Severe pulmonary hypertension in a patient with systemic lupus erythematosus and minimal lupus activity.
Pulmonary hypertension (PH) sometimes occurs in patients with systemic lupus erythematosus (SLE). We report a case of 51-year-old-woman with PH associated with SLE. She had been diagnosed as SLE on the basis of pericardial effusion, hematological disorder, positive antinuclear antibody, and hypocomplementemia. Despite minimal lupus activity, she had marked elevation of pulmonary arterial pressure (101/53 mmHg) and decreased cardiac index (1.5 l/min/m2). Symptoms related to PH were progressive under treatment with oral corticosteroids, oxygen, calcium antagonists, and warfarin. After 17 months of epoprostenol treatment, she died of pulmonary infarction. SLE-associated PH is often severe and progressive even in association with minimal activity.
Kawamura
Nobutoshi
N
Department of Cardiovascular Medicine, Kyushu University, Fukuoka.
Tsutsui
Hiroyuki
H
Fukuyama
Kae
K
Hayashidani
Shunji
S
Koike
George
G
Egashira
Kensuke
K
Abe
Yasunobu
Y
Yamamoto
Hidetaka
H
Tsuneyoshi
Masazumi
M
Takeshita
Akira
A
D000925:Anticoagulants / Q000627:therapeutic use; D001327:Autoimmune Diseases / Q000150:complications*; D002121:Calcium Channel Blockers / Q000627:therapeutic use; D018450:Disease Progression; D011464:Epoprostenol / Q000627:therapeutic use; D017809:Fatal Outcome; D005260:Female; D006439:Hemodynamics; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy / Q000209:etiology*; D017380:Hypertrophy, Right Ventricular / Q000150:complications; D008180:Lupus Erythematosus, Systemic / Q000150:complications*; D008875:Middle Aged; D010100:Oxygen / Q000627:therapeutic use; D011239:Prednisolone / Q000627:therapeutic use; D011655:Pulmonary Embolism / Q000209:etiology; D014859:Warfarin / Q000627:therapeutic use
D000925:Anticoagulants; D002121:Calcium Channel Blockers; D014859:Warfarin; D011239:Prednisolone; D011464:Epoprostenol; D010100:Oxygen