Most Sunday mornings, I make myself an exceptional cup of pour-over coffee and sit down on my deck with the latest issue of the New England Journal of Medicine. I check out the image of the week. I read the case report with pen in hand, racing to diagnose the patient before the authors spill…
Kidney involvement is a major cause of morbidity and mortality in systemic lupus erythematosus (SLE). Collectively termed lupus nephritis, SLE with kidney involvement comes in many subtypes. The current classification by the International Society of Nephrology/Renal Pathology Society (ISN/RPS), however, does not include lupus podocytopathy, which, through various clinical and epidemiologic studies, has recently been…
SAN DIEGO—At ACR Convergence 2023, Philip Seo, MD, MHS, presented an overview of clinical updates in rheumatology from the past year.
Pneumatosis cystoides intestinalis (PCI) is a rare gastrointestinal pathological process defined by the presence of gas within the layers of the intestinal wall, commonly within the mucosa and submucosa of the small and large intestines.2,3 PCI has been described in the literature in association with various connective tissue diseases, including scleroderma, mixed connective tissue disease,…
Hajdu-Cheney syndrome (HCS) is a rare connective tissue disease, with fewer than 100 cases reported worldwide.1 Hallmark features include acro-osteolysis (i.e., resorption of the distal phalanges of the hands and feet), osteoporosis, facial dysmorphisms, and craniofacial and dental abnormalities. Patients often have short stature and can have neuroanatomical deformities causing intellectual disabilities. These patients can…
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,…
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…
PHILADELPHIA—At ACR Convergence 2022, the much-anticipated ACR Review Course featured talks from eight experts. Topics reflected the heterogeneity of our field and included Sjögren’s disease, spondyloarthritis (SpA), osteoarthritis (OA), paraneoplastic rheumatic syndromes, metabolic bone disease, statin myopathy, Raynaud’s phenomenon and autoinflammatory syndrome. Here, I share highlights from this comprehensive, six-hour session. Sjögren’s Disease Sara S….
Erin Wilfong, MD, PhD, shared her niche expertise in connective tissue disease ILD (CTD-ILD) via case-based learning during a session of the 2022 ACR Education Exchange.