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Cervera ricard antiphospholipid syndrome update on pathogenesis diagnosis and management-torino gennaio
1. ANTIPHOSPHOLIPID SYNDROME:
UPDATE ON PATHOGENESIS,
DIAGNOSIS AND MANAGEMENT
Ricard Cervera, MD, PhD, FRCP
Department of Autoimmune Diseases
Hospital Clínic
Barcelona
3. ANTIPHOSPHOLIPID
SYNDROME
Epidemiology
• 20% of deep vein thrombosis
• 10% of recurrent abortions
• 30% of cerebro-vascular accidents in
<50 yr-olds
NIH, 2001
6. ANTIPHOSPHOLIPID
SYNDROME
Clinical manifestations:
Classical, unusual and silent
European Forum on 2002
Antiphospholipid Antibodies
7. ANTIPHOSPHOLIPID
SYNDROME
Clinical manifestations:
Classical, unusual and silent
E U R O -P H O S P H O L IP I D
P R O JE C T
N e u ro lo g ic m a n ife s t a tio n s
E n c e p h a lo p a th
V e n o u s th ro m b o s is
M u ltiin f d e m e n tia
E p ile p s y
T IA
S tro k e
M ig ra in e
0 5 10 15 20
8. ANTIPHOSPHOLIPID
SYNDROME
Clinical manifestations:
Classical, unusual and silent
9. ANTIPHOSPHOLIPID
SYNDROME
Clinical manifestations:
Classical, unusual and silent
E U R O -P H O S P H O L IP ID
P R O JE C T
C a rd ia c m a n ifesta tio n s
B y p a s s o c c lu s io n s
A c u te m y o c a rd
C h ro n ic m y o c a rd
V e g e ta tio n s
A n g in a
M I
V a lv e le s io n s
0 2 4 6 8 10
10. ANTIPHOSPHOLIPID
SYNDROME
Clinical manifestations:
Classical, unusual and silent
11. ANTIPHOSPHOLIPID
SYNDROME
Clinical manifestations:
Classical, unusual and silent
12. ANTIPHOSPHOLIPID
SYNDROME
Clinical manifestations:
Classical, unusual and silent
13. ANTIPHOSPHOLIPID
SYNDROME
Clinical manifestations:
Classical, unusual and silent
14. ANTIPHOSPHOLIPID
SYNDROME
Clinical manifestations:
Classical, unusual and silent
15. ANTIPHOSPHOLIPID
SYNDROME
Clinical manifestations:
Classical, unusual and silent
16. ANTIPHOSPHOLIPID
SYNDROME
Clinical manifestations:
Classical, unusual and silent
17. ANTIPHOSPHOLIPID
SYNDROME
Clinical manifestations:
Classical, unusual and silent
32. Pre-Conference Workshop on CAPS
and non-criteria APS manifestations
Smita Vaidya, Horacio Adrogué
Doruk Erkan, Gerard Espinosa,
Maria Tektonidou, Antonio Cabral
Yehuda Shoenfeld, Emilio González
Chair: Ricard Cervera
April 13, 2010
33. APS NEPHROPATHY
RECOMMENDATIONS
• 1. Routine performance of renal biopsy is not recommended in
APS.
• 2. In APS patients with clinical and laboratory findings that
suggest renal involvement (new onset of hypertension,
proteinuria, hematuria or renal insufficiency), renal biopsy
should be performed (Evidence level II).
• 3. In patients with APS nephropathy, especially in SLE and in
the absence of other causes associated with similar lesions, aPL
testing is recommended (Evidence level II).
• 4. In patients with APS nephropathy and persistently positive
aPL, the diagnosis of APS should be considered, provided that
other conditions resulting in similar renal lesions are excluded.
34. HEART VALVE LESIONS
RECOMMENDATIONS
1.In patients with APS and previous thrombosis, mainly with
arterial involvement, a TTE is recommended (Evidence level II)
2.1.With normal valves and in the absence of atherosclerotic
factors, follow up controls might not be necessary.
2.2.If VHD exists, serial echocardiographic follow up controls are
warranted (1 prospective study) (Evidence level II)
3.1.No attempt to treat VHD with curative intention is
recommended (Evidence level II)
3.2. A trial of steroids might be considered in APS-related VHD
(Evidence level IV)
35. THROMBOCYTOPENIA
RECOMMENDATIONS
• 1. Multicentric, international, prospective long-term follow-up
study of patients with ITP (APIgG, aPL, LAC, anti-β2GP-I…),
thrombosis being the primary outcome.
• 2. International Registry of aPL-positive “ITP” patients
(“Hematologic APS”).
• 3. We suggest that TCP may be incorporated as an isolated
clinical criteria for APS.
