A Novel Overlap Syndrome

Zandman‐Goddard, Gisele; Tweezer-Zaks, Nurit; Shalev, Tamar; Levy, Yair; Ehrenfeld, Michael; Langevitz, Pnina

doi:10.1196/annals.1422.053

Cited by 16 publications

(11 citation statements)

References 7 publications

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“…Recent evidence suggests that antiphospholipid antibodies, which alter the regulation of hemostasis and induce the activation of complement (83), are detected in several autoimmune disorders (84). They are recognized as a group of antibodies directed not only toward phospholipids such as cardiolipin, β 2 glycoprotein I ( β 2GPI), but also toward lupus anticoagulant antibodies, and antiphosphatidylserine-prothrombin complex antibodies that are detectable in SSc patients (67,69).…”

Section: Resultsmentioning

confidence: 99%

“…SSc patients with aCL/a β 2GPI antibodies are more likely to develop microvascular dysfunction (pulmonary arterial hypertension, digital infarcts) and macrovascular impairment (69). Next, there is a strong correlation between the presence of aCL and pulmonary hypertension and the possibility that aCL/a β 2GPI and lupus anticoagulant antibodies may increase local production of endothelin and cause pulmonary hypertension (84). Also, patients with anti-PS-PT antibodies may represent a unique group of SSc patients with peripheral ischemia and lung disease, but these antibodies have not been found to be associated with severity of skin disease (86).…”

Section: Resultsmentioning

confidence: 99%

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Coagulation and Autoimmunity in Scleroderma Interstitial Lung Disease

Ludwicka-Bradley¹,

Silver²,

Bogatkevich³

2011

Seminars in Arthritis and Rheumatism

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Objectives Interstitial lung disease in systemic sclerosis (SSc-ILD) is often an irreversible and progressive fibrosing process that now is the leading cause of scleroderma-related deaths. In this review we present our current understanding of the role played by coagulation and particularly by thrombin in autoimmune-mediated tissue injury and fibrosis, mainly as it relates to SSc-ILD. Methods We used PubMed to search for articles published up to October 2010 for keywords referring to autoimmunity, coagulation, pulmonary fibrosis, and scleroderma. Results SSc-ILD is an autoimmune disease associated with lymphocyte activation and release of various cytokines and growth factors. The production of autoantibodies is a central feature in SSc. Activation of the coagulation cascade with release of thrombin is 1 of the earliest events following tissue injury. Thrombin contributes to autoimmune responses by activating of pathogenic Th2 lymphocyte profile in SSc. Thrombin also modulates tissue repair responses, stimulates transformation of epithelial cells, endothelial cells, and fibroblasts into myofibroblast phenotype, and induces secretion of several pro-immune and profibrotic factors, which serve as antigens for pathogenic autoantibodies production in SSc-ILD. Conclusions The identification of links between autoimmunity and coagulation would provide new insights into the pathogenesis of pulmonary fibrosis associated with autoimmune diseases and further acknowledge the importance of thrombin in the development of SSc-ILD.

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Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

Coagulation and Autoimmunity in Scleroderma Interstitial Lung Disease

Ludwicka-Bradley¹,

Silver²,

Bogatkevich³

2011

Seminars in Arthritis and Rheumatism

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“…Minatani et al previously reported the first presentation of this overlap syndrome in a male patient [4]. One case series with five female patients [5] and other three cases of female patients [6–8] were also reported in the literature.…”

Section: Discussionmentioning

confidence: 99%

Treatment Resistant Severe Digital Ischemia Associated with Antiphospholipid Syndrome in a Male Patient with Systemic Sclerosis

Küçükşahin

Ateş

Okoh

et al. 2014

Case Reports in Rheumatology

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We report the case of a male patient with limited cutaneous systemic sclerosis (SSc) that was complicated with severe digital ischemia, resistant to medical treatment. Due to the lack of treatment response, further laboratory and imaging studies were conducted. Findings were compatible with antiphospholipid syndrome and oral warfarin was added to the treatment regimen. After successful anticoagulation no further recurrences of digital ischemia were seen. An underlying etiology in SSc patients with treatment resistant digital ischemic necrosis should be suspected for accompanying antiphospholipid syndrome (APS).

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“…Although cases of antiphospholipid (APL) syndrome have been described in a patient with scleroderma [259][260][261], the role of APL antibodies in the pathogenesis of the disease is not fully defined. Anticardiolipin antibodies (aCL) have been detected in about 4.8-45.5% of patients with scleroderma [262][263][264][265][266][267][268][269][270].…”

Section: Antiphospholipidsmentioning

confidence: 99%

Vascular Disease in Scleroderma

Wigley

2008

Clinic Rev Allerg Immunol

113

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Although scleroderma is generally considered a fibrosing disease of the tissues, it is now recognized that the underlying vascular disease is playing a fundamental role in its pathogenesis and associated tissue injury. The exact mechanism for the widespread scleroderma vascular disease is still unknown, but endothelial cell injury induced by infection, immune-mediated cytotoxicity, antiendothelial antibodies, and/or ischemia-reperfusion have all been implicated. The downstream effects of blood vessel perturbation produce "biomarkers" of vascular damage that reflect disease and may predict clinical outcomes. A complex interaction between endothelial cells, smooth muscle cells, extracellular matrix, and intravascular circulating factors is now recognized to contribute to the vascular reactivity, remodeling, and occlusive disease of scleroderma. Understanding the mechanisms underlying these processes provides rationale of novel therapeutic strategies and specific targeted therapy. This review will outline some of the evidence for the causes and consequences of scleroderma vascular disease.

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A Novel Overlap Syndrome

Cited by 16 publications

References 7 publications

Coagulation and Autoimmunity in Scleroderma Interstitial Lung Disease

Coagulation and Autoimmunity in Scleroderma Interstitial Lung Disease

Treatment Resistant Severe Digital Ischemia Associated with Antiphospholipid Syndrome in a Male Patient with Systemic Sclerosis

Vascular Disease in Scleroderma

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