Vasculitides

INTRODUCTION: Rapidly progressive glomerulonephritis (RPGN) is characterized by a rapid loss of kidney function, affecting both renal and overall patient survival. Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a small vessel vasculitis affecting multiple organ systems including the kidney, and among most frequent causes of RPGN. We here aimed to validate a recently described scoring system for short-term treatment response to therapeutic plasma exchange (PLEX) in a well-characterized and independent cohort of severe renal AAV presenting with RPGN...

The term occluding vasculopathies covers a large number of different conditions. These often manifest as skin ulcers. Occluding vasculopathies should be considered in the differential diagnosis of leg ulcers. The term "occlusive vasculopathies" encompasses pathophysiologically related entities that share structural or thrombotic obliteration of small cutaneous vessels. In this article, we will focus on livedoid vasculopathy with and without antiphospholipid syndrome and calciphylaxis with differentiation from hypertonic leg ulcer as the most relevant differential diagnoses of leg ulcer...

T1-weighted black blood FS sequences may provide a useful addition to imaging protocols in detection of subtle changes in venous vasculitides and, therefore, may have an impact on treatment options.

Systemic vasculitides are the most complex and problematic autoimmune rheumatic diseases characterized by affections of large, medium, or small vessels. Although the immunopathogenesis of vasculitides is thoroughly studied, the epidemiology and etiology are poorly explored. The main triggers of vasculitides are environmental, genetic, and various infectious factors. Diagnosis of vasculitides is complicated due to the non-specific nature of their symptoms. Vasculitides affect various organ systems with abrupt or slow (weeks-months) development of symptoms...

OBJECTIVE: Immunoglobulin A vasculitis (IgAV) is the most prevalent primary childhood vasculitis in Sweden, but is considerably rarer in adults. This study aims to describe the epidemiology, clinical characteristics and renal outcome of adult-onset IgAV in Skåne, Sweden. METHODS: The study area consisted of Skåne, the southernmost region of Sweden, with a population ≥18 years of 990 464 on 31 December 2010. Adult patients assigned the International Classification of Diseases-10 code for IgAV (D69...

OBJECTIVE: Inflammation markers, e.g. C- reactive protein (CRP) and sedimentation rate, can be normal despite active vasculitis. Von Willebrand factor (vWF) is secreted from endothelial cells in response to vascular damage. Some reports suggest increased vWF levels in vasculitis. This study aimed to evaluate vWF serum concentration in vasculitis patients as a possible biomarker of disease activity and to review the current literature. METHOD: Adult patients with systemic vasculitis were prospectively enrolled...

BACKGROUND: Patient-reported outcomes (PROs) play a crucial role in assessing rheumatic diseases, offering insights into disease evaluation and treatment efficacy. This study focuses on PRO assessment in large vessel vasculitides, including Takayasu Arteritis and Giant Cell Arteritis (GCA). METHODS: We retrospectively analyzed routine data from patients treated at our rheumatology clinic over a 10-year span. Patient and physician-rated global disease activity scale (G-DAS) scores, measured on a numeric rating scale (0-10 points), were collected at each visit...

A category of very uncommon systemic inflammatory blood vessel illnesses known as vasculitides. The pathogenesis and etiology of vasculitis are still poorly known. Despite all of the progress made in understanding the genetics and causes behind vasculitis, there is still more to learn. Epigenetic dysregulation is a significant contributor to immune-mediated illnesses, and epigenetic aberrancies in vasculitis are becoming more widely acknowledged. Less than 2 % of the genome contains protein-encoding DNA...

Vasculitides that occur in association with underlying primary diseases are called secondary vasculitides. In the diverse differential diagnostics of vasculitides, a large variety of secondary vasculitides have to be considered. Secondary vasculitides cover the full spectrum of vasculitides, presenting in manifold clinical manifestations. This article provides an overview of systemic diseases and etiological factors, such as infections, drugs, and malignancies, which can be associated with vasculitides...

In addition to venous and lymphatic diseases, there is increasing scientific evidence that inflammatory dermatoses of the legs are also indications for compression therapy. Specifically, diseases such as pyoderma gangrenosum, livedoid vasculopathy, cutaneous vasculitides, necrobiosis lipoidica, psoriasis, or erysipelas are conditions for which adjunctive compression therapy may be used when manifestations occur on the lower extremities. When inflammatory dermatoses are accompanied by edema, compression therapy is not an off-label use...

Involvement of the complement system is key to the pathogenesis of antineutrophil cytoplasmic antibody (ANCA)-associated renal vasculitis, but immunometabolic implications, especially on serum uric acid (UA) levels, still need to be elucidated. A total of 34 patients with biopsy-proven ANCA-associated renal vasculitis between 2015 and 2020 were retrospectively enrolled. Serum UA levels were correlated with clinical and histopathological characteristics, separated for critically ill (CI, n = 19), myeloperoxidase (MPO)-ANCA ( n = 21) and proteinase 3 (PR3)-ANCA ( n = 13) subgroups...

