LearningRadiology - Goodpastures Syndrome

Idiopathic Pulmonary Hemorrhage (IPH)

and

Goodpasture’s Syndrome

• Both are characterized by repeated episodes of pulmonary hemorrhage

• Both produce iron-deficiency anemia and both can produce pulmonary insufficiency

• Pathology

• Hemorrhage is typically confined to peripheral airspaces

• Diffuse interstitial fibrosis, hemosiderosis are common

• Vasculitis doesn't always occur even though these are autoimmune diseases

• Hemoptysis more copious in IPH

• Prognosis for both diseases is grave – both are treated with steroids and cytotoxic agents

Idiopathic pulmonary hemorrhage

• Occurs most commonly in children under the age of ten

• When it occurs in adults, it is twice as common in men

• Anti-glomerular basement membrane antibody should be absent (unlike Goodpasture’s)

Goodpasture’s syndrome

• Goodpasture’s includes renal disease

• Renal lesion is glomerulonephritis

• It is a disease of young adults

• Most are men

• Autoimmune etiology

• Both lung and renal pathology believed 2° to anti-glomerular basement membrane antibody cross reacting with lung basement membrane

X-ray

• Identical changes in both diseases

• Early in the disease, it is alveolar in nature, more prominent at the bases and perihilar regions — simulates pulmonary edema

• Within 2-3 days, the blood is absorbed in to the interstitium and the pattern changes to interstitial reticular

• By about 10 days, the reticular disease disappears

• With repeated bleeds, there is hemosiderin deposit in the lungs and progressive pulmonary fibrosis occurs

• Once this occurs, the new hemorrhage is superimposed on the old interstitial disease, so the reticular pattern remains rather than disappears when the blood is absorbed

• May have pulmonary hypertension

• May have hilar adenopathy

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