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Immunology

Immunology Goodpasture Syndrome G oodpasture syndrome, also known as anti–glomerular basement membrane disease, is a

Goodpasture Syndrome

Immunology Goodpasture Syndrome G oodpasture syndrome, also known as anti–glomerular basement membrane disease, is a
Immunology Goodpasture Syndrome G oodpasture syndrome, also known as anti–glomerular basement membrane disease, is a

Goodpasture syndrome, also known as anti–glomerular basement membrane disease, is a rare autoim- mune disease. Patients with the syn- drome experience a rapid progression to renal failure and death if the dis- ease is not recognized and treated early. 1-3 First described by Ernest Goodpasture during the influenza pandemic of 1919, the syndrome has been extensively studied and has led to a better understanding of autoim- munity. 2,4,5 In this article, I review the pathophysiology, diagnosis, and treatment of the acute phase of Goodpasture syndrome and describe the nursing interventions. A case study illustrates the multisystem failure encountered by patients with the syndrome.

Online
Online

To receive CE credit for this article, visit the American Association of Critical-Care Nurses’ (AACN) Web site at http://www .aacn.org, click on “Education” and select “Continuing Education,” or call AACN’s Fax On Demand at (800) 222-6329 and request item No. 1117.

Laura Bergs, RN, BSN, CNN

Definition

Goodpasture syndrome, a disease of the glomerular and alveolar base- ment membranes, is characterized by pulmonary hemorrhage and cres- centic glomerulonephritis. It is asso- ciated with serum antibodies to glomerular basement membrane and linear deposits of antibodies along the glomerular and alveolar basement membrane and may result in rapidly progressive glomerulonephritis. 4,6 However, some confusion exists between the terms Goodpasture syn- drome and Goodpasture disease. Reisli et al 7 note that Goodpasture disease is characterized by the pres- ence of antibodies to glomerular basement membrane, whereas the syndrome is the rapidly progressive glomerulonephritis and pulmonary hemorrhage alone. According to Bolton, 8 patients who have antibod- ies to glomerular basement mem- brane deposited in tissue have Goodpasture disease and patients who have Goodpasture disease along with glomerulonephritis and/or hemoptysis have Goodpasture syn- drome. Salama et al 4 note that the

Author

Laura Bergs currently works as a family nurse practitioner for Eastern Nephrology Associ- ates in
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
home
healthcare nursing.
To purchase electronic or print reprints,
contact The InnoVision Group, 101 Columbia,
Aliso Viejo, CA
92656.
Phone,
(800)
809-2273 or
(949) 362-2050
(ext 532); fax, (949) 362-2049; e-mail,
reprints@aacn.org.

term Goodpasture syndrome is rarely used in Europe; there the disorder is known as Goodpasture disease.

Epidemiology

Goodpasture syndrome is diag- nosed in 1 in 1 million persons each year. 4,8 Whites are affected more than is any other race, and the inci- dence is slightly higher in men than in women. 1,4,6,8 The syndrome affects 2 different age groups: persons in their mid 30s and persons in their late 50s. 1 With current therapy, sur- vival through the acute phase is more than 90%. 4 However, 2-year survival is less than 50%, and the mortality rate in patients with chronic renal failure is even worse. 9 End-stage renal disease develops in 40% to 70% of patients who have nephritis mediated by antibodies to glomerular basement membrane. 10 Glomerulonephritis, a potential consequence of Goodpas- ture syndrome, is responsible for 10% to 15% of all patients with end-stage renal disease in the United States. 11 The syndrome has a genetic compo- nent and has been linked to the HLA DRBI 1501 region. 8,12 Recurrence of the syndrome is infrequent, and pul- monary hemorrhage seldom occurs in patients who do not smoke tobacco. 1,7

Pathophysiology

Patients with Goodpasture syn- drome have a type II hypersensitivity reaction 9 (Figure 1). A type II hyper-

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Antigens attack Basement cell Form antibodies membrane affects Results in basement membrane leaking Kidney Lung
Antigens
attack
Basement cell
Form antibodies
membrane
affects
Results in basement
membrane leaking
Kidney
Lung
causing
Oliguria
Renal and
Hematuria
respiratory
Proteinuria
failure
causing
Pulmonary hemorrhage
Figure 1 Type II hypersensitivity reaction in Goodpasture
syndrome.

