Current perspectives on the diagnosis, assessment, and management of vasculitic neuropathy.

INTRODUCTION: Vasculitic neuropathy can present associated with both primary and secondary systemic vasculitis as a result from underlying diseases such as rheumatic diseases and infections, Moreover, confined vasculitis in the peripheral nervous system may be present. Thus, the diagnosis and management of vasculitic neuropathy require multidisciplinary approaches. AREAS COVERED: Current views as well as relevant clinical research on the diagnosis, assessment, and management of vasculitic neuropathy are reviewed to suggest appropriate treatment strategies. We searched PubMed and Google Scholar for reports published between July 2017 and July 2022. EXPERT OPINION: For the treatment of vasculitic neuropathy, determining the causative primary disease is important and often requires diagnosis by tissue biopsy. Due to the scarce research on the treatment of vasculitic neuropathy, treatment is empirically based on findings from studies of systemic vasculitides involving other organs, particularly antineutrophil cytoplasmic antibody-associated vasculitis. In addition to conventional glucocorticoids and immunosuppressive agents, complement-targeted therapy, anti-B-cell therapy, and disease-specific molecular targeted therapies have recently gained relevance. Future research is needed to develop new patient-specific therapeutic options.

INCA (Peru) Study: Impact of Non-Invasive Cardiac Magnetic Resonance Assessment in the Developing World.

Background Advanced cardiac imaging permits optimal targeting of cardiac treatment but needs to be faster, cheaper, and easier for global delivery. We aimed to pilot rapid cardiac magnetic resonance ( CMR ) with contrast in a developing nation, embedding it within clinical care along with training and mentoring. Methods and Results A cross-sectional study of CMR delivery and clinical impact assessment performed 2016-2017 in an upper middle-income country. An International partnership (clinicians in Peru and collaborators from the United Kingdom, United States, Brazil, and Colombia) developed and tested a 15-minute CMR protocol in the United Kingdom, for cardiac volumes, function and scar, and delivered it with reporting combined with training, education and mentoring in 2 centers in the capital city, Lima, Peru, 100 patients referred by local doctors from 6 centers. Management changes related to the CMR were reviewed at 12 months. One-hundred scans were conducted in 98 patients with no complications. Final diagnoses were cardiomyopathy (hypertrophic, 26%; dilated, 22%; ischemic, 15%) and 12 other pathologies including tumors, congenital heart disease, iron overload, amyloidosis, genetic syndromes, vasculitis, thrombi, and valve disease. Scan cost was $150 USD, and the average scan duration was 18±7 minutes. Findings impacted management in 56% of patients, including previously unsuspected diagnoses in 19% and therapeutic management changes in 37%. Conclusions Advanced cardiac diagnostics, here CMR with contrast, is possible using existing infrastructure in the developing world in 18 minutes for $150, resulting in important changes in patient care.

Therapeutic advances in the treatment of vasculitis.

Considerable therapeutic advances for the treatment of vasculitis of the young have been made in the past 10 years, including the development of outcome measures that facilitate clinical trial design. Notably, these include: a recognition that some patients with Kawasaki Disease require corticosteroids as primary treatment combined with IVIG; implementation of rare disease trial design for polyarteritis nodosa to deliver the first randomised controlled trial for children; first clinical trials involving children for anti-neutrophil cytoplasmic antibody (ANCA) vasculitis; and identification of monogenic forms of vasculitis that provide an understanding of pathogenesis, thus facilitating more targeted treatment. Robust randomised controlled trials for Henoch Schönlein Purpura nephritis and Takayasu arteritis are needed; there is also an over-arching need for trials examining new agents that facilitate corticosteroid sparing, of particular importance in the paediatric population since glucocorticoid toxicity is a major concern.

A rheumatology perspective on cutaneous vasculitis: assessment and investigation for the non-rheumatologist.

