Vasculitis in primary connective tissue diseases

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1 Oxford Medicine Online You are looking at 1-10 of 26 items for: MRI rheumatoid MED00240 Vasculitis in primary connective tissue diseases Laura B. Hughes DOI: /med/ Vasculitis occurring in the context of connective tissue diseases (including rheumatoid arthritis, Sjögren s syndrome, systemic sclerosis, systemic lupus erythematosus, polychondritis, seronegative spondyloarthropathies, idiopathic inflammatory myopathies, aortitis, periaortitis, and idiopathic retroperitoneal fibrosis) presents difficult diagnostic challenges due to the variety of vessels and organ systems that can be involved, and the range in degree of severity. Vasculitis in connective tissue diseases is often associated with more severe disease activity and longer disease duration. Fortunately, most are responsive to currently available therapies, especially when the diagnosis is made and appropriate therapy instituted in a timely fashion. The most important factors in successfully making these diagnoses are familiarity with common patterns of vasculitis within a given disease process and vigilance for the identification of vasculitis in the differential diagnosis. Imaging in psoriatic arthritis Dafna D. Gladman, Cheryl F. Rosen, and Vinod Chandran Print Publication Year: 2014 Published Online: Apr 2014 ISBN: eisbn: DOI: /med/ Imaging is important to determine the degree and severity of joint and entheseal inflammation Radiographic evidence of peripheral joint damage includes erosions, joint space narrowing, juxta-articular fluffy periostitis, severe osteolysis, pencil-in-cup change, ankylosis and subluxation. Axial radiographs often reveal sacroiliitis, syndesmophytes (marginal or paramarginal), facet joint fusion, discitis, and occasionally atlanto-axial subluxation Ultrasonography may be used to detect inflammatory and destructive changes in joints, tendons, and entheses, especially at point of care Magnetic resonance imaging is especially useful to demonstrate early peripheral joint damage as well as axial inflammation Page 1 of 5

2 Cutaneous vasculitis, connective tissue diseases, and urticaria Susan Burge and Graham S. Ogg Print Publication Year: 2010 Published Online: Nov 2013 ISBN: eisbn: DOI: /med/ _update_001 Vasculitis (angiitis) denotes necrotizing inflammation of the blood vessels; occlusive vasculopathy implies vascular occlusion without significant vascular inflammation. A small-vessel cutaneous vasculitis is the most common vasculitis affecting the skin, and may be the first sign of a systemic vasculitis, but 50% of patients have no systemic disease. The clinical findings must be integrated with the results of serological, pathological, and imaging studies to reach a diagnosis... Vasculitic manifestations in the gastrointestinal tract Gaafar Ragab DOI: /med/ Early diagnosis and prompt management of vasculitic lesions in the gastrointestinal (GI) tract is essential to avoid its potentially serious sequelae. In order to better understand the manifestations and consequences of vasculitic lesions in the GI tract, and the different approaches to diagnosis, the chapter presents a brief review of the anatomy and physiology of the GI tract. The recent developments in our understanding of the human gut microbiome is also discussed, as this is expected to contribute to our knowledge of the role of the GI tract in autoimmunity. This is followed by an outline of the various pathogenic mechanisms causing bowel ischaemia, the manifestation of vasculitis in the GI tract, and the use of imaging in diagnosis, and a discussion of the wide differential diagnosis-an important topic in the setting of suspected of acute GI vasculitis. Finally, an outline is given of GI involvement in the specific vasculitides. Cross-sectional imaging in vasculitis Enrique A. Sabater and Anthony W. Stanson DOI: /med/ While angiography demonstrates high-resolution images of blood vessel lumens, one of its main limitations is the inability to demonstrate disease occurring in the vessel wall where the primary pathological changes of vasculitis occur. More importantly, mural findings may precede luminal abnormalities and, indeed, luminal changes may never be evident. Thus, the non-invasive, cross-sectional imaging modalities of ultrasonography (US), computed tomography (CT), and magnetic resonance imaging (MRI) have important roles in the diagnosis of vasculitis and in treatment follow-up. All three modalities have seen continued Page 2 of 5

