Pulmonary hypertension in systemic lupus erythematosus: Report of four cases and review of the literature

doi:10.1016/0049-0172(81)90098-6

Seminars in Arthritis and Rheumatism

Volume 11, Issue 1, August 1981, Pages 177-181

https://doi.org/10.1016/0049-0172(81)90098-6 Get rights and content

Abstract

Pulmonary hypertension has been reported rarely in patients with systemic lupus erythematosus (SLE). During the past 3 $12$ yr we have observed pulmonary hypertension as a major clinical manifestation of their disease in four of 43 patients with well-documented SLE followed at our institution.

Pulmonary hypertension could be atrributed to underlying lung disease in three and was considered to be primary in the remaining patient. Neither hydralazine nor prednisone administration had any effect on the course of the pulmonary hypertension in these patients.

The presence of pulmonary hypertension in the course of active SLE may be more common than previously recognized.

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Cited by (83)

Pulmonary hypertension in systemic lupus erythematosus: Prevalence, predictors and diagnostic strategy
2013, Autoimmunity Reviews
To investigate the prevalence and predictors of pulmonary hypertension (PH) in patients with systemic lupus erythematosus (SLE) and to validate a diagnostic strategy.
245 patients with SLE entered a screening program. Possible PH was defined as two consecutive systolic pulmonary arterial pressure (PAP) values ≥ 40 mm Hg by echocardiography. The subsequent diagnostic procedure, including right heart catheterization if needed, confirmed or excluded the diagnosis of PH secondary to cardiopulmonary disease or SLE-related pulmonary arterial hypertension (PAH). Independent predictors of PH were identified by multivariant multiple linear or logistic regression models. The sensitivity (S), specificity (SP), positive (PPV) and negative predictive values (NPV) were calculated for different screening cutoff values.
88% patients were women. The mean (SD) age at the time of enrolment was 45 (16) years. 12 cases of PH were detected, all secondary, with a resulting prevalence of 5%. Two consecutive echocardiographic PAP measurements ≥ 40 mm Hg performed best as the cutoff point for screening (S 100%, SP 97%, PPV 70, NPV 100), as compared with single PAP measurements ≥ 30 mm Hg or ≥ 40 mm Hg The age at the time of enrolment was the only variable independently associated with PAP values (p = 0.0001), with the SLICC damage index score showing a borderline association (p = 0.08). Only the age at the time of enrolment showed an independent association with PH (OR 1.10, 95% CI 1.06–1.17).
We found a low prevalence of PH. Screening echocardiograms in asymptomatic lupus patients are thus not recommended. Two consecutive PAP values ≥ 40 mm Hg by echocardiogram is the best screening cutoff for starting investigations in SLE patients with suspected PH.
Pulmonary arterial hypertension in systemic lupus erythematosus
2011, Revue de Medecine Interne
L’hypertension pulmonaire (HTP) est une complication rare du lupus érythémateux systémique (LES). Sa prévalence varie de 0,5 à 17,5 % selon les séries et l’utilisation de l’échocardiographie ou du cathétérisme cardiaque droit comme méthode de référence. Au sein de la nouvelle classification clinique de l’hypertension pulmonaire (2008), cinq catégories étiologiques peuvent être associées au LES : l’hypertension artérielle pulmonaire (HTAP) associée aux connectivites, qui résulte d’une atteinte primitive des vaisseaux artériolaires pulmonaires ; l’HTP associée à une cardiopathie gauche (HTP post-capillaire), elle-même en rapport avec une dysfonction cardiaque systolique ou diastolique (atteinte myocardique spécifique ou coronaropathie par athérosclérose accélérée) ; l’HTP secondaire à une pneumopathie infiltrative diffuse fibrosante ou hypoxémiante ; l’HTP secondaire à une maladie thromboembolique, notamment dans le cadre du SAPL ; et enfin, très exceptionnellement, l’HTP en rapport avec une maladie veino-occlusive associée au LES. La pathogénie de l’HTAP survenant au cours du LES est complexe, et le rôle du terrain génétique, des anticorps antiphospholipides, et de la dysfonction endothéliale a été suggéré. L’évolution spontanée de l’HTP associée au LES semble être variable, mais l’hétérogénéité entre les séries rend la comparaison de celles-ci difficile. Le traitement de l’HTP associée au LES dépend des mécanismes impliqués dans sa survenue, et l’absence d’essai clinique dédié complique la prise en charge thérapeutique de ces patients. À côté des mesures non spécifiques, la prescription d’un traitement vasodilatateur pulmonaire et d’un traitement immunosuppresseur s’avère souvent nécessaire lorsque les patients sont en classe fonctionnelle II, III ou IV de la NYHA.
Pulmonary hypertension (PH) is a serious complication of connective tissue diseases. The prevalence of PH in systemic lupus erythematosus (SLE) ranges from 0.5 to 17.5%, depending on whether echocardiography or right heart catheterization is used as the gold standard for diagnosis. The recent guidelines for the diagnosis and treatment of pulmonary hypertension include several potential causes of PH in SLE, including: a primary vasculopathy similar to idiopathic pulmonary arterial hypertension (PAH); left heart diseases; post-thromboembolic disease; hypoxia and fibrosis resulting from interstitial lung disease; and the infrequent SLE-associated pulmonary veno-occlusive disease. The pathogenesis of PAH associated with lupus is yet unclear, but likely includes a role for the genetic background, the presence of antiphospholipid antibodies, and some level of endothelial dysfunction. The evolution of SLE-associated PH is highly variable and difficult to elicit because the published series have used heterogeneous inclusion criteria. Optimal therapeutic management of PAH associated with lupus is unclear because no dedicated randomized controlled trial is yet available. Treatment usually includes arterial pulmonary vasodilators and immunosuppressive agents when the patients have NYHA functional class II, III or IV dyspnea.
