GOODPASTURE

SYNDROME
CASE STUDY
Kristen Pastore Spring
2014
What is Goodpasture
Syndrome?
Uncommon, pulmonary-renal autoimmune
disorder
Can cause rapidly worsening kidney failure
and lung disease
Can involve all, but not always, of the
following:
Glomerulonephritis
Presence of anti-glomerular basement membrane
antibodies (anti-GBM antibodies)
Pulmonary hemorrhage
Also known as anti-GBM disease
Founded in 1919 by American pathologist and
physiologist Ernest Goodpasture
Goodpasture Syndrome:
Pathophysiology
Immune system
produces antibodies to
fight off infections and
bacteria
Healthy Body
Immune system produces anti-
GBM antibodies that attack the
healthy membranes in the lungs
and the glomerular basement of the
kidneys which can lead to bleeding
in the lungs, kidney damage, and
inflammation of the kidneys blood
vessels
Goodpasture
Syndrome
Goodpasture Syndrome:
Pathophysiology
Goodpasture Syndrome:
Causes
Genetics
Smoking
Viral infections
Exposure to hydrocarbon fumes/solvents
Exposure to metallic dust
Use of hair dyes
Certain drugs, such as cocaine

*Causes are still not fully understood*
Who does Goodpasture Syndrome
effect?
Most commonly effects:
Caucasians
Males more often than females
Age groups 20-30 and 60-70 years old

The incidence of Goodpasture syndrome is
approximately 0.5-1.8 cases per million per
year

Goodpasture Syndrome:
Symptoms
Dry cough
Hemoptysis (coughing up
blood)
Shortness of breath
Hematuria
Burning sensation during
urination
Proteinuria
Decreased urine production
High blood pressure
Edema in any of the body parts
(especially legs)
N/V
Pale skin
Lung Symptoms Kidney Symptoms
First common signs include fatigue, weakness, nausea,
and loss of appetite
Goodpasture Syndrome:
Diagnosis
1. Blood test
Can show the presence of anti-GBM antibodies
2. Urinalysis
Can test for presence of protein or blood in urine
3. Physical exam
Can determine high BP and fluid overload
4. Chest X-ray or Lung Biopsy
Can show any abnormalities in the lungs if
present
5. Kidney Biopsy
Can see if any kidney tissue is damaged by anti-
GBM antibodies
Goodpasture Syndrome:
Diagnosis
Goodpasture Syndrome:
Treatment
Plasmapheresis - form of treatment that
cleanses the blood by removing the harmful
antibodies, which may reduce inflammation in
kidneys and lungs
Medications
Immunosuppressive Medications (i.e.,
Cyclophosphamides) - can keep body from making
antibodies
Corticosteroids (i.e., Prednisone) - control bleeding
in lungs and suppress bodys autoimmune response
Antibiotics treat infections
Mechanical Ventilation or Supplemental Oxygen
Hemodialysis or Kidney Transplantation

Goodpasture Syndrome:
Medical Nutrition Therapy
Nutrition and Diet usually cannot prevent or cause
Goodpasture syndrome
Those with Goodpasture syndrome will usually have to
make changes for lower kidney function and high BP
Generally follow diet for chronic kidney disease
Low K+, low phosphorus, low Na, fluid restriction
Low-moderate protein if not on HD = 0.6-0.8 g/kg BW
High protein if receiving HD = >1.2 g/kg BW
50-60% kcal CHO <30% kcal FAT %PRO and total
grams varies
800-1000mg
Phosphorus
2-3 g Potassium <2000 mg Sodium
Goodpasture Disease: Survival
Rate
In the past Goodpasture syndrome was
usually fatal
Prognosis has been greatly improved with
aggressive treatment of plasmapheresis,
immunosuppressives, and
corticosteroids medications
With proper treatment, 5-year survival rate
exceeds 80% and less than 30% require
dialysis
Meet my Patient: A.A.
Meet my Patient: A.A.
A.A. is a 66-year old female
A.A. was admitted to Winthrop University
Hospital on 1/24/14 (was still currently
admitted as of 3/28/14)
Admitting Dx in ER: acute renal failure
complained of lower left quadrant pain and
weakness
BUN and Creatinine
PMHx: Hypertension

A.A.: Psychosocial Hx
White, Polish, Middle-class
Retired hairdresser
Speaks English and Polish fluently
Married and lives with husband in home in East
Meadow
Has 2 grown daughters and several grandchildren
No handicaps ambulates on own
Smoked one pack cigarettes/day x20 years and
quit 25 years ago
Drinks alcohol occasionally; no drug use

