Localized Scleroderma in Anti-NXP2-Antibody Positive Dermatomyositis A 67-year-old woman presented with erythematous, indurated skin on her left flanks. She had been diagnosed with dermatomyositis one year earlier when proximal muscle weakness, dysphagia and skin rash developed (see Figure A). Tests at the time showed the presence of anti-NXP2 and anti-Ro52 antibodies, as well as pathological…
Tumoral Calcinosis in Systemic Sclerosis Plain radiograph and computed tomography images depict calcinosis in the region of the right shoulder, left elbow and cervical spine of a 71-year-old woman with a 20-year history of systemic sclerosis. Manifestations include high-titer anti-Scl-70 antibody, diffuse skin involvement, Raynaud’s syndrome, acro-osteolysis, gastroesophageal reflux disease, interstitial lung disease and complete…
Tongue Twister 12-year-old girl who had been diagnosed with Moebius syndrome a year earlier presented for evaluation of progressive tongue hemiatrophy that was causing her difficulty with eating and speaking. Magnetic resonance imaging (MRI) of the brain demonstrated tongue hemiatrophy without involvement of the hypoglossal nerve. A tongue biopsy showed atrophy of skeletal muscle, variably…
PHILADELPHIA—Amy Gietzen started feeling the pain when she was 19. Her forearms, elbows, wrists and fingers were constantly swollen and sensitive. Six months later, she saw a doctor and was diagnosed with systemic diffuse scleroderma—a particularly hard-to-manage rheumatic disease with wide-ranging effects. Ms. Gietzen, who spoke at ACR Convergence 2022 and is a public speaker,…
PHILADELPHIA—At the first Plenary Session of ACR Convergence 2022, on Saturday, Nov. 12, speakers shared key research findings on the efficacy and safety of methotrexate as a co-therapy with pegloticase in refractory gout, the effectiveness of rituximab and cyclophosphamide in connective tissue disease associated-interstitial lung disease and the value of remote education for primary care…
Scleromyxedema is a primary cutaneous mucinosis characterized by a diffuse and generalized papular skin eruption of mucinous deposits throughout the upper dermis. In addition to dermatologic manifestations, scleromyxedema may involve the cardiopulmonary, gastrointestinal, renal and nervous systems. Dermato-neuro syndrome (DNS) is a rare, severe neurologic complication of scleromyxedema.1,2 The pathogenesis of DNS is unknown, but…
No one-size-fits-all approach exists for the care and treatment of patients with systemic sclerosis (SSc) and SSc with pulmonary involvement. Here, experts discuss some best clinical practices for these patients.
McMahan et al. examined how abnormal gastrointestinal (GI) transit may contribute to GI severity and symptoms in patients with systemic sclerosis (SSc). About 90% of people with SSc have GI tract involvement, and understanding the connection between GI symptoms, their severity and abnormal GI transit may permit targeted therapeutic approaches for these patients.
Laura Hummers, MD, MSc, provided an update on the extra-pulmonary manifestations of scleroderma & the best ways to approach management of these issues.
ACR Convergence 2021—The past year has been an exciting time for researchers and clinicians focused on the topic of connective tissue disease-associated interstitial lung disease (CTD-ILD), with advances in our understanding of these disorders and newly approved medications for their treatment. At the ACR Convergence 2021 annual Review Course, Kristin Highland, MD, MSCR, director of…