Materiały źródłowe

• #1 How I treat common variable immune deficiency

  https://pmc.ncbi.nlm.nih.gov/articles/PMC2904582/

  Common variable immunodeficiency is a rare immune deficiency, characterized by low levels of serum immunoglobulin G, A, and/or M with loss of antibody production. […] The primary treatment of CVID is replacement of antibody, achieved by either an intravenous or subcutaneous route of Ig, usually in doses of 400 to 600 mg/kg body weight per month. […] The goal of Ig therapy is to prevent infections; however, the target trough serum IgG to attain varies depending on the baseline level of IgG. […] Both intravenous and subcutaneous methods provide both safe and effective replacement strategies; convenience to the patient can best guide these choices. […] By definition, most patients with CVID have little or no serum IgA; although anti-IgA antibodies have been reported, these are quite rare, and from a pragmatic point of view, the determination of whether IgG anti-IgA is present is not clinically important.

• #1 Treatment and prognosis of common variable immunodeficiency – UpToDate

  https://www.uptodate.com/contents/treatment-and-prognosis-of-common-variable-immunodeficiency

  Treatment and prognosis of common variable immunodeficiency […] The cornerstone of therapy is immune globulin replacement, which has dramatically altered the clinical course of CVID by reducing the burden of recurrent infections and subsequent complications. Management also involves vigilant monitoring and appropriate treatment for associated problems, such as pulmonary damage, gastrointestinal, autoimmune, and granulomatous diseases, and malignancy. […] Immune globulin replacement therapy may be administered either intravenously or subcutaneously. A typical approach is to begin therapy with intravenous immune globulin (IVIG), although one can also start with subcutaneous immune globulin (SCIG), with or without an initial loading regimen. If the intravenous route is used to initiate therapy, the subcutaneous route may be substituted after two or more months on IVIG, if this is preferred.

• #1 Common Variable Immunodeficiency Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/1051103-treatment

  Common variable immunodeficiency (CVID) patients were divided into four distinct clusters correlating to perceived health, a potentially important factor in providing care. […] The mainstay of treatment for common variable immunodeficiency (CVID) is Ig replacement therapy. Although expensive, Ig replacement therapy stops the cycle of recurrent infections. […] Ig may be administered intravenously or subcutaneously. Solutions of 3-12% intravenous immunoglobulin (IVIG) can be used on a regular basis to maintain a trough level of 400-500 mg/dL in adults. A dose of 400-600 mg/kg every 2-4 weeks is usually required. In patients with structural lung damage, a trough level of 700-800 mg/dL is required. […] A solution of 16% subcutaneous injection of IV immunoglobulin (SCIG) is also an effective treatment in patients with poor intravenous access.

• #1 What Is Common Variable Immunodeficiency (CVID)?

  https://www.webmd.com/a-to-z-guides/what-is-common-variable-immunodeficiency-cvid

  Can You Treat Common Variable Immunodeficiency (CVID)? […] Theres no cure for CVID. But there are ways to manage the condition. […] Once youve been diagnosed with CVID, to prevent repeated infections, your doctor may give your immunoglobulin (IgG) replacement therapy. Its a blood-based treatment. Doctors take IgG proteins that are collected from healthy blood donors and give them to you to boost your antibody levels and immune function. […] The therapy is delivered as an IV directly into a vein through a needle once a month or as a shot under the skin (subcutaneously) once a week or every other week. […] To keep CVID under check, youll need IgG replacement therapy for the rest of your life. […] Most people have no issues with this form of therapy. But it may cause side effects such as headaches or allergic reactions. Talk to your doctor about what form of therapy works best for you. […] Your doctor may also prescribe antibiotics or antiviral medications to help you clear germs from repeated infections.

• #1 Common Variable Immunodeficiency Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/1051103-treatment

  Adverse reactions to Ig administration must be monitored during therapy. The most common reactions include backache, nausea, vomiting, chills, low-grade fever, myalgias, and fatigue. […] In most patients, CVID responds well to Ig therapy. The recurrence of infections, arthritic symptoms, and the severity and/or incidence of the autoimmune disease are reduced. […] Cyclosporin A has been successfully used in patients with CVID and lymphoid interstitial pneumonitis. […] Antimicrobial therapy should be initiated at the first sign of infection. […] Specific therapy is often necessary to target the organ system involved. […] Patients with CVID at risk of coronavirus disease 2019 (COVID-19) represent a challenge. […] In pregnant patients with CVID and lung disease, the pulmonary deficit is often exacerbated in the third trimester.

• #1 Common variable immunodeficiency – an update | Arthritis Research & Therapy | Full Text

  https://arthritis-research.biomedcentral.com/articles/10.1186/ar4032

  First line therapy in autoimmune cytopenias and lympho proliferation are steroids. In case of failure, immunosuppressive drugs, rituximab or splenectomy have been reported as options. […] The inflammatory and granulomatous lesions of the lungs, liver and intestine respond poorly to the immunoglobulin replacement therapy alone and therefore often require corticosteroids, eventually in combination with immunosuppressants (cyclosporin A, azathioprin and others). […] The life expectancy of CVID patients has considerably improved over the past 30 years, from initially 12 years to currently over 50 years. […] Thus, the development of better surrogate diagnostic markers for the presence and activity of these secondary complications as well as new therapeutic approaches are a major challenge for the coming years in the care of CVID patients.

• #1 Common variable immunodeficiency – Wikipedia

  https://en.wikipedia.org/wiki/Common_variable_immunodeficiency

  Treatment options are limited and usually include lifelong immunoglobulin replacement therapy. This therapy is thought to help reduce bacterial infections. This treatment alone is not wholly effective, and many people still experience other symptoms such as lung disease and noninfectious inflammatory symptoms. This treatment replenishes Ig subtypes that the person lacks, is given at frequent intervals for life, and is thought to help reduce bacterial infections and boost immune function. Before therapy begins, plasma donations are tested for known blood-borne pathogens, then pooled and processed to obtain concentrated IgG samples. Infusions can be administered in three different forms: intravenously (IVIg), subcutaneously (SCIg), and intramuscularly (IMIg). […] In addition to Ig replacement therapy, treatment may also involve immune suppressants to control autoimmune symptoms of the disease and high-dose steroids like corticosteroids. In some cases, antibiotics are used to fight chronic lung disease resulting from CVID. The outlook for people varies greatly depending on their level of lung and other organ damage prior to diagnosis and treatment.

• #1 Common Variable Immunodeficiency (CVID): Comprehensive Guide

  https://www.rupahealth.com/post/common-variable-immunodeficiency-cvid-comprehensive-guide

  CVID management aims to reduce infections, support immune function, and prevent complications. […] While there is no cure, treatments like immunoglobulin therapy and antibiotics help improve quality of life. […] Since people with CVID have low antibody levels, immunoglobulin replacement therapy (IgRT) is the primary treatment. […] This therapy provides the body with antibodies from healthy donors, helping reduce infection risk and support immune function. […] Some people with CVID take preventive (prophylactic) antibiotics to reduce the risk of recurrent infections. […] These medications help control bacterial infections before they become severe. […] Antibiotics can lower the chances of respiratory and sinus infections, reducing hospital visits and long-term lung damage. […] Doctors often prescribe azithromycin, amoxicillin, or trimethoprim-sulfamethoxazole for prophylactic use.