Vasculitis, by definition, is inflammation of the vasculature. This inflammation can result in either vessel wall destruction causing aneurysm or rupture, or stenosis causing ischaemia or necrosis. This autoimmune response does not always have a clear cause. Vasculitis is a heterogeneous group of disorders that has been categorised not only by primary and secondary causes, but also by the size of the affected vessel. The secondary causes that can trigger vasculitis include infection (particularly hepatitis B and C and haemorrhagic fever);cancer, autoimmune diseases such as rheumatoid arthritis (RA), systemic lupus erythematosus (SLE) and Sjogren's; drugs or allergic reactions. As vasculitis can affect any part of the vasculature, it can result in a wide range of signs and symptoms. However, one of the most common presentations of vasculitis is a rash, due to small vessel vasculitis, which is most common. A vasculitic rash has certain characteristics that are easily identifiable and differentiate it from other rashes. This is a review from a rheumatologist's perspective of how to identify vasculitis skin changes. If cutaneous vasculitis is suspected, this article identifies other areas of skin that can be affected that need identification, in addition to what to screen for in the history and other differential diagnoses to consider. Subsequently, the article addresses the key investigations to request and a brief overview of the treatment principles for primary vasculitis.

What matters for patients with vasculitis?

Advances in clinical care for patients with vasculitis have improved survival rates and created new challenges related to the ongoing management of chronic disease. Lack of curative therapies, burden of disease, treatment-related side effects, and fear of relapse contribute to patient-perceived reduction in quality of life. Patient-held beliefs about disease and priorities may differ substantially from the beliefs of their health care providers, and research paradigms are shifting to reflect more emphasis on understanding vasculitis from the patient's perspective. Efforts are ongoing to develop disease outcome measures in vasculitis that better represent the patient experience. Health care providers who care for patients with vasculitis should be sensitive to the substantial burdens of disease commonly experienced by patients living with the disease and should strive to provide comprehensive care directed towards the medical and biopsychological needs of these patients.

Coronary artery vasculitis: assessment with cardiac multi-detector computed tomography.

Coronary artery vasculitis is rare and comprises an array of inflammatory diseases. It often results in severe and life-threatening complications, including coronary artery aneurysm, coronary artery stenosis, intraluminal thrombosis, and microcirculation abnormalities. These may occur at a young age and are often silent in the early phases. Invasive coronary angiography is the gold standard for diagnosing coronary artery disease (CAD); however, multi-detector computed tomography (MDCT) is now widely regarded as a powerful non-invasive tool for the detection of CAD. It is important for clinicians to recognize the various CT findings associated with coronary artery vasculitis in order to promote accurate diagnosis and proper patient management. The purpose of this article is to present an overview of the conditions associated with coronary artery vasculitis, with an emphasis on etiology and cardiac MDCT diagnosis of CAD. Cardiac MDCT is clinically useful and can provide information for the accurate diagnosis and treatment of coronary vasculitis.

The invisible part of the iceberg: qualitative aspects of childhood vasculitis.

OBJECTIVES: The aim of this study was to develop a multidimensional assessment instrument named 'Juvenile Vasculitis Multidimensional Assessment Report' (J-VAMAR) to measure all the domains of the vasculitis. In this qualitative study, it is primarily aimed to enrich the item generation for the J-VAMAR. METHODS: Twelve children with vasculitis and their mothers (n=12) were enrolled in this study. The data were collected using both a demographic data form and a semi-structured interview form. The study was performed on individual patient face-to face interview. Data were analysed by grounded theory and the N Vivo 9 software program. RESULTS: Four categories were obtained. These categories were (i) physical effects of the illness, (ii) emotional effects of the illness, (iii) social effects of the illness and (iv) experienced challenges related to treatment process. In the physical effect category severe pain, physical limitations, weakness and fatigue; in emotional effect category thought of death, hopelessness and dissatisfaction about body image; in the social effects category decrease in academic performance, absenteeism to school and concealing the sickness from friends were the most common features. In the fourth category, subjects complained of lifelong drug use and frequency of daily drug consumptions. CONCLUSIONS: These results provide evidence-based data for the assessment of children with vasculitis by several domains including physical, emotional and social aspects as well as treatment protocols. The study provides the basis and/or justification for selecting the domains that the developing multidimensional instrument should include.

