Extranodal lymphoid microstructures in inflamed muscle and disease severity of new-onset Juvenile dermatomyositis

Consuelo M.López De Padilla, Abbe N. Vallejo, David Lacomis, Kelly Mcnallan, Ann M. Reed

Research output: Contribution to journalArticlepeer-review

70 Scopus citations


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 infil- trates 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 intrave- nous Ig or rituximab. Conclusion. These data suggest that lymphoneo- genesis is a component of the early disease process in juvenile DM. Ectopic lymphoid structures could indi- cate a severe course of disease; their early detection could be a tool for disease management.

Original languageEnglish (US)
Pages (from-to)1160-1172
Number of pages13
JournalArthritis and rheumatism
Issue number4
StatePublished - Apr 2009

ASJC Scopus subject areas

  • Immunology and Allergy
  • Rheumatology
  • Immunology
  • Pharmacology (medical)


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