Guhjy: 新頁面: *Pulmonary-renal syndrome <<< #Goodpasture's syndrome #ANCA small-vessel vasculitis #Wegener's granulomatosis #Microscopic polyangitis #Churg-Strauss syndrome #[[Henoch-Schönlei...

2018-03-14T01:51:32Z

新頁面: *Pulmonary-renal syndrome <<< #Goodpasture's syndrome #ANCA small-vessel vasculitis #Wegener's granulomatosis #Microscopic polyangitis #Churg-Strauss syndrome #[[Henoch-Schönlei...

新頁面

*Pulmonary-renal syndrome
<<<
#Goodpasture's syndrome
#ANCA small-vessel vasculitis
#[[Wegener's granulomatosis]]
#Microscopic polyangitis
#[[Churg-Strauss syndrome]]
#[[Henoch-Schönlein purpura]]
#[[Cryoglobulinemia]]
*D.D.
**Lupus GN plus pneumonitis or pneumonia
**Uremia plus pneumonia
<<<
*Diagnostic triad
**RPGN + Lung hemorrhage (hemoptysis) + anti-GBM antibody
**Diagnosis
***''Serum anti-GBM antibody detected by antibody to α3 ~NC1 domain of type IV collagen''
****Serum anti-GBM antibody may be positive in patients with paraneoplastic syndrome if nonspecific anti-GBM antibodies are used
***10-15% p'ts also have serum ANCA to myeloperoxidase
****Good prognosis
**It appears in two age groups: in young men in late 20s and in men and women in 60–70s
**Disease in the younger age group is usually explosive, with hemoptysis, a sudden fall in hemoglobin, fever, dyspnea, and hematuria. Hemoptysis is largely confined to ''smokers''
**Treatment
***[[Plasmapheresis]] (for lung hemorrhage), steroids, cyclophosphamide
***Renal transplantation (wait for 6 months and until serum antibodies are undetectable)
**Prognosis
***Poor if >50% crescents, advanced fibrosis, serum creatinine >5–6 mg/dL, oliguria, need for acute dialysis

Goodpasture's syndrome - 修訂歷史

Guhjy: 新頁面: *Pulmonary-renal syndrome <<< #Goodpasture's syndrome #ANCA small-vessel vasculitis #Wegener's granulomatosis #Microscopic polyangitis #Churg-Strauss syndrome #[[Henoch-Schönlei...