THE "CAPS" REGISTRY-1

Table 1. Demographic and clinical characteristics of patients with catastrophic APS.

PATIENT No.	SEX	AGE*	DIAGNOSIS	PREVIOUS APS MANIFESTATIONS	PRECIPITATING FACTOR	CAPS AS FIRST APS MANIFESTATION	FIRST MANIFESTATION OF CAPS
1	F	32	SLE	Thrombosis deep dermal, subclavian vein thrombosis, abortion, thrombocytopenia, TMHA	Post-abortion	No	Renal
2	M	43	Lupus-like			Yes	ARDS
3	F	22	Lupus- like	DVT		No	Neurologic
4	F	22	SLE			Yes	Neurologic
5  1ºst	M	40	PAPS	Myocardial infarction, parietal infarction		No	Neurologic
5 2ºnd		42
6	F	22	SLE		Post-abortion	Yes
7	F	13	SLE			Yes
8	F	37	SLE	Digital ischemia.		No	Skin
9	F	33	SLE	Fetal loss, thrombocytopenia	Upper respiratory infection	No
10	M	14	Lupus-like			Yes	Adrenal
11	F	23	PAPS			Yes
12	F	25	PAPS	Skin ulcers	Hysterectomy	No	Bone
13	M	57	PAPS			Yes	Testicular
14	F	11	SLE		Intestinal infection	Yes	ARDS
15	M	37	PAPS	DVT(4), myocardial infarction, thrombocytopenia, hemolytic anemia	Dental extraction & anticuagulation withdrawal	No
16	F	23	SLE		ERCP	Yes	ARDS
17	F	49	SLE	DVT, Thrombocytopenia, haemolityc anemia, Addison	Anticoagulation withdrawal	No
18	M	46	PAPS			Yes	ARDS
19	F	34	RA			Yes	Cardiac
20	F	43	PAPS	DVT, fetal losses		No	ARDS
21 (1^st)	F	68	PAPS	Fetal losses		No	Neurologic
(2^nd)		68					Neurologic
22	F	37	Lupus-like	Fetal losses	Dilatation & curettage	No	Preeclampsia-DIC
23	F	60	SLE	DVT, TIA, Skin ulcers, LR		No	Pancreatic
24	F	39	SLE			Yes
25	F	29	PAPS		OC	Yes
26	M	45	SLE	DVT, PE, Superior vena cava thrombosis		No
27	F	32	PAPS			Yes	Pulmonary
28	M	55	PAPS	CVA, myocardial infarction, mesenteric and splenic thrombosis		No	Pulmonary
29	M	50	PAPS	DVT, PE, peripheral artery thrombosys		No
30	M	53	PAPS	DVT,PE, myocardial infarction, skin ulcers		No
31	F	17	PAPS	DVT	Puerperium	No	Pulmonary
32	M	59	PAPS			Yes	Digital ischemia
33	F	52	Lupus-like	Fetal loss	Thiazide	No
34	F	22	PAPS	Chorea		No	Neurologic
35	F	45	PAPS	DVT,chorea, fetal loss		No	Neurologic
36	F	36	PAPS	DVT		No	ARDS
37	M	39	PAPS	Optic neuropathy		No
38	F	46	PAPS	Fetal loss, popliteal artery thrombosis		No
39	F	38	SLE			Yes
40	F	33	PAPS			Yes	Renal
41	F	35	PAPS	Fetal loss, femoral artery thrombosis, skin ulcers, digital necrosis		No	ARDS
42	F	28	PAPS	DVT(3), PE		No
43	M	47	PAPS			Yes
44	M	22	PAPS	Budd-Chiari syndrome		No
45	M	56	PAPS	DVT, PE		No
46	M	55	Lupus-like	DVT	Captopril	No	Digital ischemia
47	F	52	SLE			Yes	Neurologic
48	F	37	SLE	DVT, fetal losses, hemolytic anemia		No	Skin
49	F	74	PAPS	DVT, PE, LR, skin ulcers		No	Renal
50	M	45	PAPS	DVT, PE, LR, Raynaud		No
51	F	38	SLE		Urinary infection	Yes	Abdominal
52	F	37	PAPS			Yes	Hepatic
53	F	36	Systemic sclerosis	Abortion (2)		No	Digital ischemia
54	F	58	PAPS	DVT, migraine	Leg ulcer infection	No	Neurologic
55	F	29	SLE		sepsis	Yes	Skin
56	F	41	PAPS	DVT, femoral artery thrombosis, thrombocytopenia	Needle stick injury, angiography	No	Peripheral
57	F	60	PAPS		Trauma	Yes	Cardiac
58	F	30	SLE		Typhoid fever	Yes	Neurologic
59	M	37	PAPS		Cholecystectomy	Yes	Hepatic
60	F	28	PAPS			Yes	Bone marrow
61	F	16	PAPS	Perforated nasal septum, leg ulcers, thrombocytopenia		No	Neurologic, skin
