About cvid

What is cvid?

Common Variable Immune Deficiency (CVID) is a type of primary immunodeficiency, which is defined as an immune system dysfunction typically caused by a mutation in a gene or genes. The World Health Organization (WHO) recognizes more than 150 primary immunodeficiencies ranging from relatively common to quite rare.

CVID is one of the most prevalent of primary immunodeficiencies and manifests a wide variability of symptoms and range of severity. It is considered a diverse group of diseases of unknown cause (etiology) as many different immune system defects have been reported. CVID is characterized by a low level of specific proteins (antibodies, also called immunoglobulins) in the fluid portion of the blood which results in a decreasedability to fight invading microorganisms, toxins, or other foreign substances. These immunoglobulins are produced by specialized white blood cells (B cells) as they mature.

The cause of CVID is unknown in 75-80% of cases, and a genetic cause has been identified in 10-20%. Sporadic cases, with no apparent history of the disorder in their family, may be caused by a complex interaction of environmental and genetic components (multifactorial inheritance), but genes that are involved in the development and function of B cells are believed to be the primary cause.

What are the symptoms for cvid?

Anemiab symptom was found in the cvid condition

The severity of symptoms can vary greatly between people with CVID. Symptoms of common variable immunodeficiency may appear during childhood or adolescence, though many people don't experience them until adulthood.

If you have CVID, you'll likely experience repeated infections before being diagnosed. The most common types of infections include pneumonia, sinusitis, ear infections and gastrointestinal infections.

What are the causes for cvid?

In the vast majority of CVID cases, the cause is unknown. In around 10% of people with CVID, a genetic mutation has been identified. Researchers believe that the condition is caused by a combination of both environmental and genetic factors. As of now, the environmental factors are unclear.

What are the treatments for cvid?

The treatment of CVID requires the coordinated efforts of a team of specialists who may need to systematically and comprehensively plan an affected individual’s treatment. Such specialists may include physicians who diagnose and treat disorders of the blood (hematologists), the digestive tract (gastroenterologists), and/or the lungs (pulmonologists); specialists in the treatment of immune system disorders (immunologists); and/or other health care professionals.

The primary treatment for CVID consists of regular immunoglobulin (gammaglobulin) therapy, which is administered by intravenous or subcutaneous infusion with antibodies obtained from the fluid portion of the blood (gammaglobulin). Such therapy may help to prevent the recurrent infections characteristic of CVID as well as treat the disorder’s associated symptoms.

Individuals with CVID who experience adverse reactions to intravenous gammaglobulin may benefit from the subcutaneous delivery of this medication. In some, the administration of medications that block the effects of the chemical histamine (antihistamines), which is released during allergic reactions, or nonsteroidal anti-inflammatory agents (NSAIDs) are used. Rarely, hydrocortisone, a corticosteroid medication, may be needed prior to gammaglobulin therapy. Because corticosteroids may actually suppress an already weakened immune system, NSAIDs may be helpful in controlling autoimmune-like symptoms while avoiding the use of corticosteroids. However, after being immunoglobulin therapy for several months, most patients no longer require any premedication.

Some researchers have recommended that when a patient is diagnosed with an autoimmune disease, the possibility of an underlying CVID should be evaluated before the administration of immunosuppressive drugs for the autoimmune disease.

Antibiotic medications often prove beneficial for the treatment of various bacterial infections associated with CVID. Patients with irregularities involving the malabsorption of vitamin B12 may also benefit from monthly B12 injections.

Affected individuals with severely low levels of circulating platelets may be cautioned to avoid the use of aspirin, since this medication may interfere with the ability of platelets to assist in the blood-clotting process. In addition, as is the case with individuals affected by many other primary immunodeficiency disorders, individuals with CVID should not receive live virus vaccines since there is the remote possibility that the vaccine strains of virus may cause disease as a result of their defective immune systems.

Surveillance for complications include periodic complete blood count (CBC), and differential white blood counts to detect lymphoma, annual thyroid examination and thyroid function testing, annual lung (pulmonary) function testing beginning about age eight to ten years, biopsy of enlarged lymphoid tissue, and other imaging techniques for assessment of granulomatous disease and gastrointestinal complications.