Timeline: 1/24/14 2/4/14
1/24/14
Admitted to
Winthrop
1/27/14
Initial
Nutrition
Assessment
1/30/14
Dx with
Goodpasture
Syndrome
2/4/14
My First
Encounter
with A.A.
Initial Nutrition Assessment:
1/27/14
Admitting Dx: Acute Renal Failure
Pertinent Meds: Prednisone, Flagyl, Zofran, Vitamin D, Calcium
Carbonate
Labs: Glucose 172, BUN 100, Creatinine 5.0, K+ 4.3
WNL, Phosphorus 6.8, Albumin 3.4, HgbA1C
6.0%, gFR 8
Diet Order: Renal 90g protein, 2g K+, 2g Na, Low Phos, Consistent
CHO, 1200 Fluid Restriction
Anthropometric
s:
Ht: 55 Wt: 206# UBW:~205# IBW:125+10%
BMI: 34.2 Adj. BW= 157#
Estimated
Needs:
Calories = 1785-2140 kcal (25-30 kcal/kg adj. BW)
Protein = 57-71 g protein (0.8-1.0 g pro/kg adj. BW)
Fluids = ~1400 ml (20 ml/kg adj. BW)
PES: Altered nutrition related lab values (NC-2.2) related to
pulmonary renal syndrome as evidenced by BUN 100
and Creatinine 5.0
Timeline: 1/30/14
Renal Biopsy performed and A.A. was found to be
positive for renal failure and positive for anti-GBM
antibodies
Urinalysis showed +2 protein in urine
Blood work showed presence of anti-
myeloperoxidase, which is a marker for ANCA
vasculitis
Dx with Goodpasture syndrome - at this time
only renal involvement with no pulmonary
symptoms.
Bilateral leg edema as well as bacteremia,
anemia, and sacral decubitus
Timeline: 1/31/14
Put on cyclophosphamides for treatment
A.A. began two rounds of plasmapheresis and
continued for the next 9 straight days
resulted in improvement in her antibody levels
but renal function was worsening
My First Encounter with A.A.:
2/4/14
BUN increased to 165 and Creatinine increased
to 5.8
Weight = 220#; 11# and 5% gain since
admission 11 days prior likely 2 bilateral leg
edema?
Good po consuming ~75-100% of meals
A.A. expressed feelings of helplessness,
sadness, worry and concern about her medical
status
Suggested change diet to 90g 60 g protein
Timeline: 2/6/14 - 2/20/14
2/6/14
First episode of hemoptysis key pulmonary symptom of Goodpasture
syndrome
2/7/14
Tesio catheter placed
Began Hemodialysis received HD for the next 4 days and began every
other day after that with plasmapharesis on the days in between
2/12/14
Diet changed to Renal with 60 grams protein (6 days later after
recommending diet change) now on HD so protein needs are higher
Significant decrease in urine production
2/20/14
Tested positive for Urosepsis
Timeline: 2/21/14 - 2/25/14
2/21/14
Significant decline in health and medical status
Significant decrease in appetite consuming ~25% meals
Weight = ~203#; 17# and 11% decrease from two weeks prior
2 fluid losses, decreased po, post-HD weight loss?
2/23/14
A.A. had a seizure that was witnessed by rheumatology and resolved on
its own
Started on Levetiracetam, antiepileptic med
NPO x 3days
Transferred to medical ICU for closer supervision
A.A. became increasingly more confused, agitated, lethargic, and non-
verbal after seizure
2/25/14
A.A. received a lumbar puncture which showed seizure was
likely 2 pulmonary infection
Timeline: 2/28/14
A.A. not eating, sedated, intubated, non-responsive
Suggested Nepro @ goal rate of 40 cc/hr
provides 1728 kcal, 77 grams protein, and 960 ml
meeting needs
started on Nepro @ 15 cc/hour and titrated to goal rate
of 40 cc/hour via NG tube
Tolerating well, no residuals
Developed Stage II sacral pressure ulcer, gluteal
fold, sacrum excoriated
Put on Nephro-vite MVI
Renal labs trending downwards
BUN 50, Creatinine 3.0
Timeline: 3/2/14 3/6/14
Nepro TF was increased to 60 cc/hr x 5 days
providing 2592 kcal, 117 grams protein, 1440 ml
exceeding needs
+Diarrhea (non-C.diff) 2 not tolerating higher
rate?
Bilateral leg edema decreased+1 pitting edema
Now with facial edema as well
Plasmapheresis treatment discontinued
A.A. still undergoing hemodialysis; remains
intubated, unresponsive, sedated

