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1.
Allergy ; 77(7): 1961-1990, 2022 07.
Artigo em Inglês | MEDLINE | ID: mdl-35006617

RESUMO

Hereditary angioedema (HAE) is a rare and disabling disease for which early diagnosis and effective therapy are critical. This revision and update of the global WAO/EAACI guideline on the diagnosis and management of HAE provides up-to-date guidance for the management of HAE. For this update and revision of the guideline, an international panel of experts reviewed the existing evidence, developed 28 recommendations, and established consensus by an online DELPHI process. The goal of these recommendations and guideline is to help physicians and their patients in making rational decisions in the management of HAE with deficient C1 inhibitor (type 1) and HAE with dysfunctional C1 inhibitor (type 2), by providing guidance on common and important clinical issues, such as: (1) How should HAE be diagnosed? (2) When should HAE patients receive prophylactic on top of on-demand treatment and what treatments should be used? (3) What are the goals of treatment? (4) Should HAE management be different for special HAE patient groups such as children or pregnant/breast-feeding women? and (5) How should HAE patients monitor their disease activity, impact, and control? It is also the intention of this guideline to help establish global standards for the management of HAE and to encourage and facilitate the use of recommended diagnostics and therapies for all patients.


Assuntos
Angioedemas Hereditários , Angioedemas Hereditários/prevenção & controle , Angioedemas Hereditários/terapia , Criança , Proteína Inibidora do Complemento C1/genética , Proteína Inibidora do Complemento C1/uso terapêutico , Consenso , Feminino , Humanos , Gravidez
3.
Immunol Cell Biol ; 95(8): 716-728, 2017 09.
Artigo em Inglês | MEDLINE | ID: mdl-28546549

RESUMO

Production of IFN-γ contributes to host defense against Mycobacterium tuberculosis (Mtb) infection. We previously demonstrated that Signaling lymphocytic activation molecule-associated protein (SAP) expression on cells from tuberculosis (TB) patients was inversely correlated with IFN-γ production. Here we first investigated the role of NK, T- and B-cell antigen (NTB-A)/SAP pathway in the regulation of Th1 response against Mtb. Upon antigen stimulation, NTB-A phosphorylation rapidly increases and afterwards modulates IFN-γ and IL-17 secretion. To sustain a healthy immune system, controlled expansion and contraction of lymphocytes, both during and after an adaptive immune response, is essential. Besides, restimulation-induced cell death (RICD) results in an essential homeostatic mechanism for precluding excess T-cell accumulation and associated immunopathology during the course of certain infections. Accordingly, we found that the NTB-A/SAP pathway was required for RICD during active tuberculosis. In low responder (LR) TB patients, impaired RICD was associated with diminished FASL levels, IL-2 production and CD25high expression after cell-restimulation. Interestingly, we next observed that SAP mediated the recruitment of the Src-related kinase FYNT, only in T cells from LR TB patients that were resistant to RICD. Together, we showed that the NTB-A/SAP pathway regulates T-cell activation and RICD during human TB. Moreover, the NTB-A/SAP/FYNT axis promotes polarization to an unfavorable Th2-phenotype.


Assuntos
Mycobacterium tuberculosis/imunologia , Família de Moléculas de Sinalização da Ativação Linfocitária/metabolismo , Células Th2/imunologia , Tuberculose/imunologia , Adulto , Morte Celular , Diferenciação Celular , Células Cultivadas , Feminino , Homeostase , Humanos , Imunidade , Terapia de Imunossupressão , Interferon gama/metabolismo , Interleucina-17/metabolismo , Ativação Linfocitária , Masculino , Proteínas de Neoplasias/metabolismo , Proteínas Tirosina Quinases/metabolismo , Transdução de Sinais
4.
Medicina (B Aires) ; 77(4): 279-282, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-28825570

