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Vasculitis

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Overview
Symptoms and Complications of Vasculitis
Photos of Vasculitis
Causes of Vasculitis
Diagnosis of Vasculitis
Vasculitis and Rheumatic Diseases
Vasculitis and Scleroderma
Treatments for Vasculitis
Personal Stories about Vasculitis

Overview: What is Vasculitis?

Vasculitis KaleidoscopeVasculitis refers to diseases that affect blood vessels. There are many different types and causes of vasculitis, however most forms of it are treated with glucocorticoids (steroids).

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.

Symptoms and Complications of Vasculitis

The Utility of Urinalysis in Determining the Risk of Renal Relapse in ANCA–Associated Vasculitis. In patients with ANCA–associated vasculitis and kidney involvement who achieve remission after induction therapy, the presence of persistent hematuria, but not proteinuria, is a significant predictor of future renal relapse. PubMed, Clin J Am Soc Nephrol, 01/25/2018.

Emphysematous change with scleroderma–associated interstitial lung disease (SSc–ILD): the potential contribution of vasculopathy? Peripheral vasculopathy may help to destroy the fibrously thickened alveolar walls, resulting in emphysematous change in SSc–ILD. PubMed, BMC Pulm Med, 2018 Jan 30;18(1):25.

Targeted proteomics reveals promising biomarkers of disease activity and organ involvement in antineutrophil cytoplasmic antibody–associated vasculitis (AAV). Promising biomarkers of disease activity, disease severity, and organ involvement in AAV with a targeted proteomics approach using serum samples have been obtained. PubMed, Arthritis Res Ther, 2017 Sep 29;19(1):218.

The Birmingham Vasculitis Activity Score (BVAS) is an independent predictor of cardiovascular events and cardiovascular disease–related mortality in patients with ANCA–associated vasculitis (AAV). Besides the traditional risk factors, BVAS at presentation was an independent predictor of cardiovascular events and cardiovascular diseases–related mortality in patients with AAV. Seminars in Arthritis and Rheumatism, 09/07/2017.

Paediatric anti–neutrophil cytoplasmic antibody (ANCA)–associated vasculitis (AAV): an update on renal management. The aim of this article is to provide a comprehensive review of paediatric AAV, with a focus on renal manifestations, and to highlight the recent advances made in therapeutic management. PubMed, Pediatr Nephrol, 01/07/2017.

Disease Activity, Glucocorticoid (GC) Exposure, and Rituximab Determine Body Composition Changes during Induction Treatment of ANCA–Associated Vasculitis (AAV). Findings suggest that changes in BMI are independently associated with improvements in disease activity as well as GC exposure in AAV and rituximab may also have effects on BMI independent of its impact on disease activity. PubMed, Arthritis Care Res (Hoboken), 10/01/2016. (Also see Biologic Agents)

The clinical spectrum of Medium-sized Vessel Vasculitis. Systemic polyarteritis nodosa, cutaneous arteritis and gastrointestinal vasculitis demonstrate different clinical courses and therefore may be different diseases rather than a spectrum of the same disease. PubMed, Arthritis Care Res (Hoboken), 08/26/2016.

Respiratory manifestations of anca-associated vasculitis (AAV). Pulmonary manifestations of AAV were common, however only a small proportion of patients had had thorough respiratory investigations, suggesting that the pulmonary manifestations of AAV may be under–diagnosed. Wiley Online Library, 04/08/2016.

Pulmonary Vasculitis. Pulmonary vasculitis encompasses inflammation in the pulmonary vasculature with involved vessels varying in caliber from large elastic arteries to capillaries. Rheumatic Disease Clinics of North America, 03/06/2015.

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

(Case Report) A rare association of localized scleroderma (LS) type morphea, vitiligo, autoimmune hypothyroidism, pneumonitis, autoimmune thrombocytopenic purpura and central nervous system vasculitis. It is likely that localized scleroderma has an autoimmune origin and in this case becomes part of multiple autoimmune syndrome (MAS), which consist of the presence of three or more well-defined autoimmune diseases in a single patient. PubMed, BMC Res Notes. (Also see Causes of Morphea, Vitiligo, Hashimoto's Thyroiditis, Idiopathic Thrombocytopenic Purpura and Multiple Autoimmune Syndrome)

Diagnosis of Vasculitis

How is Vasculitis Diagnosed? The most common tests are biopsy, angiography, and blood tests. American College of Rheumatology.