sensitivity reaction occurs when anti- bodies are directed against specific antigens on cell surfaces or connective tissues. In Goodpasture syndrome, the antibodies attack the NC1 domain of the α 3 chain of type IV collagen located in the basement membranes of the kidneys and alveoli. 10 Basement membranes are thin structures that form a barrier between epithelial cells and connective tissue. These membranes, consisting of type IV collagen, laminin, proteoglycans, entactin, and other proteins, assist in the maintenance of tissue differ- entiation and function. 2 Although the hypersensitivity reaction affects all collagen contained within the body, organs containing alveolar and glomerular basement membranes are affected more than are other organs containing type IV collagen. 6 This difference occurs because an increase in the accessibility of epitopes (antigen molecules that permit attachment of a matching antibody)

associated with an increase in the expression of α 3 collagen chains in these base- ment mem- branes allows access and for- mation of anti- bodies. 6 In the kidney, the basement membrane is the principal selec- tive barrier of the glomerulus, pre- venting plasma proteins, erythro- cytes, leukocytes, and platelets

from passing through into the nephron. 13 Deposits of IgG, IgA, and sometimes IgM antibodies in the basement membrane break down collagen and interrupt membrane integrity. 3 A loss of membrane integrity causes leakage of blood and a rapid inflammatory response, lead- ing to proteinuria, hematuria, olig- uria, and alveolar damage with subsequent pulmonary hemorrhage. 3 Activated monocytes cause new moon–shaped formations known as crescents within the kidney; the formation is enhanced by the attraction of interleukin-1 by fibro- blasts in the renal interstitium. 2

Clinical Manifestations

The clinical manifestations of Goodpasture syndrome vary. In some patients, pulmonary signs and symp- toms occur weeks to months before renal manifestations are evident. 14(p206) Commonly, signs and symptoms of Goodpasture syndrome are associ-

ated with a history of smoking or exposure to other hydrocarbons, including trichloroethane, carbon tetrachloride, xylene, and ethyl ether. 1 This exposure is thought to damage the basement membrane and expose antigenic sites within the lung, causing alveolar damage and subsequent pulmonary hemorrhage. The initial signs and symptoms consist of a flulike malaise followed by a rapid onset of microscopic hematuria and proteinuria. 1 More than half of patients with Goodpas- ture syndrome have pulmonary signs and symptoms, including cough, mild shortness of breath, and hemop- tysis. 6,8 The skin appears pale because of iron deficiency anemia. 2 Renal manifestations can also include edema and hypertension, which can rapidly progress to acute renal fail- ure. 15 Acute renal failure may progress to chronic renal failure, and the need for lifetime hemodialysis or a kidney transplant can be a manifestation of Goodpasture syndrome. Rarely, patients also have arthritis, periph- eral neuropathy, uveitis, or leukocy- toclastic vasculitis of the skin. 1

Differential Diagnosis

Goodpasture syndrome mimics many other conditions, including systemic vasculitis, Wegener granu- lomatosis, polyarteritis nodosa, sys- temic lupus erythematosus, infection, and any cause of pulmonary hemor- rhage with decreased renal function known as pulmonary-renal syn- drome. 3,4 The occurrence of hemop- tysis may be caused by other disease states, including atrioventricular fis- tula, left ventricular failure, pul- monary embolism, pulmonary hypertension, thrombocytopenia, 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

               

Renal

long-standing

involvement

related

diabetes

to

oral

hypoglycemic

Hypertension

Cardiovascular

agents

disease

Oliguria

Peripheral

Diminished

edema

peripheral

pulses

Decreased

filtration

Increased

serum

urea

rate

level

glomerular

of

nitrogen

May

and

after

have

incidence

of

complications

biopsy

greater

bleeding

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

deposits 18

Childhood

disease

17

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

deposits

19

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

antigens 19

Abbreviation: GBM, glomerular basement membrane.

