Successful switch from inhaler iloprost to treatment in a patient who initially presented with pulmonary arterial hypertension and diagnosed as SLE.

BACKGROUND

SLE-induced Pulmonary Arterial Hypertension increases morbidity and mortality. Vasoconstruction, thrombosis, inflammation are the mostly proposed factors in PAH. Time until PAH develops is variable in patients with SLE. It has been shown that vasoactive agents improve symptoms and clinical findings in patients with SLE-induced-PAH.

METHODOLOGY: The case note of the patient with with pulmonary arterial hypertension and SLE was retrieved and reviewed, and literature search was done. We reported a patient whose initially symptoms and clinical findings were consistent with idiopathic PAH and then had been diagnosed as SLE.

RESULT: The patient tolerated the switch treatment from inhaler Iloprost from oral Bosentan well. Her symptoms, clinical findings and 6 minutes walking distance improved with treatment.

CONCLUSION

Vasodilator treatment appears to be beneficial in patients with SLE-associated PAH and switch therapy also seems to be well tolerated. Additionally, in connective tissue diseases it must be kept in mind that PAH might be the presenting symptom.