THE "CAPS" REGISTRY-7

Table 7. Evolution of patients with catastrophic APS.

PATIENT No.	TIME EVOLUTION	THROMBOTIC MICROANGIPATHY BIOPSY	TREATMENT	OUTCOME
1	Days 7	Renal	S, Cyclo, ancrod, diuretics, methildopa, aspirin	Recovery
2	“rapidly”	Skin	S, P, aspirin, dipyridamole, cyclo, AC	Recovery
3	Days 2	Skin	S, P, cyclo, AC	Recovery
4	Day 1	Heart, lung, kidney, liver, pancreas	S, cyclo, P, HD	Death
5 (1^st )	NR	Skin, kidney	S, AC	Recovery
5 (2^nd)	Days 7	Heart, kidney, brain		Death
6	Day 1	Heart, kidney, myometrium, gut		Death
7	Months 2		S, P, cyclo, AC, antihypertensive	Recovery
8	Days 7		S, aspirin, dipirydamole, antibiotics, vasodilatadors	Death
9	Days 7	Liver	S, P, dialysis	Recovery
10	Days 4	Kidney	S, AC, Hidralazyne	Recovery
11			S, P, AC	Recovery
12	Days 8	Spleen, bone marrow, nose, skin	S, AC,GG, splenectomy	Recovery
13	Days 4	Testicles, skin, brain, lung, adrenal	S, P, cyclo, AC, Antibiotics	Death
14	Day 1		S, AC, aspirin	Recovery
15	Days 2		S, P, AC, HD,	Recovery
16	Day 1	Heart, liver, lung, kidney, brain	Ventilatory support, antibiotics	Death
17			S, AC	Death
18		Gut, brain, lung	AC, HD	Death
19	Days 7		Fibrinolytics, angioplasty	Death
20	Days 3	Heart, kidney, gut, adrenal.	Support treatment	Death
21 (1^st)	Days 4		S, AC, GG,P, cyclo	Recovery
(2^nd)	Simultaneously		S, AC, P, GG, Cyclo	Recovery
22	NR	Brain, skin, heart, spleen, adrenal	P, cyclo, AC, GG	Death
23	Months 2	Heart, pancreas, brain, kidney	S, AC, aspirin	Death
24	Days 4	Heart, brain, kidney, pancreas.	S	Death
25			AC, P, HD	Death
26	Days 14		S, P, AC, cyclo, GG, vincristine, splenectomy	Recovery
27			S, AC, P	Recovery
28	Days 5		AC, HD, antibiotics, diuretics, oxygen	Death
29				Death
30	Days 6		S, cyclo, antihypertensive	Death
31	Days 2		S, P, AC, HD, ventilatory support, antihypertensive, diuretics, antibiotics	Recovery
32	Days 3		S, AC, P, aspirin	Recovery
33	Days 3		S, AC, GG, ventilatory support	Recovery
34	Day 1	Skin	S, AC, P, cyclo	Recovery
35	Day 1	Many organs	S, P, AC, cyclo, HD	Death
36	Day 14	Kidney, gut, pancreas, spleen, adrenal, thyroid, muscle, nerve, lung, heart.	S, HD, ventilatory support	Death
37			S, P, AC	Death
38			S, P, AC, cyclo, HD	Death
39			AC, S, cyclo	Recovery
40	Day 1	Kidney	S, P,aspirin	Recovery
41	Day 1		S, P, AC, HD, ventilatory support	Recovery
42			AC	Recovery
43			AC	Recovery
44			AC	Recovery
45			AC	Recovery
46	Days 7		AC, aspirin, defibrotide, fibrinolytics, ventilated support	Recovery
47	Suddenly	Heart, brain, liver, adrenal	S, cyclo	Death
48	Few days	Kidney, lung	S, GG, Cyclo, danazol	Death
49			S, AC,GG	Recovery
50	Months	Gut, lung	S, AC, cyclo, GG,cyclosporine	Death
51	Days 6	Skin	S, AC	Recovery
52	Days 7	Kidney	S, cyclo	Death
53	Days 7	Heart, skin, brain, pleen, kidney	Prostacyclin, HD, ventilated support, antibiotics	Death
