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Immunology

Goodpasture Syndrome
Laura Bergs, RN, BSN, CNN

G oodpasture syndrome, also


known as anti–glomerular basement
membrane disease, is a rare autoim-
Definition
Goodpasture syndrome, a disease
of the glomerular and alveolar base-
term Goodpasture syndrome is rarely
used in Europe; there the disorder is
known as Goodpasture disease.
mune disease. Patients with the syn- ment membranes, is characterized
drome experience a rapid progression by pulmonary hemorrhage and cres- Epidemiology
to renal failure and death if the dis- centic glomerulonephritis. It is asso- Goodpasture syndrome is diag-
ease is not recognized and treated ciated with serum antibodies to nosed in 1 in 1 million persons each
early.1-3 First described by Ernest glomerular basement membrane and year.4,8 Whites are affected more
Goodpasture during the influenza linear deposits of antibodies along than is any other race, and the inci-
pandemic of 1919, the syndrome has the glomerular and alveolar basement dence is slightly higher in men than
been extensively studied and has led membrane and may result in rapidly in women.1,4,6,8 The syndrome affects
to a better understanding of autoim- progressive glomerulonephritis.4,6 2 different age groups: persons in
munity.2,4,5 In this article, I review However, some confusion exists their mid 30s and persons in their
the pathophysiology, diagnosis, and between the terms Goodpasture syn- late 50s.1 With current therapy, sur-
treatment of the acute phase of drome and Goodpasture disease. vival through the acute phase is more
Goodpasture syndrome and describe Reisli et al7 note that Goodpasture than 90%.4 However, 2-year survival
the nursing interventions. A case disease is characterized by the pres- is less than 50%, and the mortality
study illustrates the multisystem ence of antibodies to glomerular rate in patients with chronic renal
failure encountered by patients with basement membrane, whereas the failure is even worse.9 End-stage renal
the syndrome. syndrome is the rapidly progressive disease develops in 40% to 70% of
glomerulonephritis and pulmonary patients who have nephritis mediated
hemorrhage alone. According to by antibodies to glomerular basement
Bolton,8 patients who have antibod- membrane.10 Glomerulonephritis, a
Online
ies to glomerular basement mem- potential consequence of Goodpas-
brane deposited in tissue have ture syndrome, is responsible for 10%
Goodpasture disease and patients to 15% of all patients with end-stage
To receive CE credit for this article, visit
the American Association of Critical-Care who have Goodpasture disease along renal disease in the United States.11
Nurses’ (AACN) Web site at http://www
.aacn.org, click on “Education” and select with glomerulonephritis and/or The syndrome has a genetic compo-
“Continuing Education,” or call AACN’s Fax On hemoptysis have Goodpasture syn- nent and has been linked to the HLA
Demand at (800) 222-6329 and request item
No. 1117. drome. Salama et al4 note that the DRBI 1501 region.8,12 Recurrence of
the syndrome is infrequent, and pul-
monary hemorrhage seldom occurs in
Author patients who do not smoke tobacco.1,7
Laura Bergs currently works as a family nurse practitioner for Eastern Nephrology Associ-
ates in New Bern, NC. Her experience in nursing includes critical care, nephrology, and Pathophysiology
home healthcare nursing. Patients with Goodpasture syn-
To purchase electronic or print reprints, contact The InnoVision Group, 101 Columbia, Aliso Viejo, CA 92656. drome have a type II hypersensitivity
Phone, (800) 809-2273 or (949) 362-2050 (ext 532); fax, (949) 362-2049; e-mail, reprints@aacn.org. reaction9 (Figure 1). A type II hyper-

