CVID Associated with Systemic Amyloidosis
Joint Authors
Esenboga, Saliha
Çagdas Ayvaz, Deniz
Saglam Ayhan, Arzu
Peynircioglu, Banu
Sanal, Ozden
Tezcan, Ilhan
Source
Issue
Vol. 2015, Issue 2015 (31 Dec. 2015), pp.1-4, 4 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2015-08-04
Country of Publication
Egypt
No. of Pages
4
Main Subjects
Abstract EN
Common variable immunodeficiency (CVID) is a frequent primary immune deficiency (PID), which consists of a heterogeneous group of disorders and can present with recurrent infections, chronic diarrhea, autoimmunity, chronic pulmonary and gastrointestinal diseases, and malignancy.
Secondary amyloidosis is an uncommon complication of CVID.
We report an unusual case of a 27-year-old male patient who presented with recurrent sinopulmonary infections, chronic diarrhea, and hypogammaglobulinemia and was diagnosed with CVID.
The patient was treated with intravenous immunoglobulin (IVIg) therapy once every 21 days and daily trimethoprim-sulfamethoxazole for prophylaxis.
Two years after initial diagnosis, the patient was found to have progressive decline in IgG levels (as low as 200–300 mg/dL) despite regular Ig infusions.
The laboratory tests revealed massive proteinuria and his kidney biopsy showed accumulation of AA type amyloid.
We believe that the delay in the diagnosis of CVID and initiation of Ig replacement therapy caused chronic inflammation due to recurrent infections in our patient and this led to an uncommon and life-threatening complication, amyloidosis.
Patients with CVID require regular follow-up for the control of infections and assessment of adequacy of Ig replacement therapy.
Amyloidosis should be kept in the differential diagnosis when managing patients with CVID.
American Psychological Association (APA)
Esenboga, Saliha& Çagdas Ayvaz, Deniz& Saglam Ayhan, Arzu& Peynircioglu, Banu& Sanal, Ozden& Tezcan, Ilhan. 2015. CVID Associated with Systemic Amyloidosis. Case Reports in Immunology،Vol. 2015, no. 2015, pp.1-4.
https://search.emarefa.net/detail/BIM-1058682
Modern Language Association (MLA)
Esenboga, Saliha…[et al.]. CVID Associated with Systemic Amyloidosis. Case Reports in Immunology No. 2015 (2015), pp.1-4.
https://search.emarefa.net/detail/BIM-1058682
American Medical Association (AMA)
Esenboga, Saliha& Çagdas Ayvaz, Deniz& Saglam Ayhan, Arzu& Peynircioglu, Banu& Sanal, Ozden& Tezcan, Ilhan. CVID Associated with Systemic Amyloidosis. Case Reports in Immunology. 2015. Vol. 2015, no. 2015, pp.1-4.
https://search.emarefa.net/detail/BIM-1058682
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-1058682