Successful Use of Intensive Immunosuppressive Therapy for Treating Simultaneously Occurring Cerebral Lesions and Pulmonary Arterial Hypertension in a Patient with Systemic Lupus Erythematosus

Watanabe, Ryu; Fujii, Hiroshi; Shirai, Toshikazu; Saito, Shinichiro; Hatakeyama, Akira; Sugimura, Koichiro; Ishii, Tomonori; Harigae, Hideo

doi:10.2169/internalmedicine.53.0514

Cited by 9 publications

(6 citation statements)

References 24 publications

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“…However, CTD-PH also has characteristics of Group 1’ (pulmonary vein occlusion), Group 2 (pulmonary hypertension due to left sided heart disease), and Group 3 (pulmonary hypertension due to lung diseases) because it sometimes accompanies pulmonary vein occlusion, fibrosis of the left ventricular myocardium, and interstitial pneumonia. Further, CTD-PH, except in case of scleroderma, can be expected the improvement by immunosuppressive therapy [ 1 , 7 ], which is another way in which CTD-PH differs from IPAH. Thus, to approach clinical CTD-PH, an experimental model of CTD that spontaneously develops pulmonary hypertension is necessary in addition to monocrotaline-administered mice and vascular endothelial growth factor (VEGF) inhibition with hypoxic exposure mice which are popular as animal models of pulmonary arterial hypertension [ 8 , 9 ].…”

Section: Introductionmentioning

confidence: 99%

Autoimmune disease mouse model exhibits pulmonary arterial hypertension

Sugimoto¹,

Nakazato²,

Sato³

et al. 2017

PLoS ONE

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BackgroundPulmonary arterial hypertension is often associated with connective tissue disease. Although there are some animal models of pulmonary hypertension, an autoimmune disease-based model has not yet been reported. MRL/lpr mice, which have hypergammaglobulinemia, produce various autoimmune antibodies, and develop vasculitis and nephritis spontaneously. However, little is known about pulmonary circulation in these mice. In the present study, we examined the pulmonary arterial pressure in MRL/lpr mice.Methods and resultsWe used female MRL/lpr mice aged between 12 and 14 weeks. Fluorescent immunostaining showed that there was no deposition of immunoglobulin or C3 in the lung tissue of the MRL/lpr mice. Elevation of interferon-γ and interleukin-6 was recognized in the lung tissue of the MRL/lpr mice. Right ventricular systolic pressure, Fulton index and the ratio of right ventricular weight to body weight in the MRL/lpr mice were significantly higher than those in wild type mice with same background (C57BL/6). The medial smooth muscle area and the proportion of muscularized vessels in the lung tissue of the MRL/lpr mice were larger than those of the C57BL/6 mice. Western blot analysis demonstrated markedly elevated levels of prepro-endothelin-1 and survivin as well as decreased endothelial nitric oxide synthase phosphorylation in the lung tissue of the MRL/lpr mice. Terminal deoxynucleotidyl-transferase-mediated dUTP nick end-labeling assay showed the resistance against apoptosis of pulmonary arterial smooth muscle cells in the MRL/lpr mice.ConclusionWe showed that MRL/lpr mice were complicated with pulmonary hypertension. MRL/lpr mice appeared to be a useful model for studying the mechanism of pulmonary hypertension associated with connective tissue diseases.

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Section: Introductionmentioning

confidence: 99%

Autoimmune disease mouse model exhibits pulmonary arterial hypertension

Sugimoto¹,

Nakazato²,

Sato³

et al. 2017

PLoS ONE

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“…The regimen of IVCY for SLE- or MCTD-associated PAH also remains to be established. In previous studies and case reports, the dose, interval, and duration of IVCY for SLE- or MCTD-associated PAH were 500 to 1,000 mg/m 2 per pulse, monthly in most cases, and 3 to 12 months, respectively ( 1 , 3 - 5 , 8 - 17 ).…”

Section: Discussionmentioning

confidence: 92%

Treatment of Vasodilator-resistant Mixed Connective Tissue Disease-associated Pulmonary Arterial Hypertension with Glucocorticoid and Cyclophosphamide

et al. 2017

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Pulmonary arterial hypertension (PAH) associated with systemic lupus erythematosus (SLE) or mixed connective tissue disease (MTCD), in contrast to other types of PAH, may respond to immunosuppressive therapy. Most PAH cases with an immunosuppressant response were in the early stages of the disease (WHO functional class III or less). The present case was a 34-year-old woman with MCTD-associated PAH (WHO functional class IV) who was resistant to a combination of three vasodilators. Afterwards, she was treated with glucocorticoid and cyclophosphamide. This case suggested the potential benefit of immunosuppressants in patients with severe MCTD-associated PAH.

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“…6 Watanabe et al similarly described a slight alteration in mental status that was associated with imaging findings that showed cerebellar edema. 14 However, Sy et al reported symptoms include headache, visual hallucinations, and disorientation while having unremarkable MRI findings. 11 In our case, MRI did not provide any definitive cause for our patient’s disorientation.…”

Section: Discussionmentioning

confidence: 99%

Lupus Cerebellitis Presenting With Ataxia: A Case Report

Kristoff

Shoskes

Claytor

2021

The Neurohospitalist

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We report a case of a woman with a history of systemic lupus erythematosus who developed persistent ataxia and was diagnosis with lupus cerebellitis. Magnetic resonance imaging of the brain showed T2/FLAIR signal hyperintensity within the cerebellar vermis without mass effect. The patient’s condition improved with pulse IV methylprednisolone. This case highlights unique imaging findings within the cerebellum, our diagnostic and treatment regimen, and compares this case to previous literature on similar cases.

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Successful Use of Intensive Immunosuppressive Therapy for Treating Simultaneously Occurring Cerebral Lesions and Pulmonary Arterial Hypertension in a Patient with Systemic Lupus Erythematosus

Cited by 9 publications

References 24 publications

Autoimmune disease mouse model exhibits pulmonary arterial hypertension

Autoimmune disease mouse model exhibits pulmonary arterial hypertension

Treatment of Vasodilator-resistant Mixed Connective Tissue Disease-associated Pulmonary Arterial Hypertension with Glucocorticoid and Cyclophosphamide

Lupus Cerebellitis Presenting With Ataxia: A Case Report

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