Timeline: 3/8/14
NPO x1 day for another lumbar puncture
which indicated respiratory failure and Herpes
Encephalitis
Put on Acyclovir which is an aggressive form of
antiviral treatment
Nepro TF resumed and decreased back to
goal rate 40 cc/hr
Added Prostat 30 ml x1 to provide additional
101 kcal, 15 grams protein
TOTAL = 1829 kcal, 92 grams protein

Timeline: 3/17/14
NPO x 1day for tracheostomy and placement of
mechanical ventilator. A.A. was put on
intermittent ventilation with trach collar
NG was getting clogged and A.A. was receiving
poor feedings
NG tube was removed and PEG was placed
Nepro TF resumed at 40 cc/hr
Weight = ~228#; 25# and 12% increase from 1
month ago
Anthropometrics
1/27/14 2/4/14 2/21/14 3/17/14
Height 55 55 55 55
Weight 206# 220# 203 # 228#
BMI 34.3 36.6 33.8 37.9
UBW= 205%
%UBW
100% 107% 99% 111%
IBW=137#
%IBW
150% 160% 148% 166%
Weight fluctuations likely 2 fluids shifts
o bilateral leg edema, facial edema
A.A. eating well then decreased appetite and poor po
Unsure if weights taken pre- or post-dialysis?

Nutritional Analysis:
Hospital Recall 2/3/14
Breakfast corn flakes cereal with 4 oz. whole
milk, egg beaters, 1 slice whole wheat bread
with margarine, coffee with equal and half &
half
Lunch turkey sandwich on white bread with
diet mayonnaise, cooked carrots, diet fruit
cocktail, diet ginger ale
Dinner baked tilapia, white rice, peas and
pearl onions, wheat roll with margarine, diet
peaches, diet ginger ale
Snack diet oatmeal raisin cookies
Nutritional Analysis:
Hospital Recall 2/3/14
Nutrient Actual
Intake
Recommended Amount
for Diet/A.A. Needs
Kcalories 1478 kcal ~1785-2140 kcal (based
off 25-30 kcal/kw adj
BW)
Protein 76 g ~57-71 g (based off 0.8-
1.0g/kg adj BW)
% kcal varies
Fat 43 g <30% kcal
Carbohydrat
e
203 g 50-60% kcal
Potassium 1.8 g 2-3 g
Phosphorus 760 mg 800-100 mg
Sodium 2760 mg <2000 mg
Fluid 780 ml (?) 1200 ml
% calories
Protein
CHO
Fat
Diet Order:
Renal 90 grams protein,
2g K, 2gNa, Low
phosphorus, 1200 ml Fluid
Restriction
*Not on hemodialysis at
time*
Nutritional Analysis:
Tube feeding 2/28/14
Diet Order:
Nepro TF 40cc/hr
*On hemodialysis at time*
Nutrient Actual
Intake
Recommended Amount
for Diet/A.A. Needs
Kcalories 1728 kcal ~1785-2140 kcal (based
off 25-30 kcal/kw adj
BW)
Protein 77 g ~87 g (based off 1.2g/kg
adj BW)
% kcal varies
Fat 92 g <30% kcal
Carbohydrat
e
155 g 50-60% kcal
Potassium 1.0 g 2-3 g
Phosphorus N/A 800-100 mg
Sodium 1018mg <2000 mg
Fluid 700 ml 1200 ml
% calories
Protein
CHO
Fat
Medications
Medication Nutritional Indications
Flagyl
(antibiotic)
Nutr: Anorexia; Oral/GI: N/V, metallic taste, epigastric distress, diarrhea,
constipation
Zofran
(antiemetic,
antinauseant)
Oral/GI: Dry mouth, abdominal pain, constipation, diarrhea
Prednisone
(corticosteroid, anti-
inflammatory,
immunosuppressant)
Drug: take with food; Diet: Na, Ca, Vit D, pro. Caution with grapefruit
Oral/GI: esophagitis, N/V, dyspepsia, peptic ulcer, bloating
Nutr: APPETITE, WT, CA WASTING with LT use.
Other: EDEMA, BP, slow healing, MUSCLE WASTING with LT use.
Blood: Na, K, Ca, glucose
Cyclophosphamide
(antineoplastic)
Drug: take on empty stomach;
Diet: fluid before and 72 hrs after dose
Nutr: Anorexia, wt; Oral/GI: Dry mouth, stomatitis, N/V, abdominal pain,
diarrhea
S/Cond: Caution with renal function
Other: delayed wound healing
Blood: BUN, Creatinine
Acyclovir
(antiviral)
Diet: Insure adequate fluid intake, 2-3 L/day
Nutr: Anorexia. Caution with renal function
Oral/GI: N/V, abdominal pain, constipation, diarrhea; Blood: BUN, Creat
Medications, cont.
Medication Nutritional Indications
Levetiracetam
(antiepileptic)
Nutr: Anorexia. Caution with renal function
Protonix
(antiulcer,
antisecretory)
Diet: May Fe abs
Oral/GI: Gastric acid secretion, nausea, abdominal pain, diarrhea
Norvasc
(antihypertensive)
Diet: Na, cal may be recommended; Oral/GI: dysphagia, nausea, cramps
Other: BP, edema
Coreg
(CHF treatment,
antihypertensive)
Drug: take with food; Diet: Na, cal may be recommended
Nutr: wt ; Oral/GI: N/V, diarrhea
Blood: glucose, BUN, Creat, K, Na,
Vitamin D Nutr: CALCIUM ABS. Anorexia, wt, thirst
Oral/GI: Dry mouth, metallic taste, N/V, constipation, diarrhea
Calcium Carbonate Drug: Take with meals
Diet: Insure adequate fluid intake. Take separately from large amounts of high
fiber, high oxalates or high phytate foods
Nutr: Anorexia; Oral/GI: chalky taste, dry mouth, diarrhea
Nephro-vite N/A
Labs
1/27 2/4 2/11 2/20 2/28 3/7 3/14 3/21
Glucose 172 184 173 191 167 151 161 82
Na+ 141 143 137 138 137 136 137 141
K+ 4.3 4.4 4.8 4.6 4.4 4.3 4.1 4.2
Phos 6.9 7.9 7.6 5.0 6.2 4.3 3.3 3.2
BUN 100 165 146 77 52 61 49 28
Creat 5.0 5.8 5.9 4.0 3.0 2.2 2.0 1.5
Alb 3.4 3.6 3.0 3.4 2.7 2.9 2.8 2.8
Ca 8.0 7.5 7.1 7.6 7.5 8.0 7.6 7.7
Glucose: with severe infections, corticosteroids
Na+: with edema, diarrhea, hyperglycemia, severe nephritis
Phosphorus: with ESRD & severe nephritis, hypocalcemia
BUN: with renal failure, dehydration, infection, excessive pro intake
Creat: with acute & chronic renal disease
Calcium: with hypoalbuminemia, elevated phosphorus, diarrhea,
steroid use
Albumin: with edema, ESRD, diarrhea, stress

