Demographic and clinical characteristics of patients with antiphospholipid antibody-associated chorea
| PUMCH (n=13) | Literature (n=167) | Overall (n=180) | |
| Demographics | |||
| Sex, female | 8/13 (61.5) | 139/167 (83.2) | 147/180 (81.7) |
| Age of onset of chorea, years | 25.0±15.9 | 22.6±16.0 | 22.8±16.0 |
| Age of first aPL positivity, years | 25.1±16.0 | 26.3±18.6 | 26.1±18.2 |
| Time from first aPL positivity to onset of chorea, months | −1.2 (−14.1, 11.3) | 0 (−60.0, 336.0) | 0 (−60.0, 336.0) |
| Chorea as initial presentations | 10/12 (83.3) | 77/87 (88.5) | 87/99 (87.9) |
| SLE criteria met | 8/13 (61.5) | 71/167 (42.5) | 79/180 (43.9) |
| 2006 APS criteria met | 5/13 (38.5) | 69/167 (41.3) | 74/180 (41.1) |
| 2023 APS criteria met | 6/13 (46.2) | 72/167 (43.1) | 78/180 (43.3) |
| PAPS | 2/6 (33.3) | 39/72 (54.2) | 41/78 (52.6) |
| SAPS (secondary to SLE) | 4/6 (66.7) | 33/72 (45.8) | 37/78 (47.4) |
| Comorbidities | |||
| Smoking | 0/11 (0) | – | – |
| Obesity (BMI ≥30 kg/m2) | 0/11 (0) | – | – |
| Arterial hypertension | 2/11 (18.2) | – | – |
| Diabetes mellitus | 0/11 (0) | – | – |
| Coronary artery disease | 0/11 (0) | – | – |
| Hyperlipidaemia | 2/11 (18.2) | – | – |
| Hyperhomocysteinemia | 2/11 (18.2) | – | – |
| Clinical features | |||
| VTE with a high-risk VTE profile | 0/12 (0) | 0/167 (0) | 0/179 (0) |
| VTE without a high-risk VTE profile | 1/12 (8.3) | 32/167 (19.3) | 33/179 (18.4) |
| AT with a high-risk CVD profile | 0/12 (0) | 2/167 (1.2) | 2/179 (1.1) |
| AT without a high-risk CVD profile | 4/12 (33.3) | 48/167 (28.7) | 52/179 (29.1) |
| Suspected livedo racemosa | 0/12 (0) | 11/167 (6.6) | 11/179 (6.1) |
| Suspected livedoid vasculopathy lesions | 0/12 (0) | 0/167 (0) | 0/179 (0) |
| Suspected acute/chronic aPL nephropathy | 2/12 (16.7) | 0/167 (0) | 2/179 (1.1) |
| Suspected pulmonary haemorrhage | 1/12 (8.3) | 0/167 (0) | 1/179 (0.6) |
| Established livedoid vasculopathy | 0/12 (0) | 1/167 (0.6) | 1/179 (0.6) |
| Established acute/chronic aPL nephropathy | 0/12 (0) | 2/167 (1.2) | 2/179 (1.1) |
| Established pulmonary haemorrhage | 0/12 (0) | 0/167 (0) | 0/179 (0) |
| Established myocardial disease | 0/12 (0) | 0/167 (0) | 0/179 (0) |
| Established adrenal haemorrhage | 0/12 (0) | 0/167 (0) | 0/179 (0) |
| Obstetric events | 1/8 (12.5) | 22/114 (19.3) | 23/122 (18.9) |
| Cardiac valve thickening | 0/12 (0) | 6/138 (4.3) | 6/150 (4.0) |
| Cardiac valve vegetation | 1/12 (8.3) | 7/138 (5.1) | 8/150 (5.3) |
| Thrombocytopenia | 4/13 (30.8) | 50/167 (29.9) | 54/180 (30.0) |
| Laboratory features | |||
| Lymphopenia | 0/13 (0) | 14/114 (12.3) | 14/127 (11.0) |
| Anaemia | 2/13 (15.4) | 21/114 (18.4) | 23/127 (18.1) |
| Thrombocytopenia during chorea | 3/13 (23.1) | 41/114 (36.0) | 44/127 (34.6) |
