Rama, a 20 year old smoker, presents with hemoptysis and hematuria. Likely diagnosis is:
|A||Good pasture's syndrome|
Good pasture's syndrome is an autoimmune disease characterized by formation of Anti GBM antibodies that attack both pulmonary capillaries and the Glomerulus (GBM)
It refers to a group of illnesses defined by triad of:
a. Glomerulonephritis (usually crescentic)
b. Pulmonary hemorrhage (Hemoptysis may precede nephritis).
c. Anti-GBM antibody in serum.
Association of hematuria and hemoptysis suggest a diagnosis of Goodpasture's syndrome.
i. Circulating Anti GBM antibodies (lgG): Positive
ii. ANCA antibodies: Typically Negative (ANCA negative vasculitis)
iii. ANA antibodies: usually normal
iv. C3 levels: Usually normal
v. Antibodies against Non collagenous Domain (NCL) of alpha chain of collagen type IV
(Found in Glomerular Basement membrane & Pulmonary capillaries)
Antibodies against glomerular Basement membrane
Antibodies against pulmonary capillaries
Glomerulonephritis / Renal Disease
Pulmonary / Lung Manifestations
Two Age Groups
i. Young men in late 20's (Men>>females) ii. Men and women in 60-70 years
I. Hemoptysis (Frank) II. Dyspnea
iii. Hematuria iv. Proteinuria v. Edema
i. Diffuse crescentic Glomerulonephritis
ii. Linear IgG staining along basement membrane on immunofluorescence
- Diffuse bilateral pulmonary infiltrates
1. The renal and pulmonary components may be severe or clinically silent.
2. The presence of the anti-GBM antibody, is the essential feature of the diagnosis.
3. Rapidly progressive renal failure is typical
4. Circulating anti – glomerular basement membrane (GBM) antibody and linear immuno – fluorescence on renal biopsy establishes the diagnosis
1. Plasma exchange may produce remission. 2. Prednisolone 3. Immunosuppressive therapy