Hemoptysis and Anemia in a 12-Year Old
Radiology Cases in Pediatric Emergency Medicine
Volume 3, Case 7
Craig T. Nakamura, MD
Kapiolani Medical Center For Women And Children
University of Hawaii John A. Burns School of Medicine
This is a 12 year-old female who presented to the
emergency department with pallor and hemoptysis.
Seven days ago she presented to her pediatrician with
a complaint of fever, cough, and rhinorrhea. She was
evaluated and diagnosed with a viral upper respiratory
tract infection. Over the next six days, the cough
persisted and she noticed that her sputum was
streaked with blood. She continued to cough up bloody
secretions, which eventually became coffee-ground in
nature. Her mother was concerned that she "looked
pale" and brought her to the emergency department.
She denies any sore throat. She was seen one
month ago for impetigo and was treated with topical
antibiotics. This resolved and she was well until her
recent onset of symptoms. She has also noticed coffee
colored urine over the past three days. There is no
history of ill contacts or gastroenteritis. She has been
afebrile for four days.
Exam: VS T37.0, P120, R28, BP 145/78, oxygen
saturation 93% in room air. Her oxygen saturation
increases to 100% on some oxygen by nasal prongs.
She is awake, but tired appearing. No acute distress.
Skin: Pallor is noted. No bruising or petechiae.
Normal turgor. Pupils equal and reactive. EOM's full.
Conjunctiva pale. Sharp optic discs. Moist mucous
membranes. No oral erythema or exudates. Neck
supple without adenopathy. Lungs: Good aeration
bilaterally. Bibasilar inspiratory crackles. No wheezing.
Abdomen: Benign. Extremities: No rash or lesions.
Neurologic: Normal. Pulses are good.
Her laboratory workup revealed a CBC with a
normocytic, normochromic anemia. Her hemoglobin
was 4.0, hematocrit 12.2, and reticulocyte count 6.0%.
Her platelets were 300,000. Her WBC was 8,400 with
no left shift. Electrolytes were normal, however, her
BUN was 32 and her creatinine 2.3. Her urinalysis is
significant for many red blood cells. SG 1.011, pH 6.0,
6-10 WBC's, >100 RBC's, 3-5 granular casts per HPF.
A chest radiograph is obtained.
View chest radiograph.
Her chest radiograph shows bilateral diffuse hazy
infiltrates with a fine nodular appearance. The lateral
view showed a small amount of fluid or thickening in the
fissures of the right lung. This type of radiographic
finding in conjunction with hemoptysis suggests the
presence of a pulmonary hemorrhage. Pulmonary
hemosiderosis is generally confirmed by finding
hemosiderin laden macrophages in the sputum. Just
based on the CXR alone, the differential includes viral
pneumonia, mycoplasma pneumonia, fungal
pneumonia, pulmonary edema, pulmonary
lymphagiectasia, hemangiomatosis, pulmonary
hemosiderosis, and pulmonary fibrosis (lymphoma,
sarcoidosis, leukemia, tuberous sclerosis, chronic
aspiration, and cystic fibrosis).
After the initial admission, stabilization, and
treatment, further laboratory studies were drawn. Her
anti-basement membrane serology was positive. Her
ANA was 5,120 (high), but her other lupus serologies
were all negative. Anti-streptolysin and Coombs were
both negative. C3, C4, and CH50 were all normal. In
light of her pulmonary hemorrhage, glomerulonephritis,
and positive anti-basement membrane serology, a
presumptive diagnosis of Goodpasture's syndrome is
In 1919, Ernest Goodpasture, a Harvard pathologist,
analyzed autopsy cases during an influenza epidemic.
He described a case of an 18 year-old male with
hemoptysis and renal insufficiency after having
influenza (1). The syndrome of pulmonary hemorrhage
and glomerulonephritis became known as
Goodpasture's syndrome. In 1967, it was noted that
anti-glomerular basement membrane antibodies were
found in cases of the disease (1). Goopasture's
syndrome is now defined as a triad of: a) pulmonary or
alveolar hemorrhage (defined as hemoptysis,
unexplained anemia, and diffuse alveolar infiltrates on
chest x-ray) (2), b) glomerulonephritis (cresentic or
rapidly progressive), and c) serum anti-basement
membrane antibodies or linear immunofluorescence of
IgG on basement membranes.
Goodpasture's syndrome (GS) affects persons of all
ages and races, with an average age of onset of 27 (3).
Men are affected more commonly than women (about 7
to 1) (3). Although it may occur at any time of the year,
seasonal patterns have been reported (winter and early
summer). The etiology remains unclear, however,
numerous genetic and environmental relationships have
been described. The pathologic mechanism of GS is
thought to stem from antibody mediated injury of type
IV collagen (the backbone basement membranes) (4).
