scholarly journals Progressive fatigue and central cyanosis due to severe pulmonary arterial hypertension as the initial manifestation of systemic lupus erythematosus in a boy: A case report

2016 ◽  
Vol 3 (4) ◽  
pp. 28
Author(s):  
Zhang Feng ◽  
Prasad Shrestha Arnav ◽  
Jinquan Lao ◽  
Bingbing Ye ◽  
Xiaofei Peng ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Case Report ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Lupus Erythematosus ◽  
Idiopathic Pulmonary Arterial Hypertension ◽  
Systemic Lupus ◽  
Initial Manifestation ◽  
Pulmonary Arterial ◽  
Severe Pulmonary Arterial Hypertension

Severe pulmonary arterial hypertension of systemic lupus erythematosus (SLE) in pediatrics is rarely seen, especially in boys. Sometimes SLE may only present as progressive fatigue and central cyanosis that may be diagnosed initially as cardiovascular diseases, which makes harder the diagnosis of SLE. We present a 13-year-old boy with a month of progressive fatigue and central cyanosis, diagnosed only as severe idiopathic pulmonary arterial hypertension. The boy was finally diagnosed as severe pulmonary arterial hypertension of systemic lupus erythematosus. Due to different choices of therapy, underlying cause of pulmonary arterial hypertension should be diagnosed and searched thoroughly.

scholarly journals Systemic Lupus Erythematosus-associated Pulmonary Arterial Hypertension: Serial Case Report

2021 ◽  
Vol 7 (2) ◽  
pp. 5
Author(s):  
Yunia Duana ◽  
Lucia Kris Dinarti ◽  
Dyah Wulan Anggrahini ◽  
Anggoro Budi Hartopo ◽  
Bambang Irawan
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Pulmonary Hypertension ◽  
Case Report ◽  
Pulmonary Arterial Hypertension ◽  
Connective Tissue ◽  
Arterial Hypertension ◽  
Lupus Erythematosus ◽  
Systemic Lupus ◽  
Initial Manifestation ◽  
Pulmonary Arterial

One of the causes of pulmonary arterial hypertension (PAH) is connective tissue disease, including systemic lupus erythematosus (SLE). The prevalence of PAH in patients with SLE according to cohort studies varies widely between 0.5% and 43%. In some cases, PAH is the initial manifestation leading to the diagnosis of SLE. However, PAH can develop as a complication in patients who are initially diagnosed with SLE. We report two cases with a different approach to the diagnosis of pulmonary hypertension in SLE patients. These cases emphasize the importance of investigating examination results according to the guidelines to establish the diagnosis of PAH in SLE.

Severe pulmonary arterial hypertension and interstitial pneumonia related to systemic lupus erythematosus successfully treated with mycophenolate mofetil: A novel case report

Lupus ◽  
2020 ◽  
Vol 29 (14) ◽  
pp. 1955-1960
Author(s):  
Masao Nawata ◽  
Atsushi Nagayasu ◽  
Yuya Fujita ◽  
Aya Nawata ◽  
Kazuyoshi Saito ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Mycophenolate Mofetil ◽  
Case Report ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Interstitial Pneumonia ◽  
Lupus Erythematosus ◽  
High Dose ◽  
Systemic Lupus ◽  
Pulmonary Arterial

Background Pulmonary arterial hypertension (PAH) and interstitial pneumonia (IP) are relatively rare complications of systemic lupus erythematosus (SLE) and are associated with a poor prognosis. Overcoming these complications is a challenge for improving the prognosis. Case report A 41-year-old woman was diagnosed with SLE complicated by IP at the age of 21 years and with antiphospholipid syndrome at the age of 32 years at another hospital. She had been administered prednisolone (PSL) at a dose ≥15 mg daily, as well as various immunosuppressants and antiplatelet/anticoagulation therapy. On day I of hospitalization, She presented to our emergency outpatient department with fever, marked dyspnea, and skin ulcer on the left lower leg and was admitted the same day. Chest radiography revealed marked cardiomegaly and interstitial shadow, and right heart catheterization showed elevation in the mean pulmonary arterial pressure to 47 mmHg, indicating PAH. While oxygen therapy was started, high-dose steroid therapy and mycophenolate mofetil (MMF) were administered for treatment of SLE complicated by PAH/IP, and prostacyclin (prostaglandin I2), endothelin receptor antagonist, and PDE5 inhibitor were administered for PAH. Both SLE disease activity and PAH/IP improved and were maintained with no exacerbation for 2 years. The PSL dose could eventually be reduced to 5 mg/day. Conclusion In SLE complicated by PAH/IP, reports on the efficacy of MMF are scarce, and our findings suggested that MMF may be a treatment option in such cases.

