Immunodeficiency pneumonia


Introduction to immunodeficiency pneumonia

Immunodeficiency pneumonia refers to a syndrome caused by congenital, hereditary and other immune defense mechanisms. It is not appropriate to classify immune-immune diseases, such as lymphoma-induced immune damage, as immunodeficiency pneumonia. The clinical manifestations of this syndrome are repeated infections, especially respiratory infections, which are characterized by childhood onset and occasional to adulthood.

basic knowledge

The proportion of illness: 0.0005%-0.001%

Susceptible people: no special people

Mode of infection: non-infectious

Complications: respiratory infections


Causes of immunodeficiency pneumonia

(1) Causes of the disease

1% to 2% of recurrent respiratory infections are caused by primary immunodeficiency.

(two) pathogenesis

Immune deficiency refers to various diseases caused by damage caused by congenital, hereditary and other immune defense mechanisms.


Immunodeficiency pneumonia prevention

Active exercise: proper exercise can increase fat consumption, reduce cholesterol deposition in the body, improve insulin sensitivity, and have benefits in preventing obesity, controlling body weight, regulating blood lipids and lowering blood pressure. It is a positive measure to prevent and treat cerebral infarction. Patients with cerebral infarction should be selected according to the individual's physical condition. Appropriate physical exercise and physical activity should be carried out to avoid fatigue. It is not advisable to do strenuous exercise, such as running, climbing, etc., and can perform aerobics such as jogging, walking, soft gymnastics, and Tai Chi.


Immunodeficiency pneumonia complications Complications, respiratory infections

It is common to have repeated infections, especially respiratory infections.


Symptoms of immunodeficiency pneumonia Common symptoms Diarrhea Testicular development Incomplete partial malformation Lack of eczema Repeated infections Ocular tremor Oral Candida infections Photophobic hemolytic anemia

The clinical types are as follows:

1. Congenital X-linked gammaglobulinemia (congenital X-linedagammaglobulinemia)

In 1952, Bmton first reported the disease. It was found in male children due to X chromosome abnormalities, but not immunoglobulin structural gene abnormalities. The name is about 1/100,000. The immunological feature of this disease is B lymphocyte differentiation. In the pre-B lymphocyte stage, mature lymphocytes and plasma cells were not observed; serum immunoglobulins were low, even if antigen stimulation could not produce antibodies; T lymphocytes and cell-mediated immunity were completely normal, and the children were born. After 3 to 4 months, the temporary protection of maternal antibodies usually does not occur. After that, it shows increased sensitivity to pathogenic bacteria. The lower respiratory tract infections are the most common, gastrointestinal and bone joint infections, sepsis, meningitis, etc. It is also observed that the symptoms of patients may not be as severe as the corresponding infections in children, but characterized by chronic, repeated attacks. Most of the pneumonia dissipates slowly, and half of the patients have bronchiectasis. Common pathogens include Staphylococcus aureus, Streptococcus pneumoniae, and influenza. Hemobacteria and other types of staphylococci and streptococci, followed by unidentified Haemophilus influenzae, Salmonella, Pseudomonas aeruginosa, mycoplasma, etc., and early detection of Pneumocystis carinii infection and advanced viral and fungal infections have been reported in children, but in general, these pathogens are rare in this disease, gamma globulin Alternatives and supplements, the use of antibiotics to control infection is the standard treatment of this disease, prophylactic use of gamma globulin can reduce the incidence of bacterial infection, rarely useful for mucosal surface infections, therefore, the promotion of prophylactic application of gamma globulin should be at the target Prior to the destruction of organ structure, the amount and serum immunoglobulin concentration that should be maintained to prevent infection have not been determined.

