Download NEPHROLOGY BOARD REVIEW

NEPHROLOGY
BOARD REVIEW
Palak Parikh
June 19, 2009
TOPICS FOR TODAY
Acid-base disorders
 Treatment of HTN
 ARF/AKI
 Nephrotic syndrome
 Glomerulonephritis
 Vasculitis

COMPENSATION FOR
ACID-BASE DISORDERS

Metabolic Acidosis
 Winter’s
formula: Expected pCO2 = 1.5 (HCO3) + 8
+/- 2
 Every 1 mmol/L decrease in HCO3 -> 1 mm Hg
decrease in pCO2
 pCO2 should approach last two digits of pH (ex:
pCO2 of 24 should correspond to pH of 7.24)

Metabolic Alkalosis
 Every
1 mmol/L increase in HCO3 -> 0.7 mm Hg
increase in pCO2.
COMPENSATION FOR
ACID-BASE DISORDERS

Respiratory Acidosis
 Acute:
10 mm Hg increase in pCO2 -> 1 mmol/L
increase in HCO3
 Chronic:
10 mm Hg increase in pCO2 -> 4 mmol/L
increase in HCO3

Respiratory Alkalosis
 Acute:
Every 10 mm Hg increase in pCO2 ->
2 mmol/L decrease in HCO3
 Chronic:
Every 10 mm Hg increase in pCO2 ->
4 mmol/L decrease in HCO3
MKSAP QUESTION # 62
A 44-year-old woman w/ cirrhosis 2/2 autoimmune hepatitis is hospitalized for a
progressively worsening 2-day hx of fever and abd pain. She is currently on the
orthotopic liver transplant list and has been clinically stable for the past month. She
ahs previously undergone TIPS placement and a cholecystectomy. Meds are oral
spironolactone 100 mg BID, furosemide 80 mg BID, and oral lactulose 30 mL BID.
On PE, temp is 38.2 C, pulse is 72, RR is 24, and BP is 74/55. She appears cachectic.
Cardiac and pulmonary exams are normal. The abdomen is distended, and there
is diffuse tenderness. There is 1+ pitting edema in the lower extremities. SBP is
suspected, and she is admitted to the hospital.
Lab studies:
Na 128, K 5.1, Cl 104, HCO3 12, BUN 20, Cr 1.3, Glu 84, Alb 1.4
ABG (on RA): pH 7.25, pCO2 28, pO2 78
Which of the following is the most likely diagnosis in the clinical scenario?
(A)
(B)
(C)
(D)
(E)
Mixed AG metabolic acidosis and respiratory alkalosis
Mixed AG metabolic acidosis and respiratory acidosis
Mixed non-AG metabolic acidosis and respiratory acidosis
AG metabolic acidosis
Non-AG metabolic acidosis
MKSAP QUESTION # 28
A 64-year-old man is admitted to the ICU w/ PNA and septic shock. Over the
past 4 days, he has had increasing shortness of breath and fever. He has
HTN. Surgical hx is significant for a previous cholecystectomy. Meds are
amlodipine and HCTZ.
On PE, temp is 38.8 C, pulse is 110, RR is 22, and BP is 85/50. Cardiac exam
reveals a grade 2/6 systolic murmur. On pulmonary exam, there are
crackles over the entire right lung field. There is trace pedal edema.
Lab studies on admission:
Na 135, K 4.8, Cl 103, HCO3 10, BUN 22, Cr 1.4, Glu 115
ABG (on RA): pH 6.94, pCO2 48, pO2 51
Which of the following conditions is most likely present in this patient?
(A)
(B)
(C)
(D)
(E)
AG metabolic acidosis
Mixed non-AG metabolic acidosis and respiratory acidosis
Mixed AG metabolic acidosis and respiratory alkalosis
Mixed AG metabolic acidosis and respiratory acidosis
Mixed non-AG metabolic acidosis and respiratory alkalosis
MKSAP QUESTION # 65
A 21-year-old man is evaluated in the ED for severely diminished mental status. He has a 3day history of nausea and has been unable to eat well. This morning, he vomited several
times.
On physical exam, temp is 37.4 C, pulse is 105/min, RR is 28, and BP is 122/57. He is
thin and appears in moderate distress. Cardiac and pulmonary exams are normal. The
abdomen is soft and nontender. A stool specimen is neg for occult blood.
During the exam, he begins to vomit large amounts, aspirates a significant amount of his
stomach contents, and develops respiratory failure. He is intubated and started on
mechanical ventilation.
Lab studies 1 hr after initiation of mechanical ventilation:
Na 138, K 3.7, Cl 91, HCO3 16, BUN 11, Cr 1.7, Glu 980
ABG: pH 7.53, pCO2 19, pO2 67
Which of the following is the most likely acid-base disturbance present in this
patient?
(A) Mixed AG metabolic acidosis, non-AG metabolic acidosis, respiratory acidosis
(B) Mixed AG metabolic acidosis, metabolic alkalosis, respiratory alkalosis
(C) Mixed AG metabolic acidosis w/ respiratory alkalosis
(D) Mixed metabolic alkalosis w/ respiratory acidosis
METABOLIC ALKALOSIS







