J. Charles Jennette, MD, School of Medicine, University of North Carolina at Chapel Hill, Chapel Hill, NC
The importance of accurate and rapid recognition of the diseases covered in this Short Course is evidenced by worsened outcomes when diagnosis is imprecise or delayed. In some instances, this results from misinterpretation of the nature of the injury (e.g. diagnosing a thrombotic microangiopathy as a vasculitis) or the misuse of a diagnostic term (e.g. correctly observing necrotizing arteritis but incorrectly diagnosing polyarteritis nodosa rather than microscopic polyangiitis), either of which can result in inappropriate therapy. The 2012 Chapel Hill Consensus Conference Nomenclature System for Vasculitis will be reviewed and explained. In addition, the numerous advances that have occurred in recent years in understanding the pathogenesis and/or treatment of many vascular diseases will be reviewed.
Pathologic processes that will be discussed include infectious vasculitides, giant cell arteritis, Takayasu arteritis, polyarteritis nodosa, Kawasaki disease, pauci-immune small vessel vasculitis (ANCA disease), microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA, Wegener's granulomatosis), eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss syndrome), IgA vasculitis (Henoch-Schonlein purpura), cryoglobulinemic vasculitis, hypocomplementemic urticarial vasculitis (anti-C1q vasculitis), primary CNS vasculitis, thrombotic microangiopathies (hemolytic uremic syndrome, thrombotic thrombocytopenic purpura, eclampsia/preeclampsia, and anti-phospholipid antibody syndrome), disseminated intravascular coagulation, thromboangiitis obliterans, arteriosclerosis obliterans, fibromuscular dysplasia and mediolytic arteriopathies.
The target audience includes general pathologists, residents, fellows and subspecialist pathologists focusing on organ systems that often are affected by, or are the sites biopsied or resected in patients with vasculitides and vasculopathies, such as pulmonary pathologists, dermatopathologists, GI pathologists, nephropathologists, etc.
Upon completion of this educational activity, participants should be able to: 1) Recognize the acute, subacute and chronic manifestations of vasculitides, vasculopathies and coagulopathies in biopsy, surgical and autopsy specimens; 2) Formulate and accurately resolve the differential diagnosis of a vasculitis, vasculopathy or coagulopathy observed in a tissue specimen by integrating histopathologic observations with appropriate special pathologic studies, laboratory data and clinical findings; 3) Explain the 2012 Chapel Hill Consensus Conference Nomenclature for Vasculitis; 4) Understand the pathogenetic mechanisms underlying specific forms of vasculitis, vasculopathy and coagulopathy; and 5) Understand the outcomes and appropriate treatment of the diseases covered.
Materials that will be provided for advance study by registrants include clinical case vignettes with clinical summaries, virtual slides, and digital images. A handout containing the PowerPoint presentation used during the short course will be provided to participants at the meeting, and the PowerPoint show will be posted on the USCAP Website to registrants after the meeting.