2. Common variable immunodeficiency (CVI)

The disease was first reported in 1954, is a congenital, but non-hereditary immunoglobulin decreased, the name is more confusing, others have acquired low-gammaglobulinemia, idiopathic delayed immunoglobulin deficiency The name of the disease and primary hypogammaglobulinemia, the cause of CVI is unclear, unlike X-linked agammaglobulinemia, the number of B lymphocytes in most patients is normal or increased, but can not develop into secretory plasma cells. In some cases, B lymphocytes cannot proliferate or synthesize immunoglobulins, while in other cases, plasma cells can be found to produce immunoglobulins, but they cannot be secreted. Substances that inhibit B lymphocytes are found in the serum of very few patients. The function of B lymphocytes returned to normal after removal of inhibitory substances. In some cases, the increase of inhibitory T lymphocytes was also found, and its significance in the pathogenesis is unclear. Interested in H2-receptor blockers can reduce inhibition. T lymphocyte activity, some patients increase the IgG after the application of these drugs, the serum IgG of patients with this disease is usually less than 3.0mg / ml or less than half of the lower limit of normal, IgA and IgM levels are uncertain, Often one or two immunoglobulins are abnormally reduced, and occasionally both are normal. CVI is one of the diseases with increased sweat chloride, and most of the patients with cystic fibrosis have elevated gamma globulin, but About 20% of patients showed a decrease.

Therefore, all patients with elevated sweat chloride and low gamma globulin should determine lymphocyte function, the clinical manifestations of this disease are similar to X-linked no-gammaglobulinemia, but most of the symptoms appear after 30 years old, half of the patients with repeated respiratory tract Infection is the main manifestation. Nearly 90% of patients in the clinical course have repeated bacterial pneumonia, 70% have sinusitis, 35% have otitis media, and sepsis is rare. Respiratory infections are mostly Gram-positive capsules, but Non-capsulated Haemophilus influenzae, the pathogenicity of mycoplasma is increasing, non-respiratory infections are meningitis, abdominal abscess, urinary tract infection, etc., are rare, however, more than half of patients have chronic diarrhea, probably due to Lange Due to the excessive growth of Giardia or non-intestinal bacteria, CVI often combines various non-infectious diseases such as lung, spleen, liver, non-case granuloma of the skin, bone cancer, thymoma, lymphoma and various Thyroid disease, intravenous administration of gamma globulin is recommended as an alternative treatment, but the level of serum immunoglobulin and its relationship with infection needs further study.

3. Selective immunoglobulin deficiency

The lack of selective immunoglobulins is quite common, and many of the lack of patients do not show disease status, such as the lack of selective IgG4 in the normal population of up to 25%, according to the inspection of healthy blood donors found about every 700 people One of them was a selective IgA deficiency. However, it has been reported in the literature that various immunoglobulin deficiency patients, including IgA, IgG2, IgG3, IgG4, IgM, IgE, etc., may be a single deficiency, but most of them are combined deficiency, selective IgA. Insufficient serum IgA<0.05mg/ml, patient B lymphocytes are morphologically mature, but can not produce and secrete IgA to serum or secretions, children with IgA deficiency are often accompanied by IgG2, IgG3 and IgG4 deficiency, IgA The combined disease with IgG3 has an increased prevalence of lymphoma. IgA deficiency combined with IgE deficiency has few symptoms. On the contrary, 71% of patients with normal IgE have respiratory infection, and those with symptomatic IgA deficiency often have specificity. And allergic to food, especially milk, 1/3 of patients with repeated upper and lower respiratory infections, including sinusitis, otitis media, pharyngitis and pneumonia; unlike CVI, its permanent tissue damage such as less bronchiectasis , <5%, gastrointestinal infections are quite common, and another 1/3 of patients with collagen vascular disease, but this is the case, IgA deficiency treatment is mainly for infection, allergy and collagen vascular disease, efficacy and prognosis does not seem to depend on Patients with IgA levels should pay special attention to avoid allergic reactions when infusion of IgA-containing blood products. The blood cells must be washed. If there is no IgG subtype deficiency, gamma globulin should not be infused intravenously, because it also has an allergic risk. Other rare selective immunoglobulin deficiency has IgA secretion deficiency and IgM deficiency. The patient's antibody response to pathogenic microorganisms is abnormal, and repeated infections, especially Gram-negative bacilli sepsis.