Primary elevation in the serum HCO3
Accompanied by hypochloremia, such that the decrease
in chloride offsets the incremental increase in HCO3
Caused by excessive HCO3 intake or loss of H+
Most frequently caused by vomiting, NG suction, and
diuretics
Renal compensation involves increased renal excretion
of HCO3.
If low urinary Cl, treat with normal saline to expand the
extracellular space.
Hemodialysis is the preferred treatment if pH > 7.6.
MKSAP QUESTION # 82
A 66-year-old man w/ type 2 DM and HTN is evaluated for an 8-day hx of severe diarrhea,
abdominal pain, and decreased food intake. His intake of liquids has been adequate. He
believes that he became sick after babysitting his grandson, who had similar symptoms.
Three years ago, he underwent CABG surgery. Meds are enalapril 20 mg BID, ASA 81 mg
qd, atenolol 25 mg qd, HCTZ 25 mg qd, and metformin 1000 mg BID. He drinks alcoholic
beverages occasionally and does not smoke cigarettes or use illicit drugs.
On PE, temp is 37.1 C, pulse is 66 w/ no orthostatic changes, and RR is 26. A stool specimen
is positive for occult blood.
Lab studies:
Na 136, K 3.9, Cl 114, HCO3 13, BUN 21, Cr 1.2, Glu 128, Alb 4.0
UNa 32, UK 21, UCl 80
ABG (on RA): pH 7.27, pCO2 30, pO2 90
Which of the following is most likely responsible for this patient’s acid-base disorder?
(A)
(B)
(C)
(D)
(E)
Metformin
Diarrhea
Type 4 RTA
Type 1 RTA
Enalapril
NON-AG METABOLIC ACIDOSIS



Diarrhea
Ureterointestinal Diversions
Renal Tubular Acidosis



Type 1 (distal) – impairment of distal acidification
Type 2 (proximal) – decrease in proximal bicarb reabsorption
Type 4 – caused by a lack of aldosterone effect on the kidney




Frequently associated w/ DM, advanced age, AIDS, interstitial
nephritis, obstructive uropathy, post-renal transplant status, ACE
inhibitors, heparin, and cyclosporine
Appropriately low urine pH (usually 5.5)
Associated w/ hyperkalemia
Renal Failure
NON-AG METABOLIC ACIDOSIS


Urinary AG helps to assess the amount of
ammonium in the urine.
Urine AG = UNa + UK – UCl

Negative Urine AG = GI losses



High amount of ammonium in the urine
Renal response to metabolic acidosis is intact.
Positive Urine AG = Impairment of renal acid
secretion