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associated with ated with a history of smoking or
Antigens an increase in the exposure to other hydrocarbons,
expression of α3 including trichloroethane, carbon
collagen chains tetrachloride, xylene, and ethyl
in these base- ether.1 This exposure is thought to
attack Basement cell
Form antibodies ment mem- damage the basement membrane
membrane
branes allows and expose antigenic sites within the
affects access and for- lung, causing alveolar damage and
mation of anti- subsequent pulmonary hemorrhage.
Results in basement Kidney bodies.6 The initial signs and symptoms
membrane leaking Lung
In the kidney, consist of a flulike malaise followed
the basement by a rapid onset of microscopic
causing
membrane is the hematuria and proteinuria.1 More
principal selec- than half of patients with Goodpas-
Oliguria Renal and tive barrier of the ture syndrome have pulmonary signs
Hematuria respiratory
Proteinuria
glomerulus, pre- and symptoms, including cough,
causing failure
Pulmonary hemorrhage venting plasma mild shortness of breath, and hemop-
proteins, erythro- tysis.6,8 The skin appears pale because
Figure 1 Type II hypersensitivity reaction in Goodpasture cytes, leukocytes, of iron deficiency anemia.2 Renal
syndrome. and platelets manifestations can also include
from passing edema and hypertension, which can
sensitivity reaction occurs when anti- through into the rapidly progress to acute renal fail-
bodies are directed against specific nephron.13 Deposits of IgG, IgA, and ure.15 Acute renal failure may progress
antigens on cell surfaces or connective sometimes IgM antibodies in the to chronic renal failure, and the need
tissues. In Goodpasture syndrome, basement membrane break down for lifetime hemodialysis or a kidney
the antibodies attack the NC1 domain collagen and interrupt membrane transplant can be a manifestation of
of the α3 chain of type IV collagen integrity.3 A loss of membrane Goodpasture syndrome. Rarely,
located in the basement membranes integrity causes leakage of blood and patients also have arthritis, periph-
of the kidneys and alveoli.10 Basement a rapid inflammatory response, lead- eral neuropathy, uveitis, or leukocy-
membranes are thin structures that ing to proteinuria, hematuria, olig- toclastic vasculitis of the skin.1
form a barrier between epithelial uria, and alveolar damage with
cells and connective tissue. These subsequent pulmonary Differential Diagnosis
membranes, consisting of type IV hemorrhage.3 Activated monocytes Goodpasture syndrome mimics
collagen, laminin, proteoglycans, cause new moon–shaped formations many other conditions, including
entactin, and other proteins, assist known as crescents within the kidney; systemic vasculitis, Wegener granu-
in the maintenance of tissue differ- the formation is enhanced by the lomatosis, polyarteritis nodosa, sys-
entiation and function.2 Although attraction of interleukin-1 by fibro- temic lupus erythematosus, infection,
the hypersensitivity reaction affects blasts in the renal interstitium.2 and any cause of pulmonary hemor-
all collagen contained within the rhage with decreased renal function
body, organs containing alveolar and Clinical Manifestations known as pulmonary-renal syn-
glomerular basement membranes The clinical manifestations of drome.3,4 The occurrence of hemop-
are affected more than are other Goodpasture syndrome vary. In some tysis may be caused by other disease
organs containing type IV collagen.6 patients, pulmonary signs and symp- states, including atrioventricular fis-
This difference occurs because an toms occur weeks to months before tula, left ventricular failure, pul-
increase in the accessibility of epitopes renal manifestations are evident.14(p206) monary embolism, pulmonary
(antigen molecules that permit Commonly, signs and symptoms of hypertension, thrombocytopenia,
attachment of a matching antibody) Goodpasture syndrome are associ- bronchitis, cystic fibrosis, lung can-

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Immunology

Table 1 Differential diagnosis in Goodpasture syndrome

Findings on physical Findings on renal


Diagnosis and definition History examination Laboratory data biopsy

Goodpasture syndrome Smoker or exposure Weight loss Antibodies to GBM Linear deposits of IgG
Progressive glomerulonephritis to hydrocarbons Malaise Iron deficiency anemia antibodies in the
with hemoptysis Age in 30s or 50s Hemoptysis Proteinuria GBM on
White Perioribital edema Hematuria immunofluorescence
Crescents present
Diabetic nephropathy Dependence on insulin or Weight gain Proteinuria
May have greater
Renal involvement related to oral hypoglycemic agents Oliguria Decreased glomerular
incidence of bleeding
long-standing diabetes Hypertension Peripheral edema filtration rate
and complications
Cardiovascular disease Diminished Increased level of
after biopsy
peripheral pulses serum urea nitrogen
Increased creatinine
level
Alport syndrome Sensironeural hearing loss Bloody urine Hematuria Mixed nephritis with
Congenital glomerulonephritis starting in childhood Hearing loss Anemia foam cells on
Cataracts Visual disturbances Proteinuria immunofluorescence
Chronic renal failure Thickening and
beginning in second or splitting of GBM with
third decade no immune deposits18
Childhood disease17
Lupus nephritis Diagnosis of systemic Butterfly rash Antibodies to double- Mesangial and
Inflammatory disease of the lupus erythematosus Photosensitivity stranded DNA subepithelial
connective tissue within previous year Oral ulcers Antibodies to smooth immune deposits19
Fever Arthritis of 2 or more muscle
Joint pain digits Proteinuria
Malaise Skin rash from sunlight Decreased levels of C3
More frequent in young and C4 components
women than in other of complement
groups Antibodies to nuclear
antigens19

