Features of 18 patients with severe pulmonary artery hypertension (PAH) related to connective tissue diseases.
| Patient | Sex | Age, yrs | Smoker | WHO PAH Functional Class | Connective Tissue Diseases | Disease Duration, yrs | ANA Specificity |
|---|---|---|---|---|---|---|---|
| 1 | M | 62 | Ex smoker | III | SSc | 13 | ACA |
| 2 | M | 41 | No | III | SLE | 7 | Anti-nDNA |
| 3 | F | 73 | No | III | SSc | 36 | ACA |
| 4 | F | 43 | No | III | MCTD | 18 | Anti-nRNP |
| 5 | M | 53 | Ex smoker | III | SSc | 11 | ACA |
| 6 | F | 60 | No | III | SSc | 13 | ACA |
| 7 | F | 61 | 7–8 cigarettes | III | UCTD | 9 | NA |
| 8 | F | 52 | No | III | SSc | 10 | Anti-topo I |
| 9 | F | 55 | No | III | SSc | 26 | Anti-topo I |
| 10 | F | 32 | No | III | SLE | 13 | Anti-nDNA |
| 11 | M | 64 | No | III | SSc | 12 | ACA |
| 12 | F | 33 | No | III | SM | 11 | Aspecific ANA |
| 13 | F | 52 | No | III | SSc | 2 | ACA |
| 14 | F | 41 | No | III | SSc | 23 | Anti-topo I |
| 15 | F | 67 | No | III | SSc | 4 | ACA |
| 16 | F | 59 | No | III | SSc | 4 | Anti-topo I |
| 17 | F | 51 | No | III | SSc | 14 | ACA |
| 18 | M | 31 | 40 cigarettes/day | III | SSc | 4 | ACA |
SSc: systemic sclerosis; SLE: systemic lupus erythematosus; MCTD: mixed connective tissue disease; UCTD: undifferentiated connective tissue disease; SM: overlap syndrome; ACA: anticentromere; Anti-topo I: anti-topoisomerase I.