Ig vena

Package leaflet: Information for the user

Ig VENA, 50 g/l, infusion solution
Normal human immunoglobulin for intravenous administration (IVIg)
Please read all of this leaflet carefully before using this medicine, because it contains important information for you.

• Keep this leaflet, as you may need to read it again.
• If you have any further questions, ask your doctor or nurse.
• If you experience any side effects, including any not listed in this leaflet, tell your doctor or nurse. See section 4.

Contents of this leaflet:

1. What Ig VENA is and what it is used for
2. Important information before using Ig VENA
3. How to use Ig VENA
4. Possible side effects
5. How to store Ig VENA
6. Contents of the pack and other information

1. What Ig VENA is and what it is used for

Ig VENA is a solution of normal human immunoglobulin for intravenous administration.
Immunoglobulins are human antibodies also present in blood.
Ig VENA is used in the following treatments:

Treatment of adults and children and adolescents (0–18 years) when the patient has insufficient levels of antibodies (replacement therapy) in the following cases:

1. In patients with congenital deficiency in antibody production (in primary immunodeficiency syndromes).
2. In patients with acquired deficiency in antibody production (secondary immunodeficiency), who experience severe or recurrent infections due to various clinical conditions (e.g. oncological or autoimmune diseases, or as a consequence of treatment for these diseases). Antibiotic treatment in these patients was ineffective, and either they did not achieve sufficient increase in IgG antibody titres after vaccination (pneumococcal polysaccharide vaccine and vaccine containing polypeptide antigen), or their serum IgG level was <4 g/l.

Treatment of adults and children and adolescents (0–18 years) with certain inflammatory diseases (immunomodulation) in the following cases:

1. In patients with low platelet count (immune thrombocytopenia) and in patients at high risk of bleeding or prior to surgery, in order to achieve an adequate platelet count.
2. In patients with Guillain–Barré syndrome. This is an acute disease characterized by inflammation of peripheral nerves causing severe muscle weakness, primarily in the legs and upper limbs.
3. In patients with Kawasaki disease (in combination with acetylsalicylic acid). Kawasaki disease is an acute condition primarily affecting young children, characterized by inflammation of blood vessels throughout the body.
4. In patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). This chronic disease is a rare peripheral nerve disorder characterized by gradually progressive weakness of the lower limbs and, to a lesser extent, the upper limbs.
5. In multifocal motor neuropathy (MMN). This is a rare disease that affects motor nerves and manifests as slowly progressive, asymmetric limb weakness without loss of sensation.

2. Important information before using Ig VENA

When not to use Ig VENA
Do not use this medicine if the patient is allergic (hypersensitive) to human normal immunoglobulin or to any of the other components of this medicine (listed in section 6).
Do not use if the patient has antibodies against immunoglobulin A (IgA) in the blood, because administration of a product containing IgA may lead to a severe allergic reaction.

Warnings and precautions
Before starting treatment with Ig VENA, discuss this with your doctor or nurse.
Patients must be closely monitored and carefully observed during infusion due to the risk of adverse reactions.

The following adverse reactions may occur more frequently:

• when the infusion rate is too high;
• in patients with symptoms of untreated infection (e.g. fever) or chronic inflammatory conditions;
• in patients receiving human normal immunoglobulin for the first time;
• in rare cases, when switching from a previously used human normal immunoglobulin product to another, or when a long time has passed since the last infusion.

In certain cases, immunoglobulins may increase the risk of heart attack, stroke, pulmonary artery thrombosis, or worsen deep vein thrombosis.
Therefore, the doctor will exercise particular caution in the following cases:

• in obese patients,
• in elderly patients,
• in patients with diabetes,
• in patients with hypertension,
• in patients with reduced blood volume (hypovolemia),
• in patients with vascular diseases,
• in patients at risk of developing blood clotting conditions (acquired or congenital coagulation disorders),
• in patients with a history of thrombotic events,
• in patients with conditions associated with increased blood viscosity,
• in patients who are immobilized for prolonged periods,
• in patients with current or past kidney disease, or those taking medications that may damage the kidneys (nephrotoxic drugs), as cases of acute kidney injury have been reported. In case of impaired kidney function, discontinuation of immunoglobulin administration should be considered.