• #1 Clinical and immunological features of 44 common variable immunodeficiency patients: the experience of a single center in Turkey | Allergologia et Immunopathologia

  https://www.elsevier.es/en-revista-allergologia-et-immunopathologia-105-articulo-clinical-immunological-features-44-common-S030105462030032X

  Common variable immunodeficiency (CVID) is one of the most prevalent forms of primary immunodeficiency characterized by hypogammaglobinemia. […] The general treatment is immunoglobulin (Ig) replacement therapy, which reduces serious bacterial infections. […] All patients received immunoglobulin replacement therapy, and one patient died because of granulomatous lymphocytic interstitial lung disease (GLILD). […] All patients were given Ig replacement therapy at the time of diagnosis; 37 (84.1%) were treated with intravenous immunoglobulin (IVIG) and seven (15.9%) were treated subcutaneously. […] The Ig dose was adjusted according to the clinical status and immunoglobulin level of the patient. […] Anaphylactic reaction developed in four patients (9.1%) due to the IVIG treatment. […] Sixteen patients (36.4%) who suffered from severe recurrent pulmonary infections were treated with daily antibiotic prophylaxis (trimethoprim/sulfamethoxazole).

• #1 Common Variable Immunodeficiency (CVID): Comprehensive Guide

  https://www.rupahealth.com/post/common-variable-immunodeficiency-cvid-comprehensive-guide

  Prolonged antibiotic use requires careful monitoring to avoid resistance and side effects like gut imbalances or liver strain. […] Research is ongoing to find new treatments for CVID. […] Gene therapy, immune-modulating drugs, and biologics are being explored as potential options to enhance immune function and reduce autoimmune complications. […] While immunoglobulin therapy remains the standard treatment, researchers are exploring novel therapies to improve immune function.

• #1 Common Variable Immunodeficiency (CVID) – Immune Disorders – Merck Manual Consumer Version

  https://www.merckmanuals.com/home/immune-disorders/immunodeficiency-disorders/common-variable-immunodeficiency-cvid

  Immune globulin is given throughout life to provide the missing immunoglobulins, and antibiotics are given to treat the frequent infections. […] Immune globulin (antibodies obtained from the blood of people with a normal immune system) is given throughout life to provide the missing immunoglobulins. It may be injected into a vein (intravenously) once a month or under the skin (subcutaneously) once a week or once a month. […] Antibiotics are promptly given to treat infections. Sometimes antibiotics are taken regularly to prevent infections. […] Autoimmune disorders are treated as needed with medications (such as rituximab, etanercept, infliximab, or corticosteroids) that suppress or otherwise modify the immune system’s activity.

• #1 How I treat common variable immune deficiency

  https://pmc.ncbi.nlm.nih.gov/articles/PMC2904582/

  More recently, we have used rituximab in standard doses, for more refractory or recurrent ITP and/or AIHA with success in 11 patients with CVID. […] The management of inflammatory bowel disease in CVID is the same as for immunocompetent patients, including antibiotics, such as metronidazole or tinidizole or ciprofloxacin, 5-aminosalicylic acid and/or nonabsorbed oral steroids, such as budesonide. […] The incidence of malignancy appears overall increased in CVID, occurring in up to 15% of subjects. […] During the past 3 decades, the outlook for patients with CVID has greatly improved because of standard Ig replacement therapy and more effective antibiotic coverage.

• #1 How I treat common variable immune deficiency

  https://pmc.ncbi.nlm.nih.gov/articles/PMC2904582/

  The commonest clinical history in CVID includes frequent infections in most but not all subjects. […] Although pneumonia is clearly much less common after adequate Ig replacement is initiated, continued respiratory tract disease even after treatment is instituted can lead to obstructive, restrictive, bronchiectatic changes in some cases. […] Greater doses of intravenous immunoglobulin have been found in one instance to aid in controlling lymphoid interstitial disease and granuloma, but this does not appear to be a universal experience. […] For long-term therapy, I prescribe 200 to 400 mg a day (range, 3.5-6.5 mg/kg) of hydroxychloroquine on the basis of its mechanistic roles in reducing Toll-like receptor responses, antigen presentation, and its use in autoimmunity and sarcoidosis. […] Greater doses of Ig (600 mg/kg/month) may help to prevent infections and possibly chronic lung disease, but no controlled trials have been conducted to select which patients would benefit and what doses of Ig would be needed.

• #1 Common Variable Immunodeficiency in Children | UMass Memorial Health

  https://www.ummhealth.org/health-library/common-variable-immunodeficiency-in-children

  Treatment will depend on your childs symptoms, age, and general health. It will also depend on how severe the condition is. […] Treatment includes immunoglobulin replacement therapy, medicines, routine blood tests, and postural drainage of the lungs. […] Immunoglobulin replacement therapy. IV or subcutaneous infusions of immunoglobulin (antibodies) may be given to help boost the childs immune system and replace the immunoglobulins that are needed. […] Antibiotics to treat and prevent infection as prescribed by your childs healthcare provider. […] Postural drainage of the lungs. These are special exercises or treatments for the lungs that are used in cases where frequent infections have led to scarring in the lung. Postural drainage may help with lung infections and removing secretions. […] The current therapies can reduce the number and severity of infections. It’s important to help your child stay out of situations that have a greater risk for infection. Stay away from others who are sick. Have your child wash their hands often with soap and water. […] Treatment may include:

• #1 Common Variable Immunodeficiency (CVID) | Children’s Hospital of Philadelphia

  https://www.chop.edu/conditions-diseases/common-variable-immunodeficiency-cvid-0

  The goal of treatment for CVID is to control the symptoms and improve the quality of your childs life. Most children with CVID will need aggressive treatment to combat infections. In many cases, your childs doctor will prescribe antibiotics to treat or prevent infections. […] Doctors may also recommend a treatment called immunoglobulin replacement therapy to give your childs body the tools it needs to fight off infections better. In immunoglobulin (Ig) replacement therapy, some of the plasma in your childs blood is removed then replaced with antibody-rich plasma. The new plasma serves as a jump start for your childs ailing immune system and begins attacking any viruses or bacteria found in your childs body. Ig replacement therapy can be delivered by IV (intravenously) or through the skin (subcutaneously). […] When CVID is causing severe health problems, treatment may include an allogeneic bone marrow transplant or a cord blood transplant. These procedures involve using bone marrow or cord blood from a healthy individual and transplanting it into the affected patient to help rebuild their immune system.

• #1 CVID (Common Variable Immunodeficiency) | UPMC Children’s

  https://www.chp.edu/our-services/rare-disease-therapy/conditions-we-treat/cvid-syndrome

  The goal of treating CVID is to control the symptoms of this disease and improve your child’s quality of life. […] Kids with CVID may need aggressive treatment for their infections. Sometimes, doctors prescribe antibiotics to prevent, instead of treat, infections. […] Kids may have therapy to give them more antibodies, called immunoglobulin replacement therapy, on an ongoing basis. This therapy treats the body’s lack of antibodies but doesn’t cure the disease. […] If CVID is causing severe health problems, a bone marrow or cord blood transplant may help rebuild the immune system. […] Kids with lung disease or lung failure may even need a lung transplant. […] Transplants are high-risk treatments. They also mean your child may need to remain on medicine for their whole life. […] Dr. Szabolcs is a pioneer in „reduced-intensity” pediatric transplants for rare immune disorders. He also developed surgery to replace the lungs and bone marrow using tissue from the same donor to treat CVID. This replaces both damaged lungs and the immune system. […] Members of your child’s CVID care team will talk with you about: Likely next steps for your child. CVID treatment options. Ways to help your child’s quality of life at home. […] By the end of your visit, you’ll have a CVID care plan tailored to your child’s needs.

• #1 The Scope and Impact of Viral Infections in Common Variable Immunodeficiency (CVID) and CVID-like Disorders: A Literature Review

  https://www.mdpi.com/2077-0383/13/6/1717

  With regards to supporting humoral immunity, studies of commercial immunoglobulin products have demonstrated substantial levels of anti-SARS-CoV-2 IgG present, sufficient to match values found in convalescent and vaccinated individuals once administered. […] This latter study also demonstrated viral clearance following initiation of IgRT after a median of 20 days in antibody-deficient patients with pre-existing prolonged COVID-19. […] The antivirals Paxlovid (nirmatrelvir/ritonavir), remdesivir, and molnupiravir continue to be recommended, having significantly reduced hospitalisation and death with early variants and showing benefits in secondary endpoints such as time to recovery with the Omicron variant. […] The study of CVID and its relationship with viral infections underscores the critical importance of the intricate interplay between the immune system and pathogens. […] Increasingly sophisticated genetic and immunological analytical methods will likely lead to the identification of further monogenic causes of CVID, offering insight into other viral susceptibilities and the greater scope for classification, risk stratification, and targeted therapeutic approaches.

