For the 2023 Image Competition, the ACR sought images representing a diverse range of patients with idiopathic inflammatory myopathies (IIMs) or IIM mimics. The images selected as Best Overall by the judges this year come from North America. Systemic Light Chain (AL) Amyloidosis Masquerading as Inflammatory Myositis These images depict an 81-year-old man who presented…
Diffuse Cutaneous Scleroderma with Interstitial Lung Disease: Radiological Findings A 44-year-old woman with cutaneous manifestations, tightening of her face with a reduced oral aperture and a healing digital tip ulcer (Figures 1 and 2) has had a known case of diffuse scleroderma since 2008. The patient presented with complaints of shortness of breath along with…
Resolution of GAVE After aHSCT for Progressive Systemic Sclerosis A 30-year-old man with RNA polymerase III (Pol III)-positive, diffuse, progressive systemic sclerosis had a persistent microcytic anemia with a hemoglobin level of 8 g/dL and evidence of gastric antral ventral ectasia (GAVE) on gastroscopy. He underwent autologous hematopoietic stem cell transplantation (aHSCT). After six months,…
Periungual Erythema & Its Translation on Nailfold Videocapillaroscopy in a Patient with Very Early Systemic Sclerosis A 66-year-old woman presented with Raynaud’s phenomenon and periungual erythema. HEp-2 immunofluorescence assay was positive for antinuclear antibodies, showing a centromere pattern. The presence of anti-centromere antibodies was confirmed by chemiluminescent immunoassay. The patient was diagnosed with very early…
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…
Saddle Nose & Cauliflower Ear Deformities in Relapsing Polychondritis These images depict a 32-year-old man who presented with five weeks of left-sided hearing loss, weight loss and discomfort in the nose, ear, chest wall and knee. He had an erythrocyte sedimentation rate (ESR) of 120 mm/hr, and a C-reactive protein level of 225.4 mg/L. The…
Featured Image from Europe & Central Asia Rheumatic Diseases of Childhood: Scleroderma These images depict a patient with a clinical presentation of juvenile dermatomyositis with typical skin manifestations of Gottron’s papules over the extensor surfaces of the joints, as well as hyperpigmentation. Yulia Vyzhga, MD, PhD, is a pediatric rheumatologist at National Pirogov Memorial Medical…
Rheumatic Diseases of Childhood: Juvenile Dermatomyositis with Calcinosis Cutis These images depict a 14-year-old boy with a two-year history of proximal muscle weakness affecting both upper and lower limbs, and a skin rash affecting his face. He was diagnosed with juvenile dermatomyositis and developed calcinosis over both legs with skin infection and ulceration. Plain X-ray…