62	F	48	PAPS	DVT	Cholectomy, sepsis	No	Intestinal
63	M	47	PAPS	TIA, CVA, MI	Lug ulcer infection	No	Skin
64	M	52	PAPS			Yes	Skin
65	F	38	SLE	HELLP	Cesarean section	No	Hepatic
66	F	22	PAPS	Abortion (2), Raynaud		No	Skin
67	M	47	PAPS			Yes	Renal
68	F	33	SLE		Uterus carcinoma	Yes
69	F	28	SLE		Pneumonia	Yes	ARDS
70 (1st)	F	32	SLE	DVT	Anticoagulation withdrawal	No	Neurologic
(2nd)		40			Upper respiration infection	No	Renal
(3rd)		42			Upper respiration infection	No	Renal
71	M	42	Polychondritis	Stenosis of both common iliac arteries		No	Hepatic
72	F	42	SLE	CVA, retinal artery thrombosis, fetal loss, LR, thrombocytopenia		No	ARDS
73	F	28	Lupus-like		Leg ulcer infection	Yes	Skin
74	F	16	PAPS		Upper respiratory infection	Yes
75	F	17	SLE		Upper respiratory infection	Yes
76	F	21	SLE		Upper respiratory infection	Yes
77	F	54	SLE		Cutaneous & urinary infection, abdominal surgery	Yes
78	F	17	PAPS		OC, sun exposure	Yes
79	F	18	SLE			Yes
80	F	18	SLE		Upper respiratory infection	Yes
81	F	23	SLE		Gastrointestinal & cutaneous infection	Yes
82	F	26	SLE	DVT	Post-fetal loss	No	ARDS
83 (1st)	F	67	PAPS	DVT		No	Renal
(2nd )		71			Low INR	No
84	M	61	PAPS	PE, leg arterial insufficiency	Anticoagulation withdrawal	No	Neurologic
85	F	36	PAPS	DVT	Anticoagulation withdrawal,pneumonia	No	Renal
86	M	60	PAPS	Renal microangiopathy	Low INR, lung biopsy (adenocarcinoma)	No	Skin
87	F	51	Lupus- like	Abortion	Urinary infection	No	Renal
88	M	41	PAPS		Carcinoma	Yes	Neurologic
89	F	59	PAPS	Recurrent leg arterial thrombosis, abortion (2)		No	Skin
90	F	48	Systemic sclerosis	Digital ischemia		No	Skin
91	F	47	SLE	DVT, PE		No	Pulmonary
92	F	44	SLE	CVA,cerebralvenousthrombosis, DVT,digital ischemia,renal thrombosis		No
93	F	19	SLE		SLE flare	Yes	ARDS
94	M	18	SLE			Yes
95	F	42	SLE	thrombocytopenia, fetal loss, VL, LR		No	Renal
96	F	45	PAPS		Gastric adenocarcinoma	Yes	Neurologic
97	F	7	SLE			Yes	Neurologic
98	F	40	PAPS		Lung adenocarcinoma	Yes	Neurologic
99	F	33	PAPS	Abortion, superficial venous thrombosis	Pregnancy, cesarean section	No	ARDS
100	F	22	SLE		Pregnancy, HELLP	Yes
101	F	42	SLE			Yes	Abdominal
102	F	58	PAPS		Upper respiratory infection	Yes	Renal
103	M	27	SLE		Urinary infection	yes
104	F	28	SLE	DVT, thrombocytopenia, CVA		No	ARDS
105	F	26	SLE	DVT, thrombocytopenia, anemia hemolitic		No	Pulmonary
106	F	67	PAPS	CVA, MI, LV, skin ulcers.	Urinary infection	No	ARDS
107	M	64	Rheumatoid arthritis	MI , thrombophebitis	Respiratory & leg ulcer infection	No	Renal
108	F	20	PAPS		Throat infection	Yes
109	F	47	PAPS	DVT	Infection	No	Pulmonary
110	F	22	SLE	Abortion	Infection ,HELLP	No	Renal
111	M	27	PAPS	DVT, PE	Anticoagulation withdrawal	No	Hepatic
112	F	51	SLE	CVA, Libman-sacks	Upper respiratory infection	No	Abdominal
113	M	35	PAPS	DVT, PE, atrium thrombi		No	Abdominal
114	F	53	PAPS			Yes	NR
115	M	41	SLE	Gangrene of toe, leg ulcer, thrombocytopenia, hemolitic anemia		No	Abdominal
116	F	35	SLE	Still birth(24wk) with infracted placenta, digital ischemia	SLE flare	No	Abdominal
117	M	30	PAPS		Trauma	Yes	Hypertension