Abbreviation: GBM, glomerular basement membrane.

cer, systemic lupus erythematosus, mining if the blood is gastric or pul- Chest radiographs show bilateral
trauma, aortic aneurysm, and multi- monary. An acidic pH (<7.4) indi- infiltrates with apical sparing.6 If the
ple other causes.16 The most proba- cates a gastric source.16 The values on findings on chest radiographs are
ble cause of hemoptysis is bronchitis the diffusing capacity of carbon normal, a computed tomography
and primary lung disease.16 A com- monoxide test, used to determine scan may show minor parenchymal
plete analysis of the patient’s history, carbon monoxide uptake, are ele- involvement.6 Bronchoscopy may be
laboratory tests, and physical exami- vated if pulmonary hemorrhage is performed to rule out other causes
nation aids in the differential diag- present.2 Patients with Goodpasture of bleeding and determine the extent
nosis2 (Table 1). syndrome have decreased alveolar of lung involvement.16 Blood is often
gas volumes, total lung capacity, and visualized in the tracheobronchial
Diagnostic Studies vital capacity.6 (On pulmonary func- tree.6 Examination and culturing of
Table 2 gives typical laboratory tion tests, total lung capacity is equal bronchial washings are used to rule
values for patients with Goodpas- to the tidal volume plus expiratory out infectious causes of hemoptysis.16
ture syndrome. reserve volume plus residual volume Immunofluorescence of lung tissue
plus inspiratory reserve volume.20 reveals linear IgG staining of the
Pulmonary Tests Vital capacity is equal to the total basement membrane of the alveolar
Initial evaluation of the pH of the volume exhaled after maximum wall. On electron micrographs, lung
expectorated blood assists in deter- inspiration.) tissue has a thick basement mem-

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Table 2 Laboratory values in Goodpasture syndrome
Patients without Goodpasture syn-
drome are negative for these anti-
Value in Goodpasture bodies. All patients must fast 8 hours
Laboratory test Normal value syndrome
before a blood sample for the test is
Carbon monoxide 25 mg/min per m2 Increased
uptake obtained.20
Total lung capacity 5500 mL Decreased
Sonography and Renal Biopsy
Vital capacity 4000 mL Decreased Sonograms of patients with
24-hour urine 8.8-17.7 mmol/d (1-2 g/24 h) Decreased Goodpasture syndrome reveal normal-
creatinine sized kidneys.8 Renal biopsy specimens
Serum urea nitrogen 1.8-7.1 mmol/L (5-20 mg/dL) Elevated have linear deposits of IgG and fibrin
Serum creatinine Male 53-90 µmol/L (0.6-1.02 Elevated
along the glomerular basement mem-
mg/dL) brane in a crescent shape.8 A definitive
Female 44-97 µmol/L (0.5-1.1
mg/dL) diagnosis of Goodpasture syndrome is
made if a patient has circulating anti-
Hemoglobin Male 136-172 g/L Normal or decreased
Female 120-150 g/L bodies to glomerular basement mem-
brane and examination of renal biopsy
Hematocrit Male 0.42-0.52 Normal or decreased
Female 0.35-0.47 specimens reveals linear deposits of
IgG with crescent formation.11
White blood cell count 5 × 109/L to 10 × 109/L Normal or increased