RESUMO

The benefits of the worldwide approval of new drugs for the treatment of acute C1-INH-HAE attacks may still not reach all patients. Identifying the current barriers in the access to medication, as well as conducting a detailed assessment of the progress in this area, is essential to achieve universal treatment. Two hundred and twenty five patients registered in the Argentina Hereditary Angioedema Patient Association (AHAEPA) were randomly selected and invited to participate in a web based questionnaire on accessibility to icatibant and pdC1-INH, self-treatment, delay to treatment, and coverage. The data retrieved was compared to our previous reports in 2008 and 2013. We collected 156/225 answers. One hundred and eighteen (76%) patients have either pdC1-INH (n = 86), icatibant (n = 10) or both (n = 22), while 38 (24%) do not have access to treatment. In 2008, 26% had access while 82% had it in 2013. Thirty-two subjects (22%) self-inject themselves, similar to 29% in 2013, even though between studies, widespread self-injection training activities have taken place. However, considering injections by proxy, home treatment reached 56%. Only half of the patients decide to receive treatment early during the attack. Ninety-nine patients (63%) have full coverage, thirty (19%) have no coverage at all and the rest only obtain partial reimbursement. Twenty-nine families (31%) share a single treatment dose of the medication, better than 36% in 2013. Argentina's C1-INH-HAE patients had a sustained improvement in their access to medication. Efforts should continue to further improve accessibility and optimal management of HAE acute attacks to all patients in the country.


Assuntos
Angioedemas Hereditários/tratamento farmacológico , Anti-Inflamatórios não Esteroides/administração & dosagem , Bradicinina/análogos & derivados , Proteína Inibidora do Complemento C1/administração & dosagem , Inativadores do Complemento/administração & dosagem , Argentina , Bradicinina/administração & dosagem , Feminino , Acessibilidade aos Serviços de Saúde/estatística & dados numéricos , Humanos , Masculino , Inquéritos e Questionários
5.
Medicina (B Aires) ; 77(5): 382-387, 2017.
Artigo em Espanhol | MEDLINE | ID: mdl-29044014

RESUMO

Anaphylaxis during anesthesia is an unpredictable, severe, and rare reaction. It has an incidence of 1/10 000 to 1/20 000 surgeries. In most series, the responsible drugs include neuromuscular blocking agents, latex, or antibiotics. The frequency and etiology of systemic allergic reactions in other medical procedures are largely unknown. The identification of responsible drugs of anaphylaxis is a complex task, requiring testing of all medications and substances used during surgery. We describe our experience in a retrospective study of 15 patients. Ten subjects developed anaphylaxis during surgery, two in endoscopic studies and one in a trans-vaginal ultrasound. The remaining two subjects, one in a trans-vaginal ultrasound and another during a dental procedure had a systemic allergic reaction. We studied all patients with all medications administered during the procedures, including latex and detergents and disinfectants. Three surgeries had to be suspended at induction of anesthesia, five were stopped incomplete and two were completed. Both patients that presented a reaction during endoscopy required intensive care unit admission and the rest were observed in a Hospital. The responsible drugs during surgery anaphylaxis were neuromuscular blocking agents, latex, patent blue, and ranitidine. Ortho-phthalaldehyde (OPA) was identified during endoscopic studies; latex was responsible in transvaginal ultrasounds; and amoxicillin in the dental procedure. The aim of the present article is to review our experience studying allergic systemic reactions and anaphylaxis during general anesthesia and medical procedures, emphasizing the severity of these reactions and the need for causative drug identification.


Assuntos
Anafilaxia/etiologia , Ecocardiografia Transesofagiana/efeitos adversos , Endoscopia/efeitos adversos , Endossonografia/efeitos adversos , Hipersensibilidade/etiologia , Complicações Intraoperatórias/etiologia , Procedimentos Cirúrgicos Operatórios/efeitos adversos , Adulto , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos
6.
Medicina (B Aires) ; 77(3): 180-184, 2017.
Artigo em Espanhol | MEDLINE | ID: mdl-28643673

RESUMO

Drug hypersensitivity reactions (RHD) are those that present clinically as allergic. They can or cannot involve an immunologic mechanism of lesion. They are frequent and, occasionally, life threatening. Patients with RHD repeat the reaction when they are re-exposed to the drug, limiting the therapeutic options and exposing them to more expensive and toxic drugs. It is difficult to identify the responsible drug when the reaction was not recent or when it occurred in the context of therapy with multiple drugs or confusing concurrent diseases. The diagnosis should be based on clinical history, followed by drug skin tests and drug provocation tests. We describe our experience in 771 procedures, 331 cutaneous and 440 drug provocation tests, 11% of them were positive. Positive symptoms included generalized pruritus, rash, urticaria, angioedema, rhinitis, bronchospasm, nausea and anaphylaxis. All the patients with positive tests had a good response to treatment. It can therefore be concluded that drug tests undertaken on individuals with suspected drug allergy, performed by experienced personnel and in controlled settings, are useful and safe to confirm drug hypersensitivity.


