Pulmonary haemorrhage (presumed) in Goodpasture syndrome

Case contributed by Dr James Sheldon

Presentation

Dyspnoea and haemoptysis

Patient Data

Age: 19 years
Gender: Female

The mobile study demonstrates diffuse airspace opacification.

A vascular catheter has its tip in the upper superior vena cava.

CT Chest:

Technique: Non contrast scans were performed.

Findings:

Patchy regions of ground glass opacity are present throughout the lungs. No interlobular septal thickening. No pleural effusion.

No other abnormal findings.

Case Discussion

The patient had recently undergone a renal biopsy for investigation of haematuria and worsening renal function.

MACROSCOPIC DESCRIPTION: "Kidney biopsy": A core of cortex and medulla 15mm long, glomeruli difficult to see. Many dark red striations seen throughout tissue. (RA)

MICROSCOPIC DESCRIPTION: The kidney biopsy is about two thirds cortex. There are 25 glomeruli, none of which is totally sclerosed. Twenty one glomeruli have crescents, 19 large cellular and two segmental cellular. All but two of the crescents are associated with fibrinoid necrosis. In the intact glomeruli and segments, there is no increase in mesangial cells or matrix. No deposits are demonstrated. Silver stains show considerable disruption of capillary loops in areas of necrosis. In some glomeruli, Bowman's capsule has been disrupted and there are foreign body giant cells. Protein resorption droplets are seen in visceral epithelial cells in some tufts. No CMV is demonstrated. Interstitial fibrosis is minimal but there is a patchy interstitial infiltrate of lymphocytes, histiocytes and plasma cells with a few neutrophils in one area. Many tubules contain red cell casts. Some tubules are dilated and lined by a flattened or vacuolated epithelium. Many proximal tubular cells contain large eosinophilic cytoplasmic globules. Arterioles are normal. There is focal mild fibroelastic intimal thickening in arteries. IMMUNOPEROXIDASE STAINS: IgA - light staining of casts only. IgG - light linear staining of loops. IgG4 - moderate linear staining of loops. IgM - segmental staining in several tufts. Fibrin - strong staining in crescents. C3c - light linear staining of loops. C1q - moderate mesangial staining and light interrupted linear staining in some loops.

DIAGNOSIS: Native kidney biopsy: Focal proliferative and necrotising glomerulonephritis with crescents in 84% of glomeruli but minimal interstitial fibrosis/tubular atrophy. There is linear staining for IgG, in keeping with antiglomerular basement antibody disease.

 

The findings of haemoptysis and diffuse airspace opacification are most consistent with pulmonary haemorrhage in a patient with antiglomerular basement membrane antibody disease (Goodpasture's Syndrome).

The imaging ddx is pulmonary oedema (less likely in the absence of interlobular septal thickening) or infection (consider atypical organisms particularly if the patient is immunocompromised).

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Case information

rID: 38334
Case created: 15th Jul 2015
Last edited: 5th Dec 2016
Inclusion in quiz mode: Included

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