4. Complement deficiency

Primary complement deficiency is very rare, C1, C2, C3 deficiency clinical syndrome is similar to systemic lupus erythematosus, or some other connective tissue disease, the difference is that there is no anti-DNA antibody, the patient is not susceptible to infection Significantly increased, if pneumonia occurs, it is often secondary to sepsis, C1q deficiency is often accompanied by hypogammaglobulinemia, C1 inhibitor deficiency is very common, clinical manifestations of hereditary angioedema, respiratory mucosal edema can be fatal Related to infection is C3 deficiency, C5-8 deficiency and complement bypass defects. The classical activation pathway and the bypass activation pathway converge at C3, so C3 plays a key role in the host defense mechanism. Type I C3 deficiency is found in congenital Testicular hypoplasia syndrome, K1 einfelter syndrome, is caused by the constant activation of C3 inactivation factor C3, and type II C3 deficiency is often accompanied by lipodystrophy, which is caused by the presence of serum C3 convertase. As a result, patients with C3 deficiency have increased susceptibility to capsular bacteria, and frequent recurrent purulent infections of the respiratory tract, middle ear, meninges, and skin occur. C5-8 has a pathogen. The lack of its role will inevitably weaken the clearance of pathogenic microorganisms, but the lack of isolated C5-6 is rare. Complementary bypass defects have been recognized in recent years. In the case of sickle cell anemia, patients not only lack heat resistance to Streptococcus pneumoniae. Opsonin, which does not produce an antibody response, has a reduced ability to compensate for complement bypass.

5. Congenital thymic hypoplasia (congenital thymic aplasia)

The disease is caused by the third and fourth pairs of thymocytes and parathyroid primordia, and the complete clinical syndrome includes thymic hypoplasia, parathyroid tissue deficiency, congenital heart disease and facial deformity. It is called Di George syndrome. Immunologically, the patient's blood T lymphocytes are deficient. The absolute count of lymphocytes is at the normal lower boundary level, and the T lymphocytes in the deep cortex of the lymph nodes are also absent. The T lymphocyte function is like phytohemagglutinin (PHA). The proliferative response is suppressed, the serum immunoglobulin is usually in the normal range, and the antibody response to the allergen is normal or decreased. Most of the children are suspected and diagnosed due to abnormal heart and low calcium convulsions within a few days after birth. Laboratory examination Further evidence can be provided, usually within one month of death, survivors (mostly incomplete) often occur with cytomegalovirus and Pneumocystis carinii pneumonia and Gram-negative bacilli sepsis.

6. Severe combined immunodeficiency (SCID)

The disease is a group of heterogeneous diseases characterized by lymphopenia, lack of lymphoid tissue, inhibition of thymic function and reduction of immunoglobulin. T lymphocytes and B lymphocytes are abnormal and belong to X-linked or autosomal recessive. Hereditary defects, immunological abnormalities including pluripotent stem cell defects can not develop into T lymphocytes and B lymphocytes, lymphocytes decreased, but the number changes greatly, gamma globulin decreases, occasionally lymphocyte count is normal, serum gamma globulin is normal Even increased, but the response to antigenic stimulation was reduced, although the type of the disease was not subdivided, but two subtypes with biochemical abnormalities were found.

(1) adenosine deaminase deficiency: lymphoid tissue can be seen in the thymus tissue, but the maturation of the thymus tissue, adenosine deaminase is found in various mammalian cells, the lack of which generally affects only lymphoid cells, the mechanism of action is unclear, may be Its lack of accumulation of metabolites such as deoxyadenosine triphosphate, which can kill mature lymphocytes.

(2) sputum adenosine phosphorylase deficiency: the number of patients with T lymphocytes decreased, the response to mitogen or antigen stimulation decreased, and the number of B lymphocytes and immunoglobulins were normal, so it is similar to AIDS, another rare disease of this disease The variant is a combination of lymphocytes and neutrophils, called "retinal tissue suppuration". Most SCID patients develop symptoms within 1 year of age, manifesting as oral and cutaneous Candida infections, pneumonia and diarrhea. Most patients die from suppuration. Pneumonia or organizing pneumonia, and often infected with Pneumocystis carinii or herpes virus.