54	Day 1	Brain, myocardium, lung, kidney, skin, bone marrow, uterus, ovary, bladder, pancreas, gastrointestinal	S, P, AC, Cyclo, ventilated support,	Death
55	Weeks	skin	S, Cyclo, AZA	Death
56			AC, thrombectomy, anastomosis	Recovery
57	Days 7	Skin	AC, amputation	Recovery
58	Days 3	Kidney	S, cyclo, P, HD	Recovery
59	Days 3	Bone marrow	AC, ventilated support, angioplasty	Recovery
60				Recovery
61	Days 4	Skin	S, AC	Recovery
62	Days 7	Gut	S, AC,ventilated support, colectomy	Recovery
63		Skin	S, AC, GG, aspirin, antibiotic, amputation	Recovery
64	Few days		S, AC, prostacyclin, antibiotics, ventilated support, inotrópics, amputation	Recovery
65	Day 1		AC, fibrinolytics	Recovery
66	Day 4	Skin. Liver, gallbladder	AC, fibrinolytics,amputation	Recovery
67	Day 1		AC, fibrinolytics	Recovery
68	Suddenly	Myocardium, brain, spleen, gut, pancreas	S, cyclo, aspirin	Death
69	Few days	Spleen, lung, kidney, gastric, heart	Ventilated support, antibiotics	Death
70(1st)	Days 3		S, AC, diuretics	Recovery
(2nd)	Few days		S, cyclo,AC, aspirin,diuretics, antibiotics	Recovery
(3rd)	Suddenly		S, AC, HD, digoxin	Death
71	Days 7		S, AC	Recovery
72	Day 1	Kidney	S, P, cyclo, AC, GG, ventilated support, inotropics, haemophiltration	Death
73	Days 6	Skin	S, AC, cyclo, antibiotics, amputation	Recovery
74			S, AC, antibiotics,	Recovery
75			S, AC, cyclo, antibiotics	Recovery
76			S, AC, cyclo, antibiotics	Death
77			S, AC, cyclo, antibiotics	Recovery
78			S, AC, cyclo, antibiotics	Death
79			S, AC, cyclo	Recovery
80			S, AC, cyclo,GG	Recovery
81			S, AC, cyclo, antibiotics	Recovery
82	Days 2		S, AC, cyclo	Recovery
83(1st)	Days 7		S, AC, Ventilated support	Recovery
83(2nd)			S, AC	Recovery
84	Months	Skin	AC	Death
85	< days 8	Gut, pancreas, spleen, adrenal, thyroid, muscles, nerves, lung, heart	S, HD, antibiotics, ventilated support	Death
86	Day 1	Kidney	S, AC	Death
87	Days 5	Kidney	GG, splectomy, HD, ventilated support, antibiotics	Recovery
88		Renal & cerebral infarcts	AC	Death
89			S, P, cyclo, AC, fibrinolytics, HD, amputation	Recovery
90	Day 1	Skin	P, AC	Death
91	Days 3	Uterus, kidney, spleen, adrenal, lung	S, AC,cyclo	Death
92	Days 7	Kidney, lung, myocardium, liver, gallbladder, bone, adrenal	AC	Death
93	Day 1	Kidney, brain, heart, skin	S,cyclo	Death
94	Day1	Lung, pleura, heart, brain, skin	S, P, cyclo, HD	Death
95	Few days	Kidney, heart, lung, spleen	S, agrsive treatment	Detah
96	“Rapid course”	Kidney, spleen, ovaries, brain, lung	S, P, support treatment	Death
97	Day1		S, P, AC, cyclo	Recovery
98	Days 2			Death
99	Days 5		S, AC,GG, ventilated support	Death
100			P, GG	Death
101	Days 3		S,cyclo, AC,aspirin, prostacyclin, antibiotics, sipport	Death
102		Kidney	S, P, AC, cyclo, GG, HD, ventilated support	Recovery
103	Suddenly		S, AC, HD, nytroglicerine	Death
104	Few days		S, AC,GG, diuretics, digoxin	Death
105			S, AC, antibiotics	Death
106	Suddenly		S, AC, aspirin, oxygen	Death
