Goodpastures syndrome 071011 5 ICU Definition Background Pathophysiology

Goodpasture’s syndrome 07/10/11 5 ICU 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 (NC 1) domain 부위와 의 면역 반응으로 신장과 폐에 존재하는 goodpasture antigen이 공격받아 신장의 파괴와 폐출혈을 급속하게 일으킨다.

Etiology ± ± ± Unknown Association with HLA-DR 2 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, 6 month지속 ® Corticosteroids (prednisone) : 1 -1. 5 mg/kg, tapered over 6 month ®

Treatment ± Alveolar hemorrage경우 : purse methylprednisone at 1 g/D for 3 day ± 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. ac. kr www. nlm. nih. gov www. wikipediaa. org