7. Ataxia - telangiectasia (ataxia telangiectasia, AT)

This disease is an autosomal recessive hereditary disease, paralyzed and multi-system, immunological abnormalities have not been fully clarified, most immune abnormalities are not clearly related to the increase in the prevalence of infection, but patients are reported to have delayed hypersensitivity to common antigens. Insufficient, T lymphocyte inhibition of mitogen stimulation and decreased serum IgA and IgE, seems to be associated with increased risk of infection, half of patients with serum IgG reduction, most of which are accompanied by a decrease in IgG2 subtype, cellular immune abnormalities including T Decreased lymphocyte count and function, thymic dysplasia, elevated alpha-fetoprotein in all patients, and often accompanied by increased carcinoembryonic antigen, suggesting organ maturation disorders, the earliest clinical symptoms are cerebellar ataxia, mostly at 2 years of age Appearance, often combined with phasing finger disease and nystagmus, posterior signs of eyeball conjunctiva and skin (usually in the limbs) telangiectasia, about 1/3 of cases are not infected; 1/3 cases of repeated respiratory infections, but not Residual sequelae: In another 1/3 of cases, progressive respiratory disease occurs, which progresses to bronchitis and bronchiectasis. The pathogen is mainly composed of suppurative bacteria.

8. Wiskott-Aldrich syndrome

The typical manifestations of this disease are thrombocytopenia, eczema and multiple infections, which are X-linked recessive genetic abnormalities, with damage to body fluids and cellular immunity, IgG is generally normal, IgA and IgE are increased, and IgA is decreased; against S. pneumoniae The antibody reaction of polysaccharide antigen is significantly reduced. The mechanism may be the defect of the afferent branch of the immune reaction, and the carbohydrate antigen cannot be identified and processed. It is also considered that the antigen processing is abnormal, and the cell-mediated immunity is also abnormal. The initial T lymphocyte The amount can be normal, and then slowly decreased. At the age of 6 years, the lymphocyte deficiency is caused. The response of T lymphocytes to common antigen and mitogen stimulation is reduced. 80% of patients have respiratory infections, Staphylococcus aureus, Streptococcus pneumoniae, Pseudomonas and opportunistic pathogens are predominant, usually within 10 years of age, mainly due to infection (60%) and bleeding (30%), and lymphatic reticulum malignancies can occur in 12% of patients.

9. Chronic mucocutaneous candidiasis

The disease is a neonatal immune disorder, as early as 1 year old, as late as 10 years old, manifested as mucosal skin, nasal and vaginal chronic Candida infection, and no systemic infection, some cases can eventually develop into endocrine lesions such as thyroid Paragonadal dysfunction or Addison's disease, immunologically, lymphocyte count and B lymphocyte function are normal, antibody response to Candida is normal, but T lymphocyte-mediated delayed hypersensitivity to Candida is reduced, possibly Is the lack of specific lymphocytes that can be activated for Candida, treatment for endocrine diseases, anti-candida treatment of amphotericin B combined with Candida albicans skin reaction, normal donor preparation of transfer factor may be more than the use of amphiric Prime B is effective.

10. Chemotaxis reaction is abnormal

Job syndrome is one of the types of chemotaxis disorders and is also considered to be a variant of chronic granulomatous disease. Some patients are accompanied by an increase in IgE, and clinically repeated recurrent staphylococcal abscesses in the skin, subcutaneous tissue and lymph nodes. Pulmonary infections are rare.

11. Abnormal phagocytosis

Abnormal phagocytosis is a non-independent disease, often accompanied by C3 deficiency, severe hypogammaglobulinemia or a variety of opsonin-deficient sickle cell anemia, as described above.

12. Degranulation abnormality

The aniline blue granules of phagocytic cells contain lysozyme, myeloperoxidase, acid hydrolase, etc. Congenital myeloperoxidase deficiency is associated with increased susceptibility to Candida infection, but most can be asymptomatic, phagocytic cells Specific granules contain some lysozyme and lactoferrin. The abnormality is called Chediad-Higashi syndrome. It is an autosomal recessive genetic defect. The phagocytic phagocytosis and respiratory burst are normal. The main abnormality is lysosome and The phagosome fusion disorder, clinical manifestations of the eye, skin whitening, photophobia, nystagmus, and repeated purulent infections, in addition to antibacterial therapy, cholinergic drugs and vitamin C may be beneficial.