| High serum creatinine | 2/13 (15.4) | 6/46 (13.0) | 8/59 (13.6) |
| Proteinuria | 5/13 (38.5) | 13/51 (25.5) | 18/64 (28.1) |
| Haematuria | 3/13 (23.1) | 5/44 (11.4) | 8/57 (14.0) |
| High hsCRP | 1/10 (10) | 3/18 (16.7) | 4/28 (14.3) |
| CSF availability | 8/13 (61.5) | 30/167 (18.0) | 38/180 (21.1) |
| CSF abnormality | 0/8 (0) | 0/30 (0) | 0/38 (0) |
| Appearance, clear | 8/8 (100) | – | – |
| Pressure, mm H2O | 133.4±29.7 | – | – |
| CSF white cell count, × 106 /L | 0.8±0.97 | – | – |
| CSF-Glu, mmol/L | 3.3±0.5 | – | – |
| CSF-Cl, mmol/L | 125.1±1.9 | – | – |
| CSF-Pro, g/L | 0.4±0.2 | – | – |
| CSF-IgG, mg/L | 22.9±9.1 | – | – |
| CSF-OB, Neg | 4/8 (50) | – | – |
| CSF-SOB, Neg | 4/8 (50) | – | – |
| LAC | 13/13 (100) | 131/158 (82.9) | 144/171 (84.2) |
| aCL | 11/13 (84.6) | 135/158 (85.4) | 146/171 (85.4) |
| aCL IgM | 1/11 (9.1) | 53/135 (39.3) | 54/146 (37.0) |
| aCL IgG | 10/11 (90.9) | 111/135 (82.2) | 121/146 (82.9) |
| aβ2GPI | 11/13 (84.6) | 31/55 (56.4) | 42/68 (61.8) |
| aβ2GPI IgM | 1/11 (9.1) | 12/31 (38.7) | 13/42 (31.0) |
| aβ2GPI IgG | 10/11 (90.9) | 26/31 (83.9) | 36/42 (85.7) |
| Single aPL positivity | 2/13 (15.4) | 16/53 (30.2) | 18/66 (27.3) |
| Isolated LAC | 2/2 (100.0) | 12/16 (75) | 14/18 (77.8) |
| Isolated aCL IgG/M | 0/2 (0) | 4/16 (25) | 4/18 (22.2) |
| Isolated aβ2GPI IgG/M | 0/2 (0) | 0/16 (0) | 0/18 (0) |
| Double aPL positivity | 0/13 (0) | 9/53 (17.0) | 9/66 (13.6) |
| LAC+, aβ2GPI IgG/M−, aCL IgG/M+ | 0/0 (0) | 7/9 (77.8) | 7/9 (77.8) |
| LAC−, aβ2GPI IgG/M+, aCL IgG/M+ | 0/0 (0) | 2/9 (22.2) | 2/9 (22.2) |
| LAC+, aβ2GPI IgG/M+, aCL IgG/M− | 0/0 (0) | 0/9 (0) | 0/9 (0) |
| Triple aPL positivity | 11/13 (84.6) | 27/53 (50.9) | 38/66 (57.6) |
| Triple-positive IgG isotype | 11/11 (100.0) | 23/27 (85.2) | 34/38 (89.5) |
| Triple-positive IgM isotype | 1/11 (9.1) | 4/27 (14.8) | 5/38 (13.2) |
| ANA | 10/13 (76.9) | 81/130 (62.3) | 91/143 (63.6) |
| Anti-dsDNA | 4/13 (30.8) | 41/114 (36.0) | 45/127 (35.4) |
| Coombs’ test | 7/10 (70.0) | 10/42 (23.8) | 17/52 (32.7) |
Results are shown as n/N (%), mean±SD, or median (range), excluding missing/unknown unless otherwise specified.
aCL, anticardiolipin antibody; aPL, antiphospholipid antibody; APS, antiphospholipid syndrome; AT, arterial thrombosis; aβ2GPI, anti-β2 glycoprotein I antibody; BMI, body mass index; Cl, chloride; CSF, cerebrospinal fluid; CVD, cardiovascular disease; dsDNA, double-strand DNA; Glu, glucose; hsCRP, high-sensitivity C reactive protein; LAC, lupus anticoagulant; Neg, negative; n/N, n instances of positivity out of a total of N instances; OB, oligoclonal bands; PAPS, primary APS; Pro, protein; PUMCH, Peking Union Medical College Hospital; SAPS, secondary APS; SOB, specific oligoclonal bands (oligoclonal bands exit in CSF but not peripheral blood); VTE, venous thromboembolism.