Pulmonary manifestations in adult patients with a defect in humoral immunity

2016 ◽  
Vol 88 (8) ◽  
pp. 127-134
Author(s):  
T V Latysheva ◽  
E A Latysheva ◽  
I A Martynova ◽  
G E Aminova
Keyword(s):  
Humoral Immunity ◽  
Iga Deficiency ◽  
Early Age ◽  
Congenital Diseases ◽  
Selective Iga Deficiency ◽  
Adult Age ◽  
Common Misconception ◽  
Pulmonary Manifestations ◽  
The Common ◽  
Common Variable

Primary immunodeficiencies (PIDs) are a group of congenital diseases of the immune system, which numbers more than 230 nosological entities associated with lost, decreased, or wrong function of its one or several components. Due to the common misconception that these are extremely rare diseases that occur only in children and lead to their death at an early age, PIDs are frequently ruled out by physicians of related specialties from the range of differential diagnosis. The most common forms of PIDs, such as humoral immunity defects, common variable immune deficiency, X-linked agammaglobulinemia, selective IgA deficiency, etc., are milder than other forms of PID, enabling patients to attain their adult age, and may even manifest in adulthood. Bronchopulmonary involvements are the most common manifestations of the disease in patients with a defect in humoral immunity. Thus, a therapist and a pulmonologist are mostly the first doctors who begin to treat these patients and play a key role in their fate, since only timely diagnosis and initiation of adequate therapy can preserve not only the patient’s life, but also its quality, avoiding irreversible complications. Chest computed tomography changes play a large role in diagnosis. These are not specific for PID; however, there are a number of characteristic signs that permit this diagnosis to be presumed.

Reexamining the role of TACI coding variants in common variable immunodeficiency and selective IgA deficiency

2007 ◽  
Vol 39 (4) ◽  
pp. 430-431 ◽  
Author(s):  
Emanuela Castigli ◽  
Stephen Wilson ◽  
Lilit Garibyan ◽  
Rima Rachid ◽  
Francisco Bonilla ◽  
...  
Keyword(s):  
Common Variable Immunodeficiency ◽  
Iga Deficiency ◽  
Selective Iga Deficiency ◽  
Coding Variants ◽  
Common Variable

The Association Between Immunodeficiency and the Development of Autoimmune Disease

1996 ◽  
Vol 10 (1) ◽  
pp. 57-61 ◽  
Author(s):  
J.W. Sleasman
Keyword(s):  
Immune System ◽  
Autoimmune Disease ◽  
Lupus Erythematosus ◽  
Iga Deficiency ◽  
Selective Iga Deficiency ◽  
Increased Risk ◽  
High Incidence ◽  
Autoimmune Cytopenias ◽  
The Complement System ◽  
Common Variable

There is a paradoxical relationship between immunodeficiency diseases and autoimmunity. While not all individuals with immunodeficiency develop autoimmunity, nor are all individuals with autoimmunity immunodeficient, defects within certain components of the immune system carry a high risk for the development of autoimmune disease. Inherited deficiencies of the complement system have a high incidence of systemic lupus erythematosus (SLE), glomerulonephritis, and vasculitis. Carrier mothers of children with chronic granulomatous disease, an X-linked defect of phagocytosis, often develop discoid lupus. Several antibody deficiencies are associated with autoimmune disease. Autoimmune cytopenias are commonly observed in individuals with selective IgA deficiency and common variable immune deficiency. Polyarticular arthritis can be seen in children with X-linked agammaglobulinemia. Combined cellular and antibody deficiencies, such as Wiskott-Aldrich syndrome, carry an increased risk for juvenile rheumatoid arthritis and autoimmune hemolytic anemia. Several hypothetical mechanisms have been proposed to explain the associations between autoimmunity and immunodeficiency. Immunologic defects may result in a failure to exclude microbial antigens, resulting in chronic immunologic activation and autoimmune symptoms. There may be shared genetic factors, such as common HLA alleles, which predispose an individual to both autoimmunity and immunodeficiency. Defects within one component of the immune system may alter the way a pathogen induces an immune response and lead to an inflammatory response directed at self-antigens. An understanding of the immunologic defects that contribute to the development of autoimmunity will provide an insight into the pathogenesis of the autoimmune process.

scholarly journals Salmonella typhi vaccination response study reveals defective antibody production selective IgA deficiency patient

2015 ◽  
Vol 62 (4) ◽  
pp. 318-322
Author(s):  
Daniel E Pleguezuelo ◽  
Carla Gianelli
Keyword(s):  
Antibody Response ◽  
Antibody Production ◽  
Tetanus Toxoid ◽  
Common Variable Immunodeficiency ◽  
Salmonella Typhi ◽  
Iga Deficiency ◽  
Upper Respiratory Tract ◽  
Selective Iga Deficiency ◽  
Tract Infections ◽  
Common Variable

