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.


METHODS
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.


RESULTS
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.


CONCLUSIONS
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.
Taçoy
G
G
Demirag
M
M
Göker
B
B
Cengel
A
A
D000280:Administration, Inhalation; D000328:Adult; D000959:Antihypertensive Agents / Q000009:adverse effects* / Q000627:therapeutic use; D000077300:Bosentan; D005260:Female; D006801:Humans; D006973:Hypertension / Q000139:chemically induced / Q000209:etiology*; D016285:Iloprost / Q000009:adverse effects* / Q000627:therapeutic use; D034721:Mastocytosis, Systemic / Q000503:physiopathology*; D011651:Pulmonary Artery / Q000187:drug effects / Q000473:pathology*; D013449:Sulfonamides / Q000009:adverse effects* / Q000627:therapeutic use; D014665:Vasodilator Agents / Q000009:adverse effects* / Q000627:therapeutic use
D000959:Antihypertensive Agents; D013449:Sulfonamides; D014665:Vasodilator Agents; D016285:Iloprost; D000077300:Bosentan