The patient may have an allergy (hypersensitivity) to immunoglobulin (antibodies) without being aware of it.
Hypersensitivity may occur even in patients who have previously received human normal immunoglobulin and tolerated it well. It may occur particularly in cases of IgA immunoglobulin deficiency (in patients with anti-IgA antibodies). In these rare cases, allergic (hypersensitivity) reactions such as sudden drop in blood pressure or anaphylactic reaction may occur.

In case of an adverse reaction, either reduce the infusion rate or stop the administration of immunoglobulins. Treatment depends on the type and severity of the adverse reaction.
In case of shock, manage according to current medical standards for shock treatment. Inform your doctor if any of the above conditions apply to the patient. The doctor will take appropriate precautions when administering Ig VENA.

Prevention of viral infections
Medicinal products derived from human blood or plasma undergo specific procedures designed to prevent transmission of infectious agents to treated patients. These procedures include selection of blood and plasma donors to exclude those who may be sources of infection; testing of plasma for the presence of infectious agents/viruses. Manufacturers of blood- or plasma-derived medicinal products also apply processes that inactivate or remove viruses. Despite these preventive measures, the possibility of transmitting infectious agents through a medicine prepared from human blood or plasma cannot be completely excluded. This also applies to unknown or recently discovered viruses and other pathogens.

The preventive measures taken are considered effective against enveloped viruses such as human immunodeficiency virus (HIV), hepatitis B virus (HBV), hepatitis C virus (HCV), and against non-enveloped viruses such as hepatitis A virus (HAV).

These measures may have limited effectiveness against non-enveloped viruses such as parvovirus B19.

There have been no reports of immunoglobulins causing hepatitis A or parvovirus B19 infection, since the presence of antibodies may play an important protective role against viral infections.

It is recommended that each time Ig VENA is administered, the product name and batch number be recorded for traceability.

Children and adolescents
Transient and mild glucosuria (presence of glucose in urine) has been observed in children and adolescents after administration of Ig VENA, without clinical symptoms. This may be related to the maltose content in Ig VENA, which is hydrolyzed to glucose in the renal tubules. Glucose is reabsorbed and excreted in urine only to a very small extent. Glucose reabsorption depends on the patient's age. Transient increase in serum maltose concentration may exceed the renal threshold for sugar reabsorption and lead to a positive urine glucose test result.

Ig VENA and other medicines
Inform your doctor about all medicines currently or recently taken by the patient, as well as any medicines the patient plans to take.

Do not mix human normal immunoglobulin for intravenous administration with other medicines or with other intravenous immunoglobulin (IVIg) products.

Effect on live attenuated viral vaccines
Administration of immunoglobulin may reduce the effectiveness of vaccines containing live attenuated viruses, such as measles, mumps, rubella, or varicella, for a period of at least 6 weeks up to 3 months. After receiving this medicine, a 3-month interval should be maintained before vaccination with a live attenuated viral vaccine. For measles, this interference may last up to one year. Therefore, patients receiving measles vaccination should have their antibody titers measured.

Loop diuretics (medicines that increase urine flow)
Concomitant use with loop diuretics should be avoided.

Effect on blood test results
After injection of immunoglobulin, transient increase in passively transferred antibodies in the patient's blood may cause false-positive results in serological tests.

Passive transfer of antibodies against red blood cell antigens, e.g. A, B, D (responsible for blood group), may affect results of certain serological tests for red blood cell antibodies, such as the direct antiglobulin test (DAT, Coombs test).

