Postgraduate medicine
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Postgraduate medicine · Jan 2023
ReviewPolyarteritis nodosa: an evolving primary systemic vasculitis.
Polyarteritis nodosa (PAN) is a primary form of vasculitis characterized by inflammation of primarily medium-sized arteries. Several key events have shaped the current spectrum of the disease including the separation of a subgroup with microscopic polyangiitis, the discovery of the association of hepatitis B, and the discovery of adenosine deaminase 2 deficiency (DADA2). With the discovery of secondary causes of PAN and changing nomenclature, the incidence of PAN has declined over time. ⋯ DADA2 is a genetic cause of medium vessel vasculitis that is important to distinguish from primary PAN as treatment with TNF inhibitors can prevent morbidity and mortality in those with a vasculitis phenotype. Treatment of systemic primary PAN involves the use of systemic immunosuppressive therapy largely guided by the severity of disease. With current treatment regimens, the prognosis has changed from a once uniformly fatal disease to a 5-year survival rate above 80%.
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Anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) compromise a rare group of necrotizing small to medium vessel vasculitides that constitute three distinct disorders: granulomatosis with polyangiitis (GPA) (formerly known as Wegener's granulomatosis), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA) (formerly known as Churg-Strauss syndrome). AAV is characterized by the usual presence of circulating autoantibodies to the neutrophil proteins leukocyte proteinase 3 (PR3-ANCA) or myeloperoxidase (MPO-ANCA). These antibodies can activate neutrophils and the complement system resulting in vessel wall inflammation and damage. ⋯ In the past two decades, advances in understanding the pathophysiology of AAV have led to development of new treatments and resulted in significant improvement in general outcomes and survival rates. This narrative review will focus on GPA and MPA. We will highlight clinical manifestations, diagnosis, disease monitoring, and treatment strategies in patients with AAV.
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Giant cell arteritis (GCA, also known as temporal arteritis) is classified as a vasculitis of large and medium sized vessels and can involve the cranial vessels as well as the aorta and great vessels. It is a systemic rheumatic disease that virtually never occurs in adults younger than 50 years of age. GCA is the most common idiopathic systemic vasculitis. ⋯ Glucocorticoids have been the longstanding treatment option for GCA but relatively recent studies have proven additional agents like Tocilizumab are effective steroid sparing agents. GCA is a disease that is of variable duration and length of treatment differs from patient to patient. This article will review the epidemiology, pathogenesis, clinical manifestations, work up and treatment options for GCA.
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Postgraduate medicine · Jan 2023
Value of estimated glucose disposal rate to detect prevalent left ventricular hypertrophy: implications from a general population.
Insulin resistance plays a pivotal role in developing left ventricular hypertrophy (LVH). Researchers have identified the estimated glucose disposal rate (eGDR) as a simple and cost-effective surrogate of insulin resistance. Our work aims to investigate the association between eGDR and the prevalent LVH and explore the incremental value of eGDR to detect prevalent LVH. ⋯ Our analysis revealed a linear, robust association between eGDR and prevalent LVH and demonstrated the incremental value of eGDR to optimize the detection of prevalent LVH.