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Replacement and Immunomodulatory Therapy Using Subcutaneous Immunoglobulins
Immunodeficiencies: Primary and Secondary
Immunodeficiencies are a group of more than 250 diseases in which the immune system completely or partially loses its functions.
Normally, the immune system protects the human body from pathogenic microorganisms (bacteria, viruses, and fungi) capable of causing infectious diseases. When part of this system malfunctions or is absent, the patient:
is more prone to infections;
takes longer to recover.
Primary immunodeficiencies (PIDs) arise from a genetic defect in the immune system. Their characteristics include:
a large number of subtypes;
nonspecific symptoms that make diagnosis difficult.
Examples of PIDs:
Bruton’s X-linked agammaglobulinemia;
common variable immunodeficiency (CVI);
hyper-IgM syndrome;
selective IgA deficiency, etc.
Prevalence of PIDs: approximately 1:10,000–1:50,000; about 20–30% of cases are diagnosed in adulthood.
Studies show that a PID diagnosis is made, on average:
5.5 years after the onset of symptoms in adults;
2.5 years after the onset of symptoms in children.
Typical clinical manifestations (red flags for physicians):
frequent severe respiratory tract infections (more than 4–6 episodes per year);
recurrent purulent otitis media and sinusitis;
severe bacterial infections (meningitis, osteomyelitis, sepsis);
chronic diarrhea caused by opportunistic pathogens;
delayed physical development in children.
For immunodeficiencies associated with antibody deficiency, the preferred treatment method is immunoglobulin replacement therapy. Timely initiation of therapy is crucial: it helps prevent irreversible damage to organs and systems caused by infections.
Immunodeficiencies: Primary and Secondary
Immunodeficiencies are a group of more than 250 diseases in which the immune system completely or partially loses its functions.
Normally, the immune system protects the human body from pathogenic microorganisms (bacteria, viruses, and fungi) capable of causing infectious diseases. When part of this system malfunctions or is absent, the patient:
is more prone to infections;
takes longer to recover.
Primary immunodeficiencies (PIDs) arise from a genetic defect in the immune system. Their characteristics include:
a large number of subtypes;
nonspecific symptoms that make diagnosis difficult.
Examples of PIDs:
Bruton’s X-linked agammaglobulinemia;
common variable immunodeficiency (CVI);
hyper-IgM syndrome;
selective IgA deficiency, etc.
Prevalence of PIDs: approximately 1:10,000–1:50,000; about 20–30% of cases are diagnosed in adulthood.
Studies show that a PID diagnosis is made, on average:
5.5 years after the onset of symptoms in adults;
2.5 years after the onset of symptoms in children.
Typical clinical manifestations (red flags for physicians):
frequent severe respiratory tract infections (more than 4–6 episodes per year);
recurrent purulent otitis media and sinusitis;
severe bacterial infections (meningitis, osteomyelitis, sepsis);
chronic diarrhea caused by opportunistic pathogens;
delayed physical development in children.
For immunodeficiencies associated with antibody deficiency, the preferred treatment method is immunoglobulin replacement therapy. Timely initiation of therapy is crucial: it helps prevent irreversible damage to organs and systems caused by infections.
Targets for replacement therapy:
maintaining a pre-transfusion IgG level of at least 7 g/L;
initial dose of immunoglobulin: 0.4–0.8 g/kg of body weight;
at the start of therapy or after treatment interruptions—a loading dose of 0.6–0.8 g/kg.
Main methods for confirming the diagnosis:
quantitative determination of immunoglobulin classes (IgG, IgA, IgM, IgE);
IgG subclasses;
analysis of specific antibodies following vaccination;
study of lymphocyte subpopulations (T and B cells);
genetic testing when PID is suspected.
Secondary immunodeficiencies (SIDs) are caused by various factors, such as:
malignant neoplasms (including those affecting the hematopoietic and lymphoreticular systems);
metabolic diseases, etc.
Immunoglobulin therapy is used for hypogammaglobulinemia associated with:
chronic lymphocytic leukemia (CLL);
multiple myeloma (MM), often caused by concomitant immunosuppressive therapy.
Patients with these conditions may benefit from immunoglobulin replacement therapy in addition to standard treatment for the underlying disease.
Dysimmune neuropathies are a group of rare and disabling neurological disorders. They include: chronic inflammatory demyelinating polyneuropathy (CIDP);
multifocal motor neuropathy (MMN);
Guillain–Barré syndrome (GBS);
Lewis–Sumner syndrome.
These conditions can:
affect the ability to walk or grasp objects;
cause numbness, tingling, or pain in the arms and legs;
progress, with periods of recovery and relapse, or become chronic.
maintaining a pre-transfusion IgG level of at least 7 g/L;
initial dose of immunoglobulin: 0.4–0.8 g/kg of body weight;
at the start of therapy or after treatment interruptions—a loading dose of 0.6–0.8 g/kg.
