Disease Activity In Vasculitis Research Articles

Von Willebrand factor antigen compared with other factors in vasculitic syndromes.

In order to analyse their role as a specific marker of vascular damage and their value in monitoring disease activity the plasma concentrations of von Willebrand factor antigen (vWFAg) and the ristocetin cofactor (RiCoF) activities were determined in 43 children with vasculitis and 20 controls. These patients were sub-divided into three groups according to diagnosis: Henoch-Schönlein purpura (n = 18), polyarteritis nodosa (n = 16), and systemic lupus erythematosus (n = 9). High concentrations of vWFAg and activities of RiCoF were found in all the patient groups. vWFAg and RiCoF returned to normal as the patients became symptom free and remained above normal in those with continuing symptoms. The amount of vWFAg did not correlate with the acute phase reactants. vWFAg acted as a specific marker of vascular damage and was useful for the monitoring of disease activity both in small vessel vasculitis and systemic necrotising arteritis.

Elevation of Cerebrospinal Fluid Interleukin-6 Activity in Patients with Vasculitides and Central Nervous System Involvement

The pathogenesis of central nervous system (CNS) involvement in vasculitides remains unclear. We evaluated cerebrospinal fluid (CSF) interleukin-6 (IL-6) activity in relation to the CNS disease activity in vasculitides. Three patients with vasculitides of different categories who showed CNS manifestations were studied, including polyarteritis nodosa, temporal arteritis, and Behcet's disease. All three patients showed marked elevation of CSF IL-6 activity in parallel with the CNS disease activity. In one of the three patients, cerebral vasculitis was demonstrated histologically. All these patients also showed elevation of serum IL-6 activity in parallel with systemic symptoms, such as fever and/or elevation of C-reactive protein and erythrocyte sedimentation rate. These results strongly suggest that elevation of CSF IL-6 activity may underly the common pathogenetic mechanism of CNS involvement of vasculitides irrespective of their category. Taken together with the histopathological findings in one patient, the data also suggest that inflammation might not be restricted within the CNS blood vessels, but rather be extended to brain parenchyma to promote IL-6 production presumably by glial cells.

Correlation of disease activity in systemic vasculitis with serum C-reactive protein measurement. A prospective study of thirty-eight patients.

In a prospective study over 2 years, serum C-reactive protein (CRP) concentration and erythrocyte sedimentation rate were measured serially in thirty-eight patients with various types of necrotizing systemic vasculitis. The CRP concentration was always elevated in patients with active vasculitis and fell rapidly in association with clinical remission induced by immunosuppression. During periods of complete remission, in the absence of any intercurrent condition, the value remained within the normal range. In contrast the sedimentation rate responded more slowly to changes in disease activity and did not necessarily reflect the level of inflammation at a particular time. These results, together with the commercial availability of rapid and precise assays for CRP, indicate that serial measurement of the serum CRP fills the urgent need for an objective index of the activity of the systemic vasculitides and their response to therapy.