Bleeding from pulmonary
capillaries occurs in many settings.
pulmonary hemorrhage is generally a complication of other diseases, such
as mitral stenosis or rarely infection.
It also occurs in Goodpasture’s
Hemorrhage is common at autopsy in severe
heart failure where it probably results from a combination of hemodynamic
factors and alveolar injury.
In thrombocytopenic patients minor alveolar
injury can result in hemorrhages in leukemic patients.
There remains a small group of patients in
whom repeated episodes of pulmonary hemorrhage occur in the absence of any
discernible predisposing illness.
Boston pathologist, was investigating the autopsy findings in influenza
pandemic deaths just after World War I.
In one case in which death
occurred several weeks after the attack of influenza, the lung showed
extensive bilateral hemorrhage, together with necrotizing angiitis of the
systemic vessels and glomerulonephritis.
Since then the term Goodpasture’s
syndrome has been applied to the condition of diffuse bilateral
pulmonary hemorrhage accompanied by rapidly progressive glomerulonephritis.
Antiglomerular Basement Membrane Disease:
disease has now become synonymous with the variant of pulmonary hemorrhage
associated with circulating antiglomerular baement membrane antibodies.
In this disease antibodies are
visualized in the kidney (and less well in the lung) by immunoflourescence
as linear staining of the basement membrane.
Although the patients may be adults of
any age, they are typically young men and present with hemoptysis, dyspnea,
and acute renal disease.
Either the renal symptoms or the
pulmonary symptoms may come first.
As in Wegener’s granulomatosis, the
renal symptoms are more important than the pulmonary symptoms for the
Anemia from pulmonary bleeding is
Radiographic examination reveals
diffuse bilateral alveolar filling.
The diagnosis is made on the basis of
Histologically, the alveoli are filled
with red blood cells and there is suggestive evidence of "alveolitis" in
the form of neutrophils in and around alveolar capillaries.
Radiologically, the lesions involve
rapidly in a matter of days as the red cell lyse and are then
At this stage hemosiderin-laden
macrophages are found in the alveolar space and in the interstitium.
On gross examination, the lungs are
dark red and heavy in the acute phase and rusty brown when the red cells
Treatment by bilateral nephrectomy has
been superseded by the administration of cytotoxic drugs. Plasmapheresis
has also been used to remove the antibodies. The disease is no longer
uniformly fatal. The basal laminae of the capillaries in the lungs and the
glomuruli are thought to have common antigenic determinants that are
targets for antibodies elicited by a primary lung insult or kidney injury.
The resulting damage to the basal laminae produces alveolitis and
hemorrhage in the lung and rapidly progressive glomerulonephritis in the
Other Forms of Intrapulmonary Hemorrhage:
clinical and pathologic syndrome occurs in the absence of circulating
antiglomerular basement membrane antibodies in about half the cases of
otherwise typical Goodpasture’s syndrome.
A similar syndrome may also
occur in the collagen-vascular diseases.
As in Goodpasture’s syndrome, necrotizing angiitis may be an associated feature.
granulomatosis is present (Example: upper respiratory tract lesions or
infectious granulomas of the lung).
Systemic lupus erythematosus may also
present as pulmonary hemorrhage.
pulmonary hemorrhage, usually old, is commonly found in patients with
The renal failure may be due to several other causes. Only
those with rapidly progressive glomerulonephritis should be regarded as
examples of Goodpasture’s syndrome.
interest to the pediatrician,idiopathic pulmonary hemorrhage presents as hemoptysis and anemia in patients under 20 years of age.
features are similar to those of Goodpasture’s syndrme, but this disease
differs in that renal disease is absent, the pulmonary hemorrhages are
recurrent and intermittent, and the course is much more protracted or the
disease remits spontaneously.
Because of the chronicity of the course, the
morphologic lesions differ from those in Goodpasture’s syndrome.
the acute lesions are similar, acute alveolitis may not be present, and
there may discontinuity of the alveolar basement membranes.
With repeated hemorrhages the elastic
tissue of the alveolar walls and blood vessels becomes coated with iron
and calcium (presumably dystrophic) and is rendered basophilic.
Fragmentation of elastic fibers leads to
foreign body giant cell reaction. The etiology is unknown.