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Backgrounds: SLE is a disease with diverse manifestations and occurred 6-10 times more often in women than
men. Pulmonary hypertension (PH) has been reported as clinical manifestations of SLE and its prevalence is
estimated between 0.5% and 43%. However, PH as the presenting manifestation of SLE is rarely reported. SLE
patients with PH has an unfavorable prognosis with the median survival from the onset of PH is two years.
Aim: To report a case of 37-year-old woman with PH as the presenting manifestation of SLE and review the
literature about such case.
Cases: A 37 years old female patient was admitted with the main complaint of worsening shortness of breath
since one week before. She experienced lassitude and felt more comfortable sleeping with 2 or 3 pillows. She
also experienced nausea and edema at her lower extremities. From the physical examination she was slightly
jaundice, non-scarring alopecia, mild bilateral pitting edema and holosystolic murmur were also found. Her
Antinuclear Antibody (ANA) test was positive with 1/320 titer and low complement level. Chest X-ray showed
cardiomegaly. Echocardiography and aortic CT scan showed pulmonary hypertension. She fulfilled 2019
ACR/EULAR criteria for SLE and was diagnosed as SLE-associated pulmonary hypertension (SLE-a PH). She was
treated with anticoagulant, glucocorticoid, antimalarial, immunosuppressive agent and standard treatment
of PH with improving symptoms.
Conclusion: Pulmonary hypertension is a rare initial manifestation of autoimmune disease including SLE