Undiagnosed connective tissue diseases

Cavagna, Lorenzo; Codullo, Veronica; Ghio, Stefano; Scirè, Carlo Alberto; Guzzafame, Eleonora; Rossi, Silvia; Montecucco, Carlomaurizio; Caporali, Roberto

doi:10.1097/md.0000000000004827

Cited by 17 publications

(2 citation statements)

References 35 publications

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“…SSc patients are mainly treated by anti-inflammation, immunoregulation, treatment of vascular lesion and anti-fibrosis, which has a long treatment course and causes obvious adverse drug reactions ( 15 - 17 ). With various strong effects such as self-duplication, immunoregulation, vascularization promotion and secretion, MSC becomes the hot spot of treatment for autoimmune disease.…”

Section: Discussionmentioning

confidence: 99%

Umbilical Cord Mesenchymal Stem Cells for Inhibiting the Fibrosis and Autoimmune Development in HOCl-Induced Systemic Scleroderma Mouse Model

Jin

Hou

Zheng

et al. 2021

IJSC

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Background and Objectives: Systemic scleroderma (SSc) is a rare and serious connective tissue disease, an autoimmune disease, and a rare refractory disease. In this study, preventive effect of single systemic human umbilical cord mesenchymal stem cells (UC-MSCs) transfusion on SSc was preliminarily explored. Methods and Results: SSc mouse model was established by daily intradermal injection of Hypochlorite (HOCl). SSc mice were treated by single transfusion of UC-MSCs at 0.625×10 5 , 2.5×10 5 and 1×10 6 respectively. At the 42nd day of intradermal injection of HOCl, the symptoms showed up by skin and alveolar wall thickening, lymphocytic infiltration, increased collagen in skin/lung, and the increased proportion of CD3 FoxP3＋ cells (a Treg subset) in spleen. After UC-MSCs transfusion, the degree of skin thickening, alveolar wall thickening and lymphocyte infiltration were decreased, the collagen sedimentation in skin/lung was decreased, and the proportion of CD3 FoxP3＋ cells was decreased. Conclusions: UC-MSC can achieve a preventive effect in SSc mice by fibrosis attenuation and immunoregulation.

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Section: Discussionmentioning

confidence: 99%

Umbilical Cord Mesenchymal Stem Cells for Inhibiting the Fibrosis and Autoimmune Development in HOCl-Induced Systemic Scleroderma Mouse Model

Jin

Hou

Zheng

et al. 2021

IJSC

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“…In the present study, 1 of the 40 patients (2.5%) was initially diagnosed as SS-associated PAH. This result is consistent with a similar study reported by Cavagna et al ., showing that only 1 out of 49 suspected idiopathic PAH patients (2.0%), have met the criteria of SS syndrome at the time of the initial evaluation [ 28 ]. We have additionally found that 5 out of the 25 patients who were initially diagnosed with idiopathic PAH was later diagnosed as primary SS.…”

Section: Discussionmentioning

confidence: 99%

Prevalence of primary Sjögren’s syndrome in patients undergoing evaluation for pulmonary arterial hypertension

et al. 2018

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BackgroundThe prevalence of pulmonary arterial hypertension (PAH) in primary Sjögren’s syndrome (SS) had been reported to be rare. However, recent studies using echocardiography as a screening method showed conflicting results, and the true prevalence is still unclear. Since diagnosing primary SS is difficult because of its heterogeneous nature, a number of patients with primary-SS-associated PAH may be misdiagnosed with idiopathic PAH, losing their chance to undergo immunosuppressive therapy. Therefore, we sought to elucidate the prevalence of primary SS among patients who initially present with PAH.MethodsFrom our prospective institutional PAH database, 40 consecutive patients without any obvious cause of PAH at the time of PAH diagnosis were identified. We retrospectively evaluated the prevalence of primary SS diagnosed during or after the initial assessment of PAH.ResultsDuring the initial assessment, one patient was diagnosed with primary-SS-associated PAH. Among the 25 patients who were initially diagnosed with idiopathic PAH, five were diagnosed with primary SS during their course of the disease. Of the five patients, three had key signs suggesting primary SS and were probably underdiagnosed at the time of initial evaluation. The remaining two patients, who were finally diagnosed with primary SS, did not have any specific signs suggesting primary SS at the time of initial evaluation but showed positive conversion of their autoantibodies during the course of PAH.ConclusionThe prevalence of primary-SS-associated PAH may be relatively high among patients who undergo initial evaluation for PAH. Furthermore, primary-SS-associated PAH may be underdiagnosed with routine evaluation for the primary cause of PAH. Clinicians should pay specific attention and carefully evaluate the possibility of primary SS in patients with PAH.

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Inflammatory Mechanisms in the Pathogenesis of Pulmonary Arterial Hypertension: Recent Advances

Mercurio

Cuomo

Naranjo

et al. 2021

Comprehensive Physiology

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Inflammatory processes are increasingly recognized in the pathogenesis of the vascular remodeling that characterizes pulmonary arterial hypertension (PAH). Chronic inflammation may contribute to disease progression or serve as a biomarker of PAH severity. Furthermore, inflammatory pathways may represent possible therapeutic targets for novel PAH-specific drugs beyond the currently approved therapies targeting the endothelin, nitric oxide/cyclic GMP, and prostacyclin biological pathways. The main focus of this article is to provide recent advances in the understanding of the role of inflammatory pathways in the pathogenesis of PAH from preclinical studies and current clinical data supporting chronic inflammation in PAH patients and to discuss emerging therapeutic implications.

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Undiagnosed connective tissue diseases

Cited by 17 publications

References 35 publications

Umbilical Cord Mesenchymal Stem Cells for Inhibiting the Fibrosis and Autoimmune Development in HOCl-Induced Systemic Scleroderma Mouse Model

Umbilical Cord Mesenchymal Stem Cells for Inhibiting the Fibrosis and Autoimmune Development in HOCl-Induced Systemic Scleroderma Mouse Model

Prevalence of primary Sjögren’s syndrome in patients undergoing evaluation for pulmonary arterial hypertension

Inflammatory Mechanisms in the Pathogenesis of Pulmonary Arterial Hypertension: Recent Advances

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