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| Vasculitis |
| This page was written by Shelley Ensz, and has not yet been medically edited. See Disclaimer. |
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| What is Vasculitis? |
| Vasculitis is a general term for a group of diseases that involve inflammation in blood vessels. Blood vessels of all sizes may be affected, from the largest vessel in the body (the aorta) to the smallest blood vessels in the skin (capillaries). The size of blood vessel affected varies according to the specific type of vasculitis. Johns Hopkins Vasculitis Center. |
| Allergic Vasculitis is also known as Allergic Hypersensitivity or Cutaneous Leukocytoclastic Vasculitis. Medline Plus. |
| NIH Launches Clinical Studies Nationwide to Investigate Rare Diseases. The National Institutes of Health (NIH) announced today it is launching the first clinical studies of its Rare Diseases Clinical Research Network (RDCRN). More than 20 studies are expected to open in the next few months at about 50 sites across the United States and in several other countries including the United Kingdom, Japan, and Brazil. National Institutes of Health News. 05/05/06. (Also see: Antiphospholipid Syndrome) |
| Symptoms of Vasculitis |
| Renal transplantation in systemic vasculitis: when is it safe? There are no clear guidelines on renal transplantation in patients with antineutrophil cytoplasmic antibody (ANCA)-positive vasculitis. Circulating ANCA at transplant was associated with the development of vascular lesions in the graft but was not significantly correlated with graft survival. Most grafts were lost due to patient death, which was more likely if transplantation occurred less than 12 months following induction of remission of ANCA-positive vasculitis. M. Little. NDT. July 13, 2009. |
| Primary central nervous system vasculitis (PCNSV): comparison of patients with and without cerebral amyloid angiopathy (CAA). PCNSV with CAA appears to form a clinical subset of PCNSV. The vasculitis influences the clinical findings to a greater degree than the presence of amyloid deposits in the vessels. C. Salvarini. Rheumatology. August 27, 2008. |
| Ankle-brachial pressure index (ABPI): a simple tool for assessing cardiovascular risk in patients with systemic vasculitis. There is an increased prevalence of an abnormal ABPI in patients with systemic vasculitides implying an increased risk of cardiovascular disease. S. R. Sangle. Rheumatology Advance Access. May 22, 2008. |
| Glaucomatous Type Abnormalities in Patients with Systemic Sclerosis. Glaucomatous type abnormalities were more frequent in patients with systemic sclerosis (SSc) compared to the control group. Such abnormalities may be attributed to the vasculopathy that is observed in patients with SSc, affecting the vessels of the optic disc. G. Kitsos. FRI0347 EULAR 2006. (Also see: Normal Tension Glaucoma) |
| Therapy insight: The recognition and treatment of retinal manifestations of systemic vasculitis. Treatment of these retinal manifestations is usually the treatment of the systemic disease, specific treatment is occasionally indicated to preserve vision. PubMed. Nat Clin Pract Rheumatol. 2006 Aug;2(8):443-51. (Also see: Eye Involvement) |
| Renal manifestations of systemic autoimmune disease: diagnosis and therapy. Renal involvement is relatively common in certain systemic autoimmune diseases, but can be clinically silent. Scleroderma renal crises are managed by blood pressure control using angiotensin-converting enzyme inhibitors and other agents as required. PubMed. Nephrol Ther. 2006 Jul;2(3):140-151. (Also see: Renal Involvement and Lupus) |
| Vasculitis and gastrointestinal involvement. Gastrointestinal involvement is present in up to 50% of the various forms of systemic vasculitis. PubMed. Minerva Gastroenterol Dietol. 2006 Jun;52(2):195-214. |
| Central nervous system vasculitis. Vasculitis of the central nervous system (CNS) is classified as primary angiitis or as vasculitis secondary to a variety of diseases. The prognosis is greatly improved with early recognition and therapy. PubMed. Curr Rheumatol Rep 2003 Apr;5(2):116-27. |
| Localized versus systemic vasculitis: diagnosis and management. Localized vasculitis restricted to a specific anatomic site or organ is often histologically indistinguishable from more severe, systemic forms of vasculitis. By definition, localized vasculitis involves blood vessels within a confined vascular distribution or single organ without clinical evidence of generalized inflammation. PubMed. Curr Rheumatol Rep 2003 Apr;5(2):93-9. |