Little or no ammonium in the urine
Paucity of chloride in the urine relative to the concentration of
measured cations.
MKSAP QUESTION # 92
A 44-year-old man diagnosed w/ cryptogenic cirrhosis 2 years ago is hospitalized for a
fractured left hip sustained after a car accident. He is asymptomatic except for pain
in his hip. He has felt well recently and is currently on the liver transplant list. He
smokes 1 pack of cigarettes daily and does not drink alcoholic beverages or use illicit
drugs. Meds are spironolactone 50 mg BID, lactulose 30 mL BID, propranolol 20 mg
BID, and furosemide 20 mg BID.
On PE, temp is 36 C, pulse is 72, RR is 18, and BP is 98/55. He is cachectic. There is
scleral icterus. He has normal mentation, and no asterixis is noted. Cardiac exam
reveals no murmurs or rubs, and his lungs are clear to auscultation. The abdomen is
distended but nontender. There is 2+ peripheral edema and palmar erythema.
Lab studies:
Na 130, K 3.3, Cl 107, HCO3 18, BUN 14, Cr 0.9, Glu 88, Alb 2.6
ABG (on RA): pH 7.48, pCO2 25, pO2 92
Which of the following is the most likely cause of this patient’s acid-base disorder?
(A)
(B)
(C)
(D)
(E)
Renal tubular acidosis
Impaired hepatic conversion of lactate
Lactulose-induced diarrhea
Reduced acid buffering capacity of the blood
Increased minute ventilation
RESPIRATORY ALKALOSIS
Causes a compensatory renal response if
persistent
 May cause alterations in consciousness,
perioral paresthesias, muscle spasms, and
cardiac arrhythmias

MKSAP QUESTION # 63
A 83-year-old male nursing home resident w/ a hx of dementia is evaluated
in the ED for abdominal pain. According to the nursing home staff, he
had become increasingly agitated over the past day.
On PE, temp is 36.7 C, pulse is 96, and BP is 150/92. The patient appears
frail and confused and is clutching his abdomen and writhing in pain.
He is unable to answer questions. Pulmonary exam reveals crackles at
both lung bases. Skin turgor is normal. There is suprapubic
tenderness. The prostate is smooth, enlarged, and has an estimated
mass of 40 g. There is trace ankle edema bilaterally.
Lab studies: Na 137, K 6.2, Cl 107, HCO3 18, BUN 63, Cr 3.6
U/A: Sp Grav 1.014, Trace protein, 2-3 leukocytes/hpf, 3-5 erythrocytes/hpf
Which of the following is most likely to establish a diagnosis?
(A)
(B)
(C)
(D)
Response to normal saline
Blood urea nitrogen-creatinine ratio
Fractional excretion of sodium
Placement of a urinary bladder catheter
ACUTE POSTRENAL FAILURE

The presence of hydronephrosis is 90% sensitive and
specific for obstruction but may not be evident in patients
with concurrent volume depletion or retroperitoneal
fibrosis.

Urinary tract obstruction is most common in men with
prostatic hypertrophy or cancer and in patients with intraabdominal and pelvic malignancies.

The clinical presentation of urinary tract obstruction may
vary from anuria to polyuria alternating with oliguria.
CLASSIFICATION OF
BLOOD PRESSURES
Category
Systolic BP
Diastolic BP
Normal
<120
and
<80
Pre-HTN
120-139
or
80-89
Stage I HTN
140-159
or
90-99
Stage 2 HTN
>= 160
or
>=100
MKSAP QUESTION # 66
A 45-year-old woman is evaluated for newly diagnosed HTN. She has a
family history of essential HTN, and both her parents have type 2 DM.
On PE, BP is 150/95. BMI is 32. The remainder of the exam is normal.
Lab studies:
Electrolytes, BUN, Cr Normal
Fasting Glucose 90
Total Cholesterol 220, HDL 35, LDL 140, TG 250
In addition to repeating blood pressure measurement to confirm the
diagnosis of hypertension and counseling regarding lifestyle
modification, therapy with which of the following agents is indicated
for this patient?
(A)
(B)
(C)
(D)
Hydrochlorothiazide
Doxazosin
Atenolol
Irbesartan
THIAZIDE DIURETICS


INDICATIONS

Heart failure
 Advanced age
 Systolic HTN

CONTRAINDICATIONS

Gout
SIDE EFFECTS

GLUCose intolerance
 HyperLipidemia
 HyperUricemia
 HyperCalcemia
 Hyponatremia
 Hypokalemia
MKSAP QUESTION # 13
A 45-year-old woman is referred evaluation for a BP
measurement of 150/94. Her husband is a nurse and
regularly measures her BP at home. Her usual home BP
measurement is between 110/76 and 120/80. She does
not smoke cigarettes. Her mother has HTN.
On PE, her average BP is 148/98. Results of laboratory
studies, including the creatinine level, are normal.
In addition to counseling regarding lifestyle
modifications, which of the following is the most
appropriate management for this patient?
(A)
(B)
(C)
(D)
Begin hydrochlorothiazide
Begin enalapril
Perform ambulatory blood pressure monitoring
Continue home blood pressure measurements
AMBULATORY BLOOD
PRESSURE MONITORING