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is an autoimmune-mediated inflammation of small and medium-sized vessels that can affect virtually any organ system and bears the risk of irreversible organ damage. Without treatment the mortality rates are high, which necessitates rapid diagnosis and initiation of treatment. Histological confirmation, which is not feasible in all cases, should be strived for, especially to delineate differential diagnoses and vasculitis mimics. The new American College of Rheumatology (ACR)/European Alliance of Associations for Rheumatology (EULAR) classification criteria are primarily designed for study purposes and show limitations in the routine application...

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) may affect the eye or orbit, and ophthalmic manifestations of AAV are associated with higher mortality than other inflammatory eye diseases. Perinuclear ANCA (p-ANCA) vasculitis is an uncommon cause of orbital inflammation. A 70-year-old woman with chronic kidney disease presented with a 1-year history of orbital mass and edema around her OD. Fundoscopy revealed 360° optic disc elevation OD. MRI orbits showed an infiltrative, intra- and extraconal lesion extending through the right orbital apex to the cavernous sinus...

In rheumatology, this year has been characterized by a broader knowledge of the pathogenesis of rheumatoid arthritis and mechanisms involved in the onset and persistence of low back pain. Studies relevant to the management of of gout, axial spondyloarthritis, autoinflammatory diseases and systemic vasculitides were published. New data on the safety of JAK inhibitors have been published. The ASAS-EULAR recommendations for the treatment of axial spondyloarthritis were updated, and the 2023 EULAR/PReS guidelines for the diagnosis and treatment of systemic juvenile idiopathic arthritis and adult-onset Still's disease are now available...

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides are a group of immune-mediated diseases characterized by inflammation of small vessels, leading to endothelial injury with subsequent tissue damage. Current guidelines recommend induction therapy with rituximab over cyclophosphamide for severe disease activity. In this case series-based review, the authors discuss 3 cases of granulomatosis with polyangiitis (GPA) with proteinase-3 (PR3) disease that deteriorated following induction therapy with rituximab combined with mycophenolate mofetil and high-dose steroids...

OBJECTIVES: Anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitides (AAV) are life-threatening systemic autoimmune diseases manifesting in the kidneys as necrotizing crescentic glomerulonephritis (NCGN). ANCA antigens are myeloperoxidase (MPO) or proteinase 3. Current treatments include steroids, cytotoxic drugs and B cell-depleting antibodies. The use of chimeric antigen receptor (CAR) T cells in autoimmune diseases is a promising new therapeutic approach. We tested the hypothesis that CAR T cells targeting CD19 deplete B cells, including MPO-ANCA-producing B cells, thereby protecting from ANCA-induced NCGN...

The aim of this study is to present the current care situation of patients with giant cell arteritis (GCA), Takayasu arteritis (TAK), ANCA-associated vasculitis (AAV) and Behçet's disease (BD). Trends over the last 15 years will reflect improvements and remaining deficits in the management of vasculitides. Consecutive cross-sectional data from patients with vasculitides from the German National Database (NDB) of the Collaborative Arthritis Centres between 2007 and 2021 were included. Medication, physician- and patient-reported outcomes on disease activity and disease burden, inpatient stays and occupational participation are compared for different vasculitis entities and over time...

Antineutrophil cytoplasmic autoantibody associated vasculitides has 3 different types: Granulomatosis with polyangiitis, eosinophilic granulomatosis and polyangiitis and microscopic polyangiitis. These vasculitides manifest differently based on which area of small and medium size vessels in our bodies that it affects. In this case report, we discuss a unique case of microscopic polyangiitis diagnosed in a 75-year-old male who was relatively asymptomatic i.e. indolent, apart from nodules present in the lung with the use of the diagnostic criteria outlined by 2022 American College of Rheumatology and European Alliance of Associations for Rheumatology...

INTRODUCTION: Behcet's syndrome is a rare, chronic, systemic condition often categorized within the group of vasculitides. It presents a diagnostic challenge due to its varied clinical manifestations and the absence of a definitive laboratory test. Its etiology remains unclear but may involve genetic, infectious, and environmental factors. CASE PRESENTATION: We report the case of a 16-year-old male who presented with deep vein thrombosis, followed by recurrent episodes of breakthrough thrombosis, despite adequate anticoagulant therapy...

Recent years have brought progress in understanding the role of the neutrophil, dispelling the dogma of homogeneous cells mainly involved in the prime defence against pathogens, shedding light on their pathogenic role in inflammatory diseases and on the importance of antineutrophil-cytoplasmic antibodies' pathogenic role in ANCA-associated vasculitides vasculitis (AAV). Myeloperoxidase (MPO) and proteinase 3 (PR3) expressed in neutrophil granulocytes are the most common targets for ANCAs and contribute to the formation of MPO-ANCAs and PR3-ANCAs which, released to the bloodstream, become an excellent diagnostic tool for AAV...