Prothrombin time 10-15 seconds Normal or increased Other Baseline Tests


Partial thromboplastin Depends on laboratory Normal or increased A complete blood cell count with
time Standard <35 seconds
a manual differential is used to detect
Erythrocyte Age <50 years Normal or increased anemia and the white blood cell count.
sedimentation rate Male 15 mm/h
Female 20 mm/h In Goodpasture syndrome, the results
Age 50-85 years may or may not be normal (Table 2).
Male 20 mm/h
Female 30 mm/h Other tests may include prothrombin
time, partial thromboplastin time,
Antibodies to Negative Yes in 87% of cases
glomerular basement erythrocyte sedimentation rate, com-
membrane plete chemistry panel, blood cultures,
complement panel, enzyme-linked
brane with hyperplasia of type I and Measurements of the serum levels immunosorbent assay, and anti–
II pneumocytes.3,6 of urea nitrogen and creatinine are neutrophil cytoplasmic antibody test.3
used to determine the extent of renal The transferrin level may be assessed
Renal Studies function. The levels of both are ele- to detect iron deficiency anemia.
A urinalysis is necessary to deter- vated in patients who have rapidly
mine if albumin is present in the urine progressive glomerulonephritis. Treatment and
and to obtain the urinary concentra- Nursing Considerations
tion of creatinine. In Goodpasture Serum Antibodies to Table 3 is a potential care plan for
syndrome, albumin is present in the Glomerular Basement Membrane patients with Goodpasture syndrome.
urine because of the damage to the A blood test used to determine
glomerular basement membrane, the presence of circulating antibodies Pulmonary Measures
and the 24-hour urine creatinine to glomerular basement membrane In Goodpasture syndrome, pul-
concentration is decreased, indicat- is positive in 87% of patients with monary hemorrhage can be severe
ing decreased glomerular filtration Goodpasture syndrome.20 The results and iron deficiency anemia develops.4
rates.17 The urine sediment contains of this test can be falsely elevated in A blood loss of 600 mL/d increases
dysmorphic red cells and casts.8 patients receiving antibiotic therapy.20 mortality from 7% to 85%.16 This

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Immunology

Table 3 Major complications in


bodies to glomerular basement mem- nicians agree that therapeutic plasma
Goodpasture syndrome brane are no longer detected.1,11 Ther- exchange stops pulmonary hemor-
Uncontrolled bleeding
apeutic plasma exchange requires a rhage, although documentation is
large-bore dual-lumen catheter, and lacking.24 According to Salama et al,4
Pulmonary failure fluid replacement must be carefully pulsed methylprednisolone is inef-
Renal failure monitored to prevent fluid overload fective and is complicated by severe
and/or protein depletion. Hemody- infections. However, Couser11 has
Fluid overload and/or deficit
namic monitoring is necessary to help determined that pulmonary hemor-
Death determine and prevent decreased lev- rhage responds to pulse methylpred-
els of circulating fluid. nisolone therapy. Couser11 prefers to
Isotonic sodium chloride solution use therapeutic plasma exchange in
blood loss leads to anemia; there- may be administered if signs or symp- patients who have renal involvement.
fore, blood transfusions are neces- toms of rapid volume depletion occur Pulse methylprednisolone is usu-
sary to support proper hemoglobin during plasmapheresis.21 The amount ally given intravenously at a dose of
levels, reduce hypoxia, and prevent of plasma exchanged is 50 mL/kg 30 mg/kg every other day for a total
further circulatory collapse. Main- for a maximum exchange of 4 L/d.4 of 3 doses.8,11 None of the 3 doses
taining hemodynamic stability and The plasma removed is replaced with should exceed 3 g, and no diuretics
adequate oxygen delivery is para- a solution of 4% to 5% albumin, and may be given 3 hours before any dose.8
mount. Supportive airway manage- fresh-frozen plasma may be given at Once treatment with pulse methyl-
ment is necessary for adequate tissue the end of the exchange if active prednisolone is complete, patients
oxygenation. In acute hemoptysis bleeding is present or the patient has are started on oral doses of the drug
(blood loss of 600 mL/d), asphyxia- undergone a recent biopsy.4 Once of 1 mg/kg per day.11 Whether pulse
tion is the usual cause of death and the hemoptysis stabilizes, renal dys- or regular-dose methylprednisolone
airway clearance is essential to pre- function may progress and become is used, corticosteroid therapy is
vent further hypoxia.16 Bronchoscopy the next challenge. beneficial for patients with Good-
is used to detect the site of bleeding, pasture syndrome. Immunosup-
and if possible the lung involved Immunosuppression and pression therapy is adjusted
should be isolated from healthy lung Pulse Methyprednisolone according to the white blood cell
tissue by intubation of the contralat- Patients with rapidly progressive count and is continued for 12 to 18
eral lung or by separating the lungs glomerulonephritis leading to acute months after recovery from Good-
with a double-lumen endotracheal renal failure are treated with thera- pasture syndrome.14(p206)
tube.16 Ventilator management in peutic plasma exchange and immuno- For patients with severe renal
the acute respiratory phase is neces- suppression, which are effective in 80% impairment with a serum level of
sary to preserve optimal airway of cases.4,7 For immunosuppression, creatinine greater than 530 μmol/L
function and prevent further airway cyclophosphamide 40 to 50 mg/kg (6 mg/dL), long-term hemodialysis
decline. Hemoptysis may not always or 60 to 250 mg/m2 is given intra- and kidney transplantation are indi-
be alleviated. venously in divided doses over 2 to 5 cated.4 Chronic renal failure is com-
days and then orally in doses of 2 to mon in patients with Goodpasture
Therapeutic Plasma Exchange 4 mg/kg per day.22,23 Cyclophos- syndrome who have a moderate
Therapeutic plasma exchange, phamide is always given concurrently reduction in glomerular filtration
used to decrease the level of circulating with therapeutic plasma exchange to rate at the start of treatment.10
antibodies to glomerular basement prevent immunoglobulin rebound
membrane, along with administra- suppression.8 Conclusion
tion of pulse methylprednisolone, is Other clinicians prefer to use pulse Caring for patients with Good-
used to alleviate hemoptysis.8,16,21 methyprednisolone. Controversy pasture syndrome is stressful because
Patients have plasmapheresis daily exists regarding the effectiveness of of the high rate of complications
for a total of 14 days or until anti- pulse methylprednisolone. Most cli- associated with the disease (Table 4).