• #1 Common Variable Immunodeficiency in Children | Cedars-Sinai

  https://www.cedars-sinai.org/health-library/diseases-and-conditions—pediatrics/c/common-variable-immunodeficiency-in-children-cvid.html

  Treatment will depend on your childs symptoms, age, and general health. It will also depend on how severe the condition is. […] Treatment includes immunoglobulin replacement therapy, medicines, routine blood tests, and postural drainage of the lungs. […] Immunoglobulin replacement therapy. IV or subcutaneous infusions of immunoglobulin (antibodies) may be given to help boost the childs immune system and replace the immunoglobulins that are needed. […] Antibiotics to treat and prevent infection as prescribed by your childs healthcare provider. […] Postural drainage of the lungs. These are special exercises or treatments for the lungs that are used in cases where frequent infections have led to scarring in the lung. Postural drainage may help with lung infections and removing secretions. […] The current therapies can reduce the number and severity of infections. It’s important to help your child stay out of situations that have a greater risk for infection.

• #1 Common Variable Immunodeficiency in Children

  https://www.nationwidechildrens.org/conditions/health-library/common-variable-immunodeficiency-in-children

  The current therapies can reduce the number and severity of infections. It’s important to help your child stay out of situations that have a greater risk for infection. […] It is also important to help your child stay out of situations that have a greater risk for infection and wash their hands often with soap and water.

• #1 Common variable immunodeficiency – Care at Mayo Clinic – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/common-variable-immunodeficiency/care-at-mayo-clinic/mac-20355824

  Doctors trained in immune conditions and allergic diseases in the Primary Immunodeficiency Center and other areas care for people with common variable immunodeficiency at Mayo Clinic. […] Treatments for CVID focus on decreasing how often you get infections and how bad they are. Treatments may include immunoglobulin and antibiotic therapies. […] Highly skilled pediatric experts diagnose and treat all types of conditions in children. As a team, we work together to find answers, set goals and develop a treatment plan tailored to your child’s needs.

• #1

  https://link.springer.com/article/10.1007/s10875-017-0404-8

  Importantly, patient-specific features related to treatment route selection may influence subsequent QOL more than the route itself. […] These findings suggest that the method of Ig replacement (intravenous or subcutaneous) is not as important as the burden of disease in its impact upon QOL among patients with CVID. […] We suggest that improved QOL relies on early detection of disease and implementation of an individual treatment plan for the patient.

• #1 Common Variable Immunodeficiency Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/1051103-treatment

  Inpatient care may be necessary, depending on the severity of the clinical manifestations secondary to CVID. […] Surgery is required to treat the complications of common variable immunodeficiency (CVID). […] The mainstay of outpatient care is the prevention of secondary medical conditions. IVIG should be administered every 2-4 weeks to keep the level of serum antibodies in the reference range. […] Although long-term intravenous access is often required for IVIG therapy, it is not recommended. […] Patients should see their physicians annually, unless they develop associated infections, which warrant immediate treatment.

• #1 Common variable immune deficiency disorders – Immunodeficiency UKAccessibilityIncrease TextDecrease TextGrayscaleHigh ContrastNegative ContrastLight BackgroundLinks UnderlineReadable FontReset

  https://www.immunodeficiencyuk.org/common-variable-immune-deficiency-disorders/

  As much more is being learned about the disease processes in CVIDs, finding better targeted treatments is an active research area for many immunology centres. […] Not all vaccines are safe to be administered to patients with a CVID and therefore you should discuss any recommended or required vaccinations with your clinical immunology team before receiving a vaccine.

• #1 Get Immunodeficiency Treatment | Cleveland Clinic

  https://my.clevelandclinic.org/services/immunodeficiency-treatment

  Most people with an immunodeficiency can live full, productive lives. But the disorder can also create emotional challenges. Your long-term care plan will also help you cope with the daily ups and downs, like stress and anxiety. […] With the right treatment and maintenance plan, you don’t always have to feel sick and tired. Cleveland Clinic providers are here to help you get control of your immune system once again.

• #1 Common Variable Immunodeficiency (CVID): Cause & Treatment

  https://my.clevelandclinic.org/health/diseases/21143-common-variable-immunodeficiency-cvid

  CVID can be managed with replacement immunoglobulin therapy (RIgG). This provides your body with antibodies that it cant make on its own. This can either be: […] Replacement therapy isnt a cure for CVID you’ll need to be on this treatment for the rest of your life. Your provider may also give you antibiotics to prevent bacterial infections or to treat them at the first sign of infection. […] Theres no cure for CVID. But immunoglobulin replacement treatments (IVIg and SCIg) have increased survival rates in the past few decades. They’ll reduce your risk of getting a life-threatening infection. […] People with CVID are now living longer lives decades after their diagnosis thanks to the use of immunoglobulin replacement therapy.

• #1 Immunoglobulin Replacement Therapy is critical and cost-effective in increasing life expectancy and quality of life in patients suffering from Common Variable Immunodeficiency Disorders (CVID): A health-economic assessment | PLOS One

  https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0247941

  Immunoglobulin replacement therapy (IgGRT) is critical and cost-effective in increasing life expectancy and quality of life in patients suffering from Common Variable Immunodeficiency Disorders (CVID): A health-economic assessment. […] The clinical benefit of immunoglobulin replacement therapy (IgGRT) is substantial: timely treatment with appropriate doses significantly reduces mortality and the incidence of CVID-complications such as major infections and bronchiectasis. […] Immunoglobulin replacement therapy (IgGRT) is the standard of care in CVID. […] IgGRT is highly effective in reducing the mortality rate and the incidence of major infections in CVID-patients. […] Early-initiated IgGRT may improve patients health and quality of life and may also reduce overall healthcare and societal costs by avoiding or reducing the incidence of longer-term complications related to primary immunodeficiency (PID).

• #1 Immunoglobulin Replacement Therapy is critical and cost-effective in increasing life expectancy and quality of life in patients suffering from Common Variable Immunodeficiency Disorders (CVID): A health-economic assessment | PLOS One

  https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0247941

  The health-economic model suggests that early initiation of IgGRT compared to no or delayed IgGRT is highly cost-effective. […] CVID-patients access to IgGRT should be facilitated, not only because of proven clinical efficacy, but also due to the now demonstrated cost-effectiveness. […] The results demonstrate that the cost of IgGRT is associated with an incremental clinical benefit including 17 extra LYs and 11 extra QALYs. […] Treating CVID-patients promptly was also very cost-effective in the model: an average benefit of 6 LYs and 4 QALYs, resulting in an ICER of 30,374/LY and 47,495/QALY for a 4-year reduction in diagnostic delay. […] These cost-effectiveness and cost-utility results are far below the WHO-thresholds of efficiency.

• #1 Common variable immunodeficiency – an update | Arthritis Research & Therapy | Full Text

  https://arthritis-research.biomedcentral.com/articles/10.1186/ar4032

  Current therapy of CVID can be categorized as follows: regular and sufficient substitution with immunoglobulins (IgG trough levels 7.0 g/L); targeted antibiotic treatment of (breakthrough) infections; adequate treatment of complications; and in selected patients with severe hematological changes (chronic transfusion need, leukopenia, thrombocytopenia), secondary malignancies and suspected combined immunodeficiency, allogeneic peripheral stem cell transplantation is being considered in experienced centers. […] The immunoglobulin replacement therapy is the mainstay of therapy; 90% of CVID patients are on either intravenous (IVIg) or subcutaneous (SCIg) treatment. […] The current standard dosage when administered intravenously is 400 to 600 mg/kg every 3 to 4 weeks. For subcutaneous administration, this corresponds to 100 to 150 mg/kg per week.

