Clinical and biological assessment in systemic necrotizing vasculitides.

The systemic vasculitides are multi-system disorders with significant mortality and morbidity and frequent relapses. Treatment is usually effective but fraught with potentially serious effects. Disease Assessment is important to ensure that patients receive the appropriate treatment. Disease Assessment should comprise measurement of disease activity, chronic irreversible damage and impairment of function. Serological markers can be helpful in assessing disease activity but lack sufficient sensitivity and specificity to be used on their own. Radiological techniques such as Magnetic Resonance Imaging, Ultrasound and Positron Emission Tomography show promise in the large vessel vasculitides but require validation in large studies. Clinical Assessment tools are the current gold standard for the assessment of disease activity, damage and function

The future of damage assessment in vasculitis.

Damage denotes the aspects of chronic disease that do not reverse with therapy. This concept is particularly important for the primary systemic vasculitides, since the careful differentiation between activity and damage may help avoid unnecessary exposure to cytotoxic medications. Damage significantly influences both longterm prognosis and quality of life. Because the primary systemic vasculitides have diverse manifestations, the use of a damage assessment instrument is crucial to ensure reproducibility. The Vasculitis Damage Index (VDI) is the only validated measure for damage assessment in vasculitis. Use of the VDI in recent clinical trials has shown that it may not adequately determine the full spectrum of damage experienced by patients with vasculitis of small- and medium-size vessels. We propose reexamining the way in which damage is assessed, focusing on vasculitides of small- and medium-size vessels, and outline an initiative to create a substantially revised and improved damage assessment instrument using data-driven approaches. This initiative is part of a larger international effort to create a unified approach to disease assessment for the primary systemic vasculitides

Pregnancy in Systemic Vasculitis

Conception, pregnancy and childbirth are a biological necessity, a unique privilege and birthright and yet something that mothers suffering with vasculitis cannot take for granted. Takayasu arteritis and Behcet’s disease mainly, but also ANCA associated vasculitis and IgA vasculitis are of relevance in this population. A diagnosis of systemic vasculitis has meant a lower chance of stable relationships, lower fertility, lower conception rates and worse foetal outcomes. Systemic vasculitis or it’s treatment may affect fertility by direct involvement of the reproductive organs, teratogenicity, induced infertility, or by producing a state inducing an inability of the maternal body to carry a foetus to term. Unfortunately, there is sometimes the need for medical termination. Pregnancy outcomes are poorer in women with a diagnosis of vasculitis, but this may be truer for women with Takayasu arteritis than Behcet’s disease. In spite of the many challenges in looking after expectant mothers with systemic vasculitis, we suggest some basic principles in this chapter to improve maternal and foetal outcomes. This is largely an evidence-free zone and this stream of medicine involving motherhood, babies and life-threatening rare diseases will remain emotive and therefore difficult, calling for the best that clinicians can offer