THE "CAPS" REGISTRY-3

             Table 3. Thrombotic clinical manifestations of patients at the time of catastrophic APS.

PATIENT No.

CEREBRAL

RENAL

 

SKIN

 

 

CLINICAL

IMAGING

BIOPSY

CLINICAL

IMAGING

BIOPSY

CLINICAL

IMAGING

BIOPSY

1

CVA

 

 

Failure

 

Intrarterial thrombosis

LR, ulcers, necrosis

Digital thrombosis

 

2

Seizure

CVA

Infarct

 

Failure

 

 

LR, ulcers

 

Microthrombi

3

encephalopathy

Seizures 

Microinfarcts

 

 

Failure

 

GN IV

Microthrombi

LR

 

Microthrombi

4

encephalopathy

 

 

Failure

 

Ischemia

 

 

 

5 (1st)

TIA

 

 

 

 

Microinfarcts

LR

 

Thrombosis

5 (2nd)

 

 

Microthrombi

 

 

Microthrombi

 

 

 

6

 

 

 

 

 

 

 

 

 

7

CVA

Infarct

 

Failure Hypertension

Renal arteries stenosis

GN IV

Microthrombi

 

 

 

8

encephalopathy

 

Microthrombi

Failure

 

 

LR, ulcers,

cyanosis

 

 

9

 

 

 

Failure

 

Microthrombi

LR

 

 

10

encephalopathy

 

 

Hematuria

Hypertension

infartion

 

 

 

 

11

 

 

 

Yes

 

 

Necrosis

 

 

12

 

 

 

Failure

Hypertension

 

Cortical infarction

SH

 Ulcers

 

Microthrombi

13

Coma

 

Multiple infarcts

Failure

 

 

Cyanosis

Digital ischemia

 

Microthrombi

14

encephalopathy

 

 

 

 

 

Digital ischemia

 

 

15

 

 

 

Failure

Low

perfusion

 

 

 

 

16

 

 

Microinfarct

 

 

Microthrombi

 

 

 

17

 

Infarcts

 

Yes

 

 

 

 

 

18

encepahlopathy

 

Microinfarct

Failure

 

 

LR

Purpura

 

 

19

 

 

 

 

 

Large infarct

 

 

 

20

 

 

 

 

 

Microthrombi

 

 

 

21    (1st)

Encephalopathy Seizures

Microinfarcts

 

Failure

 

 

 

 

 

        (2nd)

Encephalopathy

Microinfartcs

 

 

 

 

 

 

 

22

Encephalopathy

Infarct

Microinfarct

Failure

 

 

Bullous lesions

 

Microthrombi

23

CVA

Infarct

Subdural

Hematoma

¿vasculitis?

 

 

¿vasculitis?

 

 

 

24

Seizures

 

Microinfarcts

Failure

 

GN II

Microthrombi

 

 

 

25

 

 

 

Yes

 

 

 

 

 

26

CVA

infarct

 

 

 

 

 

 

 

27

 

 

 

 

 

 

 

 

 

28

 

 

 

Failure

 

Arterie thromosis

 

 

 

29

 

 

 

Yes

 

 

 

 

 

30

 

 

 

Failure

Arterie thrombosis

Arterie thrombosis

 

 

 

31

Encephalopathy

 

 

Failure Hypertension

 

 

 

 

 

32

Headache

Venous sinus

Thrombosis

 

 

 

 

Purpura Digital ischemia

 

 

33

Encephalopathy

 

 

Failure

 

 

Gangrene

 

 

34

Encephalopathy

Multiple infarcts

 

Failure Hematuria

 

 

LR

Ulcers

 

Microthrombi

35

Encephalopatthy

Multiple infarcts

 

Failure

 

 

LR

 

 

36

 

 

 

Failure

 

Crescenti GN

 

 

 

37

 

Infarcts

 

Yes

 

 

LR

 

 

38

 

 

 

Yes

 

 

Gangrene

 

 

39

 

Infarcts

 

Yes

 

 

LR

 

 

40

 

 

Infarcts

Failure

Hypertension

 

Microthrombi

Fragmented erythrocytes

LR

 

 

41

Encephalopathy

Seizures

 

 

Failure

 

 

 

 

 

42

 

Infarcts

 

 

 

 

 

 

 

43

 

Infarcts

 

 

 

 

 

 

 

44

 

 

 

 

 

 

 

 

 

45

 

 

 

 

 

 

 

 

 

46

 