 

cer, systemic lupus erythematosus, trauma, aortic aneurysm, and multi- ple other causes. 16 The most proba- ble cause of hemoptysis is bronchitis and primary lung disease. 16 A com- plete analysis of the patient’s history, laboratory tests, and physical exami- nation aids in the differential diag- nosis 2 (Table 1).

Diagnostic Studies

Table 2 gives typical laboratory values for patients with Goodpas- ture syndrome.

Pulmonary Tests Initial evaluation of the pH of the expectorated blood assists in deter-

mining if the blood is gastric or pul- monary. An acidic pH (<7.4) indi- cates a gastric source. 16 The values on the diffusing capacity of carbon monoxide test, used to determine carbon monoxide uptake, are ele- vated if pulmonary hemorrhage is present. 2 Patients with Goodpasture syndrome have decreased alveolar gas volumes, total lung capacity, and vital capacity. 6 (On pulmonary func- tion tests, total lung capacity is equal to the tidal volume plus expiratory reserve volume plus residual volume plus inspiratory reserve volume. 20 Vital capacity is equal to the total volume exhaled after maximum inspiration.)

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Chest radiographs show bilateral infiltrates with apical sparing. 6 If the findings on chest radiographs are normal, a computed tomography scan may show minor parenchymal involvement. 6 Bronchoscopy may be performed to rule out other causes of bleeding and determine the extent of lung involvement. 16 Blood is often visualized in the tracheobronchial tree. 6 Examination and culturing of bronchial washings are used to rule out infectious causes of hemoptysis. 16 Immunofluorescence of lung tissue reveals linear IgG staining of the basement membrane of the alveolar wall. On electron micrographs, lung tissue has a thick basement mem-

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Table 2 Laboratory values in Goodpasture syndrome

 
                       

Value

 

in

 

Goodpasture

Laboratory

test

Normal

value

syndrome

Carbon

monoxide

 

25

mg/min

   

per

m

2

     

Increased

     

uptake

Total

lung

capacity

   

5500

 

mL

             

Decreased

 

Vital

capacity

   

4000

 

mL

             

Decreased

 

24-hour

urine

   

8.8-17.7

 

mmol/d

 

(1-2

 

g/24

h)

   

Decreased

 

creatinine

Serum

urea

nitrogen

   

1.8-7.1

 

mmol/L

 

(5-20

 

mg/dL)

 

Elevated

     

Serum

creatinine

   

Male

 

53-90

µmol/L

   

(0.6-1.02

 

Elevated

       

mg/dL)

Female

44-97

µmol/L

(0.5-1.1

mg/dL)

Hemoglobin

   

Male

 

136-172

 

g/L

     

Normal

 

or

 

decreased

Female

120-150

g/L

Hematocrit

   

Male

 

0.42-0.52

         

Normal

 

or

 

decreased

Female

0.35-0.47

White

blood

cell

count

5

×

10

9

/L

to

10

×

10

9

/L

 

Normal

 

or

 

increased

Prothrombin

time

   

10-15

 

seconds

         

Normal

 

or

 

increased

Partial

thromboplastin

   

Depends

   

on laboratory

       

Normal

 

or

 

increased

time

Standard

<35

seconds

Erythrocyte

 

Age

<50

 

years

         

Normal

 

or

 

increased

sedimentation

rate

Male 15

mm/h

Female

20

mm/h

Age

50-85

years

Male

20

mm/h

Female

30

mm/h

Antibodies

to

   

Negative

             

Yes

in

87%

of

cases

glomerular

basement

membrane

brane with hyperplasia of type I and II pneumocytes. 3,6

Renal Studies A urinalysis is necessary to deter- mine if albumin is present in the urine and to obtain the urinary concentra- tion of creatinine. In Goodpasture syndrome, albumin is present in the urine because of the damage to the glomerular basement membrane, and the 24-hour urine creatinine concentration is decreased, indicat- ing decreased glomerular filtration rates. 17 The urine sediment contains dysmorphic red cells and casts. 8

Measurements of the serum levels of urea nitrogen and creatinine are used to determine the extent of renal function. The levels of both are ele- vated in patients who have rapidly progressive glomerulonephritis.

