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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

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nephrology board review

NEPHROLOGY BOARD REVIEW

Palak Parikh

June 19, 2009

topics for today
TOPICS FOR TODAY
  • Acid-base disorders
  • Treatment of HTN
  • ARF/AKI
  • Nephrotic syndrome
  • Glomerulonephritis
  • Vasculitis
compensation for acid base disorders
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 disorders4
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
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?

  • 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
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?

  • 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
MKSAP QUESTION # 65

A 21-year-old man is evaluated in the ED for severely diminished mental status. He has a 3-day 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
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
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?

  • Metformin
  • Diarrhea
  • Type 4 RTA
  • Type 1 RTA
  • Enalapril
non ag metabolic acidosis
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 acidosis11
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
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?

  • Renal tubular acidosis
  • Impaired hepatic conversion of lactate
  • Lactulose-induced diarrhea
  • Reduced acid buffering capacity of the blood
  • Increased minute ventilation
respiratory alkalosis
RESPIRATORY ALKALOSIS
  • Causes a compensatory renal response if persistent
  • May cause alterations in consciousness, perioral paresthesias, muscle spasms, and cardiac arrhythmias
mksap question 63
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?

  • Response to normal saline
  • Blood urea nitrogen-creatinine ratio
  • Fractional excretion of sodium
  • Placement of a urinary bladder catheter
acute postrenal failure
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 intra-abdominal and pelvic malignancies.
  • The clinical presentation of urinary tract obstruction may vary from anuria to polyuria alternating with oliguria.
mksap question 66
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?

  • Hydrochlorothiazide
  • Doxazosin
  • Atenolol
  • Irbesartan
thiazide diuretics
INDICATIONS

Heart failure

Advanced age

Systolic HTN

CONTRAINDICATIONS

Gout

SIDE EFFECTS

GLUCose intolerance

HyperLipidemia

HyperUricemia

HyperCalcemia

Hyponatremia

Hypokalemia

THIAZIDE DIURETICS
mksap question 13
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?

  • Begin hydrochlorothiazide
  • Begin enalapril
  • Perform ambulatory blood pressure monitoring
  • Continue home blood pressure measurements
ambulatory blood pressure monitoring
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
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
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?

  • 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
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?

  • Furosemide
  • Acetazolamide
  • Spironolactone
  • HCTZ
  • Amiloride

After discontinuing the offending agent, which of the following is the next best step in this patient’s management?

  • IV sodium chloride (3%)
  • Normal saline (0.9%)
  • Fluid restriction
  • Demeclocycline
mksap question 34
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?

  • 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
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?

  • Minimal change glomerulopathy
  • Membranous nephropathy
  • Focal segmental glomerulosclerosis
  • Membranoproliferative glomerulonephritis
  • Systemic lupus erythematosis
minimal change disease
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:
    • Sudden, massive proteinuria
    • Lower extremity edema
    • Fatigue
  • Diagnosis:
    • 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
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?

  • Minimal change glomerulopathy
  • Focal segmental glomerulosclerosis
  • Membranous nephropathy
  • IgA nephropathy
  • Antineutrophil cytoplasmic autoantibody-associated vasculitis
membranous nephropathy
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
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?

  • Thrombotic thrombocytopenic purpura
  • Antineutrophil cytoplasmic autoantibody-associated vasculitis
  • Acute tubular necrosis
  • Acute interstitial nephritis
  • Membranous glomerulonephritis
acute interstitial nephritis
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
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?

  • Acute glomerulonephritis
  • Membranous glomerulonephritis
  • Interstitial nephritis
  • Myeloma kidney
sarcoidosis
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 w/ hypercalcemia 2/2 increased Vitamin D production
hypercalcemia
Risk Factors

Malignancy

Use of thiazide diuretics

Use of vitamin D sterols

Primary hyperparathyroidism

Immobilization

Treatment

Normal saline IVF

IV Furosemide

Bisphosphonates

Calcitonin, if needed

Clinical Manifestations

Lethargy

Confusion

Coma

Nausea

Constipation

Polyuria

Hypertension

Volume depletion

Nephrolithiasis

Nephrogenic diabetes insipidus

HYPERCALCEMIA
mksap question 12
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?

  • 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
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
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 2-month 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?

  • Systemic lupus erythematosus glomerulonephritis
  • Henoch-Schonlein purpura glomerulonephritis
  • Cryoglobulinemic glomerulonephritis
  • Antineutrophil cytoplasmic antibody-associated small-vessel vasculitis
  • Anti-glomerular basement membrane glomerulonephritis
cryoglobulinemic vasculitis
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
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?

  • 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 Medium-Vessel Vasculitis

GLOMERULAR DISEASES
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

VASCULITIS