Main methods for confirming the diagnosis:
quantitative determination of immunoglobulin classes (IgG, IgA, IgM, IgE);
IgG subclasses;
analysis of specific antibodies following vaccination;
study of lymphocyte subpopulations (T and B cells);
genetic testing when PID is suspected.
Secondary immunodeficiencies (SIDs) are caused by various factors, such as:
malignant neoplasms (including those affecting the hematopoietic and lymphoreticular systems);
metabolic diseases, etc.
Immunoglobulin therapy is used for hypogammaglobulinemia associated with:
chronic lymphocytic leukemia (CLL);
multiple myeloma (MM), often caused by concomitant immunosuppressive therapy.
Patients with these conditions may benefit from immunoglobulin replacement therapy in addition to standard treatment for the underlying disease.
Dysimmune neuropathies are a group of rare and disabling neurological disorders. They include: chronic inflammatory demyelinating polyneuropathy (CIDP);
multifocal motor neuropathy (MMN);
Guillain–Barré syndrome (GBS);
Lewis–Sumner syndrome.
These conditions can:
affect the ability to walk or grasp objects;
cause numbness, tingling, or pain in the arms and legs;
progress, with periods of recovery and relapse, or become chronic.
Indications for immunoglobulin therapy:
Guillain–Barré syndrome (first-line therapy, level of evidence);
chronic inflammatory demyelinating polyneuropathy (first-line);
multifocal motor neuropathy;
Kawasaki syndrome;
idiopathic thrombocytopenic purpura.
Mechanisms of action of immunoglobulins in neuropathies:
modulation of autoimmune reactions;
neutralization of pathogenic antibodies;
suppression of inflammatory processes;
restoration of cytokine balance.
Although a complete cure is currently not possible, available treatments can significantly improve symptoms.
Immunoglobulin Therapy
Treatment methods depend on the severity of the disease. One such method is the administration of immunoglobulins (intravenously or subcutaneously).
Intravenous administration of immunoglobulin significantly alters the course of the disease:
it sharply reduces the number of infectious episodes;
it improves patients’ quality of life.
With intravenous replacement therapy:
high peak plasma immunoglobulin levels are rapidly achieved;
protective levels of circulating antibodies are maintained with infusions every 3–4 weeks in a hospital setting.
Subcutaneous immunoglobulin replacement therapy is a method that has been revived in recent years with improvements that have increased treatment adherence among patients with primary immunodeficiencies.
Technical details of administration:
Recommended infusion sites: abdomen, thighs, outer surface of the shoulder;
Minimum distance between injection sites—5 cm;
Initial infusion rate—up to 15 mL/hour/site; if well tolerated, up to 25 mL/hour/site;
In children, the infusion site is changed after every 5–15 mL; in adults, after 30 mL.
Features of subcutaneous administration:
A portable infusion pump is used, inserted through a fine needle into the subcutaneous tissue of the abdomen, shoulders, or thighs;
the amount of immunoglobulin administered is less than with intravenous administration, and absorption occurs more slowly;
several short-term home infusions are required each month (every 7–15 days);
immunoglobulins remain active in the bloodstream longer, maintaining a stable level in the blood serum;
pharmacokinetics are smooth, without the peak characteristic of intravenous administration.
Dosage regimens:
cumulative monthly dose: 2.4–4.8 mL/kg of body weight;
frequency of infusions: from once a day to once every 2 weeks;
option to use the rapid push method or a pump.
Advantages of Home Therapy with Subcutaneous Immunoglobulins
Home therapy with subcutaneous immunoglobulins for conditions involving antibody deficiency or requiring immunomodulation offers several advantages over standard intravenous therapy in a hospital setting:
1. No venous access is required.
2. There is no need for hospitalization for severely debilitated patients—this helps prevent exposure to potential infections in the hospital.
3. Infusions are administered at home using a portable infusion pump: this reduces the frequency of hospital visits and saves patients time and money.
4. Shorter infusion time: subcutaneous infusion takes 15–30 min, compared to 3–4 hours for intravenous administration.
5. Mobility during the procedure: the patient can move around and perform daily activities thanks to the infusion pump’s portable design.
6. Fewer and milder side effects: Local reactions following subcutaneous administration are generally minor and moderate compared to intravenous administration.
7. A more physiologically appropriate distribution of immunoglobulins in the body.
8. Reduced risk of systemic side effects (headache, fever, chills).
9.Better treatment adherence due to convenience and mobility.
10. The ability to train patients and family members to administer the medication themselves.
Guillain–Barré syndrome (first-line therapy, level of evidence);
chronic inflammatory demyelinating polyneuropathy (first-line);
multifocal motor neuropathy;
Kawasaki syndrome;
idiopathic thrombocytopenic purpura.