[Vasculitis. Treatment outcome parameters]. / Vaskulitis. Zielkriterien der Behandlung.

The definition of outcome parameters has improved the care of vasculitis patients dramatically in recent decades. In the first phase of joint European studies, disease stages and activity were defined. In the second and third phases results of the randomized and controlled trials were summarized and published as European recommendations for the care of small and large vessel vasculitis. Irrespective of the type of vasculitis, inducing remission, maintaining remission and preventing disease- and therapy-related complications are the main outcome criteria.

Assessment of systemic vasculitis.

The systemic vasculitides are an uncommon group of autoimmune diseases capable of causing multi organ failure and death. Current immunosuppressive strategies have substantially improved the outcome, but the natural history of treated disease is unstable, typically characterised by frequent relapses, drug toxicity and an increasing burden of damage. Early diagnosis, accurate staging and regular evaluation of disease status are important in the management of the vasculitides. Clinical evaluation tools have been developed and provide a comprehensive assessment of patients. Serological markers, especially anti-neutrophil cytoplasm antibody (ANCA), pathology and imaging investigations are a useful addition, but are more valuable in diagnosis rather than monitoring of disease activity. Advances in magnetic resonance imaging in large vessel vasculitis have improved our ability to characterise disease and may lead to earlier diagnosis and better control in future. Development of new biomarkers is required in vasculitis, and this is likely to advance our understanding as well as the management of these complex conditions.

Outpatient assessment of systemic vasculitis.

The vasculitides are complex, multisystem diseases, the natural history of which has been transformed by the use of immunosuppressive therapy. In many cases, survival is characterized by the presence of ongoing low-grade disease activity and, now that patients can survive acute, life-threatening manifestations of vasculitis, the new challenge is to provide accurate descriptions of these problems so as to plan appropriate changes in therapy to ensure optimal control of disease, with minimal toxicity, damage, and impairment of function. To achieve these aims, it is necessary to undertake structured evaluation of patients, with a significant dependence on careful clinical observation, supported by appropriate laboratory investigation at all stages from diagnosis to long-term care of these patients. This chapter reviews the important aspects of disease assessment and recommends a strategy for managing these complex patients. We can make use of standardized disease assessment instruments, such as the Birmingham Vasculitis Assessment Score and the Vasculitis Damage Index, to serve as an aide memoire in documenting the clinical features of disease. Routine monitoring for drug toxicity in the short-term and long-term is essential. Above all, however, it is very important to review these patients regularly and see them urgently in case of flares or complications, which are a common problem in most forms of vasculitis.

Diagnosis and assessment of systemic vasculitis.

The diagnosis of systemic vasculitis requires clinical evidence with appropriate symptoms and physical signs, supported by histological or radiological confirmation. Earlier recognition of these diseases has been facilitated by a greater awareness of their incidence, and also by the more widespread introduction of the anti-neutrophil cytoplasmic antibody (ANCA) test. Early diagnosis provides a greater potential for effective intervention in the course of disease and this may limit subsequent damage. However, an early diagnosis poses the more difficult challenge in the classification of the vasculitides, since traditional classification systems have depended on the presence of well-established manifestations of the disease. The accurate assessment of disease activity and damage in vasculitis has become necessary as a result of significant improvements in survival with the use of chemotherapy. The disease course however is frequently characterised by relapse as well as the scars of irreversible organ damage from disease and drug toxicity. Clinical methods of assessment are simple to apply, reliable and often more effective than any current laboratory test in evaluating the effects of therapy and determining changes in therapy. The increasing use of surrogate clinical measures of disease should provide a greater opportunity to establish the effectiveness of existing and novel therapies in the management of these complex diseases.

The national database of the German Collaborative Arthritis Centres: I. Structure, aims, and patients.