Blood glucose testing
Some types of blood glucose tests (e.g. those based on glucose dehydrogenase-pyrolloquinoline quinone (GDH-PQQ) or glucose oxidoreductase-dye methods) falsely interpret maltose (100 mg/ml) contained in Ig VENA as glucose. This may lead to falsely elevated blood glucose readings during infusion and for approximately 15 hours after the end of infusion, potentially resulting in inappropriate insulin administration leading to life-threatening hypoglycemia. Additionally, actual cases of hypoglycemia may remain untreated if hypoglycemia is masked by falsely elevated glucose readings. Therefore, when administering Ig VENA or other parenteral medicines containing maltose, blood glucose concentration should be measured using a glucose-specific method. Carefully review information regarding blood glucose testing, including test strips, to determine whether they can be used with parenteral medicines containing maltose. In case of doubt, contact the device manufacturer to confirm compatibility with maltose-containing parenteral medicines.

Children and adolescents
Although interaction studies have not been conducted in children and adolescents, no differences are expected between adult and pediatric populations.

Pregnancy, breastfeeding and fertility

• If the patient is pregnant or breastfeeding, suspects she may be pregnant, or is planning to have a child, she should consult her doctor before using this medicine. The doctor will decide whether Ig VENA can be administered during pregnancy.
• Clinical studies with Ig VENA have not been conducted in pregnant women. It has been shown that intravenous immunoglobulin products cross the placenta, particularly during the third trimester. However, long-term clinical experience with immunoglobulin use suggests that no harmful effects on pregnancy, the fetus or the newborn are expected.
• If the patient is breastfeeding and receives Ig VENA, antibodies from the medicine may pass into human milk. This may contribute to protecting the newborn against certain infections.
• Clinical experience with immunoglobulin use suggests that no harmful effect on fertility is expected.

Driving and operating machinery
Some adverse reactions associated with Ig VENA may impair the ability to drive or operate machinery. Patients who experience adverse reactions during treatment should wait until symptoms resolve before driving or operating machinery.

Ig VENA contains maltose and sodium
This medicine contains 100 mg of maltose per 1 ml.
This medicine contains approximately 69 mg of sodium per litre. This should be taken into account in patients on a low-sodium diet.

3. How to use Ig Vena

Ig Vena must be administered only by a physician or trained medical personnel under hospital or outpatient conditions.
The dose and dosing regimen depend on the indication; the physician will determine the appropriate dosage suitable for the individual patient.
Ig Vena should initially be administered slowly. If the medicine is well tolerated, the infusion rate may be gradually increased.

Use in children and adolescents
Dosing in children and adolescents (0–18 years) does not differ from that used in adults, as dosing in the individual indications is based on body weight and the patient's clinical condition.

Taking more Ig Vena than recommended
Overdose may lead to circulatory overload and excessive blood viscosity, especially in patients at risk, elderly patients, or those with heart or kidney failure, including.
If you have any further questions about the use of this medicine, consult your doctor or nurse.

4. Possible adverse reactions

Like all medicines, this medicine can cause adverse reactions, although not everyone experiences them.

The following adverse reactions may occur after administration of a medicine containing immunoglobulins:

• Chills, headache, dizziness, fever, vomiting, nausea, allergic reactions, joint pain, hypotension, and mild lower back pain may occur sporadically;
• Isolated cases of transient decrease in red blood cells (reversible haemolytic anaemia/haemolysis);
• Sudden drop in blood pressure may occur rarely, and in isolated cases, anaphylactic shock may occur even in patients who did not experience hypersensitivity during previous administrations;
• Rare cases of transient skin reactions have been observed;
• Very rarely, thromboembolic complications (blood clot formation) have been reported, which may lead to myocardial infarction, stroke, pulmonary artery thrombosis (pulmonary embolism), or deep vein thrombosis;
• Cases of transient, non-infectious meningitis (reversible aseptic meningitis);
• Increased serum creatinine levels and/or acute kidney failure have been observed;
• Cases of acute transfusion-related lung injury (TRALI – Transfusion Related Acute Lung Injury).