3 advances, allowing faster scanning and higher-resolution images. They also provide highquality images, non-invasively, matching that of angiography for lumens of large and medium-size vessels. Conventional angiography, however, provides better resolution of smaller, peripheral vessels. Ultrasound provides excellent detail to assess luminal and mural changes in peripheral and superficial vessels. Cross-sectional imaging modalities are useful for demonstrating end-organ involvement and complications of vasculitis. In small-vessel vasculitides where vessel changes are beyond the resolution of all imaging modalities, cross-sectional modalities are useful to evaluate tissue damage in organs. Skin and rheumatology Susan Burge, Rubeta Matin, and Dinny Wallis Print Publication Year: 2016 Published Online: Aug 2016 ISBN: eisbn: DOI: /med/ This chapter describes the clinical features, differential diagnosis, investigation, and management of skin problems associated with rheumatic diseases. Topics include rheumatoid arthritis, lupus erythematosus, dermatomyositis, systemic sclerosis, relapsing polychondritis, multicentric reticulohistiocytosis, and heritable collagen disorders. The approach to investigation of an underlying systemic disease is discussed. The relevance of autoantibody profiles in particular diseases is explained. Ocular manifestations of systemic vasculitis Russell W. Read DOI: /med/ The eyeball and surrounding structures are richly vascularized, supplied by branches from the internal and external carotids. A variety of ocular conditions may be caused by, or associated with, systemic vasculitis and there is potential for profound visual loss. Structures that may be involved are: conjunctiva, cornea, sclera and episclera, uveal tract, retina, orbit, and optic nerves. Examination of the ocular manifestations of vasculitis is important from the perspective of both the ophthalmologist who diagnoses an ocular condition, and must consider whether it is a manifestation of a systemic vasculitis, and the non-ophthalmologist who diagnoses a systemic vasculitis, and seeks to determine if the eye is involved. Because the eye is the only location in the human body in which vasculature can be directly visualized in the natural state, the ophthalmologist s contribution can be both vision preserving and a diagnostic aid to the referring physician. This chapter discusses ocular manifestations organized by ocular anatomy, followed by sections on ocular manifestations of the different systemic vasculitides. Page 3 of 5

4 Diagnostic and classification criteria Dafna D. Gladman, Cheryl F. Rosen, and Vinod Chandran Print Publication Year: 2014 Published Online: Apr 2014 ISBN: eisbn: DOI: /med/ Psoriatic arthritis is diagnosed using the diagnostic criteria first postulated by Moll and Wright. The recently developed CASPAR criteria are used to classify patients with psoriatic arthritis CASPAR criteria have high sensitivity and specificity in both early and established psoriatic arthritis Approach to the diagnosis of vasculitis in adult patients Barri J. Fessler DOI: /med/ Diagnosis of vasculitis is challenging due to non-specific symptoms, overlapping syndromes, lack of specific diagnostic tests, and the absence of generally accepted diagnostic criteria. While progress is being made in establishment of classification criteria for the different vasculitic syndromes (as published by the 2012 Chapel Hill Consensus Conference on nomenclature of vasculitides), there remains a strong need for classification and diagnostic criteria to aid in the early diagnosis of vasculitis and facilitate research studies. The initial, essential step in diagnosis is a comprehensive history and physical examination. The next step is selection of appropriate tests to delineate areas of disease involvement, either through tissue biopsy or imaging studies. At the same time, secondary causes of vasculitis and mimics of vasculitis must be excluded. The demonstration of vasculitis on tissue biopsy remains the gold standard for diagnosis. However, this is often not straightforward and diagnosis of a specific form of vasculitis requires familiarity with the features of the different syndromes and narrowing down the differential diagnosis based on histological, laboratory, and imaging findings in the context of the clinical pattern. Granulomatosis with polyangiitis (Wegener s granulomatosis)clinical and immunodiagnostic aspects Susanne Schinke, Wolfgang L. Gross, and Elena Csernok DOI: /med/ Granulomatosis with polyangiitis is a rare, systemic disorder defined as a necrotizing granulomatous inflammation of the respiratory tract and a necrotizing vasculitis affecting small to medium-sized vessels, with necrotizing glomerulonephritis and pulmonary Page 4 of 5

5 capillaritis but also granulomatous and non-granulomatous extravascular inflammation. Clinical suspicion of GPA should be raised by treatment-refractory chronic or destructive ENT complaints, systemic inflammation, granulomatous disease, or surrogate markers of vasculitis (nephritic sediment or purpura). In localized GPA, the respiratory tract only is affected. The combination of clinical, imaging, and histological findings with ANCA (usually canca/pr3, occasionally panca/mpo) leads to the diagnosis of GPA. However, absence of ANCA does not exclude GPA. Differential diagnoses include other primary ANCA- and non-anca-associated vasculitides and vasculitis mimics: infection, malignancy, non-vasculitic granulomatous diseases (e.g. TAP-deficiency), microangiopathies. The chapter presents established tools for evaluation of disease activity and damage (BVAS, DEI, ENTAS, VDI). Despite a high burden of disease, the outcome has significantly improved due to better diagnosis, disease awareness, and optimized immunosuppression. Nevertheless, mortality is increased, particularly within the first year, in young men, intensive care patients, and in renal or visceral organ involvement. Longterm morbidity is determined by relapsing disease leading to chronic damage, therapy sideeffects, and co-morbidities. Page 5 of 5

Atlas of the Vasculitic Syndromes

CHAPTER e40 Atlas of the Vasculitic Syndromes Carol A. Langford Anthony S. Fauci Diagnosis of the vasculitic syndromes is usually based upon characteristic histologic or arteriographic findings in a patient