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

Year 2020, , 67 - 70, 30.04.2020

Gökhan Aytekin , Fatih Çölkesen Eray Yıldız Hacı Esen , Şevket Arslan

https://doi.org/10.18678/dtfd.693297

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

Supporting Institution

none

Project Number

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Thanks

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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

Project Number

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References

• Hermaszewski RA, Webster AD. Primary hypogammaglobulinaemia: a survey of clinical manifestations and complications. Q J Med. 1993;86(1):31-42.
• Cunningham-Rundles C, Bodian C. Common variable immunodeficiency: clinical and immunological features of 248 patients. Clin Immunol. 1999;92(1):34-48.
• Spickett GP, Farrant J, North ME, Zhang JG, Morgan L, Webster AD. Common variable immunodeficiency: how many diseases? Immunol Today. 1997;18(7):325-8.
• Lachmann HJ, Hawkins PN. Systemic amyloidosis. Curr Opin Pharmacol. 2006;6(2):214-20.
• Strachan RW, Gillanders LA. A case of primary acquired hypogammaglobulinaemia with pernicious anaemia and amyloidosis: demonstrated at the Postgraduate Medical School of London. Br Med J. 1965;1(5426):35-8.
• Gaffney EF, Lee JC. Systemic amyloidosis and hypogammaglobulinemia. Arch Pathol Lab Med. 1978;102(11):558-9.
• Borte S, Celiksoy MH, Menzel V, Ozkaya O, Ozen FZ, Hammarström L, et al. Novel NLRP12 mutations associated with intestinal amyloidosis in a patient diagnosed with common variable immunodeficiency. Clin Immunol. 2014;154(2):105-11.
• Lederman HM, Winkelstein JA. X-linked agammaglobulinemia: an analysis of 96 patients. Medicine (Baltimore). 1985;64(3):145-56.
• Aytekin G, Çölkesen F, Yıldız E, Arslan S. Risk factors of bronchiectasis in adult patients with common variable immunodeficiency. Asthma Allergy Immunol. 2019;17(3):160-5.
• Urschel S, Kayikci L, Wintergerst U, Notheis G, Jansson A, Belohradsky BH. Common variable immunodeficiency disorders in children: delayed diagnosis despite typical clinical presentation. J Pediatr. 2009;154(6):888-94.
• Esenboga S, Çagdas Ayvaz D, Saglam Ayhan A, Peynircioglu B, Sanal O, Tezcan I. CVID associated with systemic amyloidosis. Case Reports Immunol. 2015;2015:879179.
• Arslan S, Ucar R, Yavsan DM, Esen H, Maden E, Reisli I, et al. Common variable immunodeficiency and pulmonary amyloidosis: a case report. J Clin Immunol. 2015;35(4):344-7.
• Aydin Z, Gursu M, Ozturk S, Kilicaslan I, Kazancioglu R. A case of primary immune deficiency presenting with nephrotic syndrome. NDT Plus. 2010;3(5):456-8.
• Turkmen K, Anil M, Solak Y, Atalay H, Esen H, Tonbul HZ. A hepatitis C-positive patient with new onset of nephrotic syndrome and systemic amyloidosis secondary to common variable immunodeficiency. Ann Saudi Med. 2010;30(5):401-3.
• Kadiroğlu AK, Yıldırım Y, Yılmaz Z, Kayabaşı H, Avcı Y, Yıldırım MS, et al. A rare cause of secondary amyloidosis: common variable immunodeficiency disease. Case Rep Nephrol. 2012;2012:860208.
• Meysman M, Debeuckelaer S, Reynaert H, Schoors DF, Dehou MF, Van Camp B. Systemic amyloidosis-induced diarrhea in sex-linked agammaglobulinemia. Am J Gastroenterol. 1993;88(8):1275-7.
• Kotilainen P, Vuori K, Kainulainen L, Aho H, Saario R, Asola M, et al. Systemic amyloidosis in a patient with hypogammaglobulinaemia. J Intern Med. 1996;240(2):103-6.
• Aytekin G, Çölkesen F, Ardeniz O, Çalışkaner A. Successful immunoglobulin replacement with subcutaneous immunoglobulin therapy in a patient with primary intestinal lymphangiectasia. Asthma Allergy Immunol. 2019;17(1):57-60.
• Esteve V, Almirall J, Ponz E, García N, Ribera L, Larrosa M, et al. [Renal involvement in amyloidosis. Clinical outcomes, evolution and survival]. Nefrologia. 2006;26(2):212-7.
• Balwani MR, Kute VB, Shah PR, Wakhare P, Trivedi HL. Secondary renal amyloidosis in a patient of pulmonary tuberculosis and common variable immunodeficiency. J Nephropharmacol. 2015;4(2):69-71.