Genetic counseling is recommended for affected individuals and their family members if a genetic type of CVID is suspected or confirmed. Other treatment is symptomatic and supportive.

What are the risk factors for cvid?

Rather than a disease, common variable immunodeficiency disorder (CVID) is a collection of hypo-gammaglobulinemia syndromes caused by various genetic defects.

  • Primarily, it is a humoral immunodeficiency disorder, characterized by reduced serum levels of immunoglobulin G and immunoglobulin A (IgA) or immunoglobulin M (IgM), recurrent sinopulmonary infections, autoimmune disorders, granulomatous diseases, elevated risk of malignancy, and ineffective antibody response despite the adequate number of B cells.
  • The disorder is diagnosed after puberty, between 20 and 45 years of age.
  • Upon the onset of the disorder, affected individuals suffer from chronic infections, lung diseases, GI tract diseases, dermatological abnormalities, and autoimmune disorders.

Risk factors:

  • There are genetic and epigenetic/environmental risk factors. A suspicion of environmental factors stems from the non-genetic causes of the disorder.
  • While the environmental factors are still unclear, the genetic factors are heterogeneous.
  • Thus, the only known risk factor is the inheritance of defective genes. Approximately, 20% of patients have a first-degree family member with IGA deficiency.
  • There is no clear pattern of inheritance. The modes of inheritance are autosomal dominant with variable penetrance, autosomal recessive, and X-linked forms.
  • Since the autosomal mode of inheritance is x-linked, the defect passes on to male and female offspring equally.
  • The cases of random, acquired mutations have also been reported.
  • Exposure of individuals to mutagenic agents is the risk factor for the non-inherited incidence.

Chronic infections,Autoimmune disorders,Lung diseases like bronchiectasis, carcinomas, rheumatoid arthritis,Gastrointestinal infections and disorders,Dermatologic manifestations,Anemia
Chronic productive cough,Dyspnea,Nasal congestion,Scars in tympanic membranes,Arthritis

Is there a cure/medications for cvid?

Common variable immunodeficiency disorder (CVID) is a collection of immunodeficiency diseases caused by various abnormal and causative changes in the immune system.

Some of the treatment options are:

  • The therapy involves undergoing a blood-based infusion to raise the level of antibodies to fight infections.
  • Generally, after the treatment of an infection, the therapy begins with adequate hydration.
  • Subsequently, a slow infusion load of intravenous immunoglobulins (IVIG) must be administered until tolerance is appropriate, followed by maintenance doses.
  • The subcutaneous route is an alternative in the maintenance phase.
  • Patients, prone to adverse reactions, are subjected to premedication of diphenhydramine and acetaminophen.
  • IgG levels should be monitored every six months to adjust the dosing of IVIG.
  • With a compromised immune system, the patients are prone to infections. Longer courses of antibiotics are the treatments for infections.
  • Prophylactic antimicrobials are not a routine recommendation. However, antiviral therapy should be prescribed to high-risk immunodeficient patients, during seasonal influenza.
  • Vaccinations must be based on the severity of antibody deficiency
  • Routine schedule for inactivated or subunit vaccines such as in mild deficiency.
  • Routine schedule for inactivated or subunit vaccines in severe deficiency
  • Recommended administration of live-attenuated vaccines in mild deficiency
  • Live-attenuated vaccines are not recommended in severe deficiency.
  • Glucocorticoids are the first-line treatment for immune thrombocytopenia and autoimmune hemolytic anemia.
  • In glucocorticoid-refractory cases, rituximab should be considered, maintaining immunoglobulin replacement therapy.
  • In severe refractory patients, splenectomy is the last resort.

Chronic infections,Autoimmune disorders,Lung diseases like bronchiectasis, carcinomas, rheumatoid arthritis,Gastrointestinal infections and disorders,Dermatologic manifestations,Anemia
Chronic productive cough,Dyspnea,Nasal congestion,Scars in tympanic membranes,Arthritis

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