Assuntos
Hipersensibilidade a Drogas/diagnóstico , Testes Cutâneos/métodos , Humanos , Testes Cutâneos/estatística & dados numéricos
7.
Medicina (B Aires) ; 76(6): 379-382, 2016.
Artigo em Espanhol | MEDLINE | ID: mdl-27959849

RESUMO

Most studies about treatment of inflammatory myopathies consist of cross-sectional analyses that do not assess long-term efficacy. In the present study we describe the follow-up of seven patients with inflammatory myopathies, 5 polymyositis and 2 dermatomyositis. We describe their clinical features, follow-up, muscle enzyme levels, and treatment responses. We define the latter as treatment cycles, every one of which end when steroid doses need to be increased or a new immunosuppressive drug has to be added because of clinical worsening or sustained increases in muscle enzyme levels. Treatment can cause remission, partially control, or fail in achieving myositis improvement when it normalizes, stabilizes, or does not affect muscle enzymes or clinical features, respectively. We analyzed 20 cycles, in which remission was achieved in 14 cases, partial control in 5 instances, and treatment failure in one case. Remission occurred after an average of 139 ± 98 days, whereas partial control took place in 160 ± 100 days. Except in one case, all treatment cycles controlled or remitted the symptoms. However, in all patients the illness recurred with time.


Assuntos
Dermatomiosite/tratamento farmacológico , Dermatomiosite/patologia , Polimiosite/tratamento farmacológico , Polimiosite/patologia , Corticosteroides/uso terapêutico , Adulto , Dermatomiosite/enzimologia , Feminino , Seguimentos , Humanos , Imunossupressores/uso terapêutico , Masculino , Pessoa de Meia-Idade , Polimiosite/enzimologia , Recidiva , Fatores de Tempo , Resultado do Tratamento
8.
Medicina (B Aires) ; 75(5): 273-6, 2015.
Artigo em Inglês | MEDLINE | ID: mdl-26502460

RESUMO

We describe the diagnostic epidemiology, the clinical course, the family history and the response to treatment of patients with angioedema without wheals (AWW) at an Allergy and Immunology Clinical Center. We reviewed the case records of all patients at our office from January 1997 to April 2013. We recorded sex, age, age at onset of symptoms, family history of angioedema, number of visits to the office, type of angioedema, and response to treatment from those patients with angioedema without wheals. We classified angioedema according to its pathophysiology. We also describe those patients with angioedema mimics. From a total of 17,823 new patients, 303 had a presumptive diagnosis of angioedema without wheals. Twenty-three patients had an angioedema mimic. Forty percent were male and 60% were female. Average age at first visit was 40.6. Average number of visits was 2.4. Fifty-seven patients referred a family history. We attributed idiopathic angioedema to 55.7% of patients, 24.3% were drug related, 15.7% were due to C1 inhibitor deficiency, 2.1% were drug related+idiopathic angioedema, 1.4% were type III and 0.7% had exercise-induced angioedema. Ninety six percent of 53 evaluable idiopathic angioedema patients referred a benefit with anti-histamine therapy. AWW was a rare cause of consultation. Most of our patients had anti H1 responsive idiopathic angioedema and none had allergic angioedema. Women cases prevailed over men's. Family history and average age of onset of symptoms were different among the different types of angioedema.


Assuntos
Angioedema/diagnóstico , Angioedema/epidemiologia , Antagonistas dos Receptores Histamínicos H1/uso terapêutico , Doenças Raras/epidemiologia , Centros de Atenção Terciária/estatística & dados numéricos , Adolescente , Adulto , Idade de Início , Idoso , Idoso de 80 Anos ou mais , Assistência Ambulatorial/estatística & dados numéricos , Angioedema/classificação , Angioedema/tratamento farmacológico , Angioedemas Hereditários/epidemiologia , Criança , Pré-Escolar , Diagnóstico Diferencial , Saúde da Família , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Prevalência , Doenças Raras/diagnóstico , Doenças Raras/tratamento farmacológico , Estudos Retrospectivos , Fatores Sexuais , Avaliação de Sintomas/estatística & dados numéricos , Urticária/epidemiologia , Adulto Jovem
9.
Medicina (B Aires) ; 75(5): 319-23, 2015.
Artigo em Espanhol | MEDLINE | ID: mdl-26502469