In clinical trials and following the marketing of Ig Vena, the following adverse reactions have been observed, listed in decreasing order of frequency.

Common (may affect less than 1 in 10 people):

• Back pain
• Nausea
• Feeling of weakness, fatigue, fever
• Muscle pain
• Headache, drowsiness

Frequency not known (cannot be estimated from available data):

• Aseptic meningitis
• Haemolysis causing anaemia
• Allergic reactions and life-threatening anaphylactic shock
• Confusion
• Stroke, dizziness, uncontrolled tremors, tingling or numbness of the skin or limbs
• Myocardial infarction, skin cyanosis, rapid heartbeat, slow heartbeat, irregular heartbeat
• Blood clots in deep veins and blood vessels, low blood pressure, high blood pressure, pallor
• Pulmonary artery thrombosis, abnormal fluid accumulation in the lungs, breathing difficulties with wheezing or cough
• Vomiting, diarrhoea, abdominal pain
• Rapidly progressing skin swelling, urticaria, redness and inflammation of the skin, skin rash, pruritus, eczema, excessive sweating
• Joint and muscle pain, back pain, neck pain, skeletal muscle stiffness
• Sudden kidney failure
• Inflammation of the vein at the injection site, fever, chest pain or discomfort, facial swelling, general malaise
• Increased blood creatinine concentration

Additional adverse reactions in children and adolescents
The frequency, type, and severity of adverse reactions in children and adolescents are expected to be the same as in adults.

Following administration of Ig Vena, transient and mild glucosuria (presence of glucose in urine) has been observed in children and adolescents, without clinical symptoms.

For information on viral safety, see section 2. "Important information before using Ig Vena".

Reporting of adverse reactions
If any adverse symptoms occur, including any adverse reactions not listed in this leaflet, inform your doctor or nurse.

Adverse reactions can be reported to the Department of Monitoring Adverse Drug Reactions of the Office for Registration of Medicinal Products, Medical Devices and Biocidal Products (Al. Jerozolimskie 181C, 02-222 Warsaw, Poland), tel: +48 22 4921301, fax: +48 22 4921309, website: https://smz.ezdrowie.gov.pl

Adverse reactions can also be reported to the responsible entity.

5. How to store Ig VENA

The medicine should be stored out of sight and reach of children.
Do not use this medicine after the expiry date stated on the vial and outer carton after "EXP". The expiry date refers to the last day of the specified month.
Store in a refrigerator (2°C - 8°C).
Before use and during its shelf life, the medicine may be stored at room temperature, not exceeding 25°C, for a maximum of 6 consecutive months. After this period, the medicine must be discarded.
Under no circumstances should the medicine be returned to the refrigerator once it has been stored at room temperature. The initial date of room temperature storage must be recorded on the carton.
After opening the vial, the contents should be used immediately.
Store vials in the outer packaging. Do not freeze.
Do not use this medicine if the solution appears cloudy, has changed colour, or contains a visible precipitate.
Medicines must not be disposed of via wastewater or household waste. Ask your pharmacist how to dispose of medicines no longer in use. This will help protect the environment.

6. Contents of the packaging and other information

What Ig VENA contains
The active substance is human normal immunoglobulin.
1 ml of solution contains 50 mg of human normal immunoglobulin.
The solution contains 50 g/l human protein, of which at least 95% is IgG (immunoglobulin G).
The distribution of IgG subclasses is as follows:
IgG\ 62.1%
IgG\ 34.8%
IgG\ 2.5%
IgG\ 0.6%
Maximum IgA content is 50 micrograms/ml.
The medicine is manufactured from plasma of blood donors.
Other components of the medicine are maltose, water for injections.