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Immunology

Table 4 Potential care plan for patients with Goodpasture syndrome

Diagnosis Desired outcome Intervention Comments/rationale for Intervention

Hypoxia related to No hypoxia Set ventilator to maintain PaO2 > Airway is unable to compensate
accumulation of fluid 90 mm Hg without ventilator
in alveoli Suction endotracheal tube as needed Removal of secretions keeps airway
to clear bloody secretions patent and prevents occlusion
Administer methylprednisolone Drug decreases inflammatory
response induced by antibodies
Treat with plasmapheresis Drug decreases level of antigen-
antibody complexes in the
basement membranes

Risk for infection related No infection Monitor temperature Increased temperature is a sign of
to invasive procedures infection
Monitor white blood cell count and White blood cell count and
erythrocyte sedimentation rate erythrocyte sedimentation rate
are elevated in infection
Use aseptic techniques when working Use of aseptic technique prevents the
with all invasive catheters introduction of bacteria into
catheter access

Fluid overload related to Fluid balance maintained Monitor intake and output Results indicate if intake and output
oliguria and proteinuria are in balance
Weigh patient daily Increases indicate if patient is gaining
excessive weight
Assess for pitting edema Results indicate if third spacing is
occurring
Monitor central venous pressure Central venous pressure is increased
in vascular overload and
decreased in volume deficit
Administer intravenous fluids as Adequate fluid intake must be
ordered maintained
Consider dialysis Dialysis may be the only option to
maintain fluid balance

Electrolyte imbalance Electrolyte balance Monitor plasma electrolyte levels Results indicate if electrolyte levels
related to ineffective maintained are within normal range
renal function Administer appropriate intravenous Inappropriate fluid can cause
fluids hyperkalemia, hypokalemia,
hypernatremia, and/or
hyponatremia

Anemia related to red No anemia Prevent blood loss Closed systems should be maintained
blood cell loss from to prevent blood loss
hemoptysis and hematuria Monitor red blood cell count Results indicate if patient has anemia
Suction endotracheal tube only when Suctioning only as needed prevents
necessary trauma and further bleeding
Administer packed red cells when Packed red cells help maintain normal
ordered red blood cell count and replace
loss

Use of therapeutic plasma exchange, members of the healthcare team and Goodpasture syndrome. Preparation
methylprednisolone, and cyclophos- collaborative team management in for lifetime hemodialysis may be
phamide and excellent critical care the treatment regimen, along with necessary, and associated lifestyle
nursing can improve outcomes in careful consideration for the concerns changes must be considered.
patients who have the syndrome. of patients’ families, may alleviate The prognosis for patients with
Open communication between all some of the stressors associated with Goodpasture syndrome has improved