Serum Antibodies to Glomerular Basement Membrane A blood test used to determine the presence of circulating antibodies to glomerular basement membrane is positive in 87% of patients with Goodpasture syndrome. 20 The results of this test can be falsely elevated in patients receiving antibiotic therapy. 20

Patients without Goodpasture syn- drome are negative for these anti- bodies. All patients must fast 8 hours before a blood sample for the test is obtained. 20

Sonography and Renal Biopsy Sonograms of patients with

Goodpasture syndrome reveal normal- sized kidneys. 8 Renal biopsy specimens have linear deposits of IgG and fibrin along the glomerular basement mem- brane in a crescent shape. 8 A definitive diagnosis of Goodpasture syndrome is made if a patient has circulating anti- bodies to glomerular basement mem- brane and examination of renal biopsy specimens reveals linear deposits of

IgG with crescent formation. 11

Other Baseline Tests A complete blood cell count with a manual differential is used to detect anemia and the white blood cell count. In Goodpasture syndrome, the results may or may not be normal (Table 2). Other tests may include prothrombin time, partial thromboplastin time, erythrocyte sedimentation rate, com- plete chemistry panel, blood cultures, complement panel, enzyme-linked immunosorbent assay, and anti– neutrophil cytoplasmic antibody test. 3 The transferrin level may be assessed

to detect iron deficiency anemia.

Treatment and Nursing Considerations

Table 3 is a potential care plan for patients with Goodpasture syndrome.

Pulmonary Measures In Goodpasture syndrome, pul- monary hemorrhage can be severe and iron deficiency anemia develops. 4

A blood loss of 600 mL/d increases

mortality from 7% to 85%. 16 This

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Immunology

Table 3 Major complications in Goodpasture syndrome

Uncontrolled

     

bleeding

 

Pulmonary

failure

Renal

failure

Fluid

overload

and/or

deficit

Death

blood loss leads to anemia; there- fore, blood transfusions are neces- sary to support proper hemoglobin levels, reduce hypoxia, and prevent further circulatory collapse. Main- taining hemodynamic stability and adequate oxygen delivery is para- mount. Supportive airway manage- ment is necessary for adequate tissue oxygenation. In acute hemoptysis (blood loss of 600 mL/d), asphyxia- tion is the usual cause of death and airway clearance is essential to pre- vent further hypoxia. 16 Bronchoscopy is used to detect the site of bleeding, and if possible the lung involved should be isolated from healthy lung tissue by intubation of the contralat- eral lung or by separating the lungs with a double-lumen endotracheal tube. 16 Ventilator management in the acute respiratory phase is neces- sary to preserve optimal airway function and prevent further airway decline. Hemoptysis may not always be alleviated.

Therapeutic Plasma Exchange Therapeutic plasma exchange, used to decrease the level of circulating antibodies to glomerular basement membrane, along with administra- tion of pulse methylprednisolone, is used to alleviate hemoptysis. 8,16,21 Patients have plasmapheresis daily for a total of 14 days or until anti-

bodies to glomerular basement mem- brane are no longer detected. 1,11 Ther- apeutic plasma exchange requires a large-bore dual-lumen catheter, and fluid replacement must be carefully monitored to prevent fluid overload and/or protein depletion. Hemody- namic monitoring is necessary to help determine and prevent decreased lev- els of circulating fluid. Isotonic sodium chloride solution may be administered if signs or symp- toms of rapid volume depletion occur during plasmapheresis. 21 The amount of plasma exchanged is 50 mL/kg for a maximum exchange of 4 L/d. 4 The plasma removed is replaced with a solution of 4% to 5% albumin, and fresh-frozen plasma may be given at the end of the exchange if active bleeding is present or the patient has undergone a recent biopsy. 4 Once the hemoptysis stabilizes, renal dys- function may progress and become the next challenge.

