Systemic Lupus Erythematosus with Pulmonary Hypertension.

Koyama, Shigeru; Ichiyoshi, Takeo; Chino, Masaaki; Kanbayashi, Takayuki

doi:10.2169/internalmedicine.35.39

Cited by 5 publications

(4 citation statements)

References 6 publications

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“…Abnormal vasospasm, Raynaud’s phenomenon, platelet abnormalities, vasculitis, thrombosis and thromboembolism have been suggested as possible pathogenetic factors [8, 24, 25]. Endothelial dysfunction is strongly implicated in the pathogenesis of autoimmune vasculitic diseases including lupus [26, 27].…”

Section: Discussionmentioning

confidence: 99%

Pulmonary hypertension in systemic lupus erythematosus: a systematic review and analysis of 642 cases in Chinese population

Yan

et al. 2012

Rheumatol Int

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Pulmonary hypertension (PH) is an increasingly recognized complication of systemic lupus erythematosus (SLE). To develop a more comprehensive understanding of the clinical and pathological characteristics of pulmonary hypertension associated with systemic lupus erythematosus (PH/SLE) in the Chinese population, a systematic review of the literature up to 2012 was conducted. Six hundred and forty-two Chinese PH/SLE cases from 22 studies were identified as well documented and further analyzed. Transthoracic echocardiography (TTE), X-ray, electrocardiogram and right heart catheterization (RHC) were performed to diagnose PH in SLE patients. The mean age of subjects was 35.5 years, the male to female ratio was 1:14, and the mean duration of SLE when PH was diagnosed was 10.7 years. The prevalence of PH in SLE was 2.8–23.3 %. Symptoms were usually nonspecific, and the observed clinical characteristics include Raynaud’s phenomenon (41.4 %), serous effusion (27.7 %), positive RNP (51.5 %) and positive ACL (46.6 %). Gold standard RHC is strongly recommended, especially for those who had resting pulmonary arterial systolic pressure >30 mmHg on TTE with the aforementioned clinical characteristics. Corticosteroids, immunosuppressants and vasodilators were the most common medications employed in treatment. Early identification and standard PH treatment with intensive SLE treatment can improve the prognosis.

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Section: Discussionmentioning

confidence: 99%

Pulmonary hypertension in systemic lupus erythematosus: a systematic review and analysis of 642 cases in Chinese population

Yan

et al. 2012

Rheumatol Int

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“…Our recent systematic review of the literature [90] identified a number of clinical factors that may be associated with survival; magnitude of the elevation in P pa at diagnosis [78,89,91], thrombosis [89,92,93], thrombocytopenia [94], presence of an anti-cardiolipin antibody [89,92,93], concurrent pregnancy [95,96], infection [77], Raynaud's phenomenon [93,97], plexiform lesion [95] and pulmonary vasculitis [96]. Conversely, lupus central nervous system disease [89], lupus nephritis [89] and lupus disease activity [89,[98][99][100] have not been associated with survival in SLE-PH. It has been suggested that SLE-PAH patients have a better prognosis than SSc-PAH patients, where CONDLIFFE et al [44] reported a 3-yr survival rate of 75% in SLE-PAH compared to 47% in SSc-PA patients in the UK (p50.01).…”

Section: Sle-associated Phmentioning

confidence: 99%

“…Several small case reports/series have reported improvement with immunosuppressive therapy [87,[101][102][103]. However, others have reported clinical deterioration in SLE-PH despite immunosuppressive therapy [99,104]. Recent observational studies suggest that a subset of SLE-PH patients will improve with immunosuppression.…”

Section: Sle-associated Phmentioning

confidence: 99%

Pulmonary hypertension in systemic sclerosis and systemic lupus erythematosus

Johnson

Granton²

2011

Eur Respir Rev

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Pulmonary arterial hypertension (PAH) is a severe manifestation of systemic sclerosis (SSc) and systemic lupus erythematosus (SLE). Due to improvements in the understanding of the pathogenesis of these diseases, improved methodological rigour in the conduct of epidemiological studies and the advent of successful therapies, our understanding of SSc-PAH and SLE-PAH has evolved considerably. In this review we will review the current evidence regarding the prevalence, prognostic factors and survival estimates for SSc-PAH and SLE-PAH. In doing so, we will compare and contrast these two diseases, highlight clinically useful features, discuss methodological limitations of existing data, and draw attention to areas where research is needed.

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“…Lupus activity was not associated with SLE-PH survival in five case reports. [40][41][42][43][44] These patients had minimal or quiescent disease and normal complement or anti-double-stranded DNA (anti-dsDNA). In one retrospective cohort of mortality in SLE, 27 43 deaths occurred, and in six cases PH was the cause of death.…”

Section: Sle Disease Activitymentioning

confidence: 99%