Goodpasture Syndrome

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Goodpasture Syndrome
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Rapidly Progressive Glomerulonephritis - Wrap it up rapidly or it's your rep, kid
RPGN is a GN you don't want to miss since, as the name suggest, the progression is quite rapid (anything from months to days). So when the alarm bells go off in your head and your inner voice is screaming "fuck, fuck, fuck, fuck", maintain a professional exterior and think F, F, F, F:
symptom progression in RPGN:
Flu-like symptoms
Falling GFR (with nephritic syndrome - Hematuria, increased BP and moderate proteinuria)
Failure (acute oliguric renal failure)
Fluid overload
If the symptoms fit or seem suspicious and your inner voice starts screaming "ass, ass, ass, ass", maintain a professional exterior and think A, A, A, A:
Blood screening for:
Anti-GBM-ab = Goodpasture
ANA and anti-DNA = SLE
Acute renal failure = elevated creatinine, urea and potassium
ANCA
PR3-ANCA = Wegener's (a W has 3 "tops"),
MPO-ANCA = Microscopic Polyangiitis (MPO = MPA),
MPO-ANCA + eosinophilia = Churg-Strauss (2 markers = 2 names)
When symptoms, blood-work and biopsy all point to RPGN and your inner voice starts screaming "cunt, cunt, cunt, cunt" maintain a professional exterior and think C, C, C, C:
Progression of treatment:
Corticosteroids (immunosuppression to halt cell proliferation)
Cyclophosphamide (immunosuppression to halt cell proliferation)
Chronic dialysis (to avoid azotemia or to remove immunocomplexes via plasmapheresis)
Call a surgeon (transplantation)
Background information:
Wikipedia, p. 295 8th OHCM and pp. 499-504 21th DPPM
Goodpasture's Syndrome
autoimmune condition characterised by anti-GBM (glomerular basement membrane) antibodies
antibodies are directed against type IV collagen
classically produces a pulmonary-renal syndrome
Features; haemoptysis, dyspnoea, pulmonary haemorrhage, rapidly progressing glomerulonephritis (haematuria, red cell casts, proteinuria, loss of kidney function)
Diagnosis - anti-GBM antibodies, renal biopsy