Dermatomyositis (Systemic Angiopathy) of Childhood

Banker, Betty Q.; Victor, Maurice

doi:10.1097/00005792-196607000-00001

Cited by 306 publications

(93 citation statements)

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“…intimal hyperplasia and occlusion of vessels by fibrin thrombi in the submucosa, muscularis, and serosal layers (2,3). This is in contrast to our patients, who showed a chronic vasculopathy rather than an acute vasculitis.…”

Section: Discussioncontrasting

confidence: 86%

Late‐onset gastrointestinal pain in juvenile dermatomyositis as a manifestation of ischemic ulceration from chronic endarteropathy

Mamyrova

Kleiner

James-Newton

et al. 2007

Arthritis & Rheumatism

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Section: Discussioncontrasting

confidence: 86%

Late‐onset gastrointestinal pain in juvenile dermatomyositis as a manifestation of ischemic ulceration from chronic endarteropathy

Mamyrova

Kleiner

James-Newton

et al. 2007

Arthritis & Rheumatism

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“…Lymphocytic aggregates are characteristic of inflammatory myopathies in both adults and children (1,6,41). Our data show that some of these cellular aggregates organize as extranodal lymphofollicular structures in the muscle of children with newly diagnosed juvenile DM.…”

Section: Discussionmentioning

confidence: 61%

“…Juvenile dermatomyositis (DM) is a chronic, multisystem inflammatory disease involving small vessels of skeletal muscle, skin, gastrointestinal tract, and other organs (1). The clinical spectrum is very variable, from mild disease that has minimal functional impact to a chronic, severely disabling condition (1)(2)(3)(4).…”

mentioning

confidence: 99%

“…The clinical spectrum is very variable, from mild disease that has minimal functional impact to a chronic, severely disabling condition (1)(2)(3)(4). Despite new therapies, juvenile DM remains chronically active in a large proportion of patients.…”

mentioning

confidence: 99%

“…The cellular infiltrate consists of T cells, B cells, and plasma cells (1,5,6). We have recently reported that many of the CD4ϩ cells in the inflamed muscle of patients with new-onset juvenile DM are CD123ϩ plasmacytoid dendritic cells (PDCs) (7).…”

mentioning

confidence: 99%

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Extranodal lymphoid microstructures in inflamed muscle and disease severity of new‐onset juvenile dermatomyositis

Padilla¹,

Vallejo²,

Lacomis³

et al. 2009

Arthritis & Rheumatism

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Objective. Juvenile dermatomyositis (DM) is an autoimmune disease of childhood characterized by lesions in skin and muscle that are populated by plasmacytoid dendritic cells (PDCs) and lymphocyte infiltrates. We undertook this study to examine the cellular composition, organization, and molecular milieu of the cellular infiltrates in muscle in juvenile DM and to correlate the infiltrates with clinical disease manifestations.Methods. Since PDCs and lymphocyte foci express CCL19 and CCL21, we investigated for in situ formation of lymphoid microstructures that could be sites of extranodal immune activation.Results. Analyses of muscle biopsy samples from children with new-onset juvenile DM showed 3 categories of lesions: diffuse infiltrates, lymphocytic aggregates lacking follicle-like organization, and follicle-like structures. The last of these exhibited elements of classic lymphoid follicles, including networks of follicular dendritic cells and high endothelial venules. They also expressed high levels of CXCL13 and lymphotoxins known to support lymphoid organogenesis. There were also resident naive CD45RA؉ T cells and maternally derived B cells and PDCs. Patients with diffuse infiltrates or lymphocytic aggregates were responsive to standard therapy with steroids and methotrexate, but those with follicle-like structures tended to have severe disease that required additional agents such as intravenous Ig or rituximab.Conclusion. These data suggest that lymphoneogenesis is a component of the early disease process in juvenile DM. Ectopic lymphoid structures could indicate a severe course of disease; their early detection could be a tool for disease management.

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Histopathology of the Skin in Dermatomyositis

Janis¹

1968

Arch Dermatol

118

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Dermatomyositis (Systemic Angiopathy) of Childhood

Cited by 306 publications

References 0 publications

Late‐onset gastrointestinal pain in juvenile dermatomyositis as a manifestation of ischemic ulceration from chronic endarteropathy

Late‐onset gastrointestinal pain in juvenile dermatomyositis as a manifestation of ischemic ulceration from chronic endarteropathy

Extranodal lymphoid microstructures in inflamed muscle and disease severity of new‐onset juvenile dermatomyositis

Histopathology of the Skin in Dermatomyositis

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