Variables Immundefektsyndrom

Common variable immunodeficiency (CVID) is a condition that is associated with an impaired immune system. It is considered the most common symptomatic primary immunodeficiency and is characterized by recurrent respiratory tract infections.

Clinical presentation

The commonest presentation is that of frequent respiratory tract infections.

Pathology

The exact cause is unknown in at least 90% of cases, while a genetic cause may be present in <10% of cases. The conditions involve:

• low levels of most or all of the immunoglobulin (Ig) classes - hypogammaglobulinaemia
• lack of B lymphocytes or plasma cells that are capable of producing antibodies
• frequent bacterial infections

Serology

The following serological abnormalities may be present:

• serum IgA and IgG: decreased but not absent
• serum IgM: occasionally decreased
• circulating T and B lymphocytes: present

Manifestations

It can affect multiple organ systems:

• cutaneous manifestations of common variable immunodeficiency
• sinonasal manifestations of common variable immunodeficiency
• pulmonary manifestations of common variable immunodeficiency
• hepatic manifestations of common variable immunodeficiency
• hematological manifestations of common variable immunodeficiency

Siehe auch:

• Multiples Myelom
• autosomal-rezessiv vererbte Agammaglobulinämie (Schweizer Typ)
• Bruton-Syndrom
• Nephrotisches Syndrom
• Hyper-IgM-Syndrom
• Immundefektsyndrome
• severe combined immunodeficiency (SCID)
• exsudative Enteropathie
• selektiver Immunglobulin-A-Mangel
• transitorische Hypogammaglobulinämie im Kindesalter

und weiter:

• Progressive multifokale Leukenzephalopathie
• Pneumonitis
• lymphozytisch interstitielle Pneumonie
• chronic granulomatous disease
• Agammaglobulinämie