RESUMO
Bronchiectasis is a well-recognised complication of primary antibody deficiency (PAD) syndromes. Previous data suggest that mortality in common variable immune deficiency (CVID) is not associated with isolated bronchiectasis. A retrospective analysis of patients with CVID and specific antibody deficiency in two tertiary referral centres with lung disease was conducted. Severity of bronchiectasis at presentation was associated with mortality. Lower FEV1, colonisation with Pseudomonas aeruginosa and a diagnosis of COPD were also associated with mortality. Bronchiectasis is an important driver of mortality in patients with PAD syndromes.
Assuntos
Bronquiectasia , Imunodeficiência de Variável Comum , Pneumopatias , Doenças da Imunodeficiência Primária , Humanos , Estudos RetrospectivosAssuntos
Adenosina Desaminase/deficiência , Transtornos da Insuficiência da Medula Óssea/genética , Peptídeos e Proteínas de Sinalização Intercelular/deficiência , Linfopenia/genética , Mutação , Neutropenia/genética , Adenosina Desaminase/sangue , Adenosina Desaminase/genética , Adulto , Sequência de Aminoácidos , Transtornos da Insuficiência da Medula Óssea/sangue , Transtornos da Insuficiência da Medula Óssea/enzimologia , Transtornos da Insuficiência da Medula Óssea/patologia , Feminino , Humanos , Peptídeos e Proteínas de Sinalização Intercelular/sangue , Peptídeos e Proteínas de Sinalização Intercelular/genética , Linfopenia/sangue , Linfopenia/enzimologia , Pessoa de Meia-Idade , Neutropenia/sangue , Neutropenia/enzimologia , LinhagemAssuntos
Anticorpos Anticitoplasma de Neutrófilos , Doenças Reumáticas , Anticorpos Anticitoplasma de Neutrófilos/análise , Anticorpos Anticitoplasma de Neutrófilos/sangue , Gerenciamento Clínico , Humanos , Seleção de Pacientes , Doenças Reumáticas/imunologia , Doenças Reumáticas/terapia , Tempo para o TratamentoRESUMO
Cytokines play an important role in the pathogenesis of systemic lupus erythematosus (SLE) and lupus nephritis. This is currently a very active area of research. Of particular interest is the use of cytokines as biomarkers of disease activity in SLE and lupus nephritis. Can cytokine measurements assist in early detection of renal flare in known lupus nephritis? Can such measurements be used to distinguish between flare and chronic damage? Or help to confirm renal remission? Could they be used to help assess the required duration of immunosuppression and reduce the need for invasive renal biopsy? This review discusses limitations of current laboratory methods in monitoring SLE, how measurements of cytokines may contribute in relation to following disease activity and summarizes what is known about cytokines as biomarkers in SLE and lupus nephritis.