What Ig VENA looks like and contents of the pack
IgVENA infusion solution is available in single vials of 50 ml, 100 ml or 200 ml with an attached handle (vial + handle). The solution is clear or slightly opalescent, colourless or pale yellow.

Pack sizes:
Single packs:
1 vial containing 2.5 g/50 ml
1 vial containing 5 g/100 ml
1 vial containing 10 g/200 ml

Multiple packs:
Multiple pack containing 2 single packs of 1 vial each, 10 g/200 ml
Multiple pack containing 3 single packs of 1 vial each, 10 g/200 ml

Not all pack sizes may be marketed.

Marketing Authorisation Holder:
Kedrion S.p.A.
Loc. Ai Conti, 55051 Castelvecchio Pascoli, Barga (Lucca), Italy

Manufacturer:
Kedrion S.p.A.
55027 Bolognana, Gallicano (Lucca), Italy

This medicinal product is authorised in the European Economic Area under the following names:

For more detailed information, please contact the local representative of the marketing authorization holder:
MB&S Medical Business and Science, ul. Chełmska 30/34, 00-725 Warsaw
Tel/fax. 22 851 52 08

Information intended exclusively for medical professionals:

Instructions for proper use

• Prior to use, Ig Vena should be brought to room temperature or body temperature.
• Before use, the solution should be visually inspected for the presence of particulate matter and discoloration. Do not use solutions that are cloudy or contain a precipitate.
• Human normal immunoglobulin should be administered intravenously at an initial infusion rate of 0.46–0.92 mL/kg/hour (10–20 drops per minute) for 20–30 minutes. If adverse reactions occur, reduce or stop the infusion. If well tolerated, the infusion rate may be gradually increased up to a maximum of 1.85 mL/kg/hour (40 drops per minute).
• In patients with primary immunodeficiency who tolerate an infusion rate of 0.92 mL/kg/hour, the rate may be gradually increased every 20–30 minutes to 2 mL/kg/hour, 4 mL/kg/hour, and up to a maximum of 6 mL/kg/hour, provided the patient continues to tolerate the infusion well. Generally, dosing and infusion rate must be individually adjusted to the patient's needs. Depending on the patient's body weight, dosage, and occurrence of adverse reactions, the maximum infusion rate may not be achieved. If adverse reactions occur, the infusion should be stopped immediately and then resumed at an appropriate rate for the patient.

Special populations
In children and adolescents (0–18 years) and elderly patients (>64 years), the initial infusion rate should be 0.46–0.92 mL/kg/hour (10–20 drops per minute) for 20–30 minutes. If well tolerated and considering the patient's clinical condition, the rate may be gradually increased up to a maximum of 1.85 mL/kg/hour (40 drops per minute).

Instructions for using the holder

1. Initial appearance of the vial with the holder label
2. Turn the vial upside down
3. Form the holder by unfolding it from the label
4. Hang the vial on the infusion stand

Precautions
Some serious adverse reactions may be related to the infusion rate. Potential complications can often be avoided by ensuring:

• Patients are not hypersensitive to human normal immunoglobulin, by initiating treatment with a slow infusion rate (0.46–0.92 mL/kg/hour);
• Patients are closely monitored during infusion for adverse reactions. Patients receiving human normal immunoglobulin for the first time, those previously treated with another IVIg product, or those with a long interval since the last infusion should be monitored during the first infusion and for at least one hour thereafter to detect potential adverse reactions. Other patients should be observed for at least 20 minutes after the infusion.

For all patients, intravenous Ig administration requires:

• Adequate hydration prior to starting the Ig infusion;
• Monitoring of urine output;
• Monitoring of serum creatinine levels;
• Avoidance of concomitant use of loop diuretics. In case of an adverse reaction, either reduce or stop the immunoglobulin infusion. Treatment depends on the type and severity of the adverse reaction. In case of shock, manage according to current medical standards for shock treatment.

