Baseline clinical characteristics of 10 pregnant patients with SLE-PAH and assessment of SLE and cardiac function
| Case number (year of pregnancy) | Baseline clinical characteristics | Baseline assessment of SLE | Baseline assessment of cardiac function | ||||||||||||||||||||
| Age | SLE duration (months) | PAH duration (months) | Planned pregnancy under awareness of risk* | Pregnancy history | SLE-associated manifestations | Autoantibody | SLEDAI | WHO functional class | Right heart catheterisation | Echocardiography | NT-proBNP (pg/mL) | ||||||||||||
| ANA | Anti-SSA | Anti-SSB | Anti-dsDNA | aPLs | mPAP (mm Hg) | PAWP (mm Hg) | CO (L/min) | PVR (WU) | PASP (mm Hg) | RVTD (mm) | EF (%) | TAPSE (mm) | Pericardial effusion | ||||||||||
| 1 (2012) | 22 | 17 | 3 | 16 weeks | G2P0A1L0 (spontaneous abortion) |
| + | + | − | − | − | 14 | IV | 33 | 6 | 5.3 | 5.09 | 98 | NR | NR | NR | NR | 9098 |
| 2 (2015) | 35 | 1 | 1 | 12 weeks | G2P1A0L1 |
| + | + | − | − | − | 2 | III | 25 | 5 | 5.8 | 3.45 | 55 | 45 | 69 | NR | Moderate | 125 |
| 3 (2019) | 35 | 52 | 51 | Yes | G3P1A1L1 (spontaneous abortion) |
| + | + | − | − | NR | 4 | II | 41 | 7 | 6.5 | 5.23 | 99 | 40 | 75 | 17 | No | 56 |
| 4 (2016) | 29 | 92 | 4 | 31 weeks | G5P1A3L1 (all spontaneous abortions) |
| + | − | − | − | − | 2 | IV | 66 | 11 | 4.4 | 12.50 | 93 | 45 | 55 | 11 | Trace | 4497 |
| 5 (2018) | 29 | 66 | 9 | No | G1P0A0L0 |
| + | − | − | + | − | 2 | II | 28 | 8 | 6.5 | 3.08 | 39 | NR | 62 | 23 | No | 293 |
| 6 (2018) | 19 | 8 | 1 | 20 weeks | G4P0A3L0 (all elective abortions) |
| + | − | − | − | − | 2 | IV | 47 | 16 | 1 | 31.00 | 102 | 42 | 71 | 10 | Trace | 18 023 |
| 7 (2019) | 34 | 1 | 1 | 22 weeks | G2P0A1L0 (induced labour) |
| + | + | + | − | − | 0 | II | 31 | NA | 5 | NA | 71 | 34 | 69 | 18 | Trace | 142 |
| 8 (2019) | 36 | 2 | 1 | 29 weeks | G4P1A2L1 (all elective abortions) |
| + | + | − | − | − | 3 | IV | NA | NA | NA | NA | 41 | 35 | 55 | NR | No | 1963 |
| 9 (2016) | 29 | 104 | 41 | Yes | G2P0A1L0 (spontaneous abortions) |
| + | + | + | + | − | 2 | III | 44 | 8 | 5.9 | 6.10 | 78 | 44 | 70 | 15 | No | 89 |
| 10 (2018) | 32 | 164 | 1 | 36 weeks | G2P1A0L1 |
| + | + | − | − | − | 0 | III | 35 | 2 | 5.2 | 6.35 | 72 | 41 | 74 | 16 | No | 1259 |
Pregnancy history: G, gravida; P, para; A, abortus; L, living.
SLE-associated manifestations (domain): constitutional (fever), haematological (leucopenia, thrombocytopaenia, autoimmune haemolysis), neuropsychiatric (delirium, psychosis, seizure), mucocutaneous (alopecia, oral ulcers, subacute cutaneous lupus erythematosus/discoid lupus erythematosus, acute cutaneous lupus erythematosus), serosal (effusion, acute pericarditis), musculoskeletal (joint involvement) and renal (proteinuria, class II/V lupus nephritis, class III/IV nephritis).
Autoantibody: +, positive; −, negative.
aPLs include anticardiolipin, anti-beta(2)-glycoprotein I and lupus anticoagulant.
Some data for case 7 and case 8 were not available due to data missing.
*In patients developing PAH during pregnancy, gestational week of PAH diagnosis is reported.
anti-dsDNA, anti-double strand DNA; anti-SSA, anti-Sjogren's syndrome antigen A; anti-SSB, anti-Sjogren's syndrome antigen B; aPLs, antiphospholipid antibodies; CO, cardiac output; EF, ejection fraction; mPAP, mean pulmonary artery pressure; NA, not available; NR, not reported; NT-proBNP, N-terminal pro-brain natriuretic peptide; PAH, pulmonary arterial hypertension; PASP, pulmonary arterial systolic pressure; PAWP, pulmonary artery wedge pressure; PVR, pulmonary vascular resistance; RVTD, right ventricular transverse diameter; SLEDAI, Systemic Lupus Erythematosus Disease Activity Index; TAPSE, tricuspid annular plane systolic excursion; WU, Woods unit.