Characteristics of patients with PHAPS.
| Patients | 1 | 2 | 3 | 4 | 5 | 6 | 7 | 8 | 9 | 10 | 11 | 12 | 13 |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Age at first PH episode, yrs | 50 | 29 | 36 | 36 | 30 | 41 | 29 | 57 | 31 | 34 | 32 | 42 | 26 |
| Age at first thrombotic or obstetric event | 29 | 25 | 34 | 22 | 25 | 33 | 19 | 56 | 30 | 33 | 26 | 32 | 26 |
| No. PH episodes | 5 | 1 | 1 | 10 | 2 | 4 | 1 | 3 | 24 | 14 | 1 | 3 | 3 |
| Sex | F | F | F | F | F | F | F | M | M | M | M | F | M |
| SLE | + | + | + | + | + | + | + | − | − | − | − | − | − |
| Pregnancy morbidity | + | + | + | + | −* | + | + | NA | NA | NA | NA | + | NA |
| Mitral disease | + | ND | + | + | − | + | + | + | + | − | + | + | − |
| RA-RV gradient by echo (mm Hg) | 43 | ND | Normal | 37** | 31 | 61 | 50 | 51 | 14 | 38** | 63** | 51 | 60** |
| CNS morbidity | + | − | + | + | − | + | + | + | − | + | + | − | − |
| Skin involvement | + | − | − | + | − | + | + | + | + | − | + | − | − |
| C3 level | 56 | 50 | 50 | 50 | 60 | 98 | 49 | 173 | 67 | 40 | 82 | 111 | 147 |
| C4 level | 25 | 10 | 42 | 7 | 27 | 36 | 6 | 42 | 10 | 16 | 11 | 14 | 27 |
| aCL IgG | 120 | 652 | 200 | 100 | 238 | 559 | 160 | 69 | 184 | 120 | 200 | 273 | 220 |
| aCL IgM | ND | ND | 10 | ND | 5 | 4 | 19 | 12 | 26 | 100 | 52 | 2 | 20 |
| β2-GPI IgG | ND | 332 | 110 | 83 | 50 | 190 | 80 | 1.7 | 104 | 200 | 352 | 176 | 1428 |
| β2-GPI IgM | ND | 29 | 13 | ND | ND | 2 | 2 | 0.1 | 31 | 190 | 50 | 2 | 16 |
| LAC ratio | 5.5 | ND | 3.3 | 2 | 1.8 | 2.5 | 3.8 | ND | Negative | 9.5 | Positive | 3 | 1.5 |
↵* Patient 5 had infertility and had no pregnancies.
↵** Mean pulmonary artery pressure measured during right heart catheterization. PHAPS: pulmonary hemorrhage and antiphospholipid antibody syndrome; SLE: systemic lupus erythematosus; RA-RV: right atrium-right ventricle; CNS: central nervous system; aCL: anticardiolipin; IgG: immunoglobulin; IgM:immunoglobulin μ; GPI: glycoprotein I; LAC: lupus anticoagulant; ND: not determined; NA: not applicable.