Nutrition Care Process
PES Statement?

Goals and Plans?
Nutrition Diagnosis
PES Statement #1:
as evidenced by hemodialysis requiring higher
protein needs, stage II sacral pressure ulcer and
gluteal fold, delayed wound healing, and infection.
related to increased demand for protein
Increased nutrient needs (NI-5.1)
Nutrition Diagnosis
PES Statement #2:

as evidenced by BUN, Creatinine,
Phosphorus gFR, rapid weight changes, and
diagnosis of Goodpasture syndrome.
related to kidney dysfunction
Altered nutrition related lab values (NC-2.2)
Goals & Plans
Improve BUN and
Creatinine levels to
within normal limits
Adhering to Renal diet
low K+, low phosphorus,
low Na+, fluid restriction
(should not restrict protein in
A.A.s case)
Remain on Nepro formula
as needed
Continue with
hemodialysis 3
days/week (M,W,F)
Goal # 1 Plans
Goals & Plans
Advance to solid
foods when
medically feasible
and tube feeding no
longer deemed
necessary to
improve quality of
life
Recommend SLP
perform swallow
evaluation to test what
type of foods and liquids
consistency can be
tolerated when A.A. has
improved cognitively
Wean off TF when
appropriate
Continue following renal
diet with supplemental
oral Nepro