13. Oxidative metabolism abnormalities

Chronic granulomatous disease is a representative disease of oxidative metabolism abnormalities in phagocytic cells. The phagocytic cells are stimulated by pathogenic microorganisms and cannot increase oxygen consumption. Therefore, superoxide anion and hydrogen peroxide cannot be produced, and the oxidative sterilization function is lost. Within the age of onset, skin, lungs, bones and lymph nodes are most commonly involved, pulmonary infections include diffuse infiltration, hilar lymphadenopathy or atelectasis, lung abscess formation, "localized" pneumonia, etc., pathogens are mostly catalase Positive bacteria such as Staphylococcus aureus and Aspergillus, etc., because the catalase-positive bacteria destroy hydrogen peroxide and make the antimicrobial defense system imperfect. In addition to the application of antimicrobial drugs to pathogens, it is reported that Smzco is effective in preventing infection of this disease. There is a certain effect, the surgical treatment of the infected foci is also important. When the conventional treatment is ineffective, the white blood cells can be infused. The infection is the most common cause of death, most of which are in childhood, but there are also those who survive to 30 years of age or older.

Another disease with abnormal oxidative metabolism is glucosamine-6-phosphate dehydrogenase deficiency, which is characterized by hemolytic anemia and repeated infections, mainly Staphylococcus and certain Gram-negative bacilli infections.


Examination of immunodeficiency pneumonia

The etiological test of infection is the best method. Detailed medical history and physical examination data are collected, and immunological examination is selected on this basis.

Nearly 90% of patients in the clinical course have recurrent bacterial pneumonia, and chest X-rays show patchy or patchy shadows in the lungs.


Diagnosis and diagnosis of immunodeficiency pneumonia

Diagnostic points

1. Primary immunodeficiency and its type to determine the early diagnosis of primary immunodeficiency is very important, as long as the early diagnosis, the corresponding immunotherapy and reasonable anti-infective treatment, it is possible to prolong the patient's survival time And improving the quality of life, such as chronic granulomatosis, was once thought to be a rapidly lethal disease, and now with reasonable antibacterial therapy combined with surgical drainage, and the use of the immunomodulator interferon, the incidence of infection and mortality has been significantly reduced It has been reported that the incidence of common variability in immunodeficiency from the onset of infection to the diagnosis of immunodeficiency is up to 10 years, which is unacceptable by current standards because it is simple in most hospitals and commercial laboratories. The method can be diagnosed, so the key to early diagnosis is to improve the awareness and vigilance of clinicians.

2. The pathogenic diagnosis of infection The various pathogenic diagnostic techniques for infection are applicable to the pathogenic diagnosis of patients with primary immunodeficiency. It is important to emphasize that:

1 immunodeficiency infection can affect multiple organ systems such as skin, gastrointestinal tract, central nervous system and even sepsis, so specimens should be collected according to the condition of the disease. In terms of pulmonary infection, in addition to collecting qualified sputum specimens, there are indications. Timely traumatic diagnostic techniques such as tracheal aspiration, pulmonary puncture, bronchoalveolar lavage, anti-pollution lower respiratory sampling and thoracotomy lung biopsy appear to be more meaningful, and a positive attitude should be taken clinically;

2 The antibody production in such patients may be impaired, and the interpretation of the results of serological diagnostic antibody detection needs to be cautious;

3 antigen detection is faster than classical microbiological methods (culture), the results are not affected by antibiotic treatment, and less contaminated.

It has been used for the detection of many pathogens such as Streptococcus pneumoniae, Haemophilus influenzae, Neisseria meningitidis, Escherichia coli, Legionella, Pneumocystis carinii and Candida. Methods include convective immunoelectrophoresis, latex agglutination. , synergistic agglutination test, enzyme-linked immunosorbent assay, etc., but there are still deficiencies such as cross-positive leading to false positives, so when the condition allows and laboratory conditions are available, the pathogen diagnosis should adopt a variety of techniques, multi-project joint detection, combined with clinical Make a proper explanation.

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