Pulmonary arterial hypertension in systemic lupus erythematosus may benefit by addition of immunosuppression to vasodilator therapy: an observational study.
OBJECTIVE
To document changes in pulmonary arterial systolic pressure (PASP) in patients with SLE who have received CYC for any indication.


METHODS
Twenty-four patients with SLE pulmonary arterial hypertension (PAH) with a PASP of >30 mmHg by transthoracic echocardiography received i.v. CYC (n = 24) or steroids (n = 24) with or without vasodilators (n = 20). Baseline clinical characteristics and PASP were evaluated before and after therapy at 6 months. Responders were defined as those who had a decrease in PASP of >15 mmHg from baseline along with improvement in their New York Heart Association functional class.


RESULTS
There were 11 responders (45.83%), with a decrease in mean PASP from 59.33 mmHg at baseline to 43.29 mmHg at the end of 6 months (P < 0.0001). The decrease in mean PASP from 39.75 mmHg at baseline to 34.4 mmHg at the end of 6 months was significant in four patients who received immunosuppression alone (P = 0.04). There was no difference in baseline PASP and disease activity between responders and non-responders. Two deaths were noted.


CONCLUSIONS
Immunosuppression and vasodilators produced significant improvement in SLE PAH over 6 months.
Departments of Rheumatology and.
Sirisha
S
Kommireddy
Cardiology, Nizam's Institute of Medical Sciences, Hyderabad, India.
Srinivas
S
Bhyravavajhala
Departments of Rheumatology and.
Kishorebabu
K
Kurimeti
Departments of Rheumatology and.
Srinivasa
S
Chennareddy
Departments of Rheumatology and.
Suresh
S
Kanchinadham
Departments of Rheumatology and.
Irlapati
I
Rajendra Vara Prasad
Departments of Rheumatology and lizarajasekhar@gmail.com.
Liza
L
Rajasekhar
D000293:Adolescent; D000328:Adult; D001794:Blood Pressure / Q000187:drug effects / Q000502:physiology; D015331:Cohort Studies; D003520:Cyclophosphamide / Q000494:pharmacology / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D004452:Echocardiography; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000209:etiology* / Q000503:physiopathology; D007166:Immunosuppressive Agents / Q000494:pharmacology / Q000627:therapeutic use*; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000188:drug therapy* / Q000503:physiopathology; D013256:Steroids / Q000494:pharmacology / Q000627:therapeutic use; D013599:Systole / Q000187:drug effects / Q000502:physiology; D016896:Treatment Outcome; D014665:Vasodilator Agents / Q000494:pharmacology / Q000627:therapeutic use*; D055815:Young Adult
D007166:Immunosuppressive Agents; D013256:Steroids; D014665:Vasodilator Agents; D003520:Cyclophosphamide
SLE; immunosuppression; pulmonary hypertension; responders; transthoracic echocardiography; vasodilators