OBJECTIVE: To describe the aims, principles, and content of the German rheumatological database and to present data on patient mix and healthcare provision for the year 1998. METHODS: The German rheumatological database contains clinical and patient derived data of the outpatients with inflammatory rheumatic diseases seen at one of the 24 collaborative arthritis centres. The case mix, institutional context, and demographic features of 25 653 patients from the year 1998 were analysed. RESULTS: 51% of the patients had rheumatoid arthritis, 23% seronegative spondyloarthropathies, including ankylosing spondylitis, psoriatic arthritis, and reactive arthritis, and 19% had vasculitis, including SLE (5%). The distribution of the age at onset of patients with RA with < or = 2 years' disease duration was comparable with recent incidence data from population studies. The case mix differed between university departments and rheumatology hospitals as well as individual practices. 65% of the male and 46% of the female patients at ages 18-60 were still in gainful employment, the rates of employment were 14% below the population rates for women, and 11% below those for men. 62% of all patients had seen a rheumatologist within the first year of disease, 73% within the first two years. Ankylosing spondylitis was seen in rheumatological care much later than all other diseases (only 39% within the first year). The mean number of contacts with a rheumatologist was five a year; rheumatologists in individual practices saw their patients seven times a year on the average. Together with visits to the non-specialist doctor mainly treating the patient, the mean number of visits to the doctor for a rheumatic condition was 20 a year. CONCLUSION: Large databases like this one give information about the patient case mix in different healthcare settings, about treatment practice, and about the consequences of disease. Patients treated in specialised rheumatology units in Germany are referred earlier than in the past, which probably reflects better regional cooperation due to the implementation of arthritis centres. University departments and outpatient clinics of rheumatology hospitals contribute considerably to the specialised care of patients with arthritis and connective tissue diseases.

The socioeconomic impact of vasculitis.

Information detailing the socioeconomic impact of the vasculitides on society has been difficult to obtain. Recent published studies provide preliminary information suggesting that the impact of systemic vasculitis may be significant and far greater than previously anticipated. A rough estimate of expenditures for vasculitis-related hospitalizations for polyarteritis nodosa, hypersensitivity vasculitis, Wegener's granulomatosis, giant cell arteritis and Takayasu's arteritis in the US amounted to $150 million per year. Costs associated with outpatient care, disability, and death were not calculated. A more comprehensive investigation into the economic and social burden of these conditions is necessary. Aspects to consider include quantifying the incidence and prevalence of disease, estimating the direct, indirect, and intangible costs of care, assessing long-term clinical outcomes, and measuring quality of life from the patient perspective.

Assessment of disease activity in systemic vasculitis.

The systemic vasculitides are a group of inflammatory disorders characterised by relapses and remission. Before the introduction of immunosuppressive drugs, mortality was unacceptably high. Immunosuppressive therapy has had a therapeutic impact, but at the cost of increased risk of infection and other adverse effects. Differentiating infection from active disease can be difficult, and the inappropriate prescription of immunosuppressive drugs can be fatal. Hence disease indices which can aid physicians to identify the active phase of disease and enable early treatment, will be valuable in the management of this group of disorders.

Disease assessment and management of the vasculitides.

The improvement in survival with chemotherapy has resulted in a change of the natural history of the systemic vasculitic syndromes. The vasculitides are now viewed as chronic disease rather than fatal conditions. Their course is frequently characterized by relapse as well as the scars of irreversible organ damage from disease and drug toxicity. Assessment tools are available which can serve as outcome measures in clinical trials as well as a guide to better management of individual patients. Improvements in therapy in future are dependent on a better understanding of the pathogenesis of these conditions and the ability to assess disease accurately.

[Diagnosis and course assessment of collagenoses and vasculitides]. / Diagnostik und Verlaufsbeurteilung von Kollagenosen und Vaskulitiden.

The different forms of vasculitis and connective tissue disorders are multisystem diseases which must be distinguished from a variety of infectious illnesses, neoplastic and degenerative diseases. The diagnosis, established by clinical, radiographic, laboratory and histologic investigation, influences treatment and thereby the outcome of the disease. A diagnostic approach to the patient with possible vasculitis and/or connective tissue disease is described.