Increased serum soluble vascular endothelial (sVE) cadherin levels in patients with chronic spontaneous urticaria (CSU).The present data showed serum levels of sVE-cadherin are increased in patients with CSU. PubMed, Ann Allergy Asthma Immunol, 2017 Jun;118(6):704-709.

Vasculitis and Rheumatic Diseases

MicroRNA expression in the affected skin of adult patients with IgA vasculitis. A positive association between the severity of purpura and skin miRNA-223-3p expression was found and aberrantly expressed miRNAs could represent a biomarker in adult IgAV. PubMed, Clin Rheumatol, 08/06/2018.

Prevalence of overlap of antineutrophil cytoplasmic antibody associated vasculitis (AAV) with systemic autoimmune diseases: an unrecognized example of poliautoimmunity. The most common AAV phenotype was renal–limited vasculitis, and the most frequent overlap disease was rheumatoid arthritis. PubMed, Clin Rheumatol, 07/14/2018. (Also see Rheumatoid Arthritis in Overlap)

Case Report: Successful treatment of cerebral large vessel vasculitis in systemic lupus erythematosus with intravenous pulse cyclophosphamide. Here, we present a rare case of cerebral large vessel vasculitis treated successfully with a clear visual presentation. PubMed, Lupus. (Also see Immunosuppressants and Symptoms and Complications of Lupus)

Case Report: Mesenteric Vasculitis in a Case of Systemic Lupus Erythematosus. We present a case of mesentric vasculitis with systemic lupus erythematosus who relapsed after high dose steroids but achieved subsequent remission after starting pulse cyclophosphamide therapy. PubMed, J Assoc Physicians India. (Also see Symptoms and Complications of Lupus)

Vasculitis and Scleroderma

Systemic sclerosis and cryoglobulinemia: Our experience with overlapping syndrome of scleroderma (SSc) and severe cryoglobulinemic vasculitis (CV) and review of the literature. The SSc-CV overlapping syndrome described here is characterized by markedly severe vascular manifestations responsible for very poor prognosis. PubMed, Autoimmun Rev. (Also see Vasculitis)

Treatments for Vasculitis

Vasculitis Treatments. Glucocorticoids (prednisone, prednisolone or others), often referred to as "steroids," are an important part of treating most forms of vasculitis. American College of Rheumatology. (Also see Steroid Warnings for Scleroderma)

Long–term follow–up of a combined rituximab and cyclophosphamide regimen in renal anti–neutrophil cytoplasm antibody–associated vasculitis (AAV). This regimen is potentially superior to current standards of care, and controlled studies are warranted to establish the utility of combination drug approaches in the treatment of AAV. Nephrology Dialysis Transplantation, 02/14/2018.

Randomised controlled trial of prolonged treatment in the remission phase of ANCA–associated vasculitis (AAV). Prolonged remission maintenance therapy with azathioprine/prednisolone, beyond 24 months after diagnosis, reduces relapse risk out to 48 months and improves renal survival in AAV. PubMed, Ann Rheum Dis, 05/25/2017.

Vasculitic and autoimmune wounds. A multidisciplinary approach with the involvement of rheumatologists allows investigation for underlying systemic disease and improves clinical outcomes for many of these challenging patients. PubMed, J Vasc Surg Venous Lymphat Disord, 2017 Mar;5(2):280-292. (Also see Treatments for Digital Ulcers)

Clinical Trials in Vasculitis. This article discusses the treatment options of (ANCA)–associated vasculitis in light of evidence from clinical trials. PubMed, Curr Treatm Opt Rheumatol, 2016;2(2):161-177. (Also see Therapeutic Plasma Exchange)

Personal Stories about Vasculitis

Dawn M: Linear/Systemic Scleroderma My family and I were informed by the doctors, that the localized/linear form of scleroderma that I was diagnosed with, would never progress into the potentially fatal, systemic form…

Jody: Fibromyalgia/Difficult Diagnosis In Sudan, I was on IV for heat stroke and food poisoning. And in Thailand, at the end of March, it all came to a crashing halt…

Krista: Scleroderma, Polymyositis, Vasculitis Many of you probably know that I have translated "SCLERO.ORG" into my language, Romanian…

Kym: Diffuse, CREST Scleroderma, Lupus and Fibromyalgia It all started with Raynaud's in my fingers and toes when I was about nineteen years old, in 1979…

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