Profile of patients with SLE-related APS
. | . | . | . | ACLAs . | . | Thrombotic Events . | . | . | . | Coexisting thrombogenic risk factors . | . | . | . | . | |||||
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Patient no. . | Age, y . | Sex . | LA . | IgG . | IgM . | Arterial . | Venous . | Pregnancy morbidity . | Non-Sapporo manifestations . | HTN . | DM . | OCP . | Smoking . | Indications for HSCT . | |||||
D-APS | |||||||||||||||||||
1 | 15 | F | PI | PI | — | — | UE DVT | None | LSE, diplopia/nystagmus | Yes | No | No | No | N, C, P | |||||
2 | 52 | F | — | PI | — | SMA + RCA | — | 5 first-trimester spontaneous abortions | None | Yes | No | No | No | V | |||||
3 | 30 | F | PT | — | — | — | LE DVT×2 | None | None | Yes | No | No | No | N | |||||
4 | 29 | F | — | PI | PI | — | UE DVT×3 | None | LR, thrombocytopenia, diplopia | No | No | Yes | No | C, V | |||||
5 | 20 | F | PT | — | PT | Splenic infarct | PE | None | None | No | No | No | Yes | AIH, AIHA | |||||
6 | 45 | F | — | PT | — | CNS infarct | LE DVT×2 | None | Myelitis/paraparesis | Yes | No | Yes | No | N, C | |||||
7 | 21 | F | PI | — | — | — | PE×2 + UE DVT×3 | None | LR, thrombocytopenia, hallucinations | Yes | No | Yes | No | N, C, V | |||||
8 | 21 | F | — | — | PT | Mesenteric artery | — | None | Calciphylaxis | No | No | No | No | N, calciphylaxis | |||||
9 | 48 | F | PT | PT | PT | — | PE + LE DVT×2 | 2 second-trimester spontaneous abortions | TIA×7 | Yes | No | Yes | No | C, P, APS | |||||
10 | 31 | F | — | — | PT | — | UE DVT | None | Seizures, thrombocytopenia | Yes | No | No | No | N, C, AIHA | |||||
11 | 38 | F | PT | PI | PT | LLE aa.×3 | PE×2 + LE DVT×2 | None | Multiple CVA | No | No | No | No | C, nonhealing LLE ulcer | |||||
12 | 36 | F | PI | — | — | CNS infarct | — | None | None | No | No | Yes | Yes | C, P | |||||
13 | 41 | F | — | PT | PT | — | UE DVT | Spontaneous abortion | Diplopia, calciphylaxis | No | No | No | No | N, C, P, calciphylaxis | |||||
14 | 27 | F | PI | — | — | — | LE DVT + PE | None | None | Yes | No | No | No | N, C, P | |||||
15 | 29 | M | PT | PI | PI | — | PE + LE DVT×2 | None | Seizures | Yes | No | No | No | N, C | |||||
16 | 22 | F | PT | — | — | — | PE + LE DVT | None | None | Yes | No | No | No | N, C, P | |||||
17 | 39 | M | PI | PI | PI | — | LE DVT×2 + UE DVT | None | Seizures, thrombocytopenia | No | No | No | No | C, thrombocytopenia | |||||
18 | 18 | F | — | PI | — | — | PE | None | Seizures, thrombocytopenia | Yes | No | No | No | N, C, myocarditis | |||||
19 | 23 | F | PT | — | — | CNS infarct × 2 | PE + LE DVT×2* | None | Multiple silent CVA, LR | Yes | No | No | No | CAPS, N, C | |||||
20 | 30 | F | — | PI | — | CNS infarct | PE×2 | None | Multiple TIA, intractable H/A | No | Yes | No | No | CAPS, C | |||||
P-APS | |||||||||||||||||||
1 | 20 | F | — | — | PT | — | None | None | LR | Yes | No | No | No | P, V | |||||
2 | 27 | F | — | PT | PT | — | None | None | Seizures, myelitis | Yes | No | No | No | N, C, P, V | |||||
3 | 20 | F | — | PT | — | — | None | None | Cranial nerve palsies | No | No | No | No | N, C | |||||
4 | 29 | F | PT | PT | — | — | None | None | Myelitis | No | No | No | No | C | |||||
5 | 29 | F | PT | — | — | — | None | None | LSE, thrombocytopenia | No | No | Yes | No | N, V | |||||
6 | 37 | M | PI | — | PT | — | None | None | Transverse myelitis | No | No | No | No | N, myelitis | |||||
7 | 20 | F | PT | PT | PT | — | None | None | Intractable headache | No | No | No | Yes | N, C, V | |||||
8 | 16 | F | — | — | PT | — | None | None | Seizures | No | No | No | No | N, C |
. | . | . | . | ACLAs . | . | Thrombotic Events . | . | . | . | Coexisting thrombogenic risk factors . | . | . | . | . | |||||
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Patient no. . | Age, y . | Sex . | LA . | IgG . | IgM . | Arterial . | Venous . | Pregnancy morbidity . | Non-Sapporo manifestations . | HTN . | DM . | OCP . | Smoking . | Indications for HSCT . | |||||