Measures BP multiple times during a 24-hr
period (during pt’s daily activities)

Can identify white coat and masked HTN

Identifies abnormalities in the normal circadian
rhythm, particularly failure of the BP to decrease
appropriately (10-20%) during sleep, which has
been associated with greater target organ
damage and long-term cardiovascular risk.
POINTERS ON HTN

The target BP for the general population is <140/90 and is <130/80
for patients with DM or renal disease.

BP during and after an acute stroke should be lowered cautiously by
about 10-15% if SBP is > 220 or DBP > 120.

More than one drug is often indicated for patients with stage 2
or higher HTN.

Diuretics are typically recommended for first-line treatment of
hypertension.

Low-dose therapy with 2 antihypertensive agents is associated with
fewer side effects than higher doses of single-agent therapy.
MKSAP QUESTION # 72
An 80-year-old woman is evaluated for resistant HTN and fatigue. Home
BP measurements are typically approximately 180/70. Meds are
metoprolol 50 mg qd, lisinopril 20 mg qd, and HCTZ 12.5 mg qd.
On PE, pulse is 72 and BP is 180/70.
Lab studies:
Na 132, K 3.3, Cl 99, HCO3 26, BUN 12, Cr 0.9
Plasma renin activity: 0.36 ng/mL per hour
Which of the following is the most appropriate next step in this
patient’s management?
(A)
(B)
(C)
(D)
Double the dose of HCTZ
Double the dose of metoprolol
Double the dose of lisinopril
Discontinue HCTZ; add spironolactone 25 mg qd.
MKSAP QUESTION # 48
A 73-year-old woman is brought to the ED after falling at home. Her family states that she has been very
confused and disoriented over the past 2 days and that she began therapy w/ a new med 1 week ago.
She also has type 2 DM.
On PE, temp is 37 C, pulse is 68, RR is 12, and BP is 115/65. She is confused and unable to appropriately
answer questions. Cardiac exam is normal. The lungs are clear to auscultation. There is no edema.
Lab studies:
Na 107, K 2.9, Cl 76, HCO3 21, BUN 17, Cr 1.1, Glu 94
Therapy with which of the following agents was most likely recently started in this patient?
(A)
(B)
(C)
(D)
(E)
Furosemide
Acetazolamide
Spironolactone
HCTZ
Amiloride
After discontinuing the offending agent, which of the following is the next best step in this patient’s
management?
(A)
(B)
(C)
(D)
IV sodium chloride (3%)
Normal saline (0.9%)
Fluid restriction
Demeclocycline
MKSAP QUESTION # 34
A 61-year-old woman is hospitalized for a 5-day history of nausea and vomiting and
a 2-day history of postural lightheadedness. Her Cr level is 7 (baseline Cr 1
month ago was 1). She has a history of HTN and Type 2 DM. Meds include
aspirin, atenolol, glipizide, enalapril, and chlorthalidone.
On PE, pulse is 68 and BP is 85/60. She is not in distress. Skin turgor is
decreased. Cardiac and pulmonary exams are normal. There is no peripheral
edema. On neurological exam, she is alert and oriented.
Lab studies:
Na 120, K 3.7, Cl 86, HCO3 26, BUN 85, Cr 8, Glu 56
U/A: Several hyaline casts/hpf
UCr 40, UNa 40
Which of the following is the next best step in this patient’s management?
(A)
(B)
(C)
(D)
(E)
Intravenous sodium chloride (3%), 100 mL
Bolus therapy with 1000 mL of normal saline (0.9%)
Dialysis
Fluid restriction
Dopamine titrated to maintain a mean arterial pressure > 60 mm Hg.
MKSAP QUESTION # 3
A 21-year-old woman is evaluated for facial and lower-extremity edema of 1 week’s
duration. For the past 3 weeks, she has had fatigue. She has no history of diabetes
mellitus, cigarette smoking, or illicit drug use.
On PE, blood pressure is 90/55. Cardiac and pulmonary exams are normal. There is
periorbital edema. The abdomen is soft and without masses. There is 2+ lower
extremity edema.
Lab studies:
Cr 0.7
Total cholesterol 325
Albumin 2.9
C3 and C4 normal
Urinalysis: Sp Grav 1.026, 3+ protein, 0-1 erythrocytes/hpf, numerous oval fat bodies/hpf
24-hour urinary protein excretion 15 g/24 hr
Which of the following is the most likely diagnosis?
(A)
(B)
(C)
(D)
(E)
Minimal change glomerulopathy
Membranous nephropathy
Focal segmental glomerulosclerosis
Membranoproliferative glomerulonephritis
Systemic lupus erythematosis
MINIMAL CHANGE DISEASE