• #2 Common variable immunodeficiency – an update | Arthritis Research & Therapy | Full Text

  https://arthritis-research.biomedcentral.com/articles/10.1186/ar4032

  Current therapy of CVID can be categorized as follows: regular and sufficient substitution with immunoglobulins (IgG trough levels 7.0 g/L); targeted antibiotic treatment of (breakthrough) infections; adequate treatment of complications; and in selected patients with severe hematological changes (chronic transfusion need, leukopenia, thrombocytopenia), secondary malignancies and suspected combined immunodeficiency, allogeneic peripheral stem cell transplantation is being considered in experienced centers. […] The immunoglobulin replacement therapy is the mainstay of therapy; 90% of CVID patients are on either intravenous (IVIg) or subcutaneous (SCIg) treatment. […] The current standard dosage when administered intravenously is 400 to 600 mg/kg every 3 to 4 weeks. For subcutaneous administration, this corresponds to 100 to 150 mg/kg per week.

• #2 Common Variable Immunodeficiency (CVID): Cause & Treatment

  https://my.clevelandclinic.org/health/diseases/21143-common-variable-immunodeficiency-cvid

  CVID can be managed with replacement immunoglobulin therapy (RIgG). This provides your body with antibodies that it cant make on its own. This can either be: […] Replacement therapy isnt a cure for CVID you’ll need to be on this treatment for the rest of your life. Your provider may also give you antibiotics to prevent bacterial infections or to treat them at the first sign of infection. […] Theres no cure for CVID. But immunoglobulin replacement treatments (IVIg and SCIg) have increased survival rates in the past few decades. They’ll reduce your risk of getting a life-threatening infection. […] People with CVID are now living longer lives decades after their diagnosis thanks to the use of immunoglobulin replacement therapy.

• #2 Common Variable Immunodeficiency Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/1051103-treatment

  Common variable immunodeficiency (CVID) patients were divided into four distinct clusters correlating to perceived health, a potentially important factor in providing care. […] The mainstay of treatment for common variable immunodeficiency (CVID) is Ig replacement therapy. Although expensive, Ig replacement therapy stops the cycle of recurrent infections. […] Ig may be administered intravenously or subcutaneously. Solutions of 3-12% intravenous immunoglobulin (IVIG) can be used on a regular basis to maintain a trough level of 400-500 mg/dL in adults. A dose of 400-600 mg/kg every 2-4 weeks is usually required. In patients with structural lung damage, a trough level of 700-800 mg/dL is required. […] A solution of 16% subcutaneous injection of IV immunoglobulin (SCIG) is also an effective treatment in patients with poor intravenous access.

• #2 Common Variable Immunodeficiency – The Rheumatologist

  https://www.the-rheumatologist.org/article/common-variable-immunodeficiency/?singlepage=1

  The primary objective of CVID therapy is reducing the frequency and duration of recurrent and chronic infections, especially sinusitis, bronchitis, otitis, pneumonia, and gastrointestinal infections and their sequelae. Immunoglobulin replacement therapy is the treatment of choice and forms the foundation of therapy. […] The standard dosing recommendation for intravenous immunoglobulin (IVIG) is 400 mg per kilogram body weight every three to four weeks. Immunoglobulins may also be administered subcutaneously in weekly intervals. An IgG trough level (the IgG level before the next infusion) of at least 5 g/L should be attained. The individual patients clinical history and outcome will determine the amount of IVIG required; some patients (e.g., those with bronchiectasis, diarrhea, or IgG hypercatabolism) may require higher IVIG doses to reach the mandatory trough level. To maximize treatment success, the dose, frequency, way of administration, and product type should be adapted to each individual needs.

• #2 Immunoglobulin Replacement Therapy is critical and cost-effective in increasing life expectancy and quality of life in patients suffering from Common Variable Immunodeficiency Disorders (CVID): A health-economic assessment | PLOS One

  https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0247941

  Immunoglobulin replacement therapy (IgGRT) is critical and cost-effective in increasing life expectancy and quality of life in patients suffering from Common Variable Immunodeficiency Disorders (CVID): A health-economic assessment. […] The clinical benefit of immunoglobulin replacement therapy (IgGRT) is substantial: timely treatment with appropriate doses significantly reduces mortality and the incidence of CVID-complications such as major infections and bronchiectasis. […] Immunoglobulin replacement therapy (IgGRT) is the standard of care in CVID. […] IgGRT is highly effective in reducing the mortality rate and the incidence of major infections in CVID-patients. […] Early-initiated IgGRT may improve patients health and quality of life and may also reduce overall healthcare and societal costs by avoiding or reducing the incidence of longer-term complications related to primary immunodeficiency (PID).

• #2 Common Variable Immunodeficiency Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/1051103-treatment

  Adverse reactions to Ig administration must be monitored during therapy. The most common reactions include backache, nausea, vomiting, chills, low-grade fever, myalgias, and fatigue. […] In most patients, CVID responds well to Ig therapy. The recurrence of infections, arthritic symptoms, and the severity and/or incidence of the autoimmune disease are reduced. […] Cyclosporin A has been successfully used in patients with CVID and lymphoid interstitial pneumonitis. […] Antimicrobial therapy should be initiated at the first sign of infection. […] Specific therapy is often necessary to target the organ system involved. […] Patients with CVID at risk of coronavirus disease 2019 (COVID-19) represent a challenge. […] In pregnant patients with CVID and lung disease, the pulmonary deficit is often exacerbated in the third trimester.

• #2 The Scope and Impact of Viral Infections in Common Variable Immunodeficiency (CVID) and CVID-like Disorders: A Literature Review

  https://www.mdpi.com/2077-0383/13/6/1717

  This review explores the clinical manifestations, outcomes, and potential therapeutic approaches for COVID-19 in CVID patients. […] It assesses the efficacy of prophylactic measures for COVID-19, including vaccination and immunoglobulin replacement therapy, as well as trialled therapies. […] Infection burden represents a major issue for patients with CVID, even once treated with either prophylactic antibiotics or immunoglobulin replacement therapy (IgRT). […] It is well understood that IgRT, the mainstay of CVID treatment, reduces the rate of respiratory infections in patients with CVID, whilst it has been specifically demonstrated to prevent the rate of RSV infections in immunocompromised patients. […] Thus, in CVID patients with persisting infections despite antibiotics, optimisation of immunoglobulin therapy, followed by viral screens for consideration of targeted antivirals seem the most rational approaches.

• #2 Common Variable Immunodeficiency (CVID) | NIAID: National Institute of Allergy and Infectious Diseases

  https://www.niaid.nih.gov/diseases-conditions/common-variable-immunodeficiency-cvid

  CVID is treated with intravenous immunoglobulin infusions or subcutaneous (under the skin) immunoglobulin injection to partially restore immunoglobulin levels. The immunoglobulin given by either method provides antibodies from the blood of healthy donors. The frequent bacterial infections experienced by people with CVID are treated with antibiotics. Other problems caused by CVID may require additional, tailored treatments.

• #2 Clinical and immunological features of 44 common variable immunodeficiency patients: the experience of a single center in Turkey | Allergologia et Immunopathologia

  https://www.elsevier.es/en-revista-allergologia-et-immunopathologia-105-articulo-clinical-immunological-features-44-common-S030105462030032X

  Common variable immunodeficiency (CVID) is one of the most prevalent forms of primary immunodeficiency characterized by hypogammaglobinemia. […] The general treatment is immunoglobulin (Ig) replacement therapy, which reduces serious bacterial infections. […] All patients received immunoglobulin replacement therapy, and one patient died because of granulomatous lymphocytic interstitial lung disease (GLILD). […] All patients were given Ig replacement therapy at the time of diagnosis; 37 (84.1%) were treated with intravenous immunoglobulin (IVIG) and seven (15.9%) were treated subcutaneously. […] The Ig dose was adjusted according to the clinical status and immunoglobulin level of the patient. […] Anaphylactic reaction developed in four patients (9.1%) due to the IVIG treatment. […] Sixteen patients (36.4%) who suffered from severe recurrent pulmonary infections were treated with daily antibiotic prophylaxis (trimethoprim/sulfamethoxazole).