 

 

Proteinuria

 

 

Digital ischemia

 

 

47

Encephalopathy

Headache

Multiple infarcts

Microthrombi

 

 

 

LR Cyanosis 

 

 

48

 

 

 

Failure Hypertension

 

GN IV

Microthrombi

Multiple hematomas

 

 

49

CVA Seizures

Infarct

 

Failure

 

 

 

 

 

50

Diplopia

Hemibalism

Vertgo

 

Multiple infarcts

Failure

Proteinuria

Hypertension

Multiple infarcts

 

Ulcers

 

 

51

Headache

 

 

 

 

 

Rash

 

Microthrombi

52

 

 

 

Failure

 

Microthrombi

Purpura

Rash

 

 

53

Encepahlopathy

 

Microthrombi

 

 

Microthrombi

Digital ischemia

Raynaud

 

Microthrombi

54

Encephalopathy

CVA

 

Microthrombs

 

Failure

 

Microthrombi

LR

Ulcers

 

Microthrombi

55

 

 

 

 

 

 

LR, Purpura

Ulcers Necrosis

 

Microthrombi

56

 

 

 

 

 

 

Raynaud

 

 

57

 

 

 

Failure

 

 

Gangrene

 

microthrombi

58

CVA

Encephalopathy

Multiple infarcts

 

Failure

 

GN IV

Microthrombi

 

 

 

59

Encephalopathy

 

 

 

 

 

LR

 

 

60

 

 

 

 

 

 

 

 

 

61

Encephalopathy

Cerebellar infarcts

 

Failure

Hypertension

 

 

LR

Ulcers

 

Microthrombi

62

 

 

 

 

 

 

 

 

 

63

Encepahlopathy

Infarcts

 

Failure

 

 

LR, ulcers, gangrene

 

Microthrombi

64

CVA

Multiple infarcts

 

Failure

 

 

 

SH, LR ulcers

 

 

65

 

Venous oclussion

 

 

 

 

 

 

 

66

 

 

 

 

 

 

LR, ulcers, gangrene

 

Microthrombi

67

 

 

 

 

 

 

 

 

 

68

Encephalopathy

Multiple infarcts

Microthrombi

Failure

 

 

Raynaud Ulcers

 

 

69

 

 

 

 

 

Microthrombi

Purpura

 

 

70(1st)

Encephalopathy

Headache

Infarct

 

 

 

 

 

 

 

 (2nd)

 

Microinfarct

 

Failure

Hypertension

 

 

LR, Purpura  Ulcers

 

 

(3rd)

 

 

 

Failure

Hypertension

 

 

Ulcers

 

 

71

 

 

 

Failure

Hypertension

Infarcts

 

 

 

 

72

Encephalopathy

Intracerebral hemorrhage

 

Failure

Perfusion defects

GN IV

Microthrombi

LR

Cyanosis

 

 

73

 

 

 

 

 

 

LR

Purpura

Gangrene

 

Microthrombi

74

Encephalopathy

Microinfarcts

 

 

 

 

LR

 

 

75

Encephalopathy

Microinfarcts

 

Yes

 

 

LR

 

 

76

CVA

Infarcts

 

Yes

 

 

LR, ulcers

 

 

77

CVA

Infarcts

 

 

 

 

LR

 

 

78

CVA

Infarcts

 

Yes

 

 

LR

 

 

79

Encephalopathy

Seizures

Microinfarcts

 

Yes

 

 

 

 

 

80

Encephalopathy

Microinfarcts

 

Yes

 

 

Ulcers

 

 

81

Encephalopathy

Microinfarcts

 

Yes

 

 

Ulcers

 

 

82

Encephalopathy

 

 

Proteinuria

 

 

Ulcers

 

 

83(1st)

Encephalopathy

Microinfarcts

 

Failure

Proteinuria

Vein thrombi

 

 

 

 

83(2nd)

Encephalopathy

Chronic subdural hematoma

 

Failure

Proteinuria

Vein thrombi

 

 

 

 

84

TIA (2)

 

 

Failure

Proteinuria

Infarcts

 

LR

 

Microthrombi

85

 

 

 

Failure

Proteinuria

 

Crescentic GN

Microthrombi

 

 

 

86

 

Infarcts

Sinus thrombi

 

Failure

Proteinuria

 

Microthrombi

Ulcers

 

 

87

Encephalopathy

Microinfarcts

 

Failure

 

Microthrombi

Petechiae

 

 