| Ocular vasculitis. Systemic vasculitis can affect practically any structure in or around the eye. In addition to preserving sight, awareness of specific ocular syndromes may afford a critical clue to undiagnosed or newly active systemic vasculitis. PubMed. Curr Rheumatol Rep 2003 Apr;5(2):100-6. (Also see: Eye Involvement) |
| Vasculitis of the nervous system. When unrecognized and therefore untreated, vasculitis of the nervous system leads to pervasive injury and disability making this a disorder of paramount importance to all clinicians. Successful treatment follows ascertainment of the specific vasculitic disorder and the underlying cytochemical mechanism of pathogenesis. PubMed. Curr Opin Neurol. 2004 Jun;17(3):317-36. |
| Photos of Vasculitis |
| Johns Hopkins Vasculitis Center Includes photos of vasculitis rashes, which can vary from small red or purple dots (usually on the legs), to bruise-like patches, to weeping sores. Johns Hopkins. |
| Causes of Vasculitis |
| Vasculitis: mechanisms involved and clinical manifestations. There is evidence, albeit limited, suggesting that ET-1 plays a role in some clinical manifestations of vasculitis. Potentially, endothelin receptor antagonism may have a place in the treatment of specific vascular manifestations of vasculitis as an adjunct to standard therapy with corticosteroids and immunosuppressant agents. Arthritis Research & Therapy 2007, 9(Suppl 2):S9. |
| Exposure to silica and risk of antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis. Long-term silica exposure may be one of the exogenous factors contributing to ANCA production, however, silica exposure alone, without typical silicosis, was not associated with ANCA positivity. PubMed. Am J Ind Med. 2006 May 11. (Also see: Antibodies, and Causes of Scleroderma: Silica) |
| Rare autoimmune rheumatic illnesses during pregnancy: systemic sclerosis, polymyositis/dermatomyositis and vasculitis. Autoimmune rheumatic diseases (ARD) affect young females durrng childbearing age. Over the last decades, improvements in survival as well as quality of life in patients affected with ARD have led to an increased number of pregnancies observed during the course of such diseases. PubMed. Z Rheumatol. 2006 May 3. (Also see: Pregnancy and Scleroderma and Dermatomyositis) |
| Diagnosis of Vasculitis |
| Are classification criteria for vasculitis useful in clinical practice? Observations and lessons from Colombia. Current vasculitis classification schemes are designed for classification, rather that diagnosis of disease and do not adequately address some common forms of inflammatory vascular diseases, including those of infectious etiology and unusual etiology seen in clinical practice. P. Patarroyo. Journal of Autoimmune Diseases. February 27, 2009. |
| Diagnosis and therapy of vasculitic neuropathy. Systemic vasculitis has an incidence of 4/100,000 per year and, untreated, has a poor prognosis, which is greatly improved by the use of immunosuppressive treatment. The prognosis of non-systemic vasculitic neuropathies is generally better, although many patients need long term immunosuppression. PubMed. Fortschr Neurol Psychiatr 2003 Apr;71(4):172-86. |
| Role of imaging in vasculitis and connective tissue diseases. Magnetic resonance imaging is the investigation of choice to detect and monitor brain disease, while computerized tomography is the best procedure for lung disease. However, since most imaging findings are not entirely specific for any given condition, it is important to interpret the results of imaging in the broader clinical context. N. Pipitone MD, PhD. (Science Direct) Best Practice & Research Clinical Rheumatology Vol 22, Issue 6, December 2008, Pages 1075-1091. (Also see: Connective Tissue Disease and Medical Tests) |
| Cutaneous Vasculitis A Case for Laparotomy? In view of the importance of gastrointestinal involvement to therapy decisions, laparotomy may be necessary to establish the presence of vasculitis. J Rheumatol NO. 7 JULY 2003;30:1621-3. |
| The morphological diagnosis of vasculitis is a challenge for the pathologist for many reasons. A number of immunopathogenic mechanisms prevailing in vasculitis can be verified with immunohistochemical and molecular biological methods, although it is frequently not known how they are initiated and perpetuated. PubMed. Verh Dtsch Ges Pathol 2002;86:83-90. |
| Vasculitis and Rheumatic Diseases |
| Prevalence, predictors and outcome of vascular damage in systemic lupus erythematosus (SLE). Vascular events (VE) occurred in 26% of SLE patients, predominantly as atherothrombotic disease. VE prevalence increased linearly over time leading to a four-fold risk of mortality. (SageJournals) A. Becker-Merok. Lupus. Vol. 18, No. 6, 508-515. April 2009. (Also see: Lupus) |
| Vasculitis and Scleroderma |