• #2 Common variable immunodeficiency – an update | Arthritis Research & Therapy | Full Text

  https://arthritis-research.biomedcentral.com/articles/10.1186/ar4032

  First line therapy in autoimmune cytopenias and lympho proliferation are steroids. In case of failure, immunosuppressive drugs, rituximab or splenectomy have been reported as options. […] The inflammatory and granulomatous lesions of the lungs, liver and intestine respond poorly to the immunoglobulin replacement therapy alone and therefore often require corticosteroids, eventually in combination with immunosuppressants (cyclosporin A, azathioprin and others). […] The life expectancy of CVID patients has considerably improved over the past 30 years, from initially 12 years to currently over 50 years. […] Thus, the development of better surrogate diagnostic markers for the presence and activity of these secondary complications as well as new therapeutic approaches are a major challenge for the coming years in the care of CVID patients.

• #2 Common Variable Immunodeficiency: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/1051103-overview

  The mainstay of treatment for CVID is immunoglobulin replacement therapy, which reduces arthritic symptoms, infection recurrence, and the severity and/or incidence of the autoimmune disease. Immunoglobulin may be administered intravenously or subcutaneously. […] Surgery is required to treat complications of CVID, such as the following: Chronic sinusitis – May require endoscopic sinus surgery; Severe autoimmune thrombocytopenia or hemolytic anemia – Can be treated with splenectomy.

• #2 Rituximab for the Treatment of Common Variable Immunodeficiency (CVID) with Pulmonary and Central Nervous System Involvement

  https://openrheumatologyjournal.com/VOLUME/15/PAGE/9/FULLTEXT/

  Rituximab might be considered as an effective and relatively safe treatment for CVID-patients with GLILD. […] In this case series, we provide our experiences in treating GLILD-patients with a particular focus on the use of rituximab. […] Due to lack of response or intolerance of azathioprine, patients then received rituximab in a dose of 2 x 1000mg abs IV, separated by two weeks. […] All three patients CVID was defined as B+smB-CD21normTrnorm. […] Our experience contributes to the already existing knowledge of the management of GLILD and the effective use of rituximab in this condition. […] Rituximab is an effective treatment for this condition in CVID due to which this subgroup of patients suffering from both, GLILD and autoimmune cytopenia, might benefit twice from B cell depletion.

• #2 Clinical trial explores biologic treatment for CVID lung condition | Immune Deficiency Foundation

  https://primaryimmune.org/resources/news-articles/clinical-trial-explores-biologic-treatment-cvid-lung-condition

  A potentially life-threatening lung condition associated with common variable immunodeficiency (CVID) is the focus of a clinical trial to determine if a biologic medication already approved for other uses will benefit patients. […] Researchers are exploring whether a drug called abatacept will reduce granulomas in the lungs caused by GLILD. Abatacept works by reducing the overactivation of T cells. […] Standard of care therapy for CVID patients with GLILD is lacking. The first line of defense is immunoglobulin (Ig) replacement therapy to prevent GLILD and infections. […] Providers typically start treatment with steroids, but that treatment can be ineffective. Other treatments include various biologic medicines designed to reduce inflammation. […] If approved, abatacept would be administered regularly as a therapeutic instead of a one-time treatment.

• #2 Successful treatment of enteropathy associated with common variable immunodeficiency

  https://www.kjim.org/journal/view.php?number=169275

  Successful treatment of enteropathy associated with common variable immunodeficiency […] The mainstay of treatment for CVID is replacement of immunoglobulins and control of infectious disease. However, the management of CVID enteropathy has been unsatisfactory. […] Some studies have shown that corticosteroids improve diarrhea in these patients. […] Combination therapy with steroids and immunomodulators such as azathioprine can be used, but the efficacy and tolerability of such combination therapy are not well documented. […] She began to receive therapy with intravenous immunoglobulin (400 mg/kg every 4 weeks). However, the diarrhea and bloating continued. A trial with corticosteroids (prednisone 10 mg/day) and azathioprine (75 mg/day) was attempted. With these therapies, the frequency of diarrhea decreased and the patient gradually regained weight. […] In summary, we have presented herein a case with CVID associated with enteropathy.

• #2 Common Variable Immunodeficiency in Children | UMass Memorial Health

  https://www.ummhealth.org/health-library/common-variable-immunodeficiency-in-children

  Treatment will depend on your childs symptoms, age, and general health. It will also depend on how severe the condition is. […] Treatment includes immunoglobulin replacement therapy, medicines, routine blood tests, and postural drainage of the lungs. […] Immunoglobulin replacement therapy. IV or subcutaneous infusions of immunoglobulin (antibodies) may be given to help boost the childs immune system and replace the immunoglobulins that are needed. […] Antibiotics to treat and prevent infection as prescribed by your childs healthcare provider. […] Postural drainage of the lungs. These are special exercises or treatments for the lungs that are used in cases where frequent infections have led to scarring in the lung. Postural drainage may help with lung infections and removing secretions. […] The current therapies can reduce the number and severity of infections. It’s important to help your child stay out of situations that have a greater risk for infection. Stay away from others who are sick. Have your child wash their hands often with soap and water. […] Treatment may include:

• #2 CVID (Common Variable Immunodeficiency) | UPMC Children’s

  https://www.chp.edu/our-services/rare-disease-therapy/conditions-we-treat/cvid-syndrome

  The goal of treating CVID is to control the symptoms of this disease and improve your child’s quality of life. […] Kids with CVID may need aggressive treatment for their infections. Sometimes, doctors prescribe antibiotics to prevent, instead of treat, infections. […] Kids may have therapy to give them more antibodies, called immunoglobulin replacement therapy, on an ongoing basis. This therapy treats the body’s lack of antibodies but doesn’t cure the disease. […] If CVID is causing severe health problems, a bone marrow or cord blood transplant may help rebuild the immune system. […] Kids with lung disease or lung failure may even need a lung transplant. […] Transplants are high-risk treatments. They also mean your child may need to remain on medicine for their whole life. […] Dr. Szabolcs is a pioneer in „reduced-intensity” pediatric transplants for rare immune disorders. He also developed surgery to replace the lungs and bone marrow using tissue from the same donor to treat CVID. This replaces both damaged lungs and the immune system. […] Members of your child’s CVID care team will talk with you about: Likely next steps for your child. CVID treatment options. Ways to help your child’s quality of life at home. […] By the end of your visit, you’ll have a CVID care plan tailored to your child’s needs.

• #2 Personalized therapy for common variable immunodeficiency: Ingenta Connect

  https://www.ingentaconnect.com/content/ocean/aap/2020/00000041/00000001/art00007

  Common variable immunodeficiency (CVID) represents a clinical descriptive diagnosis that was defined in the 1970s. […] These advances, along with developments in immune modulatory and reconstitution therapies, now permit sophisticated and specific targeting of therapies for individual patients. […] For 50 years, immune globulin therapy has been applied to patients with CVID. There are several options open to patients, including a diversity of products and modes of administration. […] Stem cell therapy is increasingly applicable in patients with severe immune dysregulation. […] In the near future, it is likely that further advances in understanding the pathophysiology of CVID, together with ongoing development of biologics and small-molecule immune modulators will lead to additional targeted therapies for these patients.