Relapsing and remitting disease for most

Cause: Unknown, but may be associated w/ NSAIDS or as a
consequence of a lymphoproliferative disease

Symptoms:




Diagnosis:



Sudden, massive proteinuria
Lower extremity edema
Fatigue
EM: effacement or flattening of glomerular epithelial cells
LM/Immunofluorescence: No abnormalities/immunoreactants
Treatment:

Corticosteroids (prednisone 60 mg qd or QOD X 4 weeks, then 40 mg QOD
X 4 weeks)


Longer treatment (12-16 weeks) for older adults
Cyclosporine and cyclophosphamide if above fails
MKSAP QUESTION # 16
A 65-year-old man is evaluated for hypoalbuminemia, hyperlipidemia, and slowly
progressive proteinuria that have developed over 1 year. One year ago, he
underwent squamous cell lung cancer resection.
On PE, BP is 150/90. Cardiac exam reveals a normal S1 and S2 w/o rubs or
gallops. Pulmonary exam shows decreased breath sounds in the right lower
lobe consistent with his previous surgery. Abdominal exam is normal. There is
3+ edema of the lower extremities.
Lab studies:
BUN 17, Cr 1.0
U/A: Sp Grav 1.020, numerous granular casts and oval fat bodies/hpf
24-hour urinary protein excretion: 15 g/24 h
CXR reveals a new 1-cm nodule in the left upper lobe.
Which of the following is the most likely cause of this patient’s renal
symptoms?
(A)
(B)
(C)
(D)
(E)
Minimal change glomerulopathy
Focal segmental glomerulosclerosis
Membranous nephropathy
IgA nephropathy
Antineutrophil cytoplasmic autoantibody-associated vasculitis
MEMBRANOUS NEPHROPATHY

Causes


Infections: Hep B, syphilis
Malignancies: Breast, colon, lung, and ovarian cancers and other
solid tumors

Diagnosis: Electron-dense immune complex deposits within
the GBM

NOTE: High risk for developing renal vein thrombosis w/
resultant pulmonary emboli

Treatment:


Pulse corticosteroids + cytotoxic therapy
Cyclosporine alone (disease returns when discontinued)
MKSAP QUESTION # 15
A 38-year-old woman is evaluated in the ED for generalized itching, an erythematous skin
rash, and joint pain. She initially tried over-the-counter diphenhydramine , but her
itching and rash did not improve. She was diagnosed with a course of amoxicillin.
Her sinus drainage and cough have improved. However, her joint pain remains, and
her temperature has been between 37.5 C and 37.8 C. She states that she has
otherwise been healthy and takes no additional meds.
On PE, temp is 37.3 C, pulse is 88, and BP is 122/68. There is a diffuse erythematous
macular papular skin rash involving her trunk, arms, and upper thighs.
Lab studies:
Hg 12.5, Leukocyte count 9800 (10% eosinophils), Platelet count 325,000
Na 138, K 4.4, HCO3 26, BUN 36, Cr 2.6
U/A: pH 5, sp grav 1.020, 2+ blood, trace protein, 4+ leukocyte esterase, 20-25
leukocytes and several leukocyte casts/hpf, 3-5 intact erythrocytes/hpf, Hansel stain
shows eosinophils
Which of the following is the most likely diagnosis in this patient?
(A)
(B)
(C)
(D)
(E)
Thrombotic thrombocytopenic purpura
Antineutrophil cytoplasmic autoantibody-associated vasculitis
Acute tubular necrosis
Acute interstitial nephritis
Membranous glomerulonephritis
ACUTE INTERSTITIAL NEPHRITIS