Selective IgA deficiency (SIgAD) is the most prevalent immunodeficiency worldwide, progressing to common variable immunodeficiency only in few reported cases. We report the case of a Spanish female aged 22 and diagnosed of selective IgA deficiency, a long history of bronchitis, several episodes of pneumonia, bilateral bronchiectasis, normal IgG, IgM, IgG subclasses, and detectable pre-vaccination IgG antibodies against tetanus toxoid and Streptococcus pneumoniae. She was evaluated in our clinic in order to rule out common variable immunodeficiency. We observed good antibody response to tetanus toxoid, absence of circulating switched memory B cells, decreased response to pneumococcal polysaccharide antigens and a lack of response to Salmonella typhi vaccine. Most SIgAD patients presents with upper respiratory tract infections or mild diarrhea. Those with lower tract infections, pneumonia or untreatable diarrhea should follow B-cell subpopulations’ study and antibody response to vaccines. Absence of response to Salmonella typhi vaccine allowed us to expose the defective antibody production.

High frequency of HLA, -A33, -B14, -DR1 in selective IgA deficiency and common variable immunodeficiency

1996 ◽  
Vol 47 (1-2) ◽  
pp. 39
Author(s):  
Despina Chryssovergi ◽  
Helen Pappas ◽  
Katerina Tarassi ◽  
Theophilos Athanasiadis ◽  
Ioanna Economidou ◽  
...  
Keyword(s):  
Common Variable Immunodeficiency ◽  
High Frequency ◽  
Iga Deficiency ◽  
Selective Iga Deficiency ◽  
Common Variable

scholarly journals Republican registry of primary immune deficiencies in the chuvash republic and description of postvaccinal immunity disorders in a pregnant patient with common variable immune deficiency

2021 ◽  
Vol 23 (2) ◽  
pp. 353-368
Author(s):  
L. M. Karzakova ◽  
O. M. Muchukova ◽  
T. S. Lutkova ◽  
S. I. Kudryashov ◽  
N. V. Zhuravleva ◽  
...  
Keyword(s):  
Immune Deficiency ◽  
T Cell ◽  
Pregnant Patient ◽  
Iga Deficiency ◽  
Common Variable Immune Deficiency ◽  
Primary Immunodeficiency Disorder ◽  
Selective Iga Deficiency ◽  
Clinical Hospital ◽  
First Case ◽  
Common Variable

In recent years, primary immunodeficiencies have turned from the class of rare diseases to the category of more common disorders which may be encountered by doctors of any clinical discipline. The first case of primary immunodeficiency disorder (PID) in Chuvashia was detected in 1993. Since that time, the Department of Internal Diseases with the Course of Clinical Immunology at the I. Ulyanov Chuvash State University registered all the cases of PID diagnosed in the region, introducing them into the Republican Registry of PID. The study was aimed for searching epidemiological indexes, clinical and laboratory manifestations of PID in Chuvash region. The study was based on the patient data obtained by retrospective analysis of 85 case histories of PID patients, treated at different departments of the Republican Clinical Hospital, and the City Chuvash Pediatric Clinical Hospital of Public Health Ministry in 2000-2019, as well as on 49 outpatient records of the patients included into the Regional PID Registry. Various forms of PIDs were diagnosed according to the criteria developed by the European Society for Immunodeficiency and the Pan-American Group on Immunodeficiency (1999). The results of this study showed that the incidence of PID in the Chuivash Region is 3.4:100,000. The incidence of common variable immune deficiency (CVID), the most common form of PID in the region, was 1.58 per 100,000 population. The average age at the time of CVID diagnosis in Chuvash patients was 30.4±16.1 years, and the age of CVID debut was 11.3±15.0 years. The delay in proper diagnosis from the moment of clinical manifestation of CVID was, on average, 17.9 years in the region. At the time of CVID diagnosis, the patients showed marked decrease in the levels of 3 or 2 immunoglobulin classes (IgG and IgA), and T-helper cell contents (CD3+CD4+) in peripheral blood. Prevalence of selective IgA deficiency with сlinical symptoms was 0.83 per 100,000 population of the region, and the incidence of the asymptomatic form of this PID was 1 : 167. In patients with selective IgA deficiency, there were also disorders in the T cell system manifesting as decreased relative number of cytotoxic T-cells as well as elevated IgG and IgM levels. The age of diagnosis of X-linked agammaglobulinemia in the region was 3.5±3.0 years. In addition to disturbances of humoral adaptive immunity in children with this disease, a decrease in absolute T cell numbers was detected. In conclusion, the article describes disturbances of postvaccinal immunity in a pregnant patient with CVID, with asymptomatic clinical course, thus leading to false interpretation of the serological markers of TORCH infections and wrong strategy of pregnancy management.

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