• #2 Pharma can lead the way toward cures for common variable immunodeficiency – The Pharmaceutical Journal

  https://pharmaceutical-journal.com/article/letters/pharma-can-lead-the-way-toward-cures-for-common-variable-immunodeficiency

  In November 2017, the National Institute for Health and Care Excellence (NICE) recommended Strimvelis (GlaxoSmithKline), a gene therapy for a form of severe combined immunodeficiency (SCID). […] However, manufacturers of immunoglobulin replacement therapy (IVIG) for less acute common variable immunodeficiency (CVID) should take note. […] Superior Strimvelis outcomes with a background of electronic health record analytical tools and inexpensive whole-genome sequencing should green-light a paradigm shift in timely CVID diagnosis and identification of the root cause. […] Treatment of CVID, which has a much higher prevalence (one in 25,000) may seem less urgent, but it is not. […] The IVIG treatment regimen is time-consuming, inconvenient, very expensive and not completely effective. […] With current and emerging technology, the days of lifetime IVIG infusions for CVID should be numbered. […] Now is the time for the pharmaceutical industry to fully embrace gene therapy and partner with health systems, genetic diagnostic centres, research institutions and regulators to lead the way in developing gene therapy cures for CVID.

• #2 Common Variable Immunodeficiency in Children

  https://www.nationwidechildrens.org/conditions/health-library/common-variable-immunodeficiency-in-children

  Treatment will depend on your childs symptoms, age, and general health. It will also depend on how severe the condition is. […] Treatment includes immunoglobulin replacement therapy, medicines, routine blood tests, and postural drainage of the lungs. […] Immunoglobulin replacement therapy. IV or subcutaneous infusions of immunoglobulin (antibodies) may be given to help boost the childs immune system and replace the immunoglobulins that are needed. […] Antibiotics to treat and prevent infection as prescribed by your childs healthcare provider. […] Postural drainage of the lungs. These are special exercises or treatments for the lungs that are used in cases where frequent infections have led to scarring in the lung. Postural drainage may help with lung infections and removing secretions.

• #2 Common Variable Immunodeficiency in Children | University Hospitals

  https://www.uhhospitals.org/rainbow/services/pediatric-allergy-and-immunology/conditions-and-treatments/article/Diseases-and-Conditions—Pediatrics/common-variable-immunodeficiency-in-children-cvid

  Treatment will depend on your childs symptoms, age, and general health. It will also depend on how severe the condition is. […] Treatment includes immunoglobulin replacement therapy, medicines, routine blood tests, and postural drainage of the lungs. […] Immunoglobulin replacement therapy. IV or subcutaneous infusions of immunoglobulin (antibodies) may be given to help boost the childs immune system and replace the immunoglobulins that are needed. […] It is also important to help your child stay out of situations that have a greater risk for infection and wash their hands often with soap and water. […] With correct treatment, the number and severity of infections should be reduced.

• #2 Get Immunodeficiency Treatment | Cleveland Clinic

  https://my.clevelandclinic.org/services/immunodeficiency-treatment

  At Cleveland Clinic, we’re experts at diagnosing and treating immunodeficiencies. And we’ll stick by your side to help you manage your condition, so you can put all those nasty bugs at bay and start living a healthy life. […] We can treat your short-term flare-ups and prevent illness. And we can help you manage your long-term immune disorders, no matter how many you have or what causes them. […] It’s important we correctly diagnose your type of immunodeficiency so we can find the right treatment for you. […] We’ll use the information we get from our conversations, physical exam and tests to create a personalized treatment plan for you. We want to help prevent chronic (lifelong), repeat infections and help you avoid life-threatening illnesses and complications. […] Your treatment plan will depend on the type of immunodeficiency disorder you have and how it affects you. We’ll work with you to make shared decisions about your goals and personal preferences.

• #2

  https://link.springer.com/article/10.1007/s10875-017-0404-8

  Importantly, patient-specific features related to treatment route selection may influence subsequent QOL more than the route itself. […] These findings suggest that the method of Ig replacement (intravenous or subcutaneous) is not as important as the burden of disease in its impact upon QOL among patients with CVID. […] We suggest that improved QOL relies on early detection of disease and implementation of an individual treatment plan for the patient.

• #2 Explainer: What Is Common Variable Immunodeficiency (CVID)?

  https://www.csl.com/we-are-csl/vita-original-stories/2024/explainer-what-is-common-variable-immunodeficiency

  Immunoglobulin replacement therapy treats symptoms of CVID and these infused medicines help support the immune system and prevent infections. The medicines may be delivered intravenously (IV) or subcutaneously (SC). […] Patients who get frequent infections also might receive broad-spectrum antibiotics for chronic infections, according to the Immune Deficiency Foundation. In that way, a PI patient is like anyone else. Antibiotics are prescribed for infections, but someone who has CVID might not respond as well and it may take longer for them to recover. […] Regular medical care is important for people with CVID. The Immune Deficiency Foundation recommends that doctors monitor patients with CVID for autoimmunity, GI symptoms, and changes in lung function.

• #2 What Is Common Variable Immunodeficiency (CVID)? | SELF

  https://www.self.com/story/what-is-common-variable-immunodeficiency-cvid

  Staying up to date on routine vaccines, like those for the flu and COVID-19, can also help some people with CVID avoid getting severely sick, Dr. Peters says. But a big caveat, as Dr. Hartog clarifies, is that people with CVID sometimes don’t respond to vaccines well. The reason for that is a little complicated, but, essentially, it’s because some vaccines elicit potentially complicated responses from people with weakened immune systems. Dr. Hartog says it’s important for people with CVID to consult with an immunologist before getting any type of viral vaccine. […] There’s still so much to learn about this primary immunodeficiency disease, but CVID doesn’t have to feel scary. As is true of many chronic illnesses, the first step to taking control of your condition is seeking a diagnosis and making a plan with your care team. People with CVID very often live happy, long, and active lives and effective, individualized treatment can be the key to making that happen.

• #2

  https://link.springer.com/article/10.1007/s10875-017-0404-8

  Common variable immunodeficiency disorder (CVID) is a primary immunodeficiency disease (PIDD) often associated with severe and chronic infections. Patients commonly receive immunoglobulin (Ig) treatment to reduce the cycle of recurrent infection and improve physical functioning. […] Route of IgG replacement did not dramatically improve QOL. SF-12 scores were highest in patients with CVID who have well-controlled PIDD, lacked physical impairments, were not bothered by treatment, and received Ig infusions at home. […] Patients with CVID often require lifelong treatment with Ig, which may be administered intravenously or via the subcutaneous route. Immunoglobulin replacement is associated with improved QOL compared with no treatment. […] We noted a modestly improved SF-12 Mental Health score for patients on SCIG replacement over IVIG and improved MCS for those receiving SCIG vs IVIG who rated themselves as less than adequately controlled disease.

• #2 Immunoglobulin Replacement Therapy is critical and cost-effective in increasing life expectancy and quality of life in patients suffering from Common Variable Immunodeficiency Disorders (CVID): A health-economic assessment | PLOS One

  https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0247941

  The health-economic model suggests that early initiation of IgGRT compared to no or delayed IgGRT is highly cost-effective. […] CVID-patients access to IgGRT should be facilitated, not only because of proven clinical efficacy, but also due to the now demonstrated cost-effectiveness. […] The results demonstrate that the cost of IgGRT is associated with an incremental clinical benefit including 17 extra LYs and 11 extra QALYs. […] Treating CVID-patients promptly was also very cost-effective in the model: an average benefit of 6 LYs and 4 QALYs, resulting in an ICER of 30,374/LY and 47,495/QALY for a 4-year reduction in diagnostic delay. […] These cost-effectiveness and cost-utility results are far below the WHO-thresholds of efficiency.

• #2 Faculty Collaboration Database – Common Variable Immunodeficiency. Med Clin North Am 2024 Jan;108(1):107-121

  https://fcd.mcw.edu/?search/showPublication/id/2338292

  Common variable immunodeficiency (CVID) is the most common primary immune deficiency characterized by impaired production of specific immunoglobulin. […] The antibody deficiency found in CVID is treated with lifelong immunoglobulin therapy, which is preventative of the majority of infections when given regularly.