88

CVA

Infarcts

 

 

 

Infarct

SH

 

 

89

 

Venous thrombi

 

Failure

 

 

Gangrene

 

 

90

 

 

 

 

 

 

Gangrene

 

Microthrombi

91

 

 

 

 

 

Microthrombi

 

 

 

92

Encephalopathy

 

Microinfarcts

Failure

 

Microthrombi

Urticaria

 

Microthrombi

93

Seizures

 

Microinfarcts

Failure

 

GN IV

Microthrombi

 

 

Microthrombi

94

 

 

Microinfarcts

Proteinuria

Hypertension

 

GN IV

Microthrombi

LR

 

Microthrombi

95

 

 

 

Failure

 

Microthrombi

 

 

 

96

CVA

Encephaopathy

Multiple infarcts

Microthrombi

 

Multiple infarcts

Microthrombi

 

 

 

97

Encephalopathy

Seizures

Microinfarcts

 

 

 

 

 

 

 

98

CVA

Infarct

 

 

 

 

 

 

 

99

 

Multiple infarcts.

Haemorrhage

 

Failure

 

 

Purpura

 

 

100

 

 

 

 

 

 

Ecchymoses

 

 

101

 

 

 

 

 

 

Raynaud

Gangrene

 

 

102

Encephalopathy

 

 

Failure

 

Microthrombi

 

 

 

103

 

 

 

Failure

 

 

 

 

 

104

Seizures

 

 

Failure

Venous thrombi

 

 

 

 

105

 

 

 

Failure

Hypertension

 

 

Echymosis

 

 

106

CVA

 

 

Failure

Proteinuria

 

 

 

 

 

107

Encephalopathy

CVA

 

Microthrombi

Faiñure

 

Microthrombi

Ulcers

 

Microthrombi

108

Encephalopathy

Seizures

 

 

Failure

 

 

Gangrene

 

 

109

Seizures

Multiple infarcts

 

Failure

 

 

Purpura

 

 

110

Seizures

Multiple infarcts

 

Failure

Proteinuria

Hypertension

 

Microthrombi

 

 

 

111

 

 

 

Failure

 

 

 

 

 

112

 

 

 

Failure

 

 

LR

 

 

113

 

 

 

Failure

Infarct

 

 

 

 

114

 

 

 

Failure

Hypertension

 

 

petechiae

 

 

115

 

 

Infarct

Hematuria

Proteinuria

 

microthrombi

 

 

 

116

 

 

 

 

 

 

 

 

 

117

 

Lacunar infarct

 

Hematuria

Hypertension

Artery thrombi

 

 

 

 

118

 

Infarcts

 

 

 

 

 

 

 

119

 

 

 

 

Artery thrombi

 

 

 

 

120

 

 

 

 

 

 

 

 

 

121

 

 

 

 

 

 

 

 

 

122

Encephalopathy

 

 

Failure

 

 

Digital necrosis

 

 

123

Coma

 

 

Failure

 

 

 

 

 

124

 

 

 

Failure

Proteinuria

Hypertension

 

 

LR, SH

Purpura

 

Vasculitis

Microthrombi

125

Encephalopathy

CVA

Multiples infarcts

 

Failure

Proteinuria

 

 

LR

Necrosis

 

 

126

CVA

 

 

Yes

 

 

Ecchymoses

LR

 

 

127

CVA

 

 

Yes

 

 

LR

Ulcers

 

 

128

 

 

 

 

 

 

LR

 

 

129

CVA (2) simultaneous in differents areas

 

 

 

 

 

 

 

 

130

CVA

 

 

 

 

 

LR, Ulcers

Digital necrosis

 

 

131

 

 

 

 

 

Multiple infarcts

 

 

 

132

Encephalopathy

Microinfarcts

 

Failure

 

 

Ulcers

 

 

133

CVA

Encephalopathy

Infarcts

 

Failure

Proteinuria

 

 

Petechiae

 

 

134

CVA

Seizures

 

 

Failure

Thrombi

 

 

 

 

135

 

 

 

Failure

 

 

 

 

 

136

 

 

Microthrombi

Failure

 

GN IIA

Microthrombi

 

 

Microthrombi

137

 

 

 

 

 

 

 

 

 

138

 

 

 

Failure

Proteinuria

Hypertension

 

 

Microthrombi

Digital ischemia

 

 

139

CVA

 

 

Failure

 

 

LR, digital ischemia

 

 

140

 

 

 

Failure