Infusion reactions
Some adverse reactions (e.g., headache, fever, chills, muscle pain, wheezing, tachycardia, lower back pain, nausea, and hypotension) may be related to the infusion rate. The recommended infusion rate must be strictly followed. Patients must be closely monitored and carefully observed during infusion due to the risk of adverse reactions.
Some adverse reactions may occur more frequently:

• In patients receiving human normal immunoglobulin for the first time, or in rare cases when switching from one normal human immunoglobulin product to another, or after a long interval since the last infusion;
• In patients with untreated infection or chronic inflammatory conditions.

Children and adolescents
No special preventive measures or monitoring recommendations are required for children and adolescents. No differences are expected in children and adolescents (from 0 to 18 years).

Thromboembolic disease
Clinical evidence links intravenous Ig administration with thromboembolic events such as myocardial infarction, cerebrovascular events (including stroke), pulmonary embolism, and deep vein thrombosis, believed to be associated with relative increases in blood viscosity following intensive immunoglobulin administration in at-risk patients. Caution is advised when prescribing and administering the product to obese patients and to patients at risk of thrombotic events (such as advanced age, hypertension, diabetes, vascular diseases, personal or family history of thrombosis, acquired or congenital coagulation disorders, prolonged immobilization, severe hypovolemia, or conditions associated with increased blood viscosity).
In patients at risk of thromboembolic adverse reactions, intravenous immunoglobulins should be administered at the lowest possible infusion rate and at the smallest effective dose.

Acute renal failure
Cases of acute renal failure have been reported in patients treated with intravenous immunoglobulins. In most cases, risk factors were identified, such as pre-existing renal insufficiency, diabetes, severe reduction in circulating blood volume, obesity, concomitant use of nephrotoxic drugs, or age over 65 years. Renal function parameters should be assessed before IVIg infusion and re-evaluated at appropriate intervals, especially in patients at potentially increased risk of acute renal failure. In patients at risk of acute renal failure, intravenous immunoglobulins should be administered at the lowest possible infusion rate and at the smallest effective dose.
If renal function deteriorates, discontinuation of intravenous Ig administration should be considered. Reports of renal dysfunction and acute renal failure have involved multiple licensed intravenous immunoglobulin products containing various excipients such as sucrose, glucose, and maltose. Among reported cases, products containing sucrose as a stabilizer were disproportionately represented. In at-risk patients, consideration may be given to using intravenous immunoglobulin products that do not contain these excipients.

Aseptic meningitis syndrome (AMS)
Aseptic meningitis syndrome (AMS) has been reported during treatment with intravenous immunoglobulins. The syndrome typically begins within several hours to two days after IVIg administration.
Cerebrospinal fluid analysis often reveals pleocytosis of up to several thousand cells/mm³, predominantly granulocytes, and elevated protein levels up to several hundred mg/dL.
AMS may occur more frequently with high-dose IVIg treatment (2 g/kg).
Patients with subjective or objective symptoms should undergo thorough neurological evaluation, including cerebrospinal fluid analysis, to exclude other causes of meningitis.
Discontinuation of IVIg therapy leads to AMS remission within a few days without sequelae.

Hemolytic anemia
Intravenous immunoglobulin products may contain blood group antibodies that can act as hemolysins and induce in vivo coating of red blood cells with immunoglobulin, leading to a positive direct antiglobulin reaction (Coombs test) and, rarely, hemolysis.
Hemolytic anemia may develop during IVIg treatment due to enhanced sequestration of red blood cells. Patients receiving intravenous immunoglobulin should be monitored for possible clinical signs of hemolysis.

Neutropenia/leukopenia
Transient decreases in neutrophil count and/or episodes of neutropenia, sometimes severe, have been reported after IVIg treatment. These typically occur within hours or days after IVIg administration and resolve spontaneously within 7 to 14 days.