• #2 Common Variable Immunodeficiency (CVID) – Immunology; Allergic Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/immunology-allergic-disorders/immunodeficiency-disorders/common-variable-immunodeficiency-cvid

  Treatment includes prophylactic IgG replacement therapy and antibiotics for infection. […] Treatment of CVID consists of immune globulin and antibiotics as needed to treat infection. […] Rituximab, vedolizumab, tumor necrosis factor (TNF)-alpha inhibitors (eg, etanercept, infliximab), corticosteroids, and/or other treatments may be required to treat complications such as autoimmune disorders, immune enteropathy, lymphoid interstitial pneumonia, and granulomatous inflammation. […] Trials have confirmed the benefit of prophylactic antibiotics in select patients with antibody deficiency.

• #3 Common Variable Immunodeficiency Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/1051103-treatment

  Common variable immunodeficiency (CVID) patients were divided into four distinct clusters correlating to perceived health, a potentially important factor in providing care. […] The mainstay of treatment for common variable immunodeficiency (CVID) is Ig replacement therapy. Although expensive, Ig replacement therapy stops the cycle of recurrent infections. […] Ig may be administered intravenously or subcutaneously. Solutions of 3-12% intravenous immunoglobulin (IVIG) can be used on a regular basis to maintain a trough level of 400-500 mg/dL in adults. A dose of 400-600 mg/kg every 2-4 weeks is usually required. In patients with structural lung damage, a trough level of 700-800 mg/dL is required. […] A solution of 16% subcutaneous injection of IV immunoglobulin (SCIG) is also an effective treatment in patients with poor intravenous access.

• #3 Common variable immunodeficiency – an update | Arthritis Research & Therapy | Full Text

  https://arthritis-research.biomedcentral.com/articles/10.1186/ar4032

  Current therapy of CVID can be categorized as follows: regular and sufficient substitution with immunoglobulins (IgG trough levels 7.0 g/L); targeted antibiotic treatment of (breakthrough) infections; adequate treatment of complications; and in selected patients with severe hematological changes (chronic transfusion need, leukopenia, thrombocytopenia), secondary malignancies and suspected combined immunodeficiency, allogeneic peripheral stem cell transplantation is being considered in experienced centers. […] The immunoglobulin replacement therapy is the mainstay of therapy; 90% of CVID patients are on either intravenous (IVIg) or subcutaneous (SCIg) treatment. […] The current standard dosage when administered intravenously is 400 to 600 mg/kg every 3 to 4 weeks. For subcutaneous administration, this corresponds to 100 to 150 mg/kg per week.

• #3 Immunoglobulin Replacement Therapy is critical and cost-effective in increasing life expectancy and quality of life in patients suffering from Common Variable Immunodeficiency Disorders (CVID): A health-economic assessment | PLOS One

  https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0247941

  Immunoglobulin replacement therapy (IgGRT) is critical and cost-effective in increasing life expectancy and quality of life in patients suffering from Common Variable Immunodeficiency Disorders (CVID): A health-economic assessment. […] The clinical benefit of immunoglobulin replacement therapy (IgGRT) is substantial: timely treatment with appropriate doses significantly reduces mortality and the incidence of CVID-complications such as major infections and bronchiectasis. […] Immunoglobulin replacement therapy (IgGRT) is the standard of care in CVID. […] IgGRT is highly effective in reducing the mortality rate and the incidence of major infections in CVID-patients. […] Early-initiated IgGRT may improve patients health and quality of life and may also reduce overall healthcare and societal costs by avoiding or reducing the incidence of longer-term complications related to primary immunodeficiency (PID).

• #3 Common Variable Immunodeficiency

  https://www.aaaai.org/conditions-treatments/primary-immunodeficiency-disease/common-variable-immunodeficiency

  CVID is treated with immunoglobulin replacement therapy (IRT), which most often relieves symptoms. IRT treatments must be given regularly and are life-long. […] Treatment for CVID involves immunoglobulin replacement, preventative antibiotics, and if indicated management of autoimmune and granulomatous disease. […] Antibiotics are used to treat most infections that result from CVID though patients may need treatment for a longer duration than a healthy individual.

• #3 Rituximab for the Treatment of Common Variable Immunodeficiency (CVID) with Pulmonary and Central Nervous System Involvement

  https://openrheumatologyjournal.com/VOLUME/15/PAGE/9/FULLTEXT/

  Rituximab might be considered as an effective and relatively safe treatment for CVID-patients with GLILD. […] In this case series, we provide our experiences in treating GLILD-patients with a particular focus on the use of rituximab. […] Due to lack of response or intolerance of azathioprine, patients then received rituximab in a dose of 2 x 1000mg abs IV, separated by two weeks. […] All three patients CVID was defined as B+smB-CD21normTrnorm. […] Our experience contributes to the already existing knowledge of the management of GLILD and the effective use of rituximab in this condition. […] Rituximab is an effective treatment for this condition in CVID due to which this subgroup of patients suffering from both, GLILD and autoimmune cytopenia, might benefit twice from B cell depletion.

• #3 Common Variable Immunodeficiency: Demystifying the Decades-Old Immune Disorder

  https://ameripharmaspecialty.com/other-health-conditions/common-variable-immunodeficiency-demystifying-the-decades-old-immune-disorder/

  No cure for CVID exists; however, there are several treatments to keep your condition in check. Once the diagnosis is confirmed, your provider will likely give you immunoglobulin (IgG) replacement therapy. It contains antibodies obtained from healthy donors. There are two ways you can receive donor-derived antibodies: […] 1. Intravenous immunoglobulin (IVIG): IVIG is administered into a vein through a needle. The usual dose is 400 – 600 mg/kg every 2 to 4 weeks. […] 2. Subcutaneous immunoglobulin (SCIG): SCIG is given as a shot under your skin. The usual dose is 160 mg/kg once a week. […] Most people respond well to IgG replacement therapy. It is highly effective in reducing the recurrence of infections and joint problems. Likewise, IgG therapy reduces the severity of complications, such as autoimmune disorders. People often need treatment for their lifetime. During the therapy, your healthcare provider will monitor you for side effects. Common side effects include backache, nausea, vomiting, chills, fever, muscle pain, and fatigue. To reduce the odds of such reactions, premedication may be necessary. Your provider may prescribe antibiotics to treat infections. In some cases, antibiotics may be used regularly to prevent infections. Other treatments are specific to the system involved. For example, vitamin B12 injections may help someone with digestive problems leading to B12 deficiency. Likewise, those with breathing difficulty may benefit from inhaled corticosteroids and similar medications. If you have a low platelet count, it is best to avoid aspirin. Moreover, people with CVID should not receive live virus vaccines. Examples include the chickenpox vaccine and the MMR vaccine. […] COVID-19 may be more severe in people with immunodeficiencies like CVID, though a clear association is lacking. Most people with CVID at risk of COVID-19 are treated with convalescent plasma or IVIG.

• #3 Personalized therapy for common variable immunodeficiency: Ingenta Connect

  https://www.ingentaconnect.com/content/ocean/aap/2020/00000041/00000001/art00007

  Common variable immunodeficiency (CVID) represents a clinical descriptive diagnosis that was defined in the 1970s. […] These advances, along with developments in immune modulatory and reconstitution therapies, now permit sophisticated and specific targeting of therapies for individual patients. […] For 50 years, immune globulin therapy has been applied to patients with CVID. There are several options open to patients, including a diversity of products and modes of administration. […] Stem cell therapy is increasingly applicable in patients with severe immune dysregulation. […] In the near future, it is likely that further advances in understanding the pathophysiology of CVID, together with ongoing development of biologics and small-molecule immune modulators will lead to additional targeted therapies for these patients.