Mechanisms of action of immunoglobulins in neuropathies:
modulation of autoimmune reactions;
neutralization of pathogenic antibodies;
suppression of inflammatory processes;
restoration of cytokine balance.
Although a complete cure is currently not possible, available treatments can significantly improve symptoms.
Immunoglobulin Therapy
Treatment methods depend on the severity of the disease. One such method is the administration of immunoglobulins (intravenously or subcutaneously).
Intravenous administration of immunoglobulin significantly alters the course of the disease:
it sharply reduces the number of infectious episodes;
it improves patients’ quality of life.
With intravenous replacement therapy:
high peak plasma immunoglobulin levels are rapidly achieved;
protective levels of circulating antibodies are maintained with infusions every 3–4 weeks in a hospital setting.
Subcutaneous immunoglobulin replacement therapy is a method that has been revived in recent years with improvements that have increased treatment adherence among patients with primary immunodeficiencies.
Technical details of administration:
Recommended infusion sites: abdomen, thighs, outer surface of the shoulder;
Minimum distance between injection sites—5 cm;
Initial infusion rate—up to 15 mL/hour/site; if well tolerated, up to 25 mL/hour/site;
In children, the infusion site is changed after every 5–15 mL; in adults, after 30 mL.
Features of subcutaneous administration:
A portable infusion pump is used, inserted through a fine needle into the subcutaneous tissue of the abdomen, shoulders, or thighs;
the amount of immunoglobulin administered is less than with intravenous administration, and absorption occurs more slowly;
several short-term home infusions are required each month (every 7–15 days);
immunoglobulins remain active in the bloodstream longer, maintaining a stable level in the blood serum;
pharmacokinetics are smooth, without the peak characteristic of intravenous administration.
Dosage regimens:
cumulative monthly dose: 2.4–4.8 mL/kg of body weight;
frequency of infusions: from once a day to once every 2 weeks;
option to use the rapid push method or a pump.
Advantages of Home Therapy with Subcutaneous Immunoglobulins
Home therapy with subcutaneous immunoglobulins for conditions involving antibody deficiency or requiring immunomodulation offers several advantages over standard intravenous therapy in a hospital setting:
1. No venous access is required.
2. There is no need for hospitalization for severely debilitated patients—this helps prevent exposure to potential infections in the hospital.
3. Infusions are administered at home using a portable infusion pump: this reduces the frequency of hospital visits and saves patients time and money.
4. Shorter infusion time: subcutaneous infusion takes 15–30 min, compared to 3–4 hours for intravenous administration.
5. Mobility during the procedure: the patient can move around and perform daily activities thanks to the infusion pump’s portable design.
6. Fewer and milder side effects: Local reactions following subcutaneous administration are generally minor and moderate compared to intravenous administration.
7. A more physiologically appropriate distribution of immunoglobulins in the body.
8. Reduced risk of systemic side effects (headache, fever, chills).
9.Better treatment adherence due to convenience and mobility.
10. The ability to train patients and family members to administer the medication themselves.
Economic aspects:
reduced hospitalization costs;
reduced need for antibiotics and antiviral medications;
fewer days of work disability.
Safety and Complications
Possible side effects:
local reactions (redness, itching, swelling at the injection site)—usually mild and resolve on their own;
systemic reactions (headache, myalgia, fever)—less common than with intravenous administration;
risk of allergic reactions (extremely rare with modern preparations).
Contraindications:
individual intolerance to the drug’s components;
history of severe anaphylactic reactions to immunoglobulins;
selective IgA deficiency with the presence of antibodies against IgA (special caution is required) .
Future Prospects
Development of new forms of immunoglobulins with improved pharmacokinetics;
Research on optimizing dosing regimens;
Expansion of indications for subcutaneous administration;
Implementation of telemedicine technologies for remote monitoring of patients receiving home therapy.
reduced hospitalization costs;
reduced need for antibiotics and antiviral medications;
fewer days of work disability.
Safety and Complications
Possible side effects:
local reactions (redness, itching, swelling at the injection site)—usually mild and resolve on their own;
systemic reactions (headache, myalgia, fever)—less common than with intravenous administration;
risk of allergic reactions (extremely rare with modern preparations).
Contraindications:
individual intolerance to the drug’s components;
history of severe anaphylactic reactions to immunoglobulins;
selective IgA deficiency with the presence of antibodies against IgA (special caution is required) .
Future Prospects
Development of new forms of immunoglobulins with improved pharmacokinetics;
Research on optimizing dosing regimens;
Expansion of indications for subcutaneous administration;
Implementation of telemedicine technologies for remote monitoring of patients receiving home therapy.