RESUMO

Rituximab, a chimeric monoclonal antibody against CD20, induces the depletion of B lymphocytes. It is used for the treatment of lymphoproliferative and autoimmune diseases. Antibody immunodeficiency associated to RTX treatment is a new motif for consultation to our service. We decided to study those patients that having been treated with RTX, consulted for hypogammaglobulinemia or recurrent infections between November 2010 and December 2014. We evaluated eight patients, seven female and one male. The average follow up time was 19.3±18.8 months, range 1 to 54, median 13. Three had a normal electrophoretic proteinogram before receiving RTX, three had hypogammaglobulinemia and in two data was not available. None of them had a quantitative determination of immunoglobulins before receiving RTX. Four received RTX as a treatment of non Hodking lymphoma, two as a treatment of chronic lymphocytic leukemia, one for immune thrombocytopenic purpura and other for microscopic polyangiitis. Six were diagnosed with hypogammaglobulinemia and one with combined IgM, IgA and IgG2 deficiency. Five presented infections, four of them with good response to intravenous immunoglobulin. RTX related antibody deficiency consultations are increasing. It is important to determine the immunoglobulin levels previously to RTX use in order to establish an etiologic relationship with RTX and a quick diagnosis of antibody deficiency. The substitutive treatment with gammaglobulin seems to be useful in patients with severe or recurrent infections.


Assuntos
Agamaglobulinemia/tratamento farmacológico , Fatores Imunológicos/uso terapêutico , Rituximab/uso terapêutico , Adulto , Idoso , Evolução Fatal , Feminino , Seguimentos , Humanos , Imunoglobulinas Intravenosas , Leucemia Linfocítica Crônica de Células B/tratamento farmacológico , Linfoma não Hodgkin/tratamento farmacológico , Masculino , Poliangiite Microscópica/tratamento farmacológico , Pessoa de Meia-Idade , Púrpura Trombocitopênica Idiopática/tratamento farmacológico , Recidiva
11.
Medicina (B Aires) ; 74(3): 198-200, 2014.
Artigo em Espanhol | MEDLINE | ID: mdl-24918666

RESUMO

In the world, hereditary angioedema (HAE) affects 1 every 50000 persons. It is characterized by highly disabling and recurrent episodes of cutaneous, abdominal and laryngeal episodes of angioedema. Asphyxia related mortality ranges from 15 to 50%. In Argentina a plasma derived C1 inhibitor concentrate (pdC1INH) has been available for the treatment of acute attacks for many decades, however, only15 (26%) out of 58 patients had received pdC1INH at least once until 2008, and only2 (3.4%) had used it regularly. After worldwide approval of the new drugs for the treatment of acute HAE attacks, adding icatibant to pdC1INH in Argentina, and after publication of the therapeutic guide for the country, 42 (82%) out of 51 patients from the original group has pdC1INH available to treat their next attack. However, 16 (18%) patients continue without access to medication and other 15 (35.7%) obtain their therapy spuriously through some other affected relative in their environment. Only 12 (28.6%) patients of the group self-treated at home. Access to treatment has greatly improved, but needs to be extended to all patients and self-treatment at home should be encouraged.


Assuntos
Angioedemas Hereditários/tratamento farmacológico , Bradicinina/análogos & derivados , Proteína Inibidora do Complemento C1/uso terapêutico , Inativadores do Complemento/uso terapêutico , Acessibilidade aos Serviços de Saúde/estatística & dados numéricos , Doença Aguda , Argentina , Bradicinina/uso terapêutico , Humanos , Inquéritos e Questionários
12.
J Infect Dis ; 207(2): 340-50, 2013 Jan 15.
Artigo em Inglês | MEDLINE | ID: mdl-23125442

RESUMO

Immune control of Mycobacterium tuberculosis depends on interferon γ (IFN-γ)-producing CD4(+) lymphocytes. Previous studies have shown that T cells from patients with tuberculosis produce less IFN-γ, compared with healthy donors, in response to mycobacterial antigens, although IFN-γ responses to mitogens are preserved. In this work, we found that M. tuberculosis-induced IFN-γ production by human T cells correlated with phosphorylation of the mitogen-activated protein kinases (MAPKs), extracellular signal-regulated kinase (ERK), and p38. Moreover, the majority of IFN-γ-producing T cells expressed signaling lymphocyte activation molecule (SLAM), and SLAM activation further increased ERK phosphorylation. Interestingly, patients with tuberculosis had delayed activation of ERK and p38, and this was most marked in patients with the poorest IFN-γ responses (ie, low responders). Besides, SLAM signaling failed to phosphorylate ERK in low responders. Our findings suggest that activation of p38 and ERK, in part through SLAM, mediates T-cell IFN-γ production in response to M. tuberculosis, a pathway that is defective in patients with tuberculosis.


