Localized scleroderma is an autoimmune disorder

Takehara, Kazuhiko; Sato, Shinichi

doi:10.1093/rheumatology/keh487

Cited by 141 publications

(130 citation statements)

References 40 publications

Supporting

Mentioning

103

Contrasting

Unclassified

Order By: Relevance

“…Localized scleroderma differs from SSc in that it is not accompanied by Raynaud's phenomenon, acrosclerosis, and internal organ involvement 1 . However, since autoimmune abnormalities of localized scleroderma have been well recognized, this disease has generally been considered to have an autoimmune background 2 . Extensive involvement of the subcutaneous tissue and underlying bony structures is known 3 .…”

Section: Introductionmentioning

confidence: 99%

Case of localized scleroderma associated with osteomyelitis

Muroi

Ogawa

Yamaoka

et al. 2010

The Journal of Dermatology

View full text Add to dashboard Cite

We report a four-year-old girl presented with progressive linear scleroderma affecting the right leg. Biopsy specimen disclosed typical histopathological findings of localized scleroderma. Right leg magnetic resonance image (MRI) showed high signal areas on T2 weighted images on the subcutaneous fatty tissue, muscles, and bone marrow, suggesting that skin inflammation extended to the bone marrow. Oral corticosteroid therapy was instituted with improvement of both skin sclerosis and MRI findings. Our observations suggest that MRI examination should be considered in patents with localized scleroderma to evaluate the extension of the inflammation.

show abstract

Section: Introductionmentioning

confidence: 99%

Case of localized scleroderma associated with osteomyelitis

Muroi

Ogawa

Yamaoka

et al. 2010

The Journal of Dermatology

View full text Add to dashboard Cite

show abstract

“…Localized scleroderma (LSc) also manifests tissue fibrosis limited to the skin and s.c. tissue, occasionally involving the muscular tissues beneath the cutaneous lesions (1,2), but the presence of Raynaud's phenomenon, acrosclerosis, and involvement of internal organs differentiates SSc from LSc (3). Abnormal collagen metabolism (4)(5)(6)(7)(8) and autoimmunity (9,10) are considered to be fundamental common characteristics of SSc and LSc, and especially, excess collagen production by dermal fibroblasts is thought to be caused by intrinsic activation of TGF-b signaling in both diseases (5,11).…”

mentioning

confidence: 99%

The Downregulation of microRNA let-7a Contributes to the Excessive Expression of Type I Collagen in Systemic and Localized Scleroderma

Makino

Jinnin

Hirano

et al. 2013

The Journal of Immunology

147

111

View full text Add to dashboard Cite

Systemic and localized scleroderma (SSc and LSc) is characterized by excessive deposition of collagen and tissue fibrosis in the skin. Although they have fundamental common characteristics including autoimmunity, little is known about the exact mechanism that mediates the excessive collagen expression in these disorders. In the current study, we tried to evaluate the possibility that microRNAs (miRNAs) play some roles in the pathogenesis of fibrosis seen in these diseases. miRNA expression patterns were evaluated by miRNA array analysis, real-time PCR, and in situ hybridization. The function of miRNAs in dermal fibroblasts was assessed using miRNA inhibitors, precursors, or protectors. In the mouse model of bleomycin-induced dermal sclerosis, the overexpression of miRNAs was performed by i.p. miRNA injection. We demonstrated let-7a expression was downregulated in SSc and LSc skin both in vivo and in vitro, compared with normal or keloid skin. The inhibition or overexpression of let-7a in human or mouse skin fibroblasts affected the protein expression of type I collagen or luciferase activity of collagen 3′-untranslated region. Also, we found let-7a was detectable and quantitative in the serum and investigated serum let-7a levels in patients with SSc or LSc. let-7a concentration was significantly decreased in these patients, especially in LSc patients. Moreover, we revealed that the intermittent overexpression of let-7a in the skin by i.p. miRNA injection improved the skin fibrosis induced by bleomycin in mice. Investigation of more detailed mechanisms of miRNA-mediated regulation of collagen expression may lead to new therapeutic approaches against SSc and LSc.

show abstract

“…Detailed studies on fibroblasts results in over expression of collagen 1, 2, 3, fibronectin and proteoglycan, fibrillin 1 genesmutations (COL1A1, COL1A2) and matrix metalloproteinases alterations. 34 This causes a release of TGF ß that promotes fibrosis and unmasks the cryptic epitopes that become the targets of autoimmune response in the host with impaired immune tolerance. Studies on endothelium in scleroderma patients exhibited the presence of endothelial cytotoxic factor in the granules (granzyme A) in activated T-lymphocytes for arterial intimal proliferation, capillary dilatation, destruction and endothelial injury.Abnormalities in molecules of endothelin family (ETA and ETB), renin-angiotensin converting enzyme (ACE) and nitric oxide synthases (NOS) alter the vascular tone in Scleroderma patients and result in Raynaud's phenomenon.…”

Section: Psoriasismentioning

confidence: 99%

Autoimmune Disorders – Immunopathogenesis and Potential Therapies

Ganapathy¹,

Vedam²,

Rajeev³

et al. 2017

JYP

View full text Add to dashboard Cite

Autoimmune disorders are formed in the body as an outcome of inappropriate immune response against its own tissues. Failure of this immune system to recognize the body's normal constituents as "self" will result in inflammation and tissue damage. Several immune based mechanisms form the basis of autoimmune disorders in the body. Management of these lesions is based on the diagnostic criteria, evaluation of clinical and oral manifestation, and laboratory investigations of these lesions. There is currently no definitive cure for all autoimmune disorders, although in rare cases they may disappear on their own. As patients experience temporary remissions in symptoms or progressive worsening, our pharmacological management are targeted only for symptomatic relief and arrest the progression of the lesion. A detailed approach to titles and abstracts of importance in this field on the topic of interest via review and case reports was included. This review article highlights on various immune pathogenic mechanisms and treatment modalities of autoimmune disorders.

show abstract

Localized scleroderma is an autoimmune disorder

Abstract: Many previous studies conclude that localized scleroderma involves autoimmune abnormalities and is one of the organ-specific autoimmune disorders targeting mainly skin, although the types of autoimmune abnormality are different from systemic sclerosis.

Cited by 141 publications

References 40 publications

Case of localized scleroderma associated with osteomyelitis

Case of localized scleroderma associated with osteomyelitis

The Downregulation of microRNA let-7a Contributes to the Excessive Expression of Type I Collagen in Systemic and Localized Scleroderma

Autoimmune Disorders – Immunopathogenesis and Potential Therapies

Contact Info

Product

Resources

About