Peripheral Blood Lymphocyte Distribution in Scleroderma

Carapeto, F. J.; Winkelmann, Ria

doi:10.1159/000251340

Cited by 14 publications

(4 citation statements)

References 12 publications

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“…Although the role of cytomegalovirus (CMV) in the pathogenesis of SSc is debated (126), indirect evidence for a role of CMV-specific antibodies in the development of this disease has also been presented (127,128). Not only are absolute lymphocyte counts reduced in Ssc (129,130), but SSc patients also have relatively fewer CD4 + CD25 + cells in the peripheral circulation compared to healthy controls (131). In this setting of diminished regulatory T cells, a dysregulation of B cells is also observed (132).…”

Section: Scleroderma-associated Pah: a Paradigm For Inflammation Amentioning

confidence: 99%

Challenges and opportunities in treating inflammation associated with pulmonary hypertension

Voelkel

Tamošiūnienė

Nicolls

2016

Expert Review of Cardiovascular Therapy

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Inflammatory cells are present in the lungs from patients with many, if not all, forms of severe pulmonary hypertension. Historically the first inflammatory cell identified in the pulmonary vascular lesions was the mast cell. T and B lymphocytes, as well as macrophages, are present in and around the pulmonary arterioles and many patients have elevated blood levels of interleukin 1 and 6; some patients show elevated levels of leukotriene B4. An overlap between collagen-vascular disease-associated pulmonary arterial hypertension (PAH) and idiopathic PAH exists, yet only a few studies have been designed that evaluate the effect of anti-inflammatory treatments. Here we review the pertinent data that connect PAH and inflammation/immune dysregulation and evaluate experimental models of severe PAH with an emphasis on the Sugen/athymic rat model of severe PAH. We postulate that there are more than one inflammatory phenotype and predict that there will be several anti-inflammatory treatment strategies for severe PAH.

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Section: Scleroderma-associated Pah: a Paradigm For Inflammation Amentioning

confidence: 99%

Challenges and opportunities in treating inflammation associated with pulmonary hypertension

Voelkel

Tamošiūnienė

Nicolls

2016

Expert Review of Cardiovascular Therapy

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“…Although the role of cytomegalovirus (CMV) in the pathogenesis of scleroderma is debated [46], indirect evidence for a role of CMV-specific antibodies in the development of this disease has also been discussed [47,48]. Not only are absolute lymphocyte counts reduced in scleroderma [49,50], but scleroderma patients also have relatively fewer CD4+CD25+ cells in the peripheral circulation compared with healthy controls [23]. In this setting of diminished Tregs, a dysregulation of B-cells is also observed [51].…”

Section: Scleroderma: a Paradigm Of Autoimmune Pahmentioning

confidence: 99%

Autoimmunity and pulmonary hypertension: a perspective

Nicolls¹

2005

Eur Respir J

238

185

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The association between autoimmunity and pulmonary arterial hypertension (PAH) has been appreciated for .40 yrs, but how autoimmune injury might contribute to the pathogenesis of this disease has only been examined in a case-specific manner.It is becoming increasingly clear that a variety of diverse clinical diseases, ranging from viral infections to connective tissue disorders, can culminate in pulmonary vascular pathology that is indistinguishable. Is there a hitherto unappreciated biology that unites these seemingly unrelated conditions?The answer to this question may come from the increasing body of evidence concerned with the central importance of regulatory T-cells in preventing inappropriate B-cell activity. Two striking similarities between conditions associated with severe angioproliferative pulmonary hypertension are a defect in the CD4 T-cell compartment and auto-antibody production. Pathogenic autoantibodies targeting endothelial cells are capable of inducing vascular endothelial apoptosis and may initiate the development of PAH.The present review will focus on what is known about autoimmune phenomena in pulmonary arterial hypertension patients, in order to better consider whether an early loss of self-tolerance followed by autoimmune injury could influence the early development of severe angioproliferative pulmonary hypertension.

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“…Furthermore, in this disease the presence of cellular immunity to tissue antigens [3,5,6,12] has recently been noticed along with T lympho cyte deficiency [1,7] and lymphocyte-mediated cytotoxicity [3]. Previously, we isolated a scleroderma-inducing glycosaminoglycan (GAG) from the urine of patients with SD [9,10].…”

mentioning

confidence: 99%

Lymphocyte Responsiveness to Urinary Glycosaminoglycan in Systemic Scleroderma

Ishikawa¹,

Hattori²

1979

Dermatology

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From the urine of patients with systemic scleroderma, we previously isolated a glycosaminoglycan, which could induce a scleroderma-like change in the skin of mouse. In the present work, the cell-mediated response to urinary glycosaminoglycans was examined by lymphocyte transformation test. The specific response was observed in lymphocytes of patients with scleroderma, when the above scleroderma-inducing glycosaminoglycan was added. By contrast, lymphocytes of patients with SLE or dermatomyositis and of normal persons hardly showed a response to this glycosaminoglycan. On the other hand, the glycosaminoglycans from normal urine could not stimulate lymphocytes of patients with scleroderma or healthy persons.

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Peripheral Blood Lymphocyte Distribution in Scleroderma

Cited by 14 publications

References 12 publications

Challenges and opportunities in treating inflammation associated with pulmonary hypertension

Challenges and opportunities in treating inflammation associated with pulmonary hypertension

Autoimmunity and pulmonary hypertension: a perspective

Lymphocyte Responsiveness to Urinary Glycosaminoglycan in Systemic Scleroderma

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