Juvenile dermatomyositis (JDM) is a childhood form of inflammatory myopathy affecting children younger than 18 years. The diagnosis of JDM is based on characteristic clinical features of proximal muscle weakness, skin rash, elevated muscle enzymes, magnetic resonance imaging and muscle biopsy.1 Autoantibodies seen in patients with inflammatory myopathy are not only associated with particular phenotypes…
Clinically amyopathic dermatomyositis (CADM), a rare subset of dermatomyositis (DM), is an autoimmune disease characterized by cutaneous findings of typical DM without evidence of myositis. Childhood presentation of CADM is rare, and not many studies describe the epidemiology of juvenile CADM.1,2 Although lung disease is rare among patients with juvenile DM, a few reports have…
Subcutaneous tocilizumab is the first biologic agent approved by the FDA treat patients with systemic sclerosis-associated interstitial lung disease.
ACR Convergence panelists discussed how to monitor SSc-ILD patients for ILD severity & the brightening prospects for therapy.
Interstitial lung disease (ILD) is a heterogeneous group of lung parenchymal disorders that share several clinical, radiologic and histopathologic features, and are therefore grouped together.1 ILD can occur in association with most rheumatic connective tissue diseases (CTDs), but patients with systemic sclerosis, polymyositis, dermatomyositis and rheumatoid arthritis have the highest risk of developing ILD.2 A…
Clinically amyotrophic dermatomyositis (CADM), a subset of dermatomyositis (DM), is a rare autoimmune disease characterized by typical DM cutaneous findings (e.g., heliotrope rash, Gottron papules, Gottron sign) without evidence of myositis.1 The incidence of DM and CADM is approximately 9.63 per 1 million people and 2.08 per 1 million people, respectively.2 The association with development…
Interstitial lung disease accompanied by anti-melanoma differentiation-associated gene 5 positive dermatomyositis is often rapidly progressive and associated with poor prognosis. In this study, a combined immunosuppressive regimen of high-dose glucocorticoids, tacrolimus and intravenous cyclophosphamide proved more effective than treatment with high-dose glucocorticoids and stepwise addition of an immunosuppressant in a historical control group.
The U.S. Food & Drug Administration (FDA) approved nintedanib for systemic sclerosis associated interstitial lung disease (SSc-ILD) on Sept. 6 after a randomized, controlled trial (SENSCIS) demonstrated significant benefit against placebo.1 At a cost of $96,000 per year, treatment reduced the adjusted annual rate of change in forced vital capacity (FVC) from –93.3 mL in…