Table 2 Clinical and serological characteristics and outcome of patients with catastrophic antiphospholipid syndrome during pregnancy or puerperium.
| Author (CAPS registry number) | Catastrophic APS features | Laboratory findings | Treatment | Outcome | |
|---|---|---|---|---|---|
| Maternal | Fetal | ||||
| Bendon et al11*(6) | Placental infarctions myocardium, renal, gastrointestinal and myometrium TMA | Severe thrombocytopenia | NR | Death | Intrauterine fetal death |
| Schistocytes | |||||
| aCL positive | |||||
| LA: ND | |||||
| Hochfeld et al12 (22) | HELLP, iliac, pelvic vein and skin thrombosis | DIC | Anticoagulation (IV heparin) | Death | Intrauterine fetal death |
| Renal failure, cerebral, cardiac | LA positive | Steroids | |||
| Pulmonary, splenic and adrenal | IgG aCL (high titres) | Cyclo | |||
| Infarcts, cerebral haemorrhage | Plasma exchange | ||||
| Kupferminc et al13†(31) | HELLP, ARDS | LA positive | Steroids | Recovery | Prematurity |
| Placental infarcts, renal failure | IgG aCL (26.5 GPL) | Plasma exchange | |||
| Alveolar haemorrhage | Dialysis | ||||
| Kitchens14*(65) | HELLP, portal vein | aCL strongly positive | Anticoagulation (IV hepatin) | Recovery | NR |
| Inferior cava, hepatic and | LA negative | Streptokinase | |||
| mesenteric vein thrombosis | |||||
| Wislowska et al15* (82) | ARDS, encephalopathy | LA positive | Anticoagulation (LMWH) | Recovery | Miscarriage |
| Rapid progressive nephritis | IgG aCL moderate levels | Steroids | |||
| Skin ulcers | Cyclo | ||||
| Sinha et al16* (100) | HELLP | Severe pancytopenia | Steroids | Death | Death (intracerebral haemorrhage) |
| Placental infarcts | IgG aCL (203 GPL) | Plasma exchange | |||
| Cerebral infarcts | IgM aCL (10 MPL) | IVIG | |||
| Bone marrow necrosis | LA positive, B2GP1 IgG | ||||
| Asherson et al7*(110) | HELLP | Thrombocytopenia | Steroids | Recovery | Death |
| Renal TMA, ARDS | Schistocytes, LA positive | Cyclo | |||
| Cerebral infarcts | IgG and IgM aCL positive | Anticoagulation (IV heparin) | |||
| Asherson et al7 (121) | PE, digital necrosis | Haemolytic anaemia | Steroids | Death | Death |
| Hepatic, renal, intestinal and | IgG aCL (72 GPL) | Anticoagulation | |||
| mesenteric thrombosis | LA negative | ||||
| Ortiz et al17† (240) | Renal TMA, valve lesions | Thrombocytopenia | Steroids, FFP | Recovery | NR |
| Multiple cerebral infarcts | Haemolytic anaemia | Anticoagulation (LMWH) | |||
| Schistocytes, LA positive | |||||
| IgG aCL (>120 GPL) | |||||
| IgM aCL (19.2 MPL) | |||||
| Koenig et al18*(251) | HELLP | Severe thrombocytopenia | Anticoagulation (LMWH) | Recovery | Death |
| Hepatic infarctions | IgG and IgM aCL positive | FFP | |||
| Bone necrosis, bowel TMA | B2GP1, LA positive | ||||
| Coward et al19†(252) | TIA, status epilepticus | LA positive | Anticonvulsants | Death | Healthy child |
| Renal failure, brain and pulmonary TMA | Dyalysis | ||||
| Adrenal haemorrhage | |||||
| Weiser M(‡)†(99) | HELLP, ARDS | Severe thrombocytopenia | Steroids, IVIG | Death | Healthy child |
| Renal failure, cerebral | IgG aCL (>100 GPL) | Anticoagulation | |||
| infarctions and haemorrhage | LA positive | Dyalysis | |||
| Present case 1* | HELLP, bone marrow | DIC | Steroids | Death | Prematurity |
| hypoplasia, renal failure | Pancytopenia | Anticoagulation (LMWH) | |||
| DVT, respiratory failure | IgG aCL high titres | ||||
| Livedo reticularis | LA negative | ||||
| Present case 2† | DVT, PE | IgG aCL high titres | Anticoagulation (LMWH) | Recovery | Healthy child |
| TIA, respiratory failure | LA negative | Steroids, IVIG | |||
| Present case 3† | Placental infarctions | Severe thrombocytopenia | Anticoagulation (LMWH) | Recovery | Healthy child |
| Renal failure, encephalopathy | LA positive, DIC, | Plasma exchange | |||
| Respiratory failure | aCL IgG (24 U/ml) | ||||
| B2GP1 IgG (44.9 U/ml) | |||||
aCL, anticardiolipin antibodies; APS, antiphospholipid syndrome; ARDS, acute respiratory distress syndrome, B2GP1, β2‐glycoprotein 1 antibodies; Cyclo, cyclophosphamide; DIC, disseminated intravascular coagulation; DVT, deep vein thrombosis; FFP, fresh frozen plasma; HELLP, haemolysis, elevated liver enzymes, low platelet count; IVIG, intravenous immunoglobulins; LA, lupus anticoagulant; LMWH, low molecular weight heparin; ND, not done; NR, not reported; PE, pulmonary embolism; TIA, transient ischaemic attack; TMA, thrombotic microangiopathy.
*Presenting during pregnancy.
†Presenting during puerperium.
‡This case was included in the CAPS registry by Manfred Weiser, Salzburg, Austria.