118	M	38	Ulcerative colitis	Thromocytopenia, abortions	Cholangiocarcinoma	No
119	F	25	SLE		Viral infection	Yes
120	F	23	PAPS		OC	Yes
121	F	27	PAPS	DVT, fetal loss	Post-fetal loss	No
122	F	69	Lupus- like		Limphoma	Yes
123	F	34	Lupus- like		Pelvic surgery for benign ovarian mass	Yes
124	M	36	SLE		SLE flare	Yes
125	F	39	Lupus- like	Amauroxis fugax, TIA, toe ulceration, LR, fetal loss		No
126	F	43	SLE		SLE flare, respiratory tract infection	Yes
127	F	49	SLE		Major abdominal surgery, suspected cholangitis	Yes
128	F	32	SLE	DVT	Abdominal surgery for reconstruction of colon previous colectomy	No
129	F	26	SLE	LR, skin ulcers		No
130	F	25	SLE	Thrombocytopenia, LR		No
131	F	47	SLE	DVT,  PE	SLEflare,Anticoagulation withdrawal	No	Pulmonary
132	M	39	PAPS	Digital ulcer		No	ARDS
133	F	28	PAPS			Yes	Neurologic
134	F	21	PAPS	DVT	A-V Shunt surgery	No
135	M	29	SLE	MI,CVA,thrombocytopenia,LR, digital ulcers, thrombosis of the  inferior & superior mesenteric artery	Repair ileostomy & Colostomy	No	Abdominal
136	F	37	SLE	Fetal loss, LR, Thrombocytopenia	Renal failure	No	Renal
137	M	66	Ulcerative colitis			Yes	Malignant hypertension
138	F	67	Dermatomyositis			Yes	Cardiac
139	F	32	PAPS			Yes	Cardiac
140	F	62	Rheumatoid arthritis			Yes
141	F	49	Rheumatoid arthritis	CVA (2) PE, MI, hepatic infarctin	Hysterectomy	No	Renal
142	F	35	SLE	Raynaud, LR, PE, abortion, finger tips ulcers, thrombocytopenia	Anticoagulation withdrawal	No	Skin
143	F	46	SLE			Yes	Neurologic
144	M	54	PAPS			Yes
145	M	37	PAPS	DVT,MI,hypertension, Leriche´s syndrome, carotid artery stenosis		No
146	M	33	SLE	Thrombocytopenia, PE	Danazol	No	Neurologic
147	M	68	PAPS			Yes	Cardiac
148	F	33	PAPS		OC	Yes
149	F	18	SLE	Thrombocytopenia	Respiratory infection	No
150	F	42	SLE		Urinary infection	Yes
151	F	47	PAPS	epilepsy, stroke		No
152	M	40	PAPS	epilepsy, thrombocytopenia, leg ulcer, Libman-Sachs	Anticoagulation withdrawal	No
153	F	24	SLE	hemolytic anemia, livedo reticularis, thrombocytopenia, diplopia	infection	No
154	F	34	SLE		Dental infection, facial cellulitis	Yes
155	F	40	Discoid lupus			Yes
156	F	43	SLE	CVA (3)	Treatment of breast cancer	No	Neurologic
157	F	61	PAPS	CVA	Adenocarcinoma in colon	No	Peripheral
158	M	55	PAPS	DVT, postphlebitic syndrome	ACE inhibitor introduction (VCE alteration)	No	Peripheral
159	F	36	PAPS	Fetal loss (2), DVT, skin ulcer		No	Ocular
160	M	39	PAPS			Yes	Renal
161	F	33	PAPS	Abortion	Ovulation induction	No
162	M	47	PAPS		Blunt abdominal trauma	Yes
163	F	38	PAPS	Thrombocytopenia, Psychiatric  features		Yes	Pulmonary
164	F	14	SLE			Yes	Renal
165	M	29	SLE	MI, thrombocytopenia, CVA, PE, avascular necrosis	Bowel surgery and withdrawal of anticoagulation	No	Abdominal
166	M	37	Behçet	Central retina vein thrombosis		No	Cardiac
167	F	66	PAPS		Surgery	Yes	Hepatic
168	M	50	PAPS		Primary lung cancer	Yes
169	F	37	SLE	Fetal loss (2), splinter hemorrhage, LR		No	Renal
170	M	63	Lupus like		Surgery	Yes	Neurologic
171	M	38	PAPS			Yes
172	F	38	PAPS	CVA, epilepsy, LR, retinal thrombosis, peripheral artery thrombosis		No
173	F	28	SLE	abortin, thrombocytopenia	Oral contraceptives	No	Cardiac