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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

Use and Reporting of Outcome Measures in Randomized Trials for Anti–Neutrophil Cytoplasmic Antibody–Associated Vasculitis (AAV): A Systematic Literature Review of Randomized Trials. Outcome measures used in AAV trials commonly included vasculitis–specific tools for disease assessment, but with heterogeneity in endpoint–definitions and timing of assessments. Seminars in Arthritis and Rheumatism, 09/29/2020.

The Longitudinal Course of Fatigue in Antineutrophil Cytoplasmic Antibody-associated Vasculitis (AAV). This study longitudinally measured fatigue levels in patients with AAV. PubMed, J Rheumatol, 2020 Apr;47(4):572-579.

Risk factors associated with IgA vasculitis with nephritis (VN) (Henoch-Schönlein purpura nephritis) progressing to unfavorable outcomes: A meta-analysis. This meta-analysis showed that older age at onset, lower GFR, initial renal features and renal biopsy with crescentic nephritis were predictive of poor prognosis in children with IgA-VN. PubMed, PLoS One, 2019 Oct 1;14(10):e0223218.

Prognostic Value of Microscopic Hematuria after Induction of Remission in Antineutrophil Cytoplasmic Antibodies–Associated Vasculitis. Hematuria at remission after a first pauci–immune glomerulonephritis flare was not associated with end–stage renal disease but with the occurrence of renal relapse within 44 months of remission. PubMed, Am J Nephrol, 2019 May 22;49(6):479-486.

Early predictors of one–year mortality in patients over 65 presenting with ANCA–associated renal vasculitis: a retrospective, multicentre study. A reduction in immunosuppression, the early detection of infections, and co–trimoxazole prophylaxis might help to reduce mortality in this population. PubMed, BMC Nephrol, 2018 Nov 9;19(1):317.

Mortality and causes of death across the systemic connective tissue diseases (CTDs) and the primary systemic vasculitides (PSVs). We observed premature deaths across the spectrum of CTDs and PSVs, with highest standard mortality ratios in diffuse scleroderma and anti–synthetase syndrome. PubMed, Rheumatology (Oxford), 10/03/2018. (Also see Connective Tissue Disease)

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.

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

ANCA vasculitis and IgA nephropathy linked to silica exposure. To our knowledge, this is the first report of both ANCA vasculitis and IgA nephropathy in the setting of silica exposure. PubMed, Occup Med (Lond), 2020 Sep 9;70(6):445-448. (Also see Diseases Caused by Silica Exposure)

(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.

Outcome Measures in Large-Vessel Vasculitis: Relationship Between Patient, Physician, Imaging, and Laboratory-Based Assessments. Measures of disease assessment in large-vessel vasculitis consist of independent outcomes, supporting the need to develop a standard set of measures covering multiple types of outcomes. PubMed, Arthritis Care Res (Hoboken), 11/30/2019.

Association between histopathologic changes and expression of selected microRNAs in skin of adult patients with IgA vasculitis. Further investigation of miRNAs expression in the affected tissues could improve knowledge of IgAV pathogenesis and possibly help to identify novel biomarkers in body fluids. PubMed, Histopathology, 05/28/2019.

Vasculitis and Rheumatic Diseases

Serum neopterin and ischemia modified albumin (IMA) levels are associated with the disease activity of adult immunoglobulin A vasculitis (Henoch-Schönlein purpura). Roles of high-sensitivity C-reactive protein, neopterin and IMA as potential markers of diagnosis and disease activity seem to be worth studying in future studies with larger study groups. PubMed, Int J Rheum Dis, 08/14/2019.

Prognostic nutritional index is associated with disease severity and relapse in ANCA–associated vasculitis. Prognostic nutritional index at diagnosis might be useful for assessing disease severity and predicting the prognosis of AAV patients. PubMed, Int J Rheum Dis, 02/06/2019.

Smoking Is a Risk Factor for Relapse of Antimyeloperoxidase Antibodies–Associated Vasculitis. Smoking is a significant and dose–dependent risk factor for relapse of microscopic polyangiitis (MPA) and patients with MPA who smoke should be encouraged to quit. PubMed, J Clin Rheumatol, 2018 Oct;24(7):361-367.

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)

Treatment of Antineutrophil Cytoplasmic Antibody-Associated Vasculitis (AAV): Is There Still a Role for Cyclophosphamide (CYC)? With the emergence of newer agents with more favorable safety profiles, CYC is no longer the cornerstone of management of AAV. PubMed, Rheum Dis Clin North Am, 2019 Aug;45(3):379-398. (Also see Immunosuppressants)

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. PubMed, Nephrol Dial Transplant, 2019 Jan 1;34(1):63-73.

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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