Reumatizam, Vol. 69 No. 2, 2022.
Review article
https://doi.org/10.33004/reumatizam-69-2-3
Long term management of complex patient with common variable immunodeficiency – a case report
Thomas Ferenc
orcid.org/0000-0002-4917-9270
; Clinical Department of Diagnostic and Interventional Radiology, Merkur University Hospital, Zagreb, Croatia
Mateja Vujica
Miroslav Mayer
Abstract
Common variable immunodeficiency (CVID) is a primary immune disorder that results from insufficient immunoglobulin (IG ) secretion. Hypogammaglobulinemia is currently treated with substitute IG s, however, the substantial variation of post-application IG levels, as well as the development of complex clinical manifestations in patients set up a significant challenge for their physicians. A 33-year-old male patient has been followed-up with the diagnosis of CVID. From early childhood, he presented with recurrent respiratory infections. In 2010, he was diagnosed with CVID. Treatment was started with substitute intravenous (IV) IG . During a 9-year follow-up in day care and stationary University Hospital Centre (UHC) departments, the patient experienced a total of 14 respiratory and 2 digestive system complications of the disease. Eventually, he developed a non-infectious pulmonary complication – granulomatous-
lymphocytic interstitial lung disease (GLILD). In 2015, treatment was altered to accomplish satisfactory IG levels from IV to conventional subcutaneous (SC) IG therapy. Due to easier application and greater volume of subcutaneously inserted IG , the patient was switched to a new generation of SCIG therapy in 2019. Additionally, corticosteroids and mesalazine were also administered, and with current treatment the patient is stable. In the 9-year period, he never reached the preferable reference levels of IG s presumed for healthy individuals. Respiratory infections are common CVID complications, but it is necessary to consider the autoimmune and neoplastic manifestations of the disease. A multidisciplinary approach, regular follow-up, and regular application of IG therapy are the key factors in decreasing
morbidity and mortality in these patients.
Keywords
common variable immunodeficiency, CVID, complications, therapy
Hrčak ID:
309197
URI
Publication date:
22.10.2023.
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