An Autopsied Case of Systemic Lupus Erythematosus with Pulmonary Hypertension—A Case Report

Kanemoto, Nariaki; Sato, Michiko; Moriuchi, Junko; Ichikawa, Yukinobu; Goto, Yuichiro; Sasadaira, Hidekazu

doi:10.1177/000331978803900210

Cited by 14 publications

(5 citation statements)

References 6 publications

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“…Low-grade lesions are characterized by "onionskin" thickening of arterioles and arteries, whereas high-grade lesions show vasoproliferative and dilatation lesions. In reported cases of PAH associated with SLE, only low-grade lesions vascular lesion have been described [1]. Our case presented with numerous high-grade pulmonary hypertensive lesions.…”

Section: Discussionmentioning

confidence: 62%

“…Clinical data suggests that a "pulmonary Raynaud's phenomenon" correlates with the development of PAH in SLE patients [1,5]. However, a recent study indicates that pulmonary hypertension develops in patients with SLE as result of abnormal inflammatory reaction which affects the blood vessels.…”

Section: Pulmonary Hypertension and Slementioning

confidence: 99%

“…ulmonary arterial hypertension (PAH), is a well recognized, albeit uncommon complication of systemic lupus erythematosous (SLE). In previously reported cases, the pulmonary lesions consisted only of onionskin thickening of the small arterioles (also termed concentric intimal fibroelastosis) [1]. Vasoproliferative/angiomatoid lesions and fibrinoid necrosis are the more severe histological manifestations of PAH and have not been observed in SLE.…”

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confidence: 95%

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Pulmonary Hypertension in Systemic Lupus Erythematosus complicated by exposure to Dexfenfluramine

Petcu¹,

Berman²,

Opris³

et al. 2009

Rom J Leg Med

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Pulmonary arterial hypertension (PAH) is a well recognized, albeit uncommon, complication of systemic lupus erythematosus (SLE) and is characterized histologically by the presence of onionskin thickening of small arterioles. We described a 24 year-old female with SLE, complicated by pulmonary hypertension after a six-week exposure to dexfenfluramine, who died of acute myocardial infarction complicated with pneumococcal sepsis. Autopsy demonstrated pulmonary arteriolar thickening with severe sclerosis as well as the more advanced plexiform and angiomatoid lesions. Based on these findings, we hypothesize that in this case the use of dexfenfluramine, albeit for a relatively short period, exerted a synergistic effect on the predisposition to PAH already inherent in this patient with SLE.

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Section: Discussionmentioning

confidence: 62%

Section: Pulmonary Hypertension and Slementioning

confidence: 99%

mentioning

confidence: 95%

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Pulmonary Hypertension in Systemic Lupus Erythematosus complicated by exposure to Dexfenfluramine

Petcu¹,

Berman²,

Opris³

et al. 2009

Rom J Leg Med

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“…38 Plexogenic arteriopathy as a cause of pulmonary hypertension in SLE was described by Roncoroni, et al in 1992, 39 and later reported in other isolated cases. [39][40][41][42] We cannot assure that it is a sign of PHT, as it may be due to recurrent pulmonary thromboembolism or the presence of capillaritis. However, the absence of any marker of those primary events is highly suggestive of lupus-related PHT.…”

Section: Discussionmentioning

confidence: 99%

Pulmonary involvement of systemic lupus erythematosus: analysis of 90 necropsies

Álvarez

Arce

et al. 2009

Lupus

108

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Pleuropulmonary manifestations of systemic lupus erythematosus (SLE) have been reported to be of variable prevalence, depending on the diagnostic methods used. The objective of this study was to determine the anatomopathological prevalence and the nature of lung involvement associated with SLE and to define if there were differences in the grade and type of pulmonary involvement in patients who had died at different time periods, before or after 1996. Complete autopsy studies of 90 patients with SLE diagnosis carried out between 1958 and 2006 and their clinical records were studied. All patients fulfilled the American College of Rheumathology (ACR) diagnostic criteria for SLE. Two groups of patients were analyzed: patients who had died before 1996 and those deceased in 1996-2006. Some pleuropulmonary involvement was detected in 97.8% of the autopsies. The most frequent findings were pleuritis (77.8%), bacterial infections (57.8%), primary and secondary alveolar haemorrhages (25.6%), followed by distal airway alterations (21.1%), opportunistic infections (14.4%) and pulmonary thromboembolism (7.8%), both acute and chronic. No cases of acute or chronic lupus pneumonitis were found. Opportunistic lung infections were invasive aspergillosis, disseminated strongyloidiasis, mucormicosis and Pneumocystis carinii. Only three of 23 patients with alveolar haemorrhage showed capillaritis. The four patients with primary pulmonary hypertension (PHT) had plexiform lesions. Deceased patients' age at death (46.09 +/- 11.01 vs 30.3 +/- 11.5 years, P < 0.0001) as well as survival time from diagnosis date (11.8 +/- 11.2 vs 4.4 +/- 4.9 years, P < 0.0001) in the second time period evaluated were significantly higher. However, there were no statistically significant differences in the prevalence of any of the pulmonary manifestations. Sepsis was considered the major cause of death without significant differences in both groups. Our results show that pulmonary manifestations directly caused by systemic lupus erythematosus are very uncommon and that their prevalence has not changed in the past 10 years. Pulmonary infection is still the most frequent affection, and it is an important cause of death in patients with lupus.

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“…In one report, most patients with SLEand PHwere classified as idiopathic (5). In several autopsied cases, plexogenic pulmonary arteriopathy was revealed, which is characteristic of primary pulmonary hypertension (PPH) (6), and the idiopathic PH may be very similar to PPH. Although the differential diagnosis between chronic pulmonary thromboembolism (CPTE) and idiopathic pulmonary hypertension is sometimes difficult, the present case was diagnosed as having idiopathic pulmonary hypertension rather than CPTE, due to the lack of evidence of arterial stenosis/webs and localized areas of decreased attenuation and vascularity sharply marginated from adjacent areas on CT (7), and the lack of evidence of coagulation and fibrinolysis on laboratory data at anytime.…”

Section: Discussionmentioning

confidence: 99%

Successful Treatment of Systemic Lupus Erythematosus and Pulmonary Hypertension with Intravenous Prostaglandin I2 Followed by Its Oral Analogue.

et al. 2000

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Systemic lupus erythematosus (SLE) is sometimes reported to complicate fatal pulmonary hypertension. A 46-year-old woman, with a ten-year history of SLE and pulmonaryhypertension, was admitted to our hospital complaining of dyspnea and chest pain. She suffered pulmonary hemorrhage and after steroid pulse therapy, she underwent continuous intravenous infusion of epoprostenol (prostaglandin I2) with corticosteroid for four weeks, which reduced the pulmonary artery pressure and resistance. Following the successful treatment, beraprost sodium, an oral PGI2 analogue, was given and it maintained pulmonary hypertension remittance for four years.

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An Autopsied Case of Systemic Lupus Erythematosus with Pulmonary Hypertension—A Case Report

Cited by 14 publications

References 6 publications

Pulmonary Hypertension in Systemic Lupus Erythematosus complicated by exposure to Dexfenfluramine

Pulmonary Hypertension in Systemic Lupus Erythematosus complicated by exposure to Dexfenfluramine

Pulmonary involvement of systemic lupus erythematosus: analysis of 90 necropsies

Successful Treatment of Systemic Lupus Erythematosus and Pulmonary Hypertension with Intravenous Prostaglandin I2 Followed by Its Oral Analogue.

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