Transfusion-related acute lung injury (TRALI)
Cases of acute non-cardiogenic pulmonary edema (transfusion-related acute lung injury – TRALI) have been reported in patients receiving IVIg medicinal products. TRALI is characterized by severe hypoxia, respiratory failure, dyspnea, cyanosis, fever, and hypotension. TRALI symptoms usually appear within 6 hours after product administration, often within 1–2 hours. Therefore, patients should be monitored; if respiratory adverse reactions occur, IVIg infusion should be stopped immediately. TRALI may be life-threatening and requires immediate management in an intensive care setting.

This medicinal product contains 100 mg of maltose per 1 mL as an excipient. The presence of maltose in the blood may interfere with glucose testing, leading to falsely elevated blood glucose readings, which may result in inappropriate insulin administration causing life-threatening hypoglycemia and death. Additionally, actual hypoglycemia may remain untreated if the hypoglycemic state is masked by a falsely elevated glucose reading. For further information, see section "Blood glucose measurement".

Dosage
Replacement therapy must be initiated and monitored by a specialist experienced in the treatment of immunodeficiency.

Dosage
The dose and dosing schedule depend on the indication. The dose should be individually determined for each patient based on clinical response. Weight-based dosing may require adjustment in patients with underweight or overweight.
The following dosing regimens are provided as guidance.

Replacement therapy in primary immunodeficiency syndromes
The dose should be adjusted to achieve an IgG level (measured before the next infusion) of at least 6 g/L or within the normal range for the patient's age group. It may take three to six months from the start of treatment to achieve a stable IgG level. An initial dose of 0.4–0.8 g/kg is recommended, followed by at least 0.2 g/kg every three to four weeks. The monthly dose required to achieve a minimum IgG concentration of 6 g/L ranges from 0.2–0.8 g/kg/month. After achieving a stable state, intervals between infusions are typically 3–4 weeks. Immunoglobulin levels should be measured and evaluated in relation to the frequency of infections. To reduce the frequency of bacterial infections, dose increases may be necessary to achieve higher concentrations.

Secondary immunodeficiencies
The recommended dose is 0.2–0.4 g/kg every three to four weeks.
The minimum IgG level should be measured and evaluated in relation to the frequency of infections. The dose should be adjusted as needed to achieve adequate protection against infections. Dose increases may be necessary in patients with persistent infections; dose reduction may be considered when the patient is free of infection.

Immune thrombocytopenic purpura (ITP)
Two alternative treatment regimens:

• Dose of 0.8–1.0 g/kg on the first day; the dose may be repeated once within 3 days
• 0.4 g/kg/day for two to five days. Treatment may be repeated if disease relapse occurs.

Guillain–Barré syndrome
0.4 g/kg/day for more than 5 days (repeat dosing may be considered in case of relapse).

Kawasaki disease
Administer 2.0 g/kg as a single dose. Patients should also receive acetylsalicylic acid concurrently.

Chronic inflammatory demyelinating polyneuropathy (CIDP)
Initial dose: 2 g/kg over 2–5 consecutive days.
Maintenance dose: 1 g/kg over 1–2 consecutive days every 3 weeks.
Treatment efficacy should be evaluated after each cycle; if no efficacy is observed after 6 months, treatment should be discontinued.
If therapy is effective, the physician should decide on long-term treatment, considering the patient's response and reaction to maintenance therapy. Dosing and intervals may require adjustment depending on the individual disease course.

Multifocal motor neuropathy (MMN)
Initial dose: 2 g/kg over 2–5 consecutive days.
Maintenance dose: 1 g/kg every 2 to 4 weeks or 2 g/kg every 4 to 8 weeks.
Treatment efficacy should be evaluated after each cycle; if no efficacy is observed after 6 months, treatment should be discontinued.
If therapy is effective, the physician should decide on long-term treatment, considering the patient's response and reaction to maintenance therapy. Dosing and intervals may require adjustment depending on the individual disease course.

Recommended dosing is presented in the table below: