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Medicine

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Kidney

Question
10 out of 11
 

Rama, a 20 year old smoker, presents with hemoptysis and hematuria. Likely diagnosis is:



A Good pasture's syndrome
B Nephrotic syndrome

C GullianBarre syndrome
D IgA Nephropathy

Ans. A Good pasture's syndrome
Goodpasture’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.

Serology

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

Clinical

I. Hemoptysis (Frank) II. Dyspnea

iii. Hematuria iv. Proteinuria v. Edema

Pathology

i. Diffuse crescentic Glomerulonephritis

ii. Linear IgG staining along basement membrane on immunofluorescence

X-ray finding

- Diffuse bilateral pulmonary infiltrates

Extra Edge:

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

Treatment :

1. Plasma exchange may produce remission. 2. Prednisolone 3. Immunosuppressive therapy

Kidney Flashcard List

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