For the 2024 Image Competition, the ACR sought images representing a diverse range of patients with educational or remarkable manifestations of pediatric rheumatic diseases, including autoimmune, inflammatory, infectious and malignant drivers of pediatric rheumatic disease. Here, we showcase the images selected as Best Overall. Cavitary Lung Nodules with Sinus & Skin Disease in Pediatric ANCA-Associated…
Anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis is a rare autoimmune condition characterized by inflammation of small- and medium-sized vessels. ANCA-associated vasculitis can lead to multisystem organ complications, including life-threatening pulmonary hemorrhage, renal failure and death. We present a case of ANCA-associated vasculitis complicated by complement-mediated thrombotic microangiopathy (c-TMA). Of the rheumatic diseases, systemic lupus erythematosus…
An unusual autoimmune inner ear condition results in hearing loss, but is reversible if treated.
Here, we present a case of inflammatory cerebral amyloid angiopathy, discussing its diverse clinical manifestations, diagnostic challenges in view of recently proposed clinical-radiologic criteria and its management.
Summaries of selected research abstracts on ANCA-associated vasculitis & IgA vasculitis from the International Vasculitis Workshop.
The large vessel vasculitides were a major focus of the 21st International Vasculitis Workshop held in Barcelona in April. The biennial conference brings together specialists from multiple disciplines, including rheumatology, nephrology and immunology, to discuss cutting-edge vasculitis research. This article reviews important points regarding the pathogenesis, diagnosis and management of both giant cell arteritis and…
Granulomatosis with polyangiitis (GPA) is a small vessel vasculitis associated with anti-neutrophil cytoplasmic antibodies (C-ANCA), specifically proteinase 3 (PR3) ANCA. GPA classically presents with multi-organ involvement. The first description of GPA defined a triad of features: 1) systemic necrotizing angiitis, 2) necrotizing inflammation of the respiratory tract and 3) necrotizing glomerulonephritis.1 Few cases of digital…
Dr. Maria Tektonidou discussed the latest recommendations for the treatment of patients with antiphospholipid syndrome (APS), including obstetric and thrombotic subtypes.
Dr. Jens Thiel provided a detailed overview of specific treatments for patients with immunoglobulin A (IgA) vasculitis and IgA nephropathy, highlighting treatments in early clinical trials.
At the end of 2023, the Society for Cardiovascular Pathology (SCVP) published consensus guidelines on the diagnostic approach to temporal artery biopsy.1 Through this publication, SCVP hopes to bring more uniformity to the processing, interpretation and reporting of these specimens, taking into consideration the most up-to-date literature available. These guidelines have obvious impact on clinical…