Korean J Med.  2015 Aug;89(2):254-258. 10.3904/kjm.2015.89.2.254.

A Case of Sjogren's Syndrome Associated with Common Variable Immunodeficiency

• Kang SH
• Cho HJ
• Hong HJ
• Hong SD
• Kim BH
• Jung SY
• Choi JJ

• KMID: 2269040
• DOI: http://doi.org/10.3904/kjm.2015.89.2.254

Abstract

Common variable immunodeficiency (CVID) is a heterogeneous group of primary immune deficiencies in adults characterized by hypogammaglobulinemia, recurrent bacterial infections, and a higher incidence of autoimmune diseases. More than 25% of CVID patients also have autoimmune diseases such as autoimmune hemolytic anemia, immune thrombocytopenic purpura, rheumatoid arthritis, and systemic lupus erythematosus. However, the pathogenesis of autoimmunity in CVID remains obscure. We report a 56-year-old woman with CVID and Sjogren's syndrome. In addition to a long history of recurrent upper respiratory infections, acute gastroenteritis, and cellulitis, she has also suffered from persistent xerostomia and xerophthalmia for the past 10 years. Serologic studies revealed hypogammaglobulinemia (low levels of IgG, IgA, and IgM in serum) and the presence of anti-Ro antibodies, and salivary scintigraphy indicated salivary gland involvement. These findings led to a diagnosis of CVID and Sjogren's syndrome, which was treated by monthly intravenous immunoglobulin therapy.

Keyword

Autoimmune diseases; Common variable immunodeficiency; Sjogren's syndrome

MeSH Terms

Adult
Agammaglobulinemia
Anemia, Hemolytic, Autoimmune
Antibodies
Arthritis, Rheumatoid
Autoimmune Diseases
Autoimmunity
Bacterial Infections
Cellulitis
Common Variable Immunodeficiency*
Diagnosis
Female
Gastroenteritis
Humans
Immunization, Passive
Immunoglobulin A
Immunoglobulin G
Immunoglobulin M
Incidence
Lupus Erythematosus, Systemic
Middle Aged
Purpura, Thrombocytopenic, Idiopathic
Radionuclide Imaging
Respiratory Tract Infections
Salivary Glands
Sjogren's Syndrome*
Xerophthalmia
Xerostomia
Antibodies
Immunoglobulin A
Immunoglobulin G
Immunoglobulin M