Most commonly occurs 2/2:



Drugs (PCNs, Cephalosporins, Fluoroquinolones, Allopurinol,
Phenytoin)
Infections (Pyelonephritis)
Inflammation (Sjogren’s, SLE, and sarcoidosis)

Urine sediment: Pyuria, leukocyte casts, microscopic
hematuria, tubular-range proteinuria

Positive Hansel’s stain

Treatment:


Discontinue offending agent
? Concomitant corticosteroids
MKSAP QUESTION # 60
A 41-year-old woman is evaluated for increased fatigue and weakness. Her breathing is more labored
when she walks to get her mail at the end of her driveway. She also has increased redness of her
eyes and a skin rash over her nose and cheeks. She has a history of osteoarthritis and HTN. Meds
are amlodipine and intermittent acetaminophen.
On PE, BP is 135/80. She has perilimbal injection (ciliary flush) and a scaly purplish rash across her nose
and cheeks. Cardiac exam reveals a soft holosystolic ejection murmur at the lower left sternal border.
There is no JVP or gallops. Pulmonary exam is unremarkable. There is no lower-extremity edema.
Lab studies:
Hg 10.5
Na 137, K 5.1, Cl 105, HCO3 22, BUN 24, Cr 1.8, Glu 113
Alb 4
Ca 11.1, Phos 2.4
U/A: 1+ protein, 1+ blood, 10-15 leukocytes/hpf, 3-5 nondysmorphic erythrocytes/hpf
Results of SPEP are normal. CXR reveals hilar lymphadenopathy. Renal ultrasound reveals a right
kidney 8.9 cm in diameter and a left kidney 9.5 cm in diameter with bilateral increased echogenicity.
Which of the following is the most likely cause of this patient’s kidney disease?
(A)
(B)
(C)
(D)
Acute glomerulonephritis
Membranous glomerulonephritis
Interstitial nephritis
Myeloma kidney
SARCOIDOSIS

Kidney disease in 20% of patients
 Nephrolithiasis
due to hypercalciuria, nephrocalcinosis,
and interstitial nephritis
 TINU



(Tubulointerstitial Nephritis and Uveitis) Syndrome
Rare presentation of sarcoidosis
More common in women
Responds to corticosteroids
 Associated
production
w/ hypercalcemia 2/2 increased Vitamin D
HYPERCALCEMIA

Risk Factors





Malignancy
Use of thiazide diuretics
Use of vitamin D sterols
Primary
hyperparathyroidism
Immobilization

Clinical Manifestations








Treatment




Normal saline IVF
IV Furosemide
Bisphosphonates
Calcitonin, if needed



Lethargy
Confusion
Coma
Nausea
Constipation
Polyuria
Hypertension
Volume depletion
Nephrolithiasis
Nephrogenic diabetes
insipidus
MKSAP QUESTION # 12
An 18-year-old man with hepatitis C virus infection is evaluated in the ED for nausea,
vomiting, anorexia, hiccups, hemoptysis, and itching. He felt well until 4 weeks ago,
when he developed an upper respiratory tract infection.
On PE, pulse is 90 and BP is 170/100. The conjunctivae are pale. Cardiac exam reveals
a grade 2/6 systolic murmur along the left sternal border. There are diffuse crackles
in both lung bases. The abdomen is soft and nontender with no masses. There is 1+
edema in the extremities.
Lab studies:
Hg 8.5, Leukocyte count 10,500, Platelet count 250,000
BUN 70, Cr 4.3
Alb 3.5
C3 140, C4 35
Antinuclear antibodies Negative
Urinalysis: 15-20 dysmorphic erythrocytes and 1 erythrocyte cast/hpf
CXR reveals bilateral fluffy pulmonary infiltrates.
Which of the following assays is most likely to be positive in this patient?
(A)
(B)
(C)
(D)
(E)
Antistreptolysin O and anti-DNAse B antibody
Anti-double-stranded DNA antibody
Antiphospholipid antibody
Anti-glomerular basement membrane antibody
Cryoglobulins
GOODPASTURE’S SYNDROME/
ANTI-GBM DISEASE