Immunosuppression and Pulse Methyprednisolone Patients with rapidly progressive glomerulonephritis leading to acute renal failure are treated with thera- peutic plasma exchange and immuno- suppression, which are effective in 80% of cases. 4,7 For immunosuppression, cyclophosphamide 40 to 50 mg/kg or 60 to 250 mg/m 2 is given intra- venously in divided doses over 2 to 5 days and then orally in doses of 2 to 4 mg/kg per day. 22,23 Cyclophos- phamide is always given concurrently with therapeutic plasma exchange to prevent immunoglobulin rebound suppression. 8 Other clinicians prefer to use pulse methyprednisolone. Controversy exists regarding the effectiveness of pulse methylprednisolone. Most cli-

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nicians agree that therapeutic plasma exchange stops pulmonary hemor- rhage, although documentation is lacking. 24 According to Salama et al, 4 pulsed methylprednisolone is inef- fective and is complicated by severe infections. However, Couser 11 has determined that pulmonary hemor- rhage responds to pulse methylpred- nisolone therapy. Couser 11 prefers to use therapeutic plasma exchange in patients who have renal involvement. Pulse methylprednisolone is usu- ally given intravenously at a dose of 30 mg/kg every other day for a total of 3 doses. 8,11 None of the 3 doses should exceed 3 g, and no diuretics may be given 3 hours before any dose. 8 Once treatment with pulse methyl- prednisolone is complete, patients are started on oral doses of the drug of 1 mg/kg per day. 11 Whether pulse or regular-dose methylprednisolone is used, corticosteroid therapy is beneficial for patients with Good- pasture syndrome. Immunosup- pression therapy is adjusted according to the white blood cell count and is continued for 12 to 18 months after recovery from Good- pasture syndrome. 14(p206) For patients with severe renal impairment with a serum level of creatinine greater than 530 μmol/L (6 mg/dL), long-term hemodialysis and kidney transplantation are indi- cated. 4 Chronic renal failure is com- mon in patients with Goodpasture syndrome who have a moderate reduction in glomerular filtration rate at the start of treatment. 10

Conclusion

Caring for patients with Good- pasture syndrome is stressful because of the high rate of complications 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

PaO 2

>

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, methylprednisolone, and cyclophos- phamide and excellent critical care nursing can improve outcomes in patients who have the syndrome. Open communication between all

members of the healthcare team and collaborative team management in the treatment regimen, along with careful consideration for the concerns of patients’ families, may alleviate some of the stressors associated with

56 CRITICALCARENURSE Vol 25, No. 5, OCTOBER 2005

Goodpasture syndrome. Preparation for lifetime hemodialysis may be necessary, and associated lifestyle changes must be considered. The prognosis for patients with Goodpasture syndrome has improved

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with the advent of plasmapheresis, but the possibility of infection related to the many invasive catheters and procedures and the use of immunosuppressive agents can cause adverse events. The benefits of these treatments certainly outweigh the associated risks. Careful monitor- ing and expert collaborative care is essential for the survival of these patients.

Case Study

A 55-year-old woman came to the emergency department because she had shortness of breath with hemoptysis. The hemoptysis had started 2 days earlier and was increasing in intensity. The patient did not smoke tobacco but was exposed to secondary smoke from her husband. She had allergies to ragweed and penicillin. While she was in the emergency department, severe hemoptysis with acute respi- ratory failure developed. She was intubated, treated with mechanical ventilation, typed and cross-matched for 2 units of blood, and transferred to the critical care unit. Results of arterial blood gas analysis were pH 7.33, PaO 2 60 mm Hg, PaCO 2 40 mm 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 × 10 9 /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 dioxide content was 35 mmol/L. The serum level of urea nitrogen was ele- vated at 11.8 mmol/L (33 mg/dL),

and the serum level of creatinine was 194 μmol/L (2.2 mg/dL). Two large-bore 14-gauge intra- venous catheters were inserted, one in each antecubital space, and 5 mg of midazolam (Versed) was adminis- tered as an intravenous bolus. Another 2 units of blood was trans- fused to alleviate the anemia. Frequent clearing of bloody secretions from the endotracheal tube was required. Ventilator settings included fraction of inspired oxygen 0.60, positive end-expiratory pressure 5 mm Hg, and respirations 16/min. An electro- cardiogram revealed sinus tachycar- dia, and a noninvasive automatic cuff monitor indicated a blood pres- sure of 90/60 mm Hg. An arterial catheter was inserted to monitor blood pressure and to obtain samples for serial arterial blood gas analyses. The patient was typed and cross- matched for 2 more units of blood, and samples for arterial blood gas analysis were obtained. Consent was obtained for bron- choscopy to determine the source of bleeding, and bronchial washings were sent to the laboratory for eval-