Assuntos
MAP Quinases Reguladas por Sinal Extracelular/metabolismo , Interferon gama/biossíntese , Mycobacterium tuberculosis/imunologia , Linfócitos T/imunologia , Tuberculose Pulmonar/imunologia , Proteínas Quinases p38 Ativadas por Mitógeno/metabolismo , Antígenos CD/metabolismo , Ativação Enzimática , Humanos , Fosforilação , Receptores de Superfície Celular/metabolismo , Transdução de Sinais , Membro 1 da Família de Moléculas de Sinalização da Ativação Linfocitária , Tuberculose Pulmonar/metabolismo , Tuberculose Pulmonar/microbiologia
13.
J Allergy Clin Immunol Pract ; 12(6): 1614-1621, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38609017

RESUMO

BACKGROUND: Clinical trials investigating drugs for the acute treatment of hereditary angioedema attacks have assessed many different outcomes. This heterogeneity limits the comparability of trial results and may lead to selective outcome reporting bias and a high burden on trial participants. OBJECTIVE: To achieve consensus on a core outcome set composed of key outcomes that ideally should be used in all clinical efficacy trials involving the acute treatment of hereditary angioedema attacks. METHODS: We conducted a Delphi consensus study involving all relevant parties: patients with hereditary angioedema, hereditary angioedema expert clinicians and clinical researchers, pharmaceutical companies, and regulatory bodies. Two Internet-based survey rounds were conducted. In round 1, panelists indicated the importance of individual outcomes used in clinical trials on a 9-point Likert scale. Based on these results, a core outcome set was developed and voted on by panelists in round 2. RESULTS: A total of 58 worldwide panelists completed both rounds. The first round demonstrated high importance scores and substantial agreement among the panelists. In the second round, a consensus of 90% or greater was achieved on a core outcome set consisting of five key outcomes: change in overall symptom severity at one predetermined time point between 15 minutes and 4 hours after treatment, time to end of progression of all symptoms, the need for rescue medication during the entire attack, impairment of daily activities, and treatment satisfaction. CONCLUSIONS: This international study obtained a high level of consensus on a core outcome set for the acute treatment of hereditary angioedema attacks, consisting of five key outcomes.


Assuntos
Angioedemas Hereditários , Humanos , Angioedemas Hereditários/tratamento farmacológico , Resultado do Tratamento , Técnica Delphi , Inquéritos e Questionários , Ensaios Clínicos como Assunto , Consenso , Feminino , Avaliação de Resultados em Cuidados de Saúde
14.
N Engl J Med ; 363(6): 532-41, 2010 Aug 05.
Artigo em Inglês | MEDLINE | ID: mdl-20818888

RESUMO

BACKGROUND: Hereditary angioedema is characterized by recurrent attacks of angioedema of the skin, larynx, and gastrointestinal tract. Bradykinin is the key mediator of symptoms. Icatibant is a selective bradykinin B2 receptor antagonist. METHODS: In two double-blind, randomized, multicenter trials, we evaluated the effect of icatibant in patients with hereditary angioedema presenting with cutaneous or abdominal attacks. In the For Angioedema Subcutaneous Treatment (FAST) 1 trial, patients received either icatibant or placebo; in FAST-2, patients received either icatibant or oral tranexamic acid, at a dose of 3 g daily for 2 days. Icatibant was given once, subcutaneously, at a dose of 30 mg. The primary end point was the median time to clinically significant relief of symptoms. RESULTS: A total of 56 and 74 patients underwent randomization in the FAST-1 and FAST-2 trials, respectively. The primary end point was reached in 2.5 hours with icatibant versus 4.6 hours with placebo in the FAST-1 trial (P=0.14) and in 2.0 hours with icatibant versus 12.0 hours with tranexamic acid in the FAST-2 trial (P<0.001). In the FAST-1 study, 3 recipients of icatibant and 13 recipients of placebo needed treatment with rescue medication. The median time to first improvement of symptoms, as assessed by patients and by investigators, was significantly shorter with icatibant in both trials. No icatibant-related serious adverse events were reported. CONCLUSIONS: In patients with hereditary angioedema having acute attacks, we found a significant benefit of icatibant as compared with tranexamic acid in one trial and a nonsignificant benefit of icatibant as compared with placebo in the other trial with regard to the primary end point. The early use of rescue medication may have obscured the benefit of icatibant in the placebo trial. (Funded by Jerini; ClinicalTrials.gov numbers, NCT00097695 and NCT00500656.)