Anti-GBM Disease – involves only the kidney (older women)
Goodpasture’s Syndrome – involves kidneys and lungs (young men)

Cause: Antibodies to type IV collagen

Pathology: Necrotizing and crescentic GL affecting most of glomeruli
(RPGN)

Immunofluorescence microscopy: Linear staining of IgG lining the GBM

Treatment:




Corticosteroids
Cyclophosphamide for 3-6 months
NOTE: Approximtely 30% also have ANCA-associated vasculitis
MKSAP QUESTION # 49
A 42-year-old man is evaluated for a 2-month history of rash on his lower extremities and
a 6-month history of cold-induced acral cyanosis and discomfort. He also has a 2month history of alcohol abuse.
On PE, pulse is 78 and BP is 150/90. Cardiac and pulmonary exams are unremarkable.
On abdominal exam, the liver is 3 cm below the right costal margin. A spleen tip is
not felt. There is 1+ lower-extremity edema. A purpuric rash also is present on the
lower extremities.
Lab studies:
Hg 11.4, Platelet count 120,000
Cr 1.7
C3 86, C4 5
AST 57, ALT 5
Urinalysis: 3+ hematuria, 1+ protein, 7-10 dysmorphic erythrocytes/hpf
Which of the following is most likely causing this patient’s renal abnormalities?
(A)
(B)
(C)
(D)
(E)
Systemic lupus erythematosus glomerulonephritis
Henoch-Schonlein purpura glomerulonephritis
Cryoglobulinemic glomerulonephritis
Antineutrophil cytoplasmic antibody-associated small-vessel vasculitis
Anti-glomerular basement membrane glomerulonephritis
CRYOGLOBULINEMIC VASCULITIS

Associated with Hepatitis C

Pertinent lab studies




Elevated LFTs
Positive RF
Low C4 (and low normal C3)
Affected organs:


Skin
Glomerulus


Membranoproliferative GN (“tram-track” appearance on light microscopy)
Treatment



Plasmapheresis to remove immune complexes
Rituximab
Eradicate Hep C, if applicable
MKSAP QUESTION # 6
A 17-year-old man is evaluated for the abrupt onset of a lower-extremity rash and
intermittent episodes of mild abdominal pain. He is otherwise asymptomatic.
On PE, respiratory rate is 18, pulse is 78, and BP is 140/90. Cardiac, pulmonary,
and abdominal exams are normal. There are lesions resembling palpable
purpura on the extremities.
Lab studies:
BUN 16, Cr 0.9
C3 100, C4 31
Urinalysis: 1+ protein, 12 dysmorphic erythrocytes and 1 erythrocyte cast/hpf
Which of the following is the most likely diagnosis?
(A)
(B)
(C)
(D)
(E)
Systemic lupus erythematosis glomerulonephritis
Antineutrophil cytoplasmic autoantibody-associated small-vessel vasculitis
Cryoglobulinomic vasculitis
Henoch-Schonlein purpura
Postinfectious glomerulonephritis
GLOMERULAR DISEASES

Nephrotic Syndrome
 Minimal
change
disease
 FSGS (inc HIVAN)
 Membranous
Nephropathy
 MPGN

Nephritic Syndrome
 IgA nephropathy
 Lupus
nephritis
 Anti-GBM Ab disease
 Small- and MediumVessel Vasculitis
VASCULITIS

Large-vessel
 Giant
cell (temporal)
arteritis
 Takayasu’s arteritis

Medium-vessel
 Polyarteritis
nodosa
 Kawasaki’s disease

Small-vessel
 Wegener’s
granulomatosis
 Churg-Strauss syndrome
 Microscopic polyangiitis
 Henoch-Schonlein purpura
 Cryoglobulinemic
vasculitis
 Cutaneous
leukocytoclastic angiitis