SummaryActivation of coagulation and endothelial cell damage was studied in 47 patients with small vessel vasculitis [Wegener’s granulomatosis (WG) and microscopic polyangiitis (MP)] by measurement of throm-bin-antithrombin III complexes (TAT), fibrin-D-dimers (D-dimers), von Willebrand-factor (vWF) concentration and plasma thrombomodulin (TM) levels. There was a close correlation between disease activity (DA) in patients with WG or MP and markers of endothelial cell damage (correlation TM/DA r = 0.46 for WG and r = 0.43 for MP) and activated coagulation (correlation TAT/DA r = 0.58 for WG and r = 0.55 for MP).Elevation of the markers of activated haemostasis and endothelial cell damage was reversed when remission was obtained by specific treatment. The markers studied were particularly helpful in cases where measurement of antineutrophil cytoplasmatic antibodies (ANCA) did fail to assess disease activity.
MRI and CSF investigations revealed meningeal involvement in a 29-year-old patient with biopsy-confirmed Wegener's granulomatosis. The intracranial manifestation of Wegener's granulomatosis was supported by the detection of pathologic circulating antineutrophil cytoplasm (c-ANCA) in the CSF. We monitored disease activity by c-ANCA measurement in the CSF. After repeated cycles of intrathecal administration of methotrexate and corticoids, progression of meningeal infiltration stopped, and CSF c-ANCA titers became negative.
Over a period of 68 months we observed 33 patients with biopsy-confirmed severe crescentic glomerulonephritis (GN) and another 5 patients who fulfilled the clinical criteria of rapidly progressive glomerulonephritis (RPGN; no biopsy confirmation) in a region comprising approximately 930,000 inhabitants. Additional 7 patients with Wegener’s granulomatosis (WG)/microscopic polyarteritis (MP) from the same region were not seen by nephrologists. The calculated annual incidence of crescentic GN/RPGN is 0.7/100,000. Of the 38 patients 13 had classical WG, 7 MP, 3 systemic lupus erythematosus, 5 Schönlein-Henoch purpura, 3 Goodpasture’s syndrome, 2 IgA glomerulonephritis. Of note is the high prevalence of WG/MP and the relative frequency of Schönlein-Henoch purpura. At the last follow-up, 3 patients were dead (8%), 7 patients were on dialysis (18%), 7 patients had elevated serum creatinine (18%) and 21 patients had normal serum creatinine (55%). We conclude that: (i) RPGN is more frequent than reported; (ii) WG and MP account for more than 50% of cases of RPGN; (iii) renal functional prognosis is good in WG/MP, but less favorable in RPGN of other causes; (iv) severe hypertension is not a feature of RPGN; (v) WG/MP, and not Goodpasture’s syndrome, is the most common cause of pulmonary hemorrhage in association with RPGN; (vi) death from infection or malignoma is uncommon (not observed in this series); (vii) de novo IgA GN may occur in patients in remission from WG (2 observations).
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.