scholarly journals SAT0240 THE PROGNOSIS OF TWO DISTINCT CLINICAL PHENOTYPES OF SLE-PAH

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1063.2-1063
Author(s):  
J. Wang ◽  
Y. Feng ◽  
Y. Lei ◽  
X. Zhang
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Lupus Erythematosus ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Systemic Lupus ◽  
Clinical Phenotypes ◽  
Weighted Score ◽  
Pulmonary Arterial

Background:Based on the characteristics of systemic lupus erythematosus-associated pulmonary arterial hypertension (SLE-PAH), Sunet alhas put forward a scoring system to distinguish two clinical phenotypes as vasculitic and vasculopathic subtypes[1]. A weighted score ≥2 suggested a vasculitic subtype by combining two factors: The time interval between SLE and PAH diagnosis <2 years and ≥2 years were 1 and 0 point; SLE Disease Activity Index (SLEDAI) >9, 5-9 and <5 were 2, 1, 0 point, respectively. While the vasculitic subtype seemed to have poorer prognosis in Sun’s research, other study has shown controversial result[2].Objectives:To find out the prognosis of two distinct clinical phenotypes of SLE-PAH.Methods:Between 2008 and 2019, a SLE-PAH cohort confirmed by right heart catheterization (RHC) from Guangdong Provincial People’s Hospital was included. Other groups of pulmonary hypertension were excluded. Based on the scoring system, patients were divided into vasculitic (weighted score≥2) and vasculopathic subtypes (weighted score<2). The endpoint was PAH-related mortality. Survival status were confirmed by clinic follow-up data or phone call.Results:A total of 53 SLE-PAH patients were enrolled. The cases of vasculitic and vasculopathic subtype were 14 and 39, respectively. Ten endpoint events occurred. Eight attributed to PAH and the cause could not be traced in two which were still included in study. The pooled 1-, 3-, 5-year survival rates were 85.7%, 78.6%, 65.5% in vasculitic subtype, and 93.9%, 87.5%, 87.5% in vasculopathic subtype, respectively. Kaplan-Meier analysis showed vasculitic subtype tended to have a poorer prognosis than vasculopathic subtype (p=0.16, HR 2.4, 95%CI 0.5-13.8, figure 1).Figure 1.Survival curves for patients with systemic lupus erythematosus-pulmonary arterial hypertension (SLE-PAH) in two distinct subtypes. RHC, Right Heart Catheterization.Conclusion:The prognosis of the two phenotypes of SLE-PAH was statistically indifferent while the vasculitic subtype showed a trend of worse prognosis. Further studies are needed.References:[1]F. Sun, Y. Lei, W. Wu, L. Guo, K. Wang, Z. Chen, W. Xu, X. Wang, T. Li, X. Zhang, S. Ye, Two distinct clinical phenotypes of pulmonary arterial hypertension secondary to systemic lupus erythematosus, Ann Rheum Dis 78(1) (2019) 148-150.[2]J. Qian, M. Li, J. Zhao, Q. Wang, Z. Tian, X. Zeng, Inflammation in SLE-PAH: good news or not?, Ann Rheum Dis (2018).0:1–2. doi:10.1136/annrheumdis-2018-214605Disclosure of Interests:None declared

Pulmonary Arterial Hypertension Responsive to Immunosuppressive Therapy in Systemic Lupus Erythematosus

Lupus ◽  
1993 ◽  
Vol 2 (6) ◽  
pp. 367-369 ◽  
Author(s):  
Sergio Morelli ◽  
Marcello Giordano ◽  
Paolo De Marzio ◽  
Roberta Priori ◽  
Alessandro Sgreccia ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Immunosuppressive Therapy ◽  
Lupus Erythematosus ◽  
Systemic Lupus ◽  
Pulmonary Arterial
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