and underwent a renal biopsy. Histopathological analysis of which supported the diagnosis by showing dense lymphocytic infiltrate with a count of IgG4+ cells/hpf higher than 60, and storiform fibrosis - a swirling, "cartwheel" pattern of fibrosis which may have a patchy distribution. The patient responded well to steroid therapy. CONCLUSION: Although respiratory symptoms are usually mild in IgG4-relatd disease, thoracic features can evolve into acute respiratory failure with few extra thoracic manifestations.
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Doença Relacionada a Imunoglobulina G4 , Doenças Pulmonares Intersticiais , Derrame Pleural , Idoso , Humanos , Imunoglobulina G , Doença Relacionada a Imunoglobulina G4/complicações , Doença Relacionada a Imunoglobulina G4/diagnóstico , Doenças Pulmonares Intersticiais/complicações , Doenças Pulmonares Intersticiais/diagnóstico , Masculino , Tomografia Computadorizada por Raios XRESUMO
Common variable immunodeficiency disorders (CVID) are the most common symptomatic primary antibody deficiency in adults with an estimated prevalence of 1/25,000. The most frequent clinical manifestations are upper respiratory tract infections (including pneumonia, bronchitis, and sinusitis) predominantly with Streptococcus pneumoniae or H. influenzae. However, CVID are complicated in 20 to 30 % of cases of non-infectious manifestations which have been well characterized in recent years. Several complications can be observed including autoimmune, lymphoproliferative, granulomatous or cancerous manifestations involving one or more organs. These complications, mostly antibody-mediated cytopenias, are correlated with a decrease in the number of circulating switched memory B cells. Replacement therapy with polyvalent gammaglobulins has greatly improved the prognosis of these patients but it remains poor in the presence of digestive complications (especially in the case of chronic enteropathy and/or porto-sinusoidal vascular disease), pulmonary complications (bronchiectasis and/or granulomatous lymphocytic interstitial lung disease) and when progression to lymphoma. Much progress is still to be made, in particular on the therapeutic management of non-infectious complications which should benefit in the future from targeted treatments based on knowledge of genetics and immunology.
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Bronquiectasia , Imunodeficiência de Variável Comum , Pneumonia , Infecções Respiratórias , Linfócitos B , Imunodeficiência de Variável Comum/complicações , Imunodeficiência de Variável Comum/diagnóstico , Imunodeficiência de Variável Comum/epidemiologia , HumanosRESUMO
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.