| Disturbed angiogenesis in systemic sclerosis(SSc) high levels of soluble endoglin(sENG). The aim of this study was to investigate, in a cross-sectional study, sENG levels together with other serum vascular markers. This study shows increased values of sENG in a large SSc cohort and a relevant association with a vascular phenotype. J. Wipff. Rheumatology Advance Access. May 13, 2008 (Also see: Antibodies) |
| Systemic Vasculitis In Progressive Systemic Sclerosis: The Frequency, Severity And Stages Of Vasculitis In Various Organs - A Retrospective Clinicopathologic Study Of 11 Autopsy Patients. The high prevalence (incidence) of vasculitis and vascular changes in the lungs, kidneys, spleen, pancreas, and heart should warn to carefully evaluate the clinical and laboratory evidence pointing to involvement of these organs in SSc. The frequently involved gastrointestinal tract may be a good target for biopsy to confirm the presence of systemic vascular changes (and consecutive submucosal fibrosis) in SSc patients. M. Bély. AB0496 EULAR 2007. |
| Urticarial vasculitis appearing in the progression of systemic sclerosis (SSc). We demonstrate that, in the present case, mast cells might be involved in both courses of urticarial vasculitis and SSc as a common factor. PubMed. J Dermatol. 2006 Nov;33(11):792-7. (Also see: Skin Fibrosis, Causes of Scleroderma: Molecular Defect, and Limited Scleroderma) |
| Scleroderma associated with ANCA-associated vasculitis (AAV). When scleroderma patients exhibit atypical features such as normotensive renal failure with signs of active inflammation, the possibility of AAV should always be considered. PubMed. Rheumatol Int. 2005 Jul 13;:1-4. (Also see: Renal Involvement) |
| Silica exposure and systemic vasculitis. A 63-year-old male who worked in Department of Energy facilities for 30 years was exposed to silica, beryllium, various solvents and heavy metals, depleted uranium, and ionizing radiation. The patient's diagnoses included microscopic polyangiitis, systemic necrotizing vasculitis, leukocytoclastic vasculitis, and glomerulonephritis. PubMed. Environ Health Perspect. 2003 Dec;111(16):1933-8 (Also see: What is Scleroderma? and Causes of Scleroderma: Environmental) |
| Treatments for Vasculitis |
| Biologic therapy in primary systemic vasculitis of the young. Our data provide retrospective evidence of efficacy of biologic therapy, and highlight the associated infectious complications. D. Eleftherion. Rheumatology Advance Access. June 17 2009. |
| Advances in the use of biologic agents for the
treatment of systemic vasculitis. Due to the well
known toxicities of cyclophosphamide, substantial interest exists in finding
other therapies to treat primary systemic vasculitis. Biologic agents
represent the next evolution in treatment for the primary systemic
vasculitides. S. Chung. Current Opinion
in Rheumatology. January 2009. |
| Induction of remission in active anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) with mycophenolate mofetil (MMF) in patients who cannot be treated with cyclophosphamide. MMF (Cellcept), in combination with prednisolone, can induce remission in patients with relapses of AAV intolerant to cyclophosphamide. Annals of the Rheumatic Diseases 2007;66:798-802. |
| B Cell Targeted Therapies in Autoimmune Diseases. Although these studies contain small cohorts of patients, they demonstrate that B cell-modulating therapies show promise in treatment of a variety of autoimmune diseases. J Rheumatol 2006 May;33 Suppl 77: 24-28. (Also see: Causes of Scleroderma: B Cells and T Cells, Lupus, and Dermatomyositis) |
| Nine patients with anti-neutrophil cytoplasmic antibody-positive vasculitis successfully treated with rituximab. RIT is used for treatment of B-cell lymphomas, but has also shown beneficial effects in autoimmune diseases. RIT seems promising and safe in ANCA-positive vasculitis, and controlled studies should be conducted. PubMed. J Intern Med. 2005 Jun;257(6):540-8. |
| Immune-mediated neuropathies: etiology and pathogenic relationship to aging processes. Accumulating evidence is linking the aging process of the immune system, immunosenescence, to the susceptibility of older individuals for paraproteinemic, vasculitic and inflammatory demyelinating neuropathies. PubMed. J Neuroimmunol 2003 Apr;137(1-2):1-11. |
| Stem cell transplantation in primary systemic vasculitis. This concept has been used mainly in patients with scleroderma, systemic lupus erythematosus, rheumatoid arthritis, and multiple sclerosis. Patients with primary systemic vasculitis (PSV) seem to be suitable candidates for high-dose chemotherapy (HDCT). PubMed. Med Klin 2003 Jan;98(1):13-8. |
| Personal Stories about Vasculitis |
