. 2019 Sep 16;12(9):e230863. doi: 10.1136/bcr-2019-230863

Table 1.

Thirty-five cases of CAPS in pregnancy and puerperium

Author (reference no.)	Maternal age	Time of onset	CAPS features	Treatment received	Maternal outcome	Fetal outcome
Khizroeva et al ¹⁴	n/a	28 weeks of gestation	Multiorgan failure	n/a	n/a	n/a
Hanouna et al ¹⁵ (Case 1)	30 years	15th day of puerperium	Renal, hepatic, cutaneous, haemolytic anaemia, placenta	Heparin, aspirin, glucocorticoids	n/a	Healthy child
Hanouna et al ¹⁵ (Case 2)	32 years	Eighth day of puerperium	Cardiac, renal, hepatic, cutaneous, haemolytic anaemia, thrombocytopaenia	Heparin, aspirin, glucocorticoids, IVIG	n/a	Healthy twins
Hanouna et al ¹⁵ (Case 3)	26 years	25 weeks of gestation	Cardiac, neurological, renal, cutaneous, haemolytic anaemia	Heparin, glucocorticoids, IVIG, plasma exchange, dialysis	n/a	Fetal death
Hanouna et al ¹⁵ (Case 4)	31 years	Third day of puerperium	Cardiac, renal, splenic, cutaneous, hepatic, thrombocytopaenia, haemolytic anaemia	Heparin, aspirin, glucocorticoids, IVIG, dialysis	n/a	Fetal death
Hanouna et al ¹⁵ (Case 5)	37 years	Fifth day of puerperium	Neurological, cutaneous, hepatic, thrombocytopaenia, haemolytic anaemia, placenta	Heparin	Venous thrombosis at 2 months, PE at 3 months	Fetal death
Hanouna et al ¹⁵ (Case 6)	33 years	On the day of the delivery at 36.5 weeks gestation	Adrenal, cutaneous, hepatic, thrombocytopaenia, haemolytic anaemia	Heparin, glucocorticoids	Adrenal insufficiency	Healthy child
Hanouna et al ¹⁵ (Case 7)	32 years	Fourth week of puerperium	Cardiac, neurological, pulmonary, renal, hepatic, pancreatic, splenic, ocular, thrombocytopaenia	Heparin, glucocorticoids, IVIG, plasma exchange, dialysis	Mild retinal sequelae	Fetal death
Hanouna et al ¹⁵ (Case 8)	29 years	15th day of puerperium	Cardiac, neurological, renal, cutaneous, hepatic, pancreatic, gastric, ocular, thrombocytopaenia, haemolytic anaemia	Heparin, glucocorticoids, plasma exchange, dialysis	Renal insufficiency, stroke at 4 years, sudden death at 6 years	Fetal death
Hanouna et al ¹⁵ (Case 9)	32 years	Second day of puerperium	Adrenal, renal, hepatic, thrombocytopaenia	Heparin, aspirin, glucocorticoids	Adrenal insufficiency	Healthy child
Hanouna et al ¹⁵ (Case 10)	36 years	Third day of puerperium	Cardiac, neurological, renal, hepatic, pancreatic, cutaneous, thrombocytopaenia, haemolytic anaemia	Heparin, glucocorticoids	n/a	Healthy child
Hanouna et al ¹⁵ (Case 11)	32 years	On the day of the delivery at 17 weeks gestation	Cutaneous, hepatic, renal, adrenal, thrombocytopaenia, gallbladder	Heparin, glucocorticoids, plasma exchange, dialysis	Renal insufficiency with proteinuria	Fetal death at 17 weeks
Hanouna et al ¹⁵ (Case 12)	27 years	On the day of the delivery at 13 weeks gestation	Cardiac, cutaneous, hepatic, placenta, thrombocytopaenia	Heparin, glucocorticoids, IVIG	n/a	Fetal death at 13 weeks
Hanouna et al ¹⁵ (Case 13)	23 years	31 weeks of gestation	Cardiac, renal, cutaneous, thrombocytopaenia, haemolytic anaemia	Heparin, aspirin, glucocorticoids, plasma exchange	Sudden death at 2.5 years	Child with developmental delay
Derks et al ¹⁶ (Case 1)	32 years	n/a	Multiple infarcts in liver and placenta	Intensive medical treatment including anticoagulation	Died of massive PE	n/a
Derks et al ¹⁶ (Case 2)	27 years	n/a	Thrombocytopaenia, Disturbances in hepatic function, epigastric pain	Glucocorticoids	n/a	Fetal death
