نظرة عامة

نقص الغلوبولين المناعي A الانتقائي هو حالة مرضية للجهاز المناعي التي يفتقر فيها المريض أو لا يتوفر لديه ما يكفي من الغلوبولين المناعي A، وهو بروتين يحارب العدوى (الجسم المضاد). لا يتعرض معظم الأشخاص المصابين بنقص الغلوبولين المناعي ‏A الانتقائي لحالات عدوى متكررة. ومع ذلك، يُصاب بعض الأشخاص الذين لديهم نقص الغلوبولين المناعي A (‏IgA) من التهاب الرئة والتهابات الأذن والتهابات الجيوب الأنفية والحساسية والربو والإسهال.

يمكن العثور على أمراض المناعة الذاتية — التي يهاجم فيها جهازك المناعي أعضاء أو أنسجة معينة في جسمك — مع نقص الجلوبولين المناعي A الانتقائي. وتتضمن حالات المناعة الذاتية الشائعة التي تم العثور عليها مع نقص الغلوبولين المناعي A التهاب المفاصل الروماتويدي أو الذئبة أو الداء البطني أو مرض الأمعاء الالتهابي.

الأعراض

Most people with selective IgA deficiency have no symptoms. Some people have illnesses more often than is typical. They also may have a particular illness that returns often. Having frequent illnesses doesn't necessarily mean a person has selective IgA deficiency.

People with selective IgA deficiency may have frequent or repeat episodes of the following:

  • Ear infections, particularly in young children.
  • Colds.
  • Sinus infections.
  • Lung illnesses, such as bronchitis or pneumonia.
  • Giardiasis, a parasitic illness of the digestive system that causes diarrhea.

Children with frequent illnesses may not eat well or may not gain weight typical for their age.

الأسباب

Selective IgA deficiency happens when immune system cells don't produce any or produce very few IgA antibodies. The exact reason cells don't produce these antibodies isn't known.

Certain medicines used to treat seizures, epilepsy or rheumatoid arthritis may cause selective IgA deficiency in some people. The deficiency may continue after the medicine is no longer taken.

عوامل الخطورة

A family history of selective IgA deficiency increases the risk of the condition. Certain variations of genes appear to be linked to selective IgA deficiency, but no gene is known to directly cause the condition.

المضاعفات

People with selective IgA deficiency are at increased risk of other long-term conditions. These include:

  • Allergies and asthma.
  • Rheumatoid arthritis.
  • Celiac disease.
  • Inflammatory bowel disease.
  • Common variable immunodeficiency, which is a lack of two or more types of immunoglobulins.

Risk of reaction to blood products

People with selective IgA deficiency are at risk of reactions to blood transfusions or blood products. Because a person's body doesn't make IgA, the immune system may see it as a foreign substance in a blood transfusion or other treatment with blood products.

A reaction may cause high fever, chills, sweating and other symptoms. Rarely, people with selective IgA deficiency have a life-threatening allergic reaction, called anaphylaxis (an-uh-fuh-LAK-sis).

Healthcare professionals recommend wearing a medical bracelet. A bracelet can show that you have selective IgA deficiency and should receive modified blood or blood products.