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Goodpasture’s syndrome 07/10/11 5ICU 박미자 목차 Definition Background Pathophysiology Frequency Etiology Signs & symptoms Dignosis Treatment Consultation Prognosis Complication Prevention Definition Goodpasture syndrome =(Goodpasture disease, anti-glumerular basement membrane disease.) :Goodpasture syndrome is a disease that affects the kidneys and lungs. It usually involves rapidly progressive kidney failure that develops in days to weeks along with lung disease. Background It was first described by Ernest Goodpasture in 1919, and subsequently his name was given to the disease. Pathophysiology Goodpasture syndrome : Autoimmune disease로 anti-GBM antibody 가 폐와 GBM의 type IV Collagen 의 alpa 3 chain의 noncollagenous (NC1) domain 부위와 의 면역 반응으로 신장과 폐에 존재하는 goodpasture antigen이 공격받아 신장의 파괴와 폐출혈을 급속하게 일으킨다. Pathophysiology 자가면역 반응에 의해 형성된 항체가 Goodpasture 항원인 alpa3 chain protein이 분포되어 있는 기저막에 결합하 는데, 이 항원은 사구체 이외에도 폐, 갑상 선, 부신 및 망막등에 있는 기저막에 분포 한다. Frequncy 백인 1명/백만명/년 다른 인종보다 백인에서 흔함. 4~80세 발생.18~30세 & 50~65세 호발. 50세 이전은 남성에서 호발 빈도가 높으 나(남:여=6:1) 50세 이후에는 남, 여 비율 이 비슷함. Mortality: 매우 치명적인 질환으로 약 10% mortality를 보이며 생존한 경우 ESRD를 갖게된다. Etiology Unknown Association with HLA-DR2 Exposure to organic solvents or hydrocarbon Infection(eg, influenza) Coccaine inhalation Exposure to metal dusts Smoking Signs & symptoms Lung disease Dry cough, breathlessness Tachypnea Hemoptysis Anemia Respiratory failure Hypoxia Chill& fever Weight loss Cyanosis & pale skin Chest pain Signs & symptoms Kidney disease Hematuria & oliguria Proteinuria & Foamy urine ARF HTN & edema Diagnosis Lab studies CBC count: anemia, intrapulmonary bleeding Electrolytes, BUN/Cr 상승: renal dysfunction ESR 상승: vasculitis Urinalysis: low-grade proteinuria, gross or microscopic hematuria Diagnosis Lab studies Anti-GBM Ab :90%이상 양성 Antineutrophilic cytoplasmic Ab(ANCA) : Sometime during the course of illness, 20%에서 양성 최대 1/3에서 양성 반응 cytoplasmic ANCA (c-ANCA) and perinuclear ANCA (p-ANCA): extraglumerular renal vasculitis 동반을 의미 Diagnosis (c-ANCA) (p-ANCA) Diagnosis Imaging Studies: Chest radiograph Patchy parenchymal consolidations Normal findings on chest radiograph(약 18%) consolidation Pleural effusions Diagnosis (A 35-year-old man who previously smoked cigarettes heavily developed massive hemoptysis) (The autopsy showed consolidated lung from extensive bleeding, which led to asphyxiation.) Diagnosis Procedures Diagnostic bronchoscopy :diffuse alveolar hemorrhage remain Lung biopsy Pulmonary function testing Diagnosis Kidney biopsy :It is the preferred invasive procedure to substantiate the diagnosis of anti-GBM disease. (The renal biopsy revealed crescentic glomerulonephritis ) Treatment Medical care rapidly remove circulating antibody, primarily by plasmapheresis stop further production of antibodies using immunosuppression with medications *치료 받지 않는 경우 mortality 90%이상 치료 받을시엔 20%이하로 줄어듬. Treatment Immunosuppressive therapy :Immunosuppressive therapy is required to inhibit antibody production Cyclophosphamide(Cytoxan) :2 mg/kg , WBC 5000이상, 6month지속 Corticosteroids (prednisone) :1-1.5 mg/kg, tapered over 6month Treatment Alveolar hemorrage경우 : purse methylprednisone at 1g/D for 3day Antibiotics(Septra, Bactrim) :treatment of lung infection and help to reduce lung hemorrhage. Treatment Plasmapheresis : remove circulating anti-GBM antibody plasmapheresis is continued for 2-3 weeks or until the patient's clinical course has improved and serum anti-GBM antibodies are not detected. Daily or QOD Treatment Hemodialysis :Underlying renal disease(ESRD…) Surgical care :Kidney transplantation Prognosis The outlook varies. Early diagnosis &treatment tend to have better outcomes 5-year survival rate 80% 30% of patients require long-term dialysis. Complication Rapidly progressive glomerulonephritis Chronic kidney failure End-stage renal disease Severe pulmonary hemorrhage (lung bleeding) Respiratory failure Consultation Renal consultation Pulmonary consultation Prevention Never sniff glue or siphon gasoline, which expose the lungs to hydrocarbon solvents and can cause the disease Early diagnosis and treatment may slow the progression of the disorder Reference 해리슨 내과학(제 1판),1997,해리슨 편찬 위원회, 정담. www.emedicine.com www.renux.dmed.ed.ac.kr www.nlm.nih.gov www.wikipediaa.org