Derks et al ¹⁶ (Case 3)	36 years	n/a	Hepatic infarcts, petechiae	Glucocorticoids, IVIG, plasmapheresis	n/a	Healthy Child
Marson et al ¹⁷	33 years	23 weeks of gestation	HELLP, thrombocytopaenia, anaemia, acalculous cholecystitis, Cutaneous	Therapeutic plasma exchange	Recovery	Intrauterine death at 23 weeks
Bendon et al ¹⁸	22 years	30 weeks of gestation	Placental infarctions myocardium, renal, gastrointestinal and myometrium TMA		Death	Intrauterine fetal death
Hochfeld et al ¹⁹	37 years	Second day after fetal death	Renal failure, cerebral, cardiac, pulmonary, splenic, and adrenal infarcts, cerebral haemorrhage	Glucocorticoids, cyclophosphamide, plasma exchange	Death	Intrauterine fetal death
Kupferminic et al ²⁰	17 years	Fifth day of puerperium	HELLP, ARDS, placental infarcts, renal failure	Glucocorticoids, plasma exchange, dialysis	Recovery	Prematurity
Kitchens²¹	38 years	38 weeks of gestation	HELLP, portal vein, inferior vena cava, mesenteric vein thrombosis	Anticoagulation	Recovery
Wislowska et al ²²	26 years	25 weeks of gestation	ARDs, encephalopathy, nephritis, Skin ulcers	LMWH, glucocorticoids, cyclophosphamide	Recovery	Miscarriage
Sinha et al ²³	22 years	25 weeks of gestation	HELLP, placental infarcts, cerebral infarcts, Bone marrow necrosis	Glucocorticoids, plasma exchange, IVIG	Death	Death
Asherson et al ²⁴	22 years	20 weeks of gestation	HELLP, ARDS, cerebral infarcts	Glucocorticoids, intravenous heparin, cyclophosphamide	Recovery	Death
Asherson et al ²⁴	27 years	Postfetal loss	PE, digital necrosis, hepatic, renal, intestinal, mesenteric thrombosis	Glucocorticoids, anticoagulation	Death	Death
Ortiz at al²⁵	32 years	Second day of puerperium	Renal, multiple cerebral infarcts	Glucocorticoids, FFP, LMWH	Recovery	n/a
Koenig et al ²⁶	19 years	17 weeks of gestation	HELLP, hepatic infarctions, bone necrosis	LMWH, FFP	Recovery	Death
Coward et al ²⁷	30 years	Third week of puerperium	TIA, status epilepticus, renal failure, adrenal haemorrhage	Anticoagulants, dialysis	Death	Healthy child
Weiser M⁶	33 years	Fifth day of puerperium	HELLP, ARDS, renal failure, cerebral infarctions and haemorrhage	Glucocorticoids, IVIG, anticoagulation, dialysis	Death	Healthy child
Gomes-Puerta et al ⁶ (case 1)	29 years	28 weeks of gestation	HELLP, bone marrow hypoplasia, renal failure, DVT, respiratory failure, livedo reticularis	Glucocorticoids, LMWH	Death	Prematurity
Gomes-Puerta et al ⁶ (case 2)	26 years	Third day of puerperium	DVT, PE, respiratory failure	LMWH, glucocorticoids, IVIG	Recovery	Healthy child
Gomes-Puerta et al ⁶ (Case 3)	28 years	Sixth day of puerperium	Placental infarctions, Respiratory failure, Renal failure, Encephalopathy	LMWH, plasma exchange	Recovery	Healthy child
Elchalal et al ²⁸	37 years	16 weeks of gestation	Thrombocytopaenia, Bilateral pleural effusions	LMWH, IVIG, Glucocorticoids	Recovery	Fetal death
Ciolkiewicz et al ⁷	24 years	Postfetal loss	PE, Myocardial infarction, multiorgan failure	n/a	Death	Fetal death

ARDS, acute respiratory distress syndrome; CAPS, catastrophic antiphospholipid syndrome; DVT, deep vein thrombosis; FFP, fresh frozen plasma; HELLP, haemolysis, elevated liver enzymes, low platelet count; IVIG, intravenous immunoglobulin; LMWH, low molecular weight heparin; PE, pulmonary embolism; TMA, thrombotic microangiopathy; n/a, not available.