Severe Pulmonary Hypertension in a Patient with Systemic Lupus Erythematosus and Minimal Lupus Activity.

Abstract

Pulmonary hypertension (PH) sometimes occurs in patients with systemic lupus erythematosus (SLE). Wereport 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 anti-body, and hypocomplementemia.Despite minimal lupus activity, she had marked elevation of pulmonary arterial pressure (101/53 mmHg)and decreased cardiac index (1.5 //min/m2). Symptomsrelated to PHwere progressive under treatment with oral corticosteroids, oxygen, calcium antagonists, and warfarin. After 17 months of epoprostenol treatment, she died of pulmonary infarction. SLE-associated PHis often severe and progressive even in association with minimal activity. (Internal Medicine 41: 109-112, 2002)

Immunosuppressive therapy in lupus‐ and mixed connective tissue disease–associated pulmonary arterial hypertension: A retrospective analysis of twenty‐three cases

“…However, our data also show that severe PAH can occur in SLE patients with minimal disease activity and that immunosuppressive therapy can sometimes be efficient in those patients. This last result seems important to highlight because it confirms some limited previous data (14, 30) and reinforces the fact that no or minimal SLE activity should not preclude the use of immunosuppressive therapy for selected patients with SLE‐associated PAH. However, since immunosuppressive therapy, particularly in patients without active SLE, is not without risk and because our study did not include controls, our results should not be interpreted as dictating how patients should be treated.…”

Pulmonary hypertension in systemic sclerosis and systemic lupus erythematosus

“…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).…”

Severe but reversible pulmonary hypertension in scleromyxedema and multiple myeloma: a case report

“…In parallel to those abnormalities, the pulmonary vascular cells of patients with PAH exhibit many features of cancerous cells from dysregulated metabolism to increased cell proliferation and resistance to apoptosis [106,109]. These observations combined with the occurrence of PAH in various connective tissue diseases support the role of inflammation, autoimmunity, and a neoplastic-like dysregulation at the center of the pathogenesis of PAH [105][106][107][110][111][112][113]. This model could provide a mechanistic explanation for the hemodynamic improvements noted in our patient and in many patients with PAH in response to immunosuppressive and anti-proliferative agents [24,25,27,.…”