Table 2

 Clinical manifestations at the time of presentation with catastrophic antiphospholipid syndrome in patients from the CAPS registry

Featuren (%)
RDS, respiratory distress syndrome.
Peripheral thrombosis 74 (34)
Deep vein thrombosis50 (23)
Femoral artery8 (4)
Radial artery4 (2)
Other arteries19 (9)
Cerebral 133 (60)
Infarcts97 (44)
Encephalopathy17 (8)
Seizures13 (6)
Microthrombosis10 (5)
Venous cerebral thrombosis5 (2)
Coma4 (2)
Transient ischaemic attack2 (1)
Cardiac 115 (52)
Valve lesion56 (26)
Myocardial infarction50 (23)
Heart failure22 (10)
Microthrombosis10 (5)
Mural thrombi9 (4)
Pulmonary 146 (66)
Acute RDS74 (34)
Pulmonary embolism54 (24)
Pulmonary haemorrhage16 (7)
Microthrombosis10 (5)
Pulmonary oedema7 (3)
Infarction6 (3)
Abdominal 189 (86)
Renal154 (70)
Hepatic62 (28)
Splenic41 (19)
Adrenal33 (15)
Intestinal27 (12)
Mesenteric23 (11)
Pancreas21 (10)
Portal vein thrombosis7 (3)
Inferior cava thrombosis7 (3)
Gallbladder thrombosis6 (3)
Skin 104 (47)
Livedo reticularis62 (28)
Skin ulcers30 (14)
Digital ischaemia21 (10)
Purpura12 (6)
Necrosis7 (3)
Microthrombosis7 (3)
Splinter haemorrhages5 (2)
Other manifestations 56 (25)
Retinal artery thrombosis11 (5)
Bone marrow necrosis7 (3)
Uterus7 (3)
Neuropathy7 (3)
Testicles4 (2)
Retinal vein thrombosis4 (2)
Thyroid thrombosis3 (1)
Avascular necrosis4 (2)
Others8 (4)