Common Variable Immunodificiency(CVID)■ difinition: common defects in B-cell function and acquired agammaglobulinaemia: heterogeneous group of immunologic disorders of unknown etiology, characterized by impaired antibody responses■ Etiology? not hereditarycommonly associated with MHC haplotype HLA-B8 & HLA-DR3- MCH : major histocompatibility complex, 주요조직적합유전자복합체- haplotypes : 한쪽 어버이에 의하여 기여- HLA : human lymphocyte antigen? pyogenic organism : pneumonia, otitis media, sinusitis■ Frequency? 1 case per 10,000-50,000 population(males and females equally)? age : peaks of onset = 1-5 years, 16-20 years■ Mortality? prognosis is good unless bronchiectasis, chronic lung damage, severe autoimmune disease? 진단 후 20년 이내 생존율 - 남성 94%, 여성 64~67%? 사망 질환lymphoma > chronic pulmonary infection > liver failure> inflammatory bowel disease > other viral infections■ Pathophysiologymany different immune system defectsinsult↙↙↘↘① specific② complement system③ cellular immunity(T-cell)④ humoral immunity(B-cell)defect →↓Ab formation X (IgG,IgM,IgA,IgE,IgD)↙↓cell membrane injuryvasodilation↓↓microvascular permeability↑SVR↓↓↙ ↓interstitial fluid↑maldysfunction ofcirculating vol.CO↑ butperfusion 유지 어려움↓↓↙↓edema, IVF↓organ & tissue hypoperfusion/damagelactic acidosis↓↓perfusion imbalanceMultiple organ dysfuction syndrom(MODS)■ Clinical presentation㉠ Temperature > 38.0℃, 90bpm㉢ RR > 20rpm or PaCO212,000cell/㎣, 500 mg/dL in adults: 400-600 mg/kg/month: f/u serum Ig level every 3monthAfter achieving normal serum IgG levels → stop & f/u every 3month? chronic lung disease: 600-800 mg/kg/month② Subcutaneous infusion of Ig(SCIG)- IV가 어려운 환자, home injection 가능- IVIG에 비해 감염률이 7배가 적다? 160 mg/kg per week ≒ IVIG 400-600 mg/kg per month③ 부작용? Nonanaphylactic reactions- first 30min. infusion: backache, nausea, chills, low-grade temperature, vomiting- end of infusion & continue for several hours: Headache, chills, fever, myalgia, fatigue⇒ trestememt : acetaminophen, diphenhydramine, hydrocortisone용량 조정 → 200-300 mg/kg? Anaphylactic reactions- IVIG are rareusually caused by fluid volume, salt, protein overload & high dose- close observation during and after infusions⇒ trestememt : 필요시 diuretics 사용? Transmission of infectious agents- 1993~1994 : hepatitis C virus 발견됨.㉡ Surgical Care? for treatment of complications : endoscopic sinus surgery, spleenectomy㉢ Diet : high-calorie diet(high-energy expenditure)㉣ Activity : Regular physical activity is encouraged.■ complication: ARDS, DIC, ARF, MODSIMMUNOLOGY, Ivan Roitt 외 2, 1993, MosbyCommon Variable Immunodeficiency, C Lucy Park, MD, 2004 May, e-Medicine