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 IVMicrothrombi |
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 |
| ||||