Pleural and pulmonary involvement in systemic lupus erythematosus
2011, Presse Medicale
Citation Excerpt :
Previous studies have largely concentrated on SSc-associated PAH (SSc-PAH), with a disease prevalence estimated to be between 7.5 and 12% [77,78]. Some degree of PHT complicates the course of SLE in 5–14% of the patients [79–81]. PHT prevalence and severity of disease in patients with SLE tend to increase with time [82].
Systemic lupus erythematosus (SLE) is a rare complex autoimmune disease with a multisystem involvement. The clinical manifestations of this disease include an erythematous rash, oral ulcers, polyarthralgia, nonerosive arthritis, polyserositis, hematologic, renal, neurologic, pulmonary and cardiac abnormalties. The involvement of the respiratory system is frequent. Pleuro-pulmonary manifestations are present in almost half of the patients during the disease course and may be the presenting symptoms in 4–5% of patients with SLE. Complications directly associated to the disease include pleuritis with or without pleural effusion, alveolitis, interstitial lung disease, lupus pneumonitis, pulmonary hemorrhage, pulmonary arterial hypertension, and pulmonary thromboembolic disease. Complications due to secondary causes include pleuro-pulmonary manifestations of cardiac and renal failure, atelectasis due to diaphragmatic dysfunction, opportunistic pneumonia, and drug toxicity. The prevalence, clinical presentation, prognosis and response to treatment vary, depending on the pattern of involvement. As with other connective tissue diseases, early and specific therapeutic intervention may be indicated for many of these pleuro-pulmonary manifestations.
Tricuspid Flow Propagation Velocity Predicts Exercise Tolerance and Readmission in Patients With Systemic Lupus Erythematosus
2009, Journal of the American Society of Echocardiography
Evaluating right ventricular dysfunction, pulmonary artery systolic pressure (PASP), and exercise tolerance is critical in patients with systemic lupus erythematosus (SLE) because of the high mortality rate in such patients with pulmonary arterial hypertension (PAH). The aim of this study was to use the flow propagation velocity (FPV) of early diastolic tricuspid inflow to evaluate exercise tolerance and PAH severity and to predict readmission in patients with SLE.
A total of 66 patients with SLE with or without PAH and 30 healthy control subjects were enrolled. Controls were age-matched to patients with SLE and without PAH. All patients completed the 6-minute walking distance (6MWD) test and underwent standard echocardiography. Tricuspid FPV was measured in the modified parasternal short-axis view using the color M-mode technique. PAH was defined as PASP > 35 mm Hg using the tricuspid regurgitant method.
Patients with SLE and PAH had significantly lower tricuspid FPVs and 6MWDs than patients in the other 2 groups (both P values < .001). Tricuspid FPV was well correlated with 6MWD (r = 0.748, P < .001). In multivariate analysis, right atrial pressure was the only independent factor affecting tricuspid FPV (R² = 0.394, P < .001), and 6MWD was affected only by tricuspid FPV and PASP (R² = 0.629, P < .001). Patients with SLE who had been readmitted had lower tricuspid FPVs than those who had not (P = .035). Furthermore, FPV ≥ 35.4 cm/s predicted 6MWD ≥ 350 m and a lower 1-year readmission rate with good sensitivity and specificity.
The tricuspid FPV technique provides a simple method for predicting exercise tolerance, the severity of PAH, and readmission among patients with SLE.
Central cyanosis due to severe pulmonary hypertension combined with pericarditis as the initial manifestation of systemic lupus erythematosus
2008, American Journal of Emergency Medicine
Citation Excerpt :
The symptoms were relieved dramatically, and she was discharged 12 days later. Pulmonary hypertension is an uncommon pulmonary manifestation of SLE, occurring in 0.5% to 14% of patients with SLE [3-7], although it has well-known association with other connective tissue disease, especially scleroderma and overlap syndrome [8]. Goupille et al [9] found that PH is often diagnosed after a well-established SLE, with a mean delay of 4.9 years.
Systemic lupus erythematosus (SLE) is a multisystemic autoimmune disorder. The initial manifestation complicating any organ system either singly or in combination is protean. Herein we report a 26-year-old female patient with fresh SLE whose initial manifestation was central cyanosis caused by severe pulmonary hypertension and acute pericarditis. The symptoms were relieved dramatically after treatment with steroid and bosentan. Accurate and timely diagnosis in SLE-associated pulmonary hypertension may be life saving.
The Lung in Systemic Lupus Erythematosus
2007, Systemic Lupus Erythematosus: A Companion to Rheumatology

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¹: From the Department of Medicine, Division of Rheumatology, New York University Medical Center, New York, New York.

²: H.D.P. is a recipient of NIAMDD Clinical Investigator Award AM-00463.

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Pulmonary hypertension in systemic lupus erythematosus: Report of four cases and review of the literature

Abstract

Am J Med

Case records of the Massachusetts General Hospital

N Engl J Med

Primary pulmonary hypertension and SLE

N Engl J Med (letter)

Pulmonary hypertension in systemic lupus erythematosus (letter)

N Engl J Med

Two cases of pulmonary hypertension with Raynaud's phenomenon. Primary pulmonary hypertension and systemic lupus erythematosus

Jap Heart J

Lupus cor pulmonale with electron microscope and immunofluorescent antibody studies

Ann Rheum Dis

Pulmonary hypertension and systemic lupus erythematosus

Arch Intern Med

Pleuropulmonary manifestations of systemic lupus erythematosus

Dis Chest

The clinical picture of systemic lupus erythematosus