Clinical and immunological characteristics of patients with IPAH and those with SSc with or without PAH
| Patient group | No of patients(M/F) | Mean (SD)age(years) | Mean (SD) IgG and IgM concentrations (mg/ml) | Autoantibodies(ANA, ACA, anti-topo 1) | Mean (SD)disease duration(months) | Mean (SD)PAP(mm Hg) | Treatments |
|---|---|---|---|---|---|---|---|
| IPAH, idiopathic pulmonary arterial hypertension; SSc, systemic sclerosis; PAH, pulmonary arterial hypertension; PAP, pulmonary artery pressure; ANA, anti-nuclear antibodies; ACA, anti-centromere antibodies; anti-topo 1, anti-topoisomerase 1 antibodies. | |||||||
| *Disease duration of SSc. | |||||||
| †Disease duration of PAH. | |||||||
| ‡Mean PAP as measured by right heart catheterisation. | |||||||
| §Systolic PAP <35 mm Hg as measured by echocardiography. | |||||||
| IPAH | 35 (9/26) | 44.9 (15.5) | IgG: 9.99 (3.19) | ANA = 3/35 (9%) | 16 (21.5) | 61.4 (16.2)‡ | Epoprostenol: 13 |
| IgM: 1.12 (0.63) | ACA = 0 | Oral anticoagulant: 22 | |||||
| Anti-topo 1 = 0 | |||||||
| SSc with PAH | 10 (4/6) | 58.4 (11) | IgG: 12.8 (3.25) | ANA = 5/10 (50%) | 128 (99)* | 50.1 (13.3)‡ | Epoprostenol: 4 |
| IgM: 2.3 (2.46) | ACA = 3/10 (30%) | 19 (19.9)† | Oral anticoagulant: 4 | ||||
| Anti-topo 1 = 2/10 (20%) | |||||||
| Limited cutaneous SSc without PAH | 10 (1/9) | 44.8 (15.6) | IgG: 11.5 (3.48) | ANA = 10/10 (100%) | 58 (58) | § | No epoprostenol or anticoagulant |
| IgM: 1.79 (0.69) | ACA = 10/10 (100%) | ||||||
| Diffuse SSc without PAH | 10 (4/6) | 50.2 (11) | IgG: 13.6 (3.17) | ANA = 10/10 (100%) | 98 (116) | § | No epoprostenol or anticoagulant |
| IgM: 1.85 (1.14) | ACA = 0 | ||||||
| Anti-topo 1 = 7/10 (70%) | |||||||