Pulmonary Hemorrhagic Disorders



The combination of hemoptysis, anemia, and diffuse pulmonary infiltrates along with the de­velopment of glomerulonephritis is known as Goodpasture’s syndrome. This is predominantly a disease of young white males. The etiology is unknown, but the presence of antiglomerular basement membrane antibodies lining both the glomerulus and the alveolus’ suggests an autoim­mune mechanism. While the lung disease may be intermittent, the kidney disease rapidly pro­gresses to renal failure. On occasion, hemoptysis by itself may be life-threatening. Bilateral neph­rectomy results in cessation of hemoptysis, but present therapy is directed at the presumed im­munological basis for the disease. Plasmapheresis is used to remove the antibodies, immunosup­pressive drugs are administered to decrease their production, and steroids are given empirically to decrease the pulmonary hemorrhage. Untreated patients usually die within two years.

Idiopathic pulmonary hemosiderosis can pre­sent similarly to Goodpasture’s syndrome, al­though it predominantly affects young girls and does not involve the kidneys. The etiology is un­known and there are no clear-cut immunological markers. Despite this, treatment similar to that for Goodpasture’s syndrome is usually attempted, al­though the efficacy in this disease is much less clear, and average survival is about two to three years.

lagen-vascular diseases, particularly SLE and per­iarteritis nodosa. It may also be seen with systemic vasculitis, in particular Wegener’s gran­ulomatosis, hypersensitivity vasculitis, mixed cryoglobulinemia, and Behget’s syndrome.

Finally, pulmonary hemorrhage, with or with­out renal disease, may accompany one of the col