Table 1
Immunosuppressive therapy in patients with pulmonary hypertension secondary to connective tissue diseases (selected literature)
| Patient no. | Sex | Age | CTD | PAP s/d/m | CI/CO | Immunosuppressive therapy | Vasodilator therapy | Outcome | Last PAP s/d/m | Last CI/CO | Remarks | References |
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 1 | F | 22 | SLE | 94/41/57 | 2,2/– | 500 mg pulse methylprednisolone for 3 days; prednisone 0.5 mg/kg; 20 cyclophosphamide courses (750 mg) over a period of 32 months | Prazosin 5 mg/day over a period of 55 months | Improvement | 54/18/32 | 3,8/ | Treatment with vasodilator alone was ineffective | 25 |
| 2 | F | 27 | SLE | 78/–/– | ND | Plasma exchange + 500 mg pulse prednisone for 3 days; 20 mg dexamethasone + 20 mg methotrexate iv every week for 4 weeks; cyclosporin A 5 mg/kg/day + fluocortolone 100 mg/week for 4 weeks | None | Improvement | 42/–/– | ND | 26 | |
| 3 | F | 31 | SLE | 67/–/– | ND | 20 mg dexamethasone iv + 20 mg methotrexate iv once a week + cyclosporin A 5 mg/kg/day for 2 months | None | Improvement | 42/–/– | ND | 26 | |
| 4 | F | 20 | MCTD | –/–/58 | ND | Sequential administration of methylprednisolone + cyclophosphamide + cyclosporin A | None | Improvement | –/–/41 | ND | 10 years of follow up | 27 |
| 5 | F | 33 | SLE | 88/34/52 | 1,8/– | Cyclophosphamide infusions 0.5 mg/m2 monthly for 6 months followed by once/3 months in combination with 7.5 mg/day prednisolone | None | Improvement | 66/34/46 | 3/ | Negative acute vasodilator testing ; last PAPs 67 mm Hg after 2 years | 28 |
| 6 | F | 29 | SLE | 115/40/65 | /3,84 | 500 mg pulse methylprednisolone for 3 days; prednisolone 80 mg/day | Diltiazem 180 mg/day | Improvement | 65/32/50 | /4,6 | Last PAPs 35 mm Hg after 18 months | 29 |
| 7 | F | 49 | P Sjo | 100/36/60 | ND | Methylprednisolone 4 mg/day, azathioprine 100 mg/day | Nifedipine and enalapril | Improvement | 55/18/32 | ND | Vasodilators were ineffective alone; two years of follow up | 30 |
| 8 | F | 28 | SLE | 74/40/52 | 3,4/– | Prednisone 60 mg/day | None | Aggravation | 78/40/56 | 2,5/– | Negative acute vasodilator testing | 8 |
| 9 | F | 29 | SLE | 70/38/47 | 2,4/– | Prednisone 80 mg/day | None | Stabilisation | 63/37/43 | 2,5/– | Negative acute vasodilator testing | 8 |
| 10 | F | 23 | SLE | 70/34/50 | ND | Prednisone 60 mg/day | None | Aggravation | ND | ND | 8 | |
| 11 | F | 36 | SLE | 58/35/40 | 1,1/– | Prednisone 60 mg/day | None | Death | ND | ND | Negative acute vasodilator testing | 8 |
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CTD = connective tissue disease; SLE = systemic lupus erythematosus; MCTD = mixed connective tissue disease; PSjö = primary Sjögren’s syndrome; PAP s/d/m = pulmonary arterial pressure systolic/diastolic/mean (mm Hg); CI = cardiac index (l/min/m2); CO = cardiac output (l/min).
