Otoimmun Komplikasyonlarla Seyreden Yaygın Değişken İmmün Yetmezlik Hastasında Renal Amiloidoz

Abstract

Yaygın değişken immün yetmezlik (YDIY), bozulmuş antikor üretimi ve yetersiz B hücresi farklılaşması ile karakterize nadir görülen bir birincil immün yetmezlik tablosudur. Her ne kadar YDIY hastalarında sık tekrarlayan solunum yolu enfeksiyonları en belirgin klinik durum olsa da; YDIY, lenfoid hiperplazi, otoimmün sitopeni, kronik akciğer hastalıkları, granülomatöz hastalıklar ve maligniteye yatkınlık gibi birçok sistem ve organı etkileyen heterojen bir immün sistem bozukluğudur. Bu durum, tanı ve immünoglobulin replasman tedavisinde gecikmeye, uygun dozda ve zamanda antibiyotik alınamamasına, kronik inflamasyona ve dolayısıyla sekonder amiloidoza yol açabilir. Bu vaka sunumunda, otoimmün komplikasyonla seyreden bir YDIY olgusu ve takip sırasında gelişen renal amiloidoz tablosunun sunulması amaçlanmıştır.

Keywords

• Yaygın değişken immün yetmezlik,renal amiloidoz,kronik enflamasyon

Renal Amyloidosis in a Common Variable Immune Deficiency Patient with Autoimmune Complications

Abstract

Common variable immune deficiency (CVID) is a rare primary immunodeficiency disorder that is characterized by defective antibody production and inadequate B cell differentiation. While frequently recurrent respiratory tract infections are the most prominent clinical feature in CVID patients, CVID is a heterogeneous immune deficiency disorder that involves many systems and organs such as lymphoid hyperplasia, autoimmune cytopenia, chronic lung diseases, granulomatous diseases and susceptibility to malignancy. This may lead to delay in diagnosis and immunoglobulin replacement therapy, not being able to receive antibiotics at the appropriate dose and time, chronic inflammation, and therefore secondary amyloidosis. In this case report it is aimed to present a CVID patient with autoimmune complications and developing renal amyloidosis during follow-up.

Keywords

• Common Variable Immune Deficiency,renal amyloidosis,chronic inflamation

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