• #3 Pharma can lead the way toward cures for common variable immunodeficiency – The Pharmaceutical Journal

  https://pharmaceutical-journal.com/article/letters/pharma-can-lead-the-way-toward-cures-for-common-variable-immunodeficiency

  In November 2017, the National Institute for Health and Care Excellence (NICE) recommended Strimvelis (GlaxoSmithKline), a gene therapy for a form of severe combined immunodeficiency (SCID). […] However, manufacturers of immunoglobulin replacement therapy (IVIG) for less acute common variable immunodeficiency (CVID) should take note. […] Superior Strimvelis outcomes with a background of electronic health record analytical tools and inexpensive whole-genome sequencing should green-light a paradigm shift in timely CVID diagnosis and identification of the root cause. […] Treatment of CVID, which has a much higher prevalence (one in 25,000) may seem less urgent, but it is not. […] The IVIG treatment regimen is time-consuming, inconvenient, very expensive and not completely effective. […] With current and emerging technology, the days of lifetime IVIG infusions for CVID should be numbered. […] Now is the time for the pharmaceutical industry to fully embrace gene therapy and partner with health systems, genetic diagnostic centres, research institutions and regulators to lead the way in developing gene therapy cures for CVID.

• #3 Common variable immunodeficiency (CVID) in children – Children’s Health Allergy Immunology

  https://www.childrens.com/specialties-services/conditions/common-variable-immunodeficiency

  If your child is diagnosed with CVID, the doctor will likely administer immunoglobin replacement therapy, which usually relieves symptoms. Your child will need to get these treatments regularly throughout their life. […] IgG therapy can be given through a catheter in your child’s vein (intravenous; IVIG) or subcutaneously (SCIG). The decision to use one or the other will depend on what’s best for your child and your family. […] Your child’s doctor will also prescribe antibiotics to treat infections, and many times, longer courses may be required than for those patients without CVID. […] Sometimes, biopsies may be needed when nodules in the lung and liver appear. This may change treatments. […] If lympho-proliferation and granulomas develop, other treatments may be needed to suppress the immune system further. These medicines include steroids and other chemotherapy medications. […] All patients with CVID should be followed at the center. If patients with CVID have complications including pulmonary, gastrointestinal, or liver problems these require the cooperative interaction of your child’s immunologist with other subspecialties.

• #3 Get Immunodeficiency Treatment | Cleveland Clinic

  https://my.clevelandclinic.org/services/immunodeficiency-treatment

  At Cleveland Clinic, we’re experts at diagnosing and treating immunodeficiencies. And we’ll stick by your side to help you manage your condition, so you can put all those nasty bugs at bay and start living a healthy life. […] We can treat your short-term flare-ups and prevent illness. And we can help you manage your long-term immune disorders, no matter how many you have or what causes them. […] It’s important we correctly diagnose your type of immunodeficiency so we can find the right treatment for you. […] We’ll use the information we get from our conversations, physical exam and tests to create a personalized treatment plan for you. We want to help prevent chronic (lifelong), repeat infections and help you avoid life-threatening illnesses and complications. […] Your treatment plan will depend on the type of immunodeficiency disorder you have and how it affects you. We’ll work with you to make shared decisions about your goals and personal preferences.

• #3

  https://step2.medbullets.com/pediatrics/322235/common-variable-immunodeficiency-disorder-cvid

  A 35-year-old man presents to the emergency room for watery diarrhea. He reports that he feels as though he is always sick with sinusitis, upper respiratory infections, or diarrhea. […] He is started on intravenous immunoglobulin therapy. […] Treatment goals focus on preventing infections and treating specific complications of the disease. […] First-line intravenous immunoglobulin (IVIG) replacement therapy may pre-medicate with antihistamines. […] Second-line antibiotics. […] Lymphoma is a common cause of death in these patients.

• #3 Common Variable Immunodeficiency: Signs, Causes, and More

  https://resources.healthgrades.com/right-care/symptoms-and-conditions/common-variable-immunodeficiency

  The treatment of CVID involves administering antibody replacement therapy and medications to relieve other symptoms. […] Immunoglobulin replacement therapy is the main treatment option for CVID. It involves the injection of antibodies to boost the recipient’s serum level of antibodies. […] Your doctor may prescribe medications to manage specific symptoms. For example, antibiotics will help treat infections. […] Doctors may prescribe inactivated vaccines for people with severe antibody deficiency. Your doctor may vaccinate you against common diseases such as: polio, typhoid fever, flu, anthrax, rabies, hepatitis A and B, tetanus. […] Immunoglobulin replacement therapy is the primary treatment method for this disorder. This therapy helps to boost the serum level of circulating antibodies, but it can cause severe reactions. […] Also, your doctor may prescribe antimicrobial medications to fight infections and administer corticosteroids for autoimmune conditions.

• #4 Common variable immunodeficiency – an update | Arthritis Research & Therapy | Full Text

  https://arthritis-research.biomedcentral.com/articles/10.1186/ar4032

  First line therapy in autoimmune cytopenias and lympho proliferation are steroids. In case of failure, immunosuppressive drugs, rituximab or splenectomy have been reported as options. […] The inflammatory and granulomatous lesions of the lungs, liver and intestine respond poorly to the immunoglobulin replacement therapy alone and therefore often require corticosteroids, eventually in combination with immunosuppressants (cyclosporin A, azathioprin and others). […] The life expectancy of CVID patients has considerably improved over the past 30 years, from initially 12 years to currently over 50 years. […] Thus, the development of better surrogate diagnostic markers for the presence and activity of these secondary complications as well as new therapeutic approaches are a major challenge for the coming years in the care of CVID patients.

• #4 Rituximab for the Treatment of Common Variable Immunodeficiency (CVID) with Pulmonary and Central Nervous System Involvement

  https://openrheumatologyjournal.com/VOLUME/15/PAGE/9/FULLTEXT/

  In the CVID-case with additional and relapsing CNS-involvement, rituximab in combination with prednisolone, primarily administered due to pulmonary manifestation achieved a clinical and cMRI-morphological regression of the neurological manifestation. […] A case of CNS-manifestation responded to rituximab-treatment.

• #4 Common Variable Immunodeficiency: Demystifying the Decades-Old Immune Disorder

  https://ameripharmaspecialty.com/other-health-conditions/common-variable-immunodeficiency-demystifying-the-decades-old-immune-disorder/

  No cure for CVID exists; however, there are several treatments to keep your condition in check. Once the diagnosis is confirmed, your provider will likely give you immunoglobulin (IgG) replacement therapy. It contains antibodies obtained from healthy donors. There are two ways you can receive donor-derived antibodies: […] 1. Intravenous immunoglobulin (IVIG): IVIG is administered into a vein through a needle. The usual dose is 400 – 600 mg/kg every 2 to 4 weeks. […] 2. Subcutaneous immunoglobulin (SCIG): SCIG is given as a shot under your skin. The usual dose is 160 mg/kg once a week. […] Most people respond well to IgG replacement therapy. It is highly effective in reducing the recurrence of infections and joint problems. Likewise, IgG therapy reduces the severity of complications, such as autoimmune disorders. People often need treatment for their lifetime. During the therapy, your healthcare provider will monitor you for side effects. Common side effects include backache, nausea, vomiting, chills, fever, muscle pain, and fatigue. To reduce the odds of such reactions, premedication may be necessary. Your provider may prescribe antibiotics to treat infections. In some cases, antibiotics may be used regularly to prevent infections. Other treatments are specific to the system involved. For example, vitamin B12 injections may help someone with digestive problems leading to B12 deficiency. Likewise, those with breathing difficulty may benefit from inhaled corticosteroids and similar medications. If you have a low platelet count, it is best to avoid aspirin. Moreover, people with CVID should not receive live virus vaccines. Examples include the chickenpox vaccine and the MMR vaccine. […] COVID-19 may be more severe in people with immunodeficiencies like CVID, though a clear association is lacking. Most people with CVID at risk of COVID-19 are treated with convalescent plasma or IVIG.

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