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with the advent of plasmapheresis, and the serum level of creatinine uation. The bronchial sample con-
but the possibility of infection was 194 μmol/L (2.2 mg/dL). tained alveolar blood, numerous
related to the many invasive Two large-bore 14-gauge intra- hemosiderin-laden macrophages,
catheters and procedures and the use venous catheters were inserted, one and a mild inflammatory infiltrate.
of immunosuppressive agents can in each antecubital space, and 5 mg Blood serum was sent for assay for
cause adverse events. The benefits of of midazolam (Versed) was adminis- antibodies to glomerular basement
these treatments certainly outweigh tered as an intravenous bolus. membrane, and treatment with levo-
the associated risks. Careful monitor- Another 2 units of blood was trans- floxacin 250 mg intravenously every
ing and expert collaborative care is fused to alleviate the anemia. Frequent 6 hours was started.
essential for the survival of these clearing of bloody secretions from The pulmonologist suspected
patients. the endotracheal tube was required. Goodpasture syndrome and consulted
Ventilator settings included fraction with the nephrologist. They agreed
Case Study of inspired oxygen 0.60, positive to perform a renal biopsy to confirm
A 55-year-old woman came to end-expiratory pressure 5 mm Hg, their suspicions. The renal biopsy
the emergency department because and respirations 16/min. An electro- revealed linear IgG deposits with
she had shortness of breath with cardiogram revealed sinus tachycar- crescent formation (Figure 2). The
hemoptysis. The hemoptysis had dia, and a noninvasive automatic patient was started on daily plasma-
started 2 days earlier and was cuff monitor indicated a blood pres- pheresis for an exchange of 1 L/d. In
increasing in intensity. The patient sure of 90/60 mm Hg. An arterial addition, methylprednisolone was
did not smoke tobacco but was catheter was inserted to monitor given intravenously on odd days at a
exposed to secondary smoke from blood pressure and to obtain samples dose of 300 mg for 3 doses and then
her husband. She had allergies to for serial arterial blood gas analyses. daily at a dose of 100 mg/d. Cyclo-
ragweed and penicillin. While she The patient was typed and cross- phosphamide was given intravenously
was in the emergency department, matched for 2 more units of blood, at a dose of 400 mg/d. The hemopt-
severe hemoptysis with acute respi- and samples for arterial blood gas ysis stopped on the patient’s second
ratory failure developed. She was analysis were obtained. day in the critical care unit. The serum
intubated, treated with mechanical Consent was obtained for bron- level of antibodies to glomerular
ventilation, typed and cross-matched choscopy to determine the source of basement membrane was 35 mg/dL.
for 2 units of blood, and transferred bleeding, and bronchial washings On the third day in the critical
to the critical care unit. Results of were sent to the laboratory for eval- care unit, the patients was weaned to
arterial blood gas analysis were pH
7.33, PaO2 60 mm Hg, PaCO2 40 mm
A B
Hg, and bicarbonate 33 mmol/L,
indicating partially compensated
respiratory acidosis. The hematocrit
was 0.21, and the hemoglobin level
was 60 g/L. The platelet count was
low at 100 × 109/L. Prothrombin and
partial thromboplastin times were
normal. A chest radiograph showed
right-sided alveolar shadowing. The
serum concentration of potassium
was 5.5 mmol/L, indicating slight
hyperkalemia, and the total carbon Figure 2 Renal biopsy specimens. Left, High-power view of a glomerulus shows a
cellular crescent compressing the glomerular tuft and a small area of fibrinoid
dioxide content was 35 mmol/L. The necrosis. Right, Strong linear staining is evident along the glomerular basement
serum level of urea nitrogen was ele- membrane.
vated at 11.8 mmol/L (33 mg/dL), Reprinted from Salama et al,4 with permission from Elsevier Science.

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Immunology

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58 CRITICALCARENURSE Vol 25, No. 5, OCTOBER 2005

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Goodpasture Syndrome
Laura Bergs
Crit Care Nurse 2005;25 50-58
Copyright © 2005 by the American Association of Critical-Care Nurses
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