Goal # 2 Plans
Goals & Plans
Improve stage II
sacral pressure
ulcer/skin integrity
Prevent further skin
breakdown
Continue on Prostat 30ml
x1 to provide additional
15g protein
Suggest increasing Prostat
x2 as necessary to provide
total 30g additional protein
Continue on Nephro-vite
multivitamin
Not able to provide Vitamin
C supp. b/c
contraindicated
Goal #3 Plans
Prognosis & Future Plans
Last day I saw A.A. was 3/21/14 - was still admitted
Total 56 days at Winthrop
21 days in MICU
Recovering from AKI and remains unresponsive but
appeared to be clinically stable
BUN = 21, Creatinine = 1.5
A.A. had been downgraded from MICU to one of the
medical floors for mechanically ventilated patients
Case Management was in discussion with daughter
about post-hospital needs
A.A. would require rehab facility that offered both
dialysis care and ventilator care
Journal Article
Effects of peridialytic oral supplements on nutritional
status and quality of life in chronic hemodialysis patients.
by Kraus M.A., Mueller B.A., Scott M.K., Shah N.A., Thomas J. 3
rd,
Vilay A.M
Purpose: Determine the effects of peridialytic oral
supplements on nutritional markers and quality of life in
patients receiving maintenance hemodialysis
Method: Open, prospective, non-randomized, and
comparative study. This study was performed at an outpatient
hemodialysis unit in a teaching hospital and included 88
adults with stage 5 chronic kidney disease. The study
involved nutrition therapy with >1 can of enteral nutrition
(Nepro) with each hemodialysis session 3x weekly for 3
months, or standard care without enteral nutrition.

Kraus M.A., Mueller B.A., Scott M.K., Shah N.A., Thomas J. 3
rd,
Vilay A.M. (2009). Effects of peridialytic oral
supplements on nutritional status and quality of life in chronic hemodialysis patients. J RENAL NUTR, 19 (2):
145-152.
Journal Article
Results:
Nutritional Markers - Results showed a significant difference in
serum albumin over 3 months time between the enteral nutrition
group and standard comparison group. Mean serum albumin
concentrations did not differ between baseline and month 3 in the
enteral nutrition group, but serum albumin levels decreased
significantly in the standard comparison group.
Quality of Life - The role-physical domain score of the Kidney
Disease Quality of Life - Short Form significantly changed over
time in the enteral nutrition group versus the comparison group.
The enteral nutrition group expressed an improvement in quality
of life with respect to impact of physical health on daily activities
Kraus M.A., Mueller B.A., Scott M.K., Shah N.A., Thomas J. 3
rd,
Vilay A.M. (2009). Effects of peridialytic oral
supplements on nutritional status and quality of life in chronic hemodialysis patients. J RENAL NUTR, 19 (2):
145-152.
Journal Article
Conclusions:
Nepro was well-tolerated among the enteral nutrition
group, and greater than 80% of the prescribed
supplements was consumed.
Oral nutrition, as part of structured, supervised
peridialytic therapy in chronic hemodialysis patients, was
well-accepted, and resulted in the maintenance of serum
albumin levels and improvement of quality of life with
respect to physical health
Kraus M.A., Mueller B.A., Scott M.K., Shah N.A., Thomas J. 3
rd,
Vilay A.M. (2009). Effects of peridialytic oral
supplements on nutritional status and quality of life in chronic hemodialysis patients. J RENAL NUTR, 19 (2): 145-
152.
References
Academy of nutrition & dietetics. (2013). International dietetics & nutrition terminology reference
manual. (pp. 149-150, 188-189, 244-245). Chicago: Academy of Nutrition and Dietetics
Kraus M.A., Mueller B.A., Scott M.K., Shah N.A., Thomas J. 3
rd,
Vilay A.M. (2009). Effects of
peridialytic oral supplements on nutritional status and quality of life in chronic hemodialysis
patients. J RENAL NUTR, 19 (2): 145-152.
Lacey, K., Nahikian-Nelms, M. (2011). Nutrition therapy and pathophysiology. (p. 536).
Belmont, California: Wadsworth Cengage Learning.
Mayo Clinic (2011). Encephalitis. Retrieved from http://www.mayoclinic.org/diseases-
conditions/encephalitis/basics/definition/con-20021917
National Kidney Foundation. (2013). Goodpastures syndrome. Retrieved from
https://www.kidney.org/atoz/content/goodpasture.cfm
National Institute of Diabetes and Digestive and Kidney Disease (2012). Goodpasture
syndrome. Retrieved from http://kidney.niddk.nih.gov/kudiseases/pubs/goodpasture/
Pranay, K. (2013). Goodpasture Syndrome. Retrieved from
http://emedicine.medscape.com/article/240556-overview.
Pronsky, M.Z., & Crowe, S.J. (2012). Food medication interactions. (17
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