uation. The bronchial sample con- tained alveolar blood, numerous hemosiderin-laden macrophages, and a mild inflammatory infiltrate. Blood serum was sent for assay for antibodies to glomerular basement membrane, and treatment with levo- floxacin 250 mg intravenously every 6 hours was started. The pulmonologist suspected Goodpasture syndrome and consulted with the nephrologist. They agreed to perform a renal biopsy to confirm their suspicions. The renal biopsy revealed linear IgG deposits with crescent formation (Figure 2). The patient was started on daily plasma- pheresis for an exchange of 1 L/d. In addition, methylprednisolone was given intravenously on odd days at a dose of 300 mg for 3 doses and then daily at a dose of 100 mg/d. Cyclo- phosphamide was given intravenously at a dose of 400 mg/d. The hemopt- ysis stopped on the patient’s second day in the critical care unit. The serum level of antibodies to glomerular basement membrane was 35 mg/dL. On the third day in the critical care unit, the patients was weaned to

A

A B Figure 2 Renal biopsy specimens. Left, High-power view of a glomerulus shows a cellular

B

A B Figure 2 Renal biopsy specimens. Left, High-power view of a glomerulus shows a cellular

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 necrosis. Right, Strong linear staining is evident along the glomerular basement membrane.

Reprinted from Salama et al, 4 with permission from Elsevier Science.

CRITICALCARENURSE Vol 25, No. 5, OCTOBER 2005 57

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Immunology

a T-tube and a fraction of inspired

oxygen of 0.45. During the plasma- pheresis, severe hypotension devel- oped, necessitating discontinuation of the treatment. The patient was given 2 L of isotonic sodium chlo-

ride solution. Her body temperature increased to 38.9°C rectally, and blood cultures were positive for Staphylococcus aureus. Treatment with intravenous vancomycin at a dose of 1 g/12 h for 7 days was started.

Plasmapheresis was continued daily for a total exchange of 3 L. On day 6, the patient’s renal status dete- riorated; the serum level of urea nitro- gen was 23.2 mmol/L (65 mg/dL) and the serum level of creatinine was 389 μmol/L (4.4 mg/dL). Uri- nary output diminished to 60 mL in 8 hours. Central venous pressure was 16 mm Hg and pulmonary cap- illary wedge pressure was 8 mm Hg. Hemodialysis was started, and 1 L of fluid was removed during the first dialysis treatment. Central venous pressure after dialysis was 10 mm Hg with a pulmonary capillary wedge pressure of 6 mm Hg. The patient continued to require hemodialysis 3 d/wk in 4-hour increments, and her condition continued to improve. On day 10, her test for antibodies to glomerular basement membrane was negative, and her urea nitrogen and creatinine levels were 15.7 mmol/L (44 mg/dL) and 265 μmol/L (3.0 mg/ dL), respectively. She was extubated on that day and placed on fluid restriction with sips and chips. Her renal function continued to improve with dialysis, and she was started on

a full liquid diet on day 11. On day

13, her serum urea nitrogen was 8.2 mmol/L (23 mg/dL), and her serum

level of creatinine was 97 μmol/L

(1.1 mg/dL), and she was transferred out of the critical care unit. She continued to improve and was discharged from the hospital on day 21. At that time, her urine out- put was 2 L/d, her serum urea nitro- gen was 6.1 mmol/L (17 mg/dL), and her serum creatinine was 80 μmol/L (0.9 mg/dL). At the time of discharge, she was taking oral methylprednisolone at 10 mg/d and oral cyclophosphamide at 100 mg twice daily. She did not require long- term hemodialysis and was to follow up with her primary care physician.

Acknowledgments

The insignificant characteristics of the case study have been changed for confidential reasons, and any resemblance to a real person is coincidental. A special thank you to Dr Brigid Lusk; without her assistance and mentoring, this article would not have been possible.

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