This document contains a summary of a noon conference presentation on hypertensive emergencies and thrombotic thrombocytopenic purpura (TTP). It includes two multiple choice questions about appropriate blood pressure reduction goals in hypertensive emergencies and the classic pentad of TTP symptoms. The document also discusses a patient case of a 28-year-old man who presented with hypertensive emergency and was later found to have IgA nephropathy on renal biopsy. It provides prognostic factors and outcomes for IgA nephropathy as well as an illness script comparing the pathophysiology, epidemiology, clinical presentation, diagnosis, and treatment of TTP versus IgA nephropathy.

1. Noon Conference
Lauren N. Ko, MD, M.Ed
08/06/2018

2. Question 1
What is the goal rate of reduction in blood
pressure in hypertensive emergencies?
A) 5-10% in the first hour and by a further 5-10% over the next 23 hours
B) 10-20% in the first hour and by a further 5-10% over the next 23 hours
C) 15-25% in the first hour and by a further 5-10% over the next 23 hours
D) 10-20% in the first hour and by a further 10-20% over the next 23 hours

3. Question 1
What is the goal rate of reduction in blood
pressure in hypertensive emergencies?
A) 5-10% in the first hour and by a further 5-10% over the next 23 hours
B) 10-20% in the first hour and by a further 5-10% over the next 23 hours
C) 15-25% in the first hour and by a further 5-10% over the next 23 hours
D) 10-20% in the first hour and by a further 10-20% over the next 23 hours

4. Patient Case
7/1
Mr. R dx’ed with
kidney stones
Mr. R awoken
with HA/N/V, mild
confusion, unable
to go to work
7/8
7/9
Presents to VMMC
with BP 240/120,
started on nicardipine
gtt  amodipine,
lisinopril, HCTZ
Mr. R– 28yo M w/ no PMHx p/w hypertensive emergency
7/10
Patient’s BP stabilizes
with multidrug therapy.
24h protein is 6g.
Nephrology consulted.
REVIEW:
In this case,
what are the
three reasons
renal biopsy is
recommended?

5. TTP?!
Our patient presented with:
- confusion, headache
- thrombocytopenia
- hemolytic anemia
- acute renal failure
(1) George JN, Nester CM. Syndromes of thrombotic microangiopathy. N Engl J Med 2014; 371:654.
(2) Laszik ZG, Kambham N, Silva FG. Thrombotic microangiopathies. In: Heptinstall's Pathology of the Kidney,
Jennett JC, D'Agati VD, Olson JL, Silva FG (Eds), Lippincott-Raven, Philadelphia 2014.

6. Question 2
Which of the following is not considered part of
the classic pentad of TTP?
A) Microangiopathic hemolytic anemia
B) Thrombocytopenia
C) Fever
D) Neurologic Abnormalities
E) Renal Failure
F) Elevated PT/PTT

7. Question 2
Which of the following is not considered part of
the classic pentad of TTP?
A) Microangiopathic hemolytic anemia
B) Thrombocytopenia
C) Fever
D) Neurologic Abnormalities
E) Renal Failure
F) Elevated PT/PTT

8. TTP?
S/Sx of TTP?
Yes
Severe
thrombocytopenia,
normotensive +/-
ADAMTS13 deficiency
Urgent plasma
exchange for suspected
TTP
Mild
thrombocytopenia,
high MAP, severe renal
dysfunction
Consider HTN induced
TMA
No Search for other
etiologies
(1) Khanal et al. Differentiating malignant hypertension-induced thrombotic microangiopathy from thrombotic
thrombocytopenic purpura. Ther Adv Hematol. 2015 Jun; 6(3): 97–102.

9. Patient Case
7/1
Mr. R dx’ed with
kidney stones
Mr. R awoken
with HA/N/V, mild
confusion, unable
to go to work
7/8
7/9
Presents to VMMC
with BP 240/120,
started on amodipine,
lisinopril, and
nicardipine gtt
Mr. R– 28yo M w/ no PMHx p/w hypertensive emergency
7/10
Patient’s BP stabilizes
with uptitration of
lisinpril. Nephrology
consulted.
Renal biopsy performed;
shows IgA deposits in
glomerular mesangium
7/12

10. Prognosis
(1) Berthoux F, Mohey H, Laurent B, Mariat C, Afiani A, Thibaudin L. Predicting the risk for dialysis or death in IgA
nephropathy. J Am Soc Nephrol. 2011;22(4):752. Epub 2011 Jan 21.
(2) Alamartine E, Sabatier JC, Guerin C, Berliet JM, Berthoux F. Prognostic factors in mesangial IgA
glomerulonephritis: an extensive study with univariate and multivariate analyses. Am J Kidney Dis.
1991;18(1):12.
• 50% slow progression to ESRD
• Prognostication factors:
• Hypertension >140/90
• Elevated creatinine
• Persistent protein excretion above 1000mg/day
• Reduction in presenting GFR
• Acute onset nephrotic syndrome
• Modifiable risk factors: obesity, hypertriglyceridemia, smoking

11. Prognosis
(1) Berthoux F, Mohey H, Laurent B, Mariat C, Afiani A, Thibaudin L. Predicting the risk for dialysis or death in IgA
nephropathy. J Am Soc Nephrol. 2011;22(4):752. Epub 2011 Jan 21.
(2) Alamartine E, Sabatier JC, Guerin C, Berliet JM, Berthoux F. Prognostic factors in mesangial IgA
glomerulonephritis: an extensive study with univariate and multivariate analyses. Am J Kidney Dis.
1991;18(1):12.
• 50% slow progression to ESRD
• Prognostication factors:
• Hypertension >140/90
• Elevated creatinine
• Persistent protein excretion above 1000mg/day
• Reduction in presenting GFR
• Acute onset nephrotic syndrome
• Modifiable risk factors: obesity, hypertriglyceridemia, smoking
Death or Dialysis
(in 20 years)
2%
(proteinuria
<1000mg/day w/
treatment)
67%
(proteinuria
>1000mg/day w/
treatment)

12. Illness Script
TTP IgA Nephropathy
Pathophysiology Deficiency of ADAMTS13 no cleavage of von
Willebrand factor  ultralarge vWf multimers
accumulate on endothelial surfaces where
platelets attach and accumulate
Immunoglobulin A (IgA) nephropathy is characterized
by predominant IgA deposition in the glomerular
mesangium.
Epidemiology Median age: 41
females>males, black race
2:1 male to female ratio
MC in caucasians, asians
Time Course Acute Varies
Clinical
Presentation
Fatigue, dyspnea, petechiae/bleeding
(accompanied by severe MAHA,
thrombocytopenia). 41% with neurologic sx
40-50% - one or recurrent episodes of gross
hematuria (w/ URI), less with microscopic hematuria
and mild proteinuria incidentally found on routine
exam, <10% with nephrotic syndrome vs.
glomerulonephritis (edema, HTN, renal insuff)
Diagnostics CBC, peripheral blood smear, high LDH/low
haptoglobin, Coags, Direct Coombs,
ADAMTS13 activity/inhibitor testing
UA, microscopy
Renal biopsy with MEST-C histologic scoring
Therapeutics Urgent plasma exchange + glucocorticoids BP control with ACEi/ARB (if proteinuria),
glucocorticoids, +/- immunosuppressive agents

13. Questions?
Thank you!