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Giant cell arteritis with low erythrocyte sedimentation rate: frequency of occurence in a population-based study
To determine the frequency of a low erythrocyte sedimentation rate (ESR) in patients with giant cell arteritis (GCA) and evaluate their clinical features in a defined population
Musculoskeletal manifestations in a population-based cohort of patients with giant cell arteritis
To define musculoskeletal manifestations occurring in a population-based cohort of patients with giant cell (temporal) arteritis (GCA)
Skin manifestations in giant cell arteritis
Giant cell arteritis is a primary systemic vasculitis involving large- and medium-sized vessels which affects almost exclusively patients aged 50 years or older. Cutaneous manifestations are rare because giant cell arteritis spares the small vessels, that is, those vessels that are typically associated with skin lesions. The most common skin abnormality observed in giant cell arteritis is thickening and, less frequently, erythema or nodules of the superficial temporal arteries. Necrosis of the scalp and tongue has also been occasionally described in patients with severe vascular ischemia induced by inflammation. Glossitis and facial edema are other rare lesions that reflect active inflammation
Primary CNS vasculitis: Pathophysiological diversity
Primary central nervous system vasculitis (PCNSV) is a rare vasculitis that affects the brain and occasionally the spinal cord of adults and children. Extensive progress has been achieved in the recognition of clinicopathological subsets that has translated into useful nosology reflective of the natural history and response to immunosuppressant treatment. Affected patients with small-artery vasculitis characterized by positive histology, negative cerebral angiography and prominent leptomeningeal enhancement on MRI after intravenous gadolinium administration have a milder disease course and more favorable outcome compared to those with large-artery PCNSV with multiple large-artery stenoses on cerebral angiography and extensive lesions on MRI. Subsets of patients presenting with rapidly progressive PCNSV, solitary tumor-like mass lesion and vascular amyloid deposits have the worst prognosis
Distal extremity swelling with pitting edema in polymyalgia rheumatica. Report on nineteen cases
To determine the frequency and clinical characteristics of diffuse distal extremity swelling with pitting edema occurring in polymyalgia rheumatica (PMR)
Skin manifestations in microscopic polyangiitis
Microscopic polyangiitis is a pauci-immune necrotizing small- and medium-vessel vasculitis mainly involving the lung and kidneys but also the peripheral nerves and skin. A variety of skin lesions are observed in about half of patients. The most common cutaneous manifestations are palpable purpura, livedo racemosa, and skin nodules. Histology of skin lesions typically shows leukocytoclasia with fibrinoid degeneration, neutrophil infiltration, and sometimes erythrocyte extravasation around the affected capillaries and small vessels in the dermis. Direct immunofluorescence shows absence of immunoglobulin deposits. A detailed clinical history and physical examination complemented by histological studies can aid in discriminating microscopic polyangiitis from other vasculitides affecting the skin
Prominent perivascular enhancement in primary central nervous system vasculitis
Clinical images: prominent perivascular enhancement in primary central nervous system vasculiti
Laboratory investigations useful in giant cell arteritis and Takayasu's arteritis
A raised erythrocyte sedimentation rate (ESR) is considered a hallmark for the diagnosis of giant cell arteritis (GCA). The American College of Rheumatology 1990 criteria for GCA include ESR greater than or equal to 50 mm/h as one of the five criteria. Although the presence of a normal ESR made GCA less likely, the results of a population-based study showed that the occurrence of a low/normal value in GCA at diagnosis is not rare. Pre-treatment ESR may be a prognostic indicator for duration of treatment. C-reactive protein (CRP) and interleukin-6 (IL-6) may be more sensitive indicators of disease activity than ESR in GCA patients. However, it is unclear whether the use in clinical practice of CRP and IL-6 has some apparent advantage over ESR. ESR is the most often used tool to assess disease activity in Takayasu's arteritis (TA). However, some studies have found that ESR and CRP are not able to differentiate patients with clinically active and inactive TA. Furthermore, histopathological studies have shown that over 40% of patients thought to be in clinical remission with normal acute phase reactants have active arteritis. IL-6 could be a promising marker of disease activity in TA; however, further studies are required to confirm its usefulness in clinical practice. Other laboratory investigations could be useful in the diagnosis or follow-up of GCA and TA, but more studies are required
Classification of inflammatory arthritis
Letter to the editor on classification of inflammatory arthriti
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