Lesional and nonlesional skin from patients with untreated juvenile dermatomyositis displays increased numbers of mast cells and mature plasmacytoid dendritic cells

Shrestha, Sheela; Wershil, Barry K.; Sarwark, John F.; Niewold, Timothy B.; Philipp, Teresa; Pachman, Lauren M.

doi:10.1002/art.27529

Cited by 65 publications

(49 citation statements)

References 45 publications

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“…As in SLE, autoantigens of apoptotic origin provide the immunogenic stimulus for the initiation of pathogenic responses (78). RNA-containing immune complexes can activate pDCs in salivary glands and enhance the production of IFN-, while IFN- itself can upregulate the expression of ISGs in the target organs (87,88). Early studies clearly identified an IFN signature in salivary glands from patients with pSS; IRF7, IRF8, and IRF9 were significantly upregulated (91,92).…”

Section: Sjögren's Syndromementioning

confidence: 99%

Type I interferon–mediated autoimmune diseases: pathogenesis, diagnosis and targeted therapy

2017

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Section: Sjögren's Syndromementioning

confidence: 99%

Type I interferon–mediated autoimmune diseases: pathogenesis, diagnosis and targeted therapy

2017

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“…Specific use of topical corticosteroids or topical immunomodulators (tacrolimus, pimecrolimus) can be helpful for limited rashes when disease activity is confined to the skin and not amenable to systemic therapy, or for more severe rashes, including scalp involvement 103 . Antihistamines and moisturizers are helpful for treating associated pruritis, given the presence of dermal mast cells in the skin of JDM patients 106 . Hydroxychloroquine, which inhibits Toll-like receptor activation, has anecdotally been helpful as an adjunctive agent and has been shown to improve cutaneous and renal disease activity and prevent disease damage in lupus 107 .…”

Section: Treatment Approaches For Juvenile Dermatomyositismentioning

confidence: 99%

Developments in the Classification and Treatment of the Juvenile Idiopathic Inflammatory Myopathies

Rider¹,

Katz²,

Jones³

2013

Rheumatic Disease Clinics of North America

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The juvenile idiopathic inflammatory myopathies (JIIM) are rare, heterogeneous autoimmune diseases that share chronic muscle inflammation and weakness. JIIM broadly includes three major clinicopathologic groups: juvenile dermatomyositis, juvenile polymyositis, and overlap myositis. A growing spectrum of clinicopathologic groups and serologic phenotypes defined by the presence of myositis-specific or myositis-associated autoantibodies are now recognized, each with differing demographics, clinical manifestations, laboratory findings, and prognoses. With the first multi-center collaborative studies and controlled trials using standardized preliminarily validated outcome measures, the therapy of juvenile myositis has advanced. Although daily oral corticosteroids remain the backbone of treatment, disease-modifying anti-rheumatic drugs (DMARDs) are almost always used as adjunctive therapy. Methotrexate is the conventional DMARD for the initial therapy, either alone or combined with intravenous pulse methylprednisolone, and/or intravenous immunoglobulin for patients with moderate to severe disease. Cyclosporine may be added to these or serve as an alternative to methotrexate. Other drugs and biologic therapies, including mycophenolate mofetil, tacrolimus, cyclophosphamide, rituximab, and infliximab, might benefit selected patients with recalcitrant disease, unacceptable steroid toxicity, or patients with risk factors for poor prognosis. The treatment of cutaneous disease, calcinosis, and the role for rehabilitation are also discussed.

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“…Indeed, pDCs are present in both muscle and skin of DM patients [16, 26, 27, 35, 46•, 47, 48]. The presence of mature pDCs has been shown in perivascular and perimysial infiltrates in muscle biopsies from DM and JDM patients [16,26,47,48]. Mature pDCs in DM muscle also express CD83 (a maturation marker) as well as chemokines CCL21, CCL19, and CXCL12, all of which are important for dendritic cell migration into regional lymph nodes [47].…”

Section: Interferon-inducible Transcripts and Proteins In Dermatomyosmentioning

confidence: 98%

Pathogenesis of Dermatomyositis: Role of Cytokines and Interferon

2011

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Dermatomyositis is a systemic autoimmune disease that primarily affects skeletal muscle, skin, and the lungs. Dermatomyositis is characterized by autoantibodies, tissue inflammation, parenchymal cell damage and death, and vasculopathy. This review focuses on recent advances regarding the role of cytokines and interferon in the pathogenesis of the disease. Evidence for the role of a particular cytokine is based on data showing dysregulated levels in tissue and/or blood; correlation with histopathologic or clinical markers of disease activity; and, rarely, clinical efficacy of targeted cytokine inhibitors. Many of the recent advances pertain to elucidation of the role of interferons in both muscle and skin disease in dermatomyositis. Although a great deal of progress has been made regarding the role of interferon in the disease, many critical questions remain unanswered.

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Lesional and nonlesional skin from patients with untreated juvenile dermatomyositis displays increased numbers of mast cells and mature plasmacytoid dendritic cells

Cited by 65 publications

References 45 publications

Type I interferon–mediated autoimmune diseases: pathogenesis, diagnosis and targeted therapy

Type I interferon–mediated autoimmune diseases: pathogenesis, diagnosis and targeted therapy

Developments in the Classification and Treatment of the Juvenile Idiopathic Inflammatory Myopathies

Pathogenesis of Dermatomyositis: Role of Cytokines and Interferon

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