107	Days 6	Brain, cerebellum, lung, pancreas, adrenal, kidney, myocardium, skin, parathyroids.	S, P, AC, GG	Death
108	Day 1		S, AC, cyclo, GG, antibiotics	Recovery
109	Day 1		S, P, AC, ventilated support	Recovery
110		Renal	S, AC, cyclo,GG, nephrectomy, antibiotics	Recovery
111	Day 2		AC, fibrinolytics, ventilated support	Recovery
112	Day 1		S, AC	Recovery
113			S, AC, GG	Recovery
114			AC, defibrotide	Recovery
115	“ Several days”	Gut, kidney		Death
116	Day1	Gallbladder	S, AC	Recovery
117		adrenal	S	Recovery
118			S, P, AC, cyclo	Recovery
119			S, P, AC	Recovery
120			S, AC	Recovey
121			S, P, AC	Recovery
122			S, P AC, cyclo, HD	Death
123			S, P AC, GG, HD	Death
124			S, P, AC, cyclo	Recovery
125			S, AC, cyclo, GG, Prostacyclin	Recovery
126			S, AC	Death
127			S, AC, cyclo, HD, P	Death
128			S, AC	Death
129			S, AC, cyclo	Death
130			S, AC	Recovery
131	Day 1	Lung	S, AC, cyclo, support	Death
132	Days 6		S ,P, AC,GG,ventilated support, dypiridamole, inotropics	Recovery
133	Days 7		S, ,P, AC, GG, ventilated support	Recovery
134	Day 1		S, P, AC, GG,HD	Recovery
135	“Suddenly”		S, AC	Death
136	“Few days”	Kidney, heart, lung, spleen, brain, skin	S, HD, “aggresive treatment”	Death
137	Day 1		S, AC, amputation	Recovery
138		Kidney	AC,  HD, diuretics, nitrates, renal replacement	Recovery
139	Day 3		S, AC, diuretics	Recovery
140	Few days		AC, antibiotics, diuretics	Recovey
141			AC	Recovery
142	Days 4	Kidney, skin	S, P, AC, cyclo, HD	Recovery
143	Weeks 2	Myocardium, brain, cerebellum, pancreas, kidney, genitourinary tract	S, P, Cyclo	Death
144	Weeks 6		S, P, AC	Recovery
145			S, P, AC, GG, HD	Death
146			S, AC, HD	Recovery
147	Day 1		AC, fibrynolitics	Recovery
148	Day 1		S, AC	Recovery
149			AC,S, GG, Cyclo, HD, P	Death
150			S, AC, GG	Recovery
151			S,AC,Cyclo,	Death
152			S,AC, P,HD	Death
153			S,AC,GG	Death
154			S, AC,GG	Recovery
155			S,AC,Aspirin	Recovery
156	Months		AC, Cyclo, aspirin	Recovery
157	Weehs 4		AC, aspirin	Recovery
158	Days 10		AC, Debibrotide, Aspirin, dypiridamole	Recovery
159		Lung, bladder, pancreas, orbital tissues	S, AC, HD, ventilated support	Death
160	Days 2		AC, HD, aspirin, thrombolysis	Recovery
161			Curettage, support treatment	Recovery
162			AC	Recovery
163	Days 3	Skin	S, HD, ventilated support	Death
164	Days 4	Brain, kidney, lung, spleen, liver	S, AC, Cyclo	Death
165	Day 1		S, AC, Antithrombin III, support treatment	Death
166	Weeks 2		S, AC Cyclo	Recovery
167	Weeks 2	Liver, bone marrow	S, P, Cyclo	Recovey
168			S, AC	Death
169	Days 7	kidney	, S, P, AC, Cyclo, HD, ventilated support	Recovery
170	“Suddenly”		S, P, AC, Cyclo, GG, ventilated support	Recovery
171			S, P, GG	Death
172			S, AC	Death
173		kidney	S, AC, diuretics	Recovery
174	“Few days”		AC	Recovery
175	Days 3		S	Death
176			S, AC, Cyclo, GG	Death
177			AC, S, Cyclo, GG, amputation	Death