Assuntos
Angioedemas Hereditários/tratamento farmacológico , Antagonistas de Receptor B2 da Bradicinina , Bradicinina/análogos & derivados , Doença Aguda , Adulto , Bradicinina/administração & dosagem , Bradicinina/efeitos adversos , Bradicinina/uso terapêutico , Método Duplo-Cego , Feminino , Humanos , Injeções Subcutâneas , Análise de Intenção de Tratamento , Masculino , Estatísticas não Paramétricas , Ácido Tranexâmico/uso terapêutico
15.
Medicina (B Aires) ; 73(5): 433-7, 2013.
Artigo em Espanhol | MEDLINE | ID: mdl-24152399

RESUMO

The processes of isolation and sterilization of intravenous gamma globulin (IVIG) affect the end product characteristics and, therefore, its tolerability. Different products have different incidences of adverse reactions. The aim of this study was to quantify the immediate adverse events (AE) caused by the different IVIG preparations. We analyzed 1 395 infusions in 28 patients, with a median of 32.5 per subject (range 2-214), using six different IVIG preparations, with an average dose 40.3 ± 8.3 g. One thousand and thirty-one infusions were analyzed retrospectively and 364 prospectively. Patients used a mean of 2.68 ± 1.8 different IVIGs, with a median of 2 (range 1-6) per person. The number of trademarks used was related to the number of infusions received, r = 0.73. AE presented in 24 (2.3%) of 1 031 infusions retrospectively analyzed, affecting 11 of 23 patients enrolled, with a mean of 2.18 ± 1.08 AE per subject. Of 24 patients and 364 infusions prospectively analyzed, AE were observed in 14 patients and in 32 (7.2%) procedures. Twenty-four (42.9%) of 56 AE were mild, 31 (55.5%) moderate and one (1.8%) severe. The infusion rate was 9.04 ± 6 g/h for those presenting AE vs. 10.6 ± 4.6 g/h for those who did not (p = 0.31, NS). The incidence, severity and proportion of patients with AE for each brand of IVIG were very different from each other. This information should be taken into account when selecting the IVIG to be used.


Assuntos
Imunoglobulinas Intravenosas/efeitos adversos , Fatores Imunológicos/efeitos adversos , gama-Globulinas/efeitos adversos , Estudos de Coortes , Feminino , Humanos , Infusões Intravenosas , Masculino , Estudos Retrospectivos , Índice de Gravidade de Doença , Fatores de Tempo , Resultado do Tratamento
16.
Medicina (B Aires) ; 73(4): 315-23, 2013.
Artigo em Espanhol | MEDLINE | ID: mdl-23924529

RESUMO

Common variable immunodeficiency (CVID) is characterized by an impaired antibody production and an increased susceptibility to recurrent infections of the respiratory tract, mainly by extracellular encapsulated bacteria. We analyzed the clinical characteristics of 69 patients evaluated over a period of 10 years at three centers in the city of Buenos Aires. At the onset of the study 14 patients were on follow up, and at its end the number of patients reached to 60. Most of them consulted for infection or hypogammaglobulinemia and nearly half had an established diagnosis of immunodeficiency. Sixty-five (94.2%) patients had infections by encapsulated bacteria, four (6.1%) sepsis and two tuberculosis. The average age of onset of infectious symptoms was 18.1 years; the average age at diagnosis was 29.6 years and the delay to diagnosis 11.9 years. Forty one (59.4%) patients reported a history of recurrent or chronic diarrhea. In 22 (31.9%) 13 autoimmune diseases were diagnosed, being the most frequent the hematological disorders and hypothyroidism. Eight patients had histological polyclonal lymphoproliferation, four (5.8%) with granulomatous disease affecting the liver, the larynx and/or the skin; and four as lymphoid interstitial pneumonitis (LIP). Nineteen (27.5%) patients had splenomegaly and 23/57 (40.3%) images suggestive of lymphocytic or granulomatous processes (including the 4 with LIP) in the chest CT. Three (4.3%) patients developed B cell lymphoma, four (5.8%) stomach adenocarcinoma and one breast cancer. The study had a median follow-up of 54 months, range 1-353 and four patients (5.8%) died during the follow up.


