Restricted accessResearch articleFirst published online 1998-09
Successful treatment with immunosuppression,anticoagulation and vasodilator therapy of pulmonary hypertension in SLE associated with secondary antiphospholipid syndrome
Severe pulmonary hypertension (PHT) occurring in patients with systemic lupus erythematosus (SLE) is uncommon. Different modalities have been tried in the treatment for this condition but have not been effective because of progressive increase of pulmonary resistance over time. Our patient with SLE and PHT with antiphospholipid syndrome (APS), a condition which has previously never been described, responded rapidly to combination treatment with immuno-suppression, anticoagulation and vasodilator therapy.
Asherson RA et al. Pulmonary hypertension in systemic lupus erythematosus . BMJ1983; 287: 1024–1025 .
2.
Asherson RA et al. Pulmonary hypertension in a lupus clinic: Experience with twenty-four patients . J Rheumatol1990: 17: 1292–1298 .
3.
Quismorio FP Jr . Pulmonary manifestations of systemic lupus erythematosus. In: Wallace DJ , Hahn BH (eds). Dubois' Lupus Erythematosus, (5th ed.) William & Wilkins: Baltimore, MD , 1997; 673–692.
4.
Winslow TM et al. Five-year follow-up study of the prevalence and progression of pulmonary hypertension in systemic lupus erythematosus . Am Heart J1995; 129: 510–515 .
5.
Rich S , Kaufmann E , Levy PS . The effect of high doses of calcium-channel blockers on survival in primary pulmonary hypertension . N Engl J Med1992; 327: 76–81 .
6.
Shinohara S et al. Combined effects of diltiazem and oxygen in pulmonary hypertension of mixed connective tissue diseaseJ Rheumatol1994; 21: 1763–1765 .
7.
Fuster V et al. Primary pulmonary hypertension: nature history and the importance of thrombosis . Circulation1984; 70: 580–587
8.
Sandoval J et al. Primary antiphospholipid syndrome presenting as chronic thromboembolic pulmonary hypertension. Treatment with thromboendarterectomy . J Rheumatol1996; 23: 772–775 .
9.
Menon N , McAlpine L , Peacock AJ , Madhok R . The acute effects of prostacyclin on pulmonary hemodynamics in patients with pulmonary hypertension secondary to systemic sclerosis . Arthritis Rheum1998; 41: 466–469 .
10.
Goupille P et al. Precapillary pulmonary hypertension dramatically improved with high doses of corticosteroids during systemic lupus erythematosus . J Rheumatol1994; 21: 10-10 .
11.
Groen H , Bootsma H , Postma DS , Kallenberg CGM . Primary pulmonary hypertension in a patient with SLE: partial improvement with cyclophosphamide . J Rheumatol1993; 20: 1055–1057 .
12.
Karmochkine M et al. Improvement of severe pulmonary hypertension in a patient with systemic lupus erythematosus . Ann Rheum Dis1996; 55: 561–562 .
13.
Ignaszewki AP , Percy JS , Humen DP , MacKenzie WC . Successful treatment of pulmonary hypertension associate with systemic lupus erythematosus with prostaglandin I2 and Prostaglandin E1 . J Rheumatol1993; 20: 595–596 .
14.
Morelli S et al. Pulmonary arterial hypertension responsive to immunosuppressive therapy in systemic lupus erythematosus . Lupus1993; 2: 367–369 .