178			S, AC, GG	Death
179	Day 1		AC	Recovery
180	Days 12		S, AC, Cyclo	Recovery
181	Days 5		AC, Amputation	Recovery
182	Day 1		AC,HD, antibiotics	Death
183	Days 7		S, AC, GG, ventilated support	Recovery
184	Day 1	Skin, kidney	AC, antihypertensives	Recovery
185	Days 4		S, AC, ventilated support	Recovery
186	Weeks 2	Brain, heat, lung, gut, liver	S, P, AC,	Recovery
187	8 ± 2	Lun, kidney	S, AC	Death
188	8 ± 2		AC	Death
189	8 ± 2	Kidney, liver. Spleen	S, AC	Death
190	8 ± 2	Brain, kidney, liver	S	Death
191	8 ± 2	Brain, adrenal, spleen, skin	S, AC	Death
192	8 ± 2	Brain, adrenal, spleen, skin	AC	Death
193	8 ± 2	Brain, kidney, skin	S, Pentoxifiline, nifedipine	Death
194	8 ± 2	Brain, kidney	AC	Death
195	8 ± 2	Brain, kidney	AC	Death
196	8 ± 2	Gut, kidney	AC	Death
197	8 ± 2	Brain, lung, skin	AC	Death
198	8 ± 2	myocardium	AC	Death
199	Day 1		S, P, AC, Cyclo, GG	Recovery
200	Day 1		S, P, AC, Cyclo, GG	Recovery
201			S, P, AC	Recovery
202			S, P, AC, Cyclo, antibiotics, ventilated support	Recovery
203	“very short period of time”		NR	Recovery
204			S, P, AC, HD	Death
205	Day 5		S, AC, immunosupresives, ticlopidine, ventilated support	Recovery
206			S, P, AC, HD	Recovery
207	Suddenly	Gut, lung	S, AC, GG	Recovery
208			S, P, AC, Cyclo, GG, thrombolysis	Death
209			S, AC, Cyclosporine, GG Mycophenolate mofetil	Death
210			S, AC	Death
211			P, AC, Cyclo, GG	Recovery
212			S, AC, Cyclo	Recovery
213			S, P, AC, GG, HD	Death
214			S, AC	Death
215			AC	Recovery
216			S, AC	Death
217			S , P, HD	Death
218			S, AC, Cyclo	Death
219			AC, prostaglandin	Recovery
220			AC	Recovery
221 (1^st)	Simultaneously		S, P, AC	Recovery
(2^nd)	Less than a week		S, AC, PE	Recovery
222	Day 1	Renal	S, Cyclo, P, AC, Dyalisis	Recovery
223	Days 6		S, AC, GG, aspirin, azathioprina	Recovery
224	Days 3		S, Cyclo, AC, GG, antibioyics.	Death
225	Simultaneously		S, P, AC, GG, HD	Recovery
	Simultaneously		S, P, AC	Recovery
226	Suddenly	Bowel, liver	AC	Death
227	Days 5	Bowel	S, P, AC, GG, intestinal resection	Recovery
228	suddenly	Lung	S, GG, ventilated support	Death
229			S, Cyclo, AC, GG	Death
230		Lung	S, Cyclo, AC, aspirin	Death
231			AC, aspirin	Death
232			S, P , AC, GG	Recovery
233			S, P dyalisis, antibiotics, ventilated suport	Death
234	Days 4		S, AC, antibiotics.	Recovery
235	Simultaneously		S, AC, antibiotics, ventilated support	Recovery
236	Simultaneously		S, AC, GG	Recovery
237	Less than a week		S, AC, P. HD	Recovery
238	Less than a week		P, AC aspirin, HD	Recovery
239	Less than a week		S, P, AC, GG, cyclo, ventilated support	Recovery
240	A week	Kidney	S, AC, aspirin, fresh frozen plasma	Recovery
241	Simultaneously	Skin	S, AC,aspirin, HD	Death
242	Simultaneously	Skin, liver	S	Recovery but recurrent CAPS on 17 ocassions
243	Less than a week	Liver	S, AC, rituximab	Recovery
244	Less than a week	Liver, spleen, gallblader	P, AC, aspirin, HD