39. European Forum on
Antiphospholipid Antibodies
THE "CAPS" REGISTRY
International Registry of Patients with
Catastrophic APS
www.med.ub.es/MIMMUN/FORUM/CAPS.HTM
40.
41. 2003
1. Clinical evidence of vessel occlusions affecting 3 or more organs or
systems.
2. Development of the manifestations simultaneously or in less than a
week.
3. Confirmation by histopathology of small vessel occlusion in at least
one organ.
4. Laboratory confirmation of the presence of aPL (LA and/or aCL).
-Definite catastrophic APS: All 4 criteria.
-Probable catastrophic APS:
-1, 2 & 4
-1, 3 & 4 and the development of the third event in more than
a week but less than a month, despite anticoagulation
42. 2005
• Sensitivity 90.3%
• Specificity 99.4%
• Positive predictive value 99.4 %
• Negative predictive value 91.1 %
43. Pre-Conference Workshop on CAPS
and non-criteria APS manifestations
Smita Vaidya, Horacio Adrogué
Doruk Erkan, Gerard Espinosa,
Maria Tektonidou, Antonio Cabral
Yehuda Shoenfeld, Emilio González
Chair: Ricard Cervera
April 13, 2010
49. APS-2011:
TREATMENT
• High/moderate INR controversy
• Heparin/aspirin for pregnancy
controversy
• Treatment of catastrophic APS
50. APS - 2011:
Heparin/aspirin for pregnancy controversy
S P E C IF IC S IT U A T IO N S :
A N T IP H O S P H O L IP ID S Y N D R O M E
T h e H o s p ita l C lín ic o f B a rc e lo n a E x p e rie n c e
M E D IC A L T R E A T M E N T
N o p re vio u s tre a tm e n t
v io
A s p irin 1 0 0 m g / d a y
fro m 1 m o n th b e fo re a tte m p tin g c o n c e p tio n
F a ilu re o f a s p irin in p re v io u s p re g n a n c y
A s p irin p lu s L M W h e p a rin
H is to ry o f th ro m b o s is
A s p irin p lu s L M W h e p a rin
P re d n is o n e d u rin g p re g n a n c y
O n ly if re q u ire d fo r m e d ic a l c o m p lic a tio n s
51. APS - 2011:
Heparin/aspirin for pregnancy controversy
SPECIFIC SITUATIONS:
ANTIPHOSPHOLIPID SYNDROME
The Hospital Clínic of Barcelona Experience
n:137(78%)
100 n=63 (81%)
90 ABORTION/FETAL
DEATH
80
LIVEBORN
70
60
50
40
30 n=14 (19%)
n=39 (22%)
20
10
0
Before After
treatment treatment
52. APS - 2011:
Heparin/aspirin for pregnancy controversy
SPECIFIC SITUATIONS:
ANTIPHOSPHOLIPID SYNDROME
The Hospital Clínic of Barcelona Experience
RESULTS (V)
Normal liveborn
AAS before conception (n=59 patients)
patients) 52 cases (88.1% )
AAS after conception (n=18 patients)
patients) 11 cases (61.1% )
p=0.01 OR (IC):4.7 (1.3-16.2)
(1.3-
53. APS-2011:
TREATMENT
• High/moderate INR controversy
• Heparin/aspirin for pregnancy
controversy
• Treatment of catastrophic APS
55. CATASTROPHIC APS
Triple Therapy
TRATAMIENTO
PLASMA EXCHANGE
STEROIDS ANTICOAGULATION
+/- IV IMMUNOGLOBULINS
Infections
SIRS
Asherson RA, Cervera et al. Medicine (Baltimore) 2001; 80:355-376
56.
57. SB1
2006
160
140 20%
120
53%
100
Died
80
33% Survived
60
40
20
0
1992-2000 2001-2005 p=0.005
Year of Diagnosis
58. Diapositiva 57
SB1 The mortality rate wsfifty-three percent in the first period, before two thousand and one.
whilst the mortality rate was thirty-three percent from 2001.
In other word the mortality decresed twenty percent from two thounsand and one with a p statistically significant.
What does depend on?
sbucciarelli; 05/03/2006
59. SB3
p=0.025 First period
29%
Second period
13%
AC+CS+PE and/or IVIG
60. Diapositiva 58
SB3 When we use the logistic regression anlysis including age, precipitating factor and rate use of combinated therapy
The precipitanting factor dissapeared.
the mortality decrease in the second period was associated with the age and the higher rate use of combinated treatment.
Likely the age is a statistical factor, because there is a little difference between two age.This difference is not enough for explaining so
significant reduction of mortality
Therefore the main reason for explaining the mortality decrese was the higher use rate of combinated therapy
In other word the reduction of twenty percent of mortality from two thounsand and one depends on the higher use rate of combinated
treatment wit AC+CS+PE and/or IVIG.
sbucciarelli; 05/03/2006
