BACKGROUND Common variable immunodeficiency (CVID) represents a group of heterogeneous, still undifferentiated, syndromes that are all characterized by defective antibody formation. It is often associated with autoimmune disease. METHODS An African-American girl was diagnosed with CVID at age 3 years. She was seen during an adrenal crisis precipitated by pneumonia at the age of 8 years and 10...

Common variable immunodeficiency (CVID) is a form of severe antibody deficiency with an estimated prevalence of 1 in 25,000 to 1 in 100,000. The disorder apparently results from currently undefined immune deregulations resulting in failed B-cell differentiation with impaired secretion of immunoglobulins. A 5-year female presented with fever, cough and shortness of breath at rest. Past medical h...

BACKGROUND Although common variable immunodeficiency (CVID) is associated with sinopulmonary disease, there are no previous systematic evaluations comparing computed tomographic (CT) sinus imaging with immunoglobulin measurements in patients with this condition. METHODS We performed a retrospective review by a blinded observer using the Lund-Mackay numerical scoring scale as well as a visual ...

Background: Patients with unclassified hypogammaglobulinemia (UCH) constitute a diagnostic and therapeutic dilemma, because information concerning the clinical and immunological characteristics of these patients is insufficient. Objective: To evaluate B-cell subsets in cases with UCH and common variable immunodeficiency (CVID) and their association with treatment requirement in UCH patients. Me...

BACKGROUND Hypogammaglobulinemia may be part of several different immunological or malignant conditions, and its origin is not always obvious. Furthermore, although autoimmune cytopenias are known to be associated with common variable immunodeficiency (CVID) and even may precede signs of immunodeficiency, this is not always recognized. Despite novel insight into the molecular immunology of comm...

Patients who have common variable immunodeficiency (CVID) and granulomatous/lymphocytic interstitial lung disease (GLILD) are at high risk for early mortality and B cell lymphomas. Infection with human herpes virus type 8 (HHV8), a B cell lymphotrophic virus, is linked to lymphoproliferative disorders in people who have secondary immunodeficiencies. Therefore, we determined the prevalence of HH...

We treated a patient with meningioma suffering from a congenital immunodeficiency syndrome. The patient was diagnosed with common variable immunodeficiency (CVID) in youth, and had suffered from repeated infections, especially in the respiratory tract, requiring continuous treatment with antibiotics. Imaging revealed a tumor located in the left frontal region with a broad attachment suggesting ...

BACKGROUND A subset of patients with chronic rhinosinusitis (CRS) has refractory disease. The risk factors for refractory CRS include atopy, a disrupted mucociliary transport system, medical conditions affecting the sinonasal tract mucosa, and immunodeficiency. METHODS We review four primary immunodeficiencies reported in individuals with CRS: common variable immune deficiency (CVID), selecti...

Common variable immunodeficiency (CVID) is one of the most common and clinically important primary immune deficiencies. CVID patients have poor humoral immunity, resulting in recurrent infections of the gastrointestinal and upper respiratory tracts, as well as increased incidence of some forms of cancers and autoimmune diseases. The treatment for CVID is IgG replacement, often given as intraven...

This paper describes the first case of common variable immunodeficiency (CVID) and AA amyloidosis. A recently treated tuberculosis, and chronic inflammation induced by frequent respiratory tract infections, were thought to be responsible for the amyloidosis. No other reason for this condition could be detected. Although T cell dysfunction in some CVID patients has been reported, pulmonary tuber...