Assuntos
Imunodeficiência de Variável Comum/complicações , Imunodeficiência de Variável Comum/epidemiologia , Adulto , Fatores Etários , Idade de Início , Idoso , Idoso de 80 Anos ou mais , Argentina/epidemiologia , Imunodeficiência de Variável Comum/imunologia , Progressão da Doença , Feminino , Doença Granulomatosa Crônica/imunologia , Humanos , Doenças Pulmonares Intersticiais/imunologia , Linfoma de Células B/imunologia , Masculino , Pessoa de Meia-Idade , Fatores Sexuais , Neoplasias Gástricas/imunologia , Fatores de Tempo , Adulto Jovem
19.
Medicina (B Aires) ; 72(2): 119-23, 2012.
Artigo em Espanhol | MEDLINE | ID: mdl-22522852

RESUMO

Hereditary angioedema (HAE) is a rare autosomal dominant disease, characterized by episodes of edema involving the skin, gastrointestinal tract and larynx. HAE has a historical asphyxia mortality of 15% to 50%. It is the consequence of functional C1 inhibitor deficiency. The identification of bradykinin as the principal mediator of the disease has lead to the development of new drugs for its treatment. HAE management and treatment are agreed by international consensus decision. A therapeutic guide for the treatment of the disease is important to improve diagnosis and treatment. We here describe the pharmacology of drugs available for the treatment of HAE in Argentina: plasma derived C1 Inhibitor, the bradykinin antagonist: icatibant, the attenuated androgen danazol and the anti-fibrinolytic agents epsilonaminocaproic acid and tranexamic acid. Furthermore, we describe drug use and adverse effects control, as well as the last international consensus document recommendations applicable to Argentina to conform a first guide to HAE treatment in our country.


Assuntos
Angioedemas Hereditários/terapia , Doenças Raras/terapia , Doença Aguda , Argentina , Proteína Inibidora do Complemento C1/uso terapêutico , Inativadores do Complemento/uso terapêutico , Humanos
20.
Medicina (B Aires) ; 72(2): 103-8, 2012.
Artigo em Espanhol | MEDLINE | ID: mdl-22522849

RESUMO

Ocular cicatricial pemphigoid (OCP) is a blistering autoimmune disease that can produce severe conjunctival damage. Its response to immunosuppressive treatment is poorly known. We describe a group of 76 patients, 62 women and 14 men. Mean age at diagnosis was 67±14 years old, with a delay to diagnosis of 7.5±10 years. Sixty patients continued their follow up in our services for 19±21 months. Nineteen out of 51 had mild disease, 19 moderate, 5 severe and 8 very severe at onset of treatment. The more frequently prescribed drugs were dapsone, in 35 (23 discontinued it because of adverse effects), and methotrexate in 42 patients, nine of them stopped it. Other patients received azathioprine, cyclophosphamide and ciclosporine. Seventeen received oral steroids in addition to immunosuppressive drugs. Four patients combined two immunosuppressive drugs to control their disease. In three refractory cases IV immunoglobulin (Ig) was administered with good response. From 48 evaluated patients, 39 improved with treatment, eight remained stable and one progressed. In our experience, methotrexate and azathioprine were effective drugs, with low toxicity. Dapsone was useful in mild cases, with frequent adverse effects. IVIg was effective for refractory cases.


Assuntos
Doenças Autoimunes/tratamento farmacológico , Imunossupressores/uso terapêutico , Penfigoide Mucomembranoso Benigno/tratamento farmacológico , Adulto , Idoso , Idoso de 80 Anos ou mais , Azatioprina/uso terapêutico , Dapsona/uso terapêutico , Diagnóstico Tardio , Feminino , Seguimentos , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Imunossupressores/efeitos adversos , Masculino , Metotrexato/uso terapêutico , Pessoa de Meia-Idade , Penfigoide Mucomembranoso Benigno/patologia , Índice de Gravidade de Doença , Resultado do Tratamento
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