Pulmonary Hypertension in Systemic Lupus Erythematosus: A Report of an Autopsied Case.

Satō, Masaki; Okazaki, Hitoaki; Okamoto, Hiroshi; Ohtaka, Akihiko; Shimizu, Hideo; Minato, Nagahiro; Kano, Shogo

doi:10.2169/internalmedicine.33.540

Cited by 6 publications

(7 citation statements)

References 12 publications

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“…The clinical relevance of NYHA functional class, 6‐minute walking distance, and hemodynamics has been previously emphasized, since these are major end points in PAH and have a strong association with mortality (25). These results confirm and extend our previous data and case reports in the literature (13–18, 26, 27). Indeed, we recently reported that among 21 patients with SLE‐ or MCTD‐associated PAH, 8 (38%) were responders after at least 1 year of immunosuppressive therapy, without the need of addition of PAH‐specific therapy (20).…”

Section: Discussionsupporting

confidence: 93%

“…The overexpression of growth factors such as platelet‐derived growth factors A and B and the chemokines RANTES/CCL5 and fractalkine/CX 3 CL1 have been demonstrated in the pulmonary arteries of patients with severe PAH (11, 12). These findings strongly support the use of immunosuppressants in CTD‐associated PAH, although conflicting results have been reported in the published case reports (13–19) and no randomized clinical trials have validated their use.…”

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confidence: 53%

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

Jaïs¹,

Launay²,

Yaïci³

et al. 2008

Arthritis & Rheumatism

325

247

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Objective. To describe the response to first-line immunosuppressive therapy with or without pulmonary vasodilators in pulmonary arterial hypertension (PAH) associated with systemic lupus erythematosus (SLE) or mixed connective tissue disease (MCTD).Methods. Twenty-three consecutive patients with SLE-or MCTD-associated PAH treated with first-line immunosuppressive therapy either alone (n ‫؍‬ 16) or in combination with pulmonary vasodilators (n ‫؍‬ 7) were evaluated according to clinical and hemodynamic criteria before and after immunosuppressive therapy. Responders were defined as patients in New York Heart Association (NYHA) functional class I or II with hemodynamic improvement after the last pulse of cyclophosphamide.Results. Among the 16 patients treated with firstline immunosuppressive therapy alone, 8 (50%) were responders. These patients had a significantly improved NYHA functional class, 6-minute walking distance, and mean pulmonary artery pressure. Patients in NYHA functional class I or II and/or a cardiac index >3.1 liters/minute/m 2 at baseline were more likely to benefit from immunosuppressive therapy. Six of the 8 nonresponders subsequently improved with pulmonary vasodilators. Among the 7 patients who were initially treated with immunosuppressive therapy and pulmonary vasodilators, 4 (57.1%) were responders.Conclusion. PAH associated with SLE or MCTD may respond to a treatment combining cyclophosphamide and glucocorticoids. Patients who could benefit from this immunosuppressive therapy could be those who have less severe disease at baseline. For patients with more severe disease, pulmonary vasodilators should be started, possibly in combination with immunosuppressants. In any case, clinical and hemodynamic evaluations are mandatory to monitor the response and adapt the treatment. These retrospective and uncontrolled data need to be confirmed by randomized controlled trials.

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Section: Discussionsupporting

confidence: 93%

supporting

confidence: 53%

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

Jaïs¹,

Launay²,

Yaïci³

et al. 2008

Arthritis & Rheumatism

325

247

View full text Add to dashboard Cite

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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

107

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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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“…Of these four, three were "primary" non-thromboembolic PHT. Sato et al [41] in 1994 published an autopsied case of SLE complicated by primary PHT and aPL. There have also been other single case reports of patients with primary APS and this type of PHT [42][43][44][45].…”

Section: Pulmonary Hypertension and Antiphospholipid Antibodiesmentioning

confidence: 99%

Pulmonary Hypertension, Antiphospholipid Antibodies, and Syndromes

Asherson

Cervera²

2007

Clinic Rev Allerg Immunol

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Antiphospholipid antibodies have been associated with two types of pulmonary hypertension (PHT), the thromboembolic type, after deep venous thromboses in the lower limbs complicated by pulmonary embolism and the "primary" plexogenic type. The PHT may occur in the absence of any other manifestations of the antiphospholipid syndrome (APS), and cases have been recorded with very high levels of antiphospholipid antibodies. It may also accompany systemic lupus erythematosus (SLE) and may manifest with or without other features of the APS. It may also form part of the clinical presentation of a "primary" APS. Its prevalence is of the order of 1.8-3.5% of the manifestations of the APS depending on the series. Primary "idiopathic" PHT has long been regarded as an "immunological" disorder. Its manifestations are essentially to the primary type seen with the connective tissue disorders such as SLE, APS, mixed connective tissue disease, calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia variety of systemic sclerosis and Sjögren's syndrome. The high prevalence of PHT in patients with human immunodeficiency virus infection who demonstrate low CD4 counts points to a close relationship between the T regulatory cells (Treg) and the development of PHT, and this hypothesis is discussed in this review. Genetic and chromosomal aspects of PHT are also discussed.

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Pulmonary Hypertension in Systemic Lupus Erythematosus: A Report of an Autopsied Case.

Cited by 6 publications

References 12 publications

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

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

Pulmonary involvement of systemic lupus erythematosus: analysis of 90 necropsies

Pulmonary Hypertension, Antiphospholipid Antibodies, and Syndromes

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