D-APS | |||||||||||||||||||
1 | 15 | F | PI | PI | — | — | UE DVT | None | LSE, diplopia/nystagmus | Yes | No | No | No | N, C, P | |||||
2 | 52 | F | — | PI | — | SMA + RCA | — | 5 first-trimester spontaneous abortions | None | Yes | No | No | No | V | |||||
3 | 30 | F | PT | — | — | — | LE DVT×2 | None | None | Yes | No | No | No | N | |||||
4 | 29 | F | — | PI | PI | — | UE DVT×3 | None | LR, thrombocytopenia, diplopia | No | No | Yes | No | C, V | |||||
5 | 20 | F | PT | — | PT | Splenic infarct | PE | None | None | No | No | No | Yes | AIH, AIHA | |||||
6 | 45 | F | — | PT | — | CNS infarct | LE DVT×2 | None | Myelitis/paraparesis | Yes | No | Yes | No | N, C | |||||
7 | 21 | F | PI | — | — | — | PE×2 + UE DVT×3 | None | LR, thrombocytopenia, hallucinations | Yes | No | Yes | No | N, C, V | |||||
8 | 21 | F | — | — | PT | Mesenteric artery | — | None | Calciphylaxis | No | No | No | No | N, calciphylaxis | |||||
9 | 48 | F | PT | PT | PT | — | PE + LE DVT×2 | 2 second-trimester spontaneous abortions | TIA×7 | Yes | No | Yes | No | C, P, APS | |||||
10 | 31 | F | — | — | PT | — | UE DVT | None | Seizures, thrombocytopenia | Yes | No | No | No | N, C, AIHA | |||||
11 | 38 | F | PT | PI | PT | LLE aa.×3 | PE×2 + LE DVT×2 | None | Multiple CVA | No | No | No | No | C, nonhealing LLE ulcer | |||||
12 | 36 | F | PI | — | — | CNS infarct | — | None | None | No | No | Yes | Yes | C, P | |||||
13 | 41 | F | — | PT | PT | — | UE DVT | Spontaneous abortion | Diplopia, calciphylaxis | No | No | No | No | N, C, P, calciphylaxis | |||||
14 | 27 | F | PI | — | — | — | LE DVT + PE | None | None | Yes | No | No | No | N, C, P | |||||
15 | 29 | M | PT | PI | PI | — | PE + LE DVT×2 | None | Seizures | Yes | No | No | No | N, C | |||||
16 | 22 | F | PT | — | — | — | PE + LE DVT | None | None | Yes | No | No | No | N, C, P | |||||
17 | 39 | M | PI | PI | PI | — | LE DVT×2 + UE DVT | None | Seizures, thrombocytopenia | No | No | No | No | C, thrombocytopenia | |||||
18 | 18 | F | — | PI | — | — | PE | None | Seizures, thrombocytopenia | Yes | No | No | No | N, C, myocarditis | |||||
19 | 23 | F | PT | — | — | CNS infarct × 2 | PE + LE DVT×2* | None | Multiple silent CVA, LR | Yes | No | No | No | CAPS, N, C | |||||
20 | 30 | F | — | PI | — | CNS infarct | PE×2 | None | Multiple TIA, intractable H/A | No | Yes | No | No | CAPS, C | |||||
P-APS | |||||||||||||||||||
1 | 20 | F | — | — | PT | — | None | None | LR | Yes | No | No | No | P, V | |||||
2 | 27 | F | — | PT | PT | — | None | None | Seizures, myelitis | Yes | No | No | No | N, C, P, V | |||||
3 | 20 | F | — | PT | — | — | None | None | Cranial nerve palsies | No | No | No | No | N, C | |||||
4 | 29 | F | PT | PT | — | — | None | None | Myelitis | No | No | No | No | C | |||||
5 | 29 | F | PT | — | — | — | None | None | LSE, thrombocytopenia | No | No | Yes | No | N, V | |||||
6 | 37 | M | PI | — | PT | — | None | None | Transverse myelitis | No | No | No | No | N, myelitis | |||||
7 | 20 | F | PT | PT | PT | — | None | None | Intractable headache | No | No | No | Yes | N, C, V | |||||
8 | 16 | F | — | — | PT | — | None | None | Seizures | No | No | No | No | N, C |
HTN, hypertension; DM, diabetes mellitus; OCP, oral contraceptive pill; D-APS, definite APS; P-APS, probable APS; PI, positive in the past but not immediately before transplantation; — negative result; UE, upper extremity; DVT, deep venous thrombosis; N, nephritis; C, cerebritis; P, pneumonitis; SMA, superior mesenteric artery; RCA, right coronary artery; V, cutaneous vasculitis; PT, positive during pretransplant testing; LE, lower extremity; PE, pulmonary embolism; AIH, autoimmune hepatitis; AIHA, autoimmune hemolytic anemia; LLE aa, left lower extremity arteries; sp.-spontaneous; LSE-Libman-Sacks endocarditis; CAPS, catastrophic APS; H/A-headache. Other abbreviations are explained in the text.
Patient 19 also had 2 myocardial infarctions.