Alveolar and glomerular basement membranes are
thought to be affected more than other type IV collagen
containing organs (ie. skin, eye, and choroid plexus)
because of increased accessibility of epitopes due to an
increased expression of alpha-3 collagen chains in
these basement membranes (4), allowing access and
formation of antibodies. In addition, disease states
such as infection, fluid overload, and increased O2
requirement causes capillary leakage and further
accessibility of the basement membranes.
In GS, the pulmonary symptoms and signs always
precede the renal symptoms. The initial manifestations
are usually a cough, mild shortness of breath, and
hemoptysis. Hypoxia may occur. There are decreased
alveolar gas volumes, and subsequently decreased
total lung and vital capacities (5). The renal
involvement follows the pulmonary symptoms. This
may range from nearly normal to a focal proliferative or
necrotizing glomerulonephritis. Other systemic
symptoms are fever and arthralgias. Pallor may occur
as an iron-deficiency anemia develops secondary to
The differential diagnoses of GS includes all other
pulmonary-renal syndromes, most of which are
vasculitic (Wegener's granulomatosis, polyarteritis
nodosa, systemic lupus erythematosis, lymphomatoid
granulomatosis, Henoch-Schonlein purpura, hemolytic
uremic syndrome, scleroderma, rheumatoid arthritis,
mixed connective tissue disease, drug-induced
vasculitis, giant-cell arteritis, and idiopathic rapidly
progressive glomerulonephritis) (5).
The diagnosis of GS should be made expeditiously,
for it can progress rapidly to fatal pulmonary or renal
failure. Diagnostic studies should consist of a complete
blood count, creatinine, sedimentation rate, serum
antibodies, and urine creatinine and protein. A chest
radiograph should be performed. The acute air space
involvement may have a patchy, diffuse, or perihilar
appearance. The infiltrates are usually bilateral with
apical sparing. Air bronchograms may be present (6).
Two to three days later, the blood begins to be
reabsorbed and the chest radiograph now takes on an
interstitial reticular appearance. With continual
hemorrhage there may be fibrosis. Several studies
have shown that a CT may detect minor parenchymal
involvement despite a normal chest radiograph (6). The
use of MRI in pulmonary hemorrhage has also been
described (increased signal density on T1 and
decreased on T2 secondary to the physical
characteristics of pulmonary hemosiderin deposits) (6).
On bronchoscopy, blood is often visualized in the
tracheobronchial tree. Although this is nondiagnostic, a
positive bronchoalveolar lavage with Prussian blue
staining (iron containing alveolar macrophages) is
diagnostic of pulmonary hemosiderosis (7). Biopsy
shows intra-alveolar hemorrhage with hemosiderin
laden macrophages. Electron microscopy shows a
thickened basement membrane with hyperplasia of type
I and II pneumocytes (4). The renal basement
membrane is also widened. There is hypertrophy of the
cells with loss of foot processes and IgG deposits.
Once the diagnosis is made, treatment should be
initiated. The patient may need an initial RBC
transfusion if clinically unstable. Hypertension may also
need to be treated. In addition, other factors which may
cause further alveolar damage (ie. infection or
pulmonary edema) must be treated. Once stabilized,
there are two objectives of therapy: 1) remove all
anti-GBM antibodies and 2) prevent their resynthesis by
immunosuppression with steroids and cytotoxic drugs
(3). The first objective is accomplished by
plasmapheresis. The second objective is met by
treatment with corticosteroids and cyclophosphamide.
Its dosage is titrated to the WBC count (1, 4). The
success of therapy can be monitored by serial
anti-GBM titers (3). The response to treatment and
course of GS is quite variable and may range from
long-term remissions to chronic pulmonary hemorrhage
with fibrosis and renal failure requiring hemodialysis or
1) Jones DA, Jennette JC, Falk RJ.
Goodpasture's syndrome revisited. North Carolina
Medical Journal 1990;51(8):411-415.
2) Rezkalla MA, Simmons JL. Idiopathic
pulmonary hemosiderosis and alveolar hemorrhage
syndrome: case report and review of the literature.
South Dakota Medical Journal. March 1995:79-84.
3) Glassock RJ, Brenner BM. Immunopathogenic
Mechanisms of Renal Injury. In: Braunwald E,
Isselbacher KJ, Petersdorf RG, Wilson JD, Martin JB,
Fauci AS (eds). Harrison's Principles of Internal
Medicine, eleventh edition, New York, McGraw-Hill,
Inc., 1987, pp. 1185-1186.
4) Kelly PT, Haponik EF. Goodpasture
syndrome: molecular and clinical advances. Medicine
5) Prakash UBS. Renal Diseases. In: Baum GL,
Wolinsky E (eds). Textbook of Pulmonary Diseases,
fifth edition. Boston, Little Brown & Company, 1984,
6) Muller NL, Miller RR. Diffuse pulmonary
hemorrhage. Radiology Clinics of North America
7) Duffy TP. The Red Baron. New England
Journal of Medicine 1992;327(6):408-411.
Return to Radiology Cases In Ped Emerg Med Case Selection Page
Return to Univ. Hawaii Dept. Pediatrics Home Page