Duration of frequent or severe respiratory tract infection in adults before diagnosis of IgG subclass deficiency.

Many adults with IgG subclass deficiency (IgGSD) experience long intervals of frequent/severe respiratory tract infection before IgGSD diagnosis, but reasons for delays in IgGSD diagnoses are incompletely understood. We performed a retrospective study of 300 white adults (ages ≥18 y) with IgGSD including frequency analyses of age at IgGSD diagnosis, duration of frequent/severe respiratory tract infection before IgGSD diagnosis, and age at onset of frequent/severe infection (calculated). We performed multivariable regressions on age at diagnosis, infection duration, and age at infection onset using these variables, as appropriate: sex; age at diagnosis; diabetes; autoimmune condition(s); atopy; allergy; corticosteroid use; body mass index; serum immunoglobulin isotype levels; blood lymphocyte subsets; three IgGSD-associated human leukocyte antigen-A and -B haplotypes; and referring physician specialties. Mean age at diagnosis was 50 ± 12 (standard deviation) y (median 50 y (range 19-79)). There were 247 women (82.3%). Mean infection duration at IgGSD diagnosis was 12 ± 13 y (median 7 y (range 1-66)). Mean age at infection onset was 38 ± 16 y (median 38 y (range 4, 76)). Age at infection onset was ≥18 y in 95.7% of subjects. Regressions on age at diagnosis and infection duration revealed no significant associations. Regression on age at infection onset revealed one positive association: age at diagnosis (p <0.0001). We conclude that the median duration of frequent/severe respiratory tract infection in adults before IgGSD diagnosis was 7 y. Older adults may be diagnosed to have IgGSD after longer intervals of infection than younger adults. Duration of frequent/severe respiratory tract infection before IgGSD diagnosis was not significantly associated with routine clinical and laboratory variables, including referring physician specialties.

Neutropenia autoinmunitaria crónica del adulto y su frecuente asociación con déficit de IgG. Estudio de un caso y revisión bibliográfica / Association with IgG3 deficiency in chronic autoimmune neutropenia in adults: case report and literatura review

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Educational paper: primary antibody deficiencies.

Primary antibody deficiencies (PADs) are the most common primary immunodeficiencies and are characterized by a defect in the production of normal amounts of antigen-specific antibodies. PADs represent a heterogeneous spectrum of conditions, ranging from often asymptomatic selective IgA and IgG subclass deficiencies to the severe congenital agammaglobulinemias, in which the antibody production of all immunoglobulin isotypes is severely decreased. Apart from recurrent respiratory tract infections, PADs are associated with a wide range of other clinical complications. This review will describe the pathophysiology, diagnosis, and treatment of the different PADs.

Characterisation of the onset and presenting clinical features of adult bronchiectasis.

BACKGROUND: There is little information available on the features of initial presentation of bronchiectasis and documentation of the onset and progress of symptoms leading up to this. Therefore a study was performed on a large cohort of adult patients presenting to Monash Medical Centre (MMC) to survey the course of their disease up to the time of diagnosis. OBJECTIVES: To characterise the onset and presenting clinical features of bronchiectasis in adults. METHODS: A cross-sectional study of 103 adults presenting to a tertiary referral hospital with newly diagnosed bronchiectasis. Clinical features of bronchiectasis and results of spirometry, sputum microbiology and radiology were assessed and correlated. RESULTS: Most patients had idiopathic bronchiectasis (74%) and did not have other significant disease. The dominant symptom was chronic productive cough present in 98% of patients with other important symptoms being chronic rhinosinusitis (70%), dyspnoea (62%), and fatigue (74%). Most patients had had a chronic productive cough for over 30 years prior to diagnosis and over 80% of patients had chronic respiratory symptoms from childhood. The dominant finding on physical examination was the presence of crackles which were generally bi-basal. Spirometry showed mild airway obstruction with an average forced expiratory volume in 1s of the cohort of 76% predicted. Radiologic imaging generally showed multilobar disease (80%). CONCLUSIONS: The typical profile of bronchiectasis in this group of patients was of longstanding productive cough, rhinosinusitis and fatigue in non-smokers with crackles on chest auscultation.

[Common variable immunodeficiency: a rare humoral immunodeficiency with frequent later change of diagnosis]. / Common variable immunodeficiency: eine seltene humorale Immundefizienz mit meist erst später Diagnosestellung.

Common variable immunodeficiency (CVIS) is a condition associated with a deficiency of humoral immunity. The typical patient is an adolescent or adult with a history of recurrent upper respiratory tract infections over many years. Despite the long history the illness is not readily recognised and diagnosis is usually delayed until the patient is admitted to hospital for a severe respiratory tract infection and is being worked up for causes of immunodeficiency. The diagnosis of CVIS is based on the finding of an immunoglobulin deficiency in immunoelectrophoresis or documentation of reduced IgG subclasses. Typically this leads to upper respiratory tract infections and malabsorption, as illustrated by the case reports. Pathophysiologically this disease is characterised by malfunction of B cells with defective gammaglobulin production or secretion. 7 patients were substituted with IgG, leading to decreased susceptibility to infection and a reduction in hospital admissions due to severe infections.

Deficiencia de subclases de IgG, asma bronquial e infección recurrente

Recientemente se ha informado un posible incremento de la incidencia de deficiencia de subclases de IgG en pacientes con asma bronquial. Esto obviamente predispone a la aparición de infección recurrente y de secuelas respiratorias inherentes a la inmunodeficiencia, la cual empeora el curso de la enfermedad asmática. Informamos acerca de un grupo de siete pacientes en los cuales estudiamos dicha asociación. Adicionalmente nos referimos, en otro contexto, al grupo de pacientes asmáticos sin infección recurrente patológica en quienes la administración de inmunoglobulinas muestra claros efectos benéficos y comentamos algunos mecanismos propuestos para la acción de las inmunoglobulinas en estos casos. Finalmente, es de especial interés para nosotros sensibilizar a los clínicos respecto a esta asociación y a la importancia de estudiar el perfil humoral de estos pacientes e instaurar una terapia oportuna con inmunoglobulinas en los casos en que esté indicada, lo cual va a beneficiar en forma decisiva la evaluación y el pronóstico de estos pacientes.

Chronic sinusitis, steroid-dependent asthma, and IgG subclass and selective antibody deficiencies.

In the past 20 years considerable progress has been made in understanding the ways in which subtle immunologic defects can adversely affect health. Immunoglobulin G subclass deficiencies have been identified and are related to an increased susceptibility to respiratory tract infections in certain patients. To assess the immunocompetence of such patients, the quantity and quality of their antibody response must be evaluated. Immunologic evaluation is best performed by measuring selective antibodies before and after a specific challenge. Patients with mild immunodeficiency may benefit from prophylactic antibiotic therapy; those with profound immunodeficiency require antibody replacement therapy.