Table 1

Immunosuppressive therapy in patients with pulmonary hypertension secondary to connective tissue diseases (selected literature)

Patient no.SexAgeCTDPAP s/d/mCI/COImmunosuppressive therapyVasodilator therapyOutcomeLast PAP s/d/mLast CI/CORemarksReferences
1F22SLE94/41/572,2/–500 mg pulse methylprednisolone for 3 days; prednisone 0.5 mg/kg; 20 cyclophosphamide courses (750 mg) over a period of 32 monthsPrazosin 5 mg/day over a period of 55 monthsImprovement54/18/323,8/Treatment with vasodilator alone was ineffective 25
2F27SLE78/–/–NDPlasma 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 weeksNoneImprovement42/–/–ND 26
3F31SLE67/–/–ND20 mg dexamethasone iv + 20 mg methotrexate iv once a week + cyclosporin A 5 mg/kg/day for 2 monthsNoneImprovement42/–/–ND 26
4F20MCTD –/–/58NDSequential administration of methylprednisolone + cyclophosphamide + cyclosporin ANoneImprovement–/–/41ND10 years of follow up 27
5F33SLE88/34/521,8/–Cyclophosphamide infusions 0.5 mg/m2 monthly for 6 months followed by once/3 months in combination with 7.5 mg/day prednisoloneNoneImprovement66/34/463/Negative acute vasodilator testing ; last PAPs 67 mm Hg after 2 years 28
6F29SLE115/40/65/3,84500 mg pulse methylprednisolone for 3 days; prednisolone 80 mg/dayDiltiazem 180 mg/dayImprovement65/32/50/4,6Last PAPs 35 mm Hg after 18 months 29
7F49P Sjo100/36/60NDMethylprednisolone 4 mg/day, azathioprine 100 mg/dayNifedipine and enalaprilImprovement55/18/32NDVasodilators were ineffective alone; two years of follow up 30
8F28SLE74/40/523,4/–Prednisone 60 mg/dayNoneAggravation78/40/562,5/–Negative acute vasodilator testing 8
9F29SLE70/38/472,4/–Prednisone 80 mg/dayNoneStabilisation63/37/432,5/–Negative acute vasodilator testing 8
10F23SLE70/34/50NDPrednisone 60 mg/dayNoneAggravationNDND 8
11F36SLE58/35/401,1/–Prednisone 60 mg/dayNoneDeathNDNDNegative acute vasodilator testing 8
  • 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).