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PubMed1993

[The von Willebrand factor antigen in systemic vasculitis].

ELISA was used to investigate concentrations of Willebrand's factor antigen (WF III Ag) in 131 patients with various forms of systemic vasculitis (SV) and 93 patients with cardiovascular diseases. Compared to donors, a mean WF III Ag level proved significantly higher in all nosological variants of SV except periarteritis nodosa. Skin livedo vasculitis did not produce noticeable deviations in WF III Ag levels versus those in donors, whereas atherosclerosis obliterans showed these levels to be significantly elevated. Essential hypertension patients had low values of WF III Ag. The highest mean level of the antigen was found in rheumatoid vasculitis and Wegener's granulomatosis in which the antigen content rose to the highest for vascular affections levels (p < 0.05-0.01). High WF III Ag in Wegener's granulomatosis, periarteritis nodosa, leukocytoclastic vasculitis were indicative of renal involvement. Moreover, there was a tendency to an increase in the antigen quantities in Wegener's granulomatosis patients with intercurrent infections versus such without them. In other SV forms and atherosclerosis obliterans concentrations of the antigen were not related to clinical features of the disease.

Adult↗
PubMed1993

Systemic vasculitis positive for circulating anti-neutrophil cytoplasmic antibodies and with predominantly neurological presentation.

A 57-year-old male patient suffering from dramatically deteriorating diffuse and focal central nervous system symptoms was admitted to hospital after a short prodromal period in a somnolent state. He was diagnosed as having systemic vasculitis positive for circulating anti-neutrophil cytoplasmic antibodies, primarily involving the brain, but also most other organ systems. Circulating anti-neutrophil cytoplasmic antibodies are highly specific for Wegener granulomatosis, though they have been detected in rare cases of other vasculitic syndromes. Central nervous system lesions as presenting signs in Wegener granulomatosis have to be regarded as rare. This case nonetheless suggests that Wegener granulomatosis has to be considered in patients with a predominantly cerebral manifestation of a vasculitic syndrome.

Antibody Specificity↗
PubMed1989

[Systemic vasculitis as a cause of fever of unknown origin].

A prospective clinical study (1974-1988) was carried out in 33 patients with several types of systemic vasculitis (SV) presenting as fever of unknown origin (FUO) according to the 1961 Petersdorf and Beeson's criteria. Histological confirmation, either from biopsy or necropsy, was available in all cases. The types of SV with FUO were: panarteritis nodosa (PAN) (14 cases), giant cell arteritis (GCA) (13 cases), and overlapping polyangiitic syndrome (OPS) (6 cases). In PAN, the clinical features associated with fever at the onset of the disease were remarkably nonspecific: constitutional symptoms (85%), arthromyalgia (50%), nonspecific abdominal pain (28%), and irritative cough (28%). In the whole course of the cases of GCA a significantly smaller frequency of presentation of local arterial symptoms and polymyalgia rheumatica (p less than 0.01) was found in the subgroup of patients with FUO than in those without it. As regard laboratory data, a higher increase of serum alkaline phosphatase (p less than 0.05) was found in the cases of PAN and OPS with FUO. Striated muscle biopsy and arteriography were the most useful investigations in the diagnosis of PAN. The study that gave the diagnosis in the cases of GCA was temporal artery biopsy.

Female↗
PubMed1998

Diagnostic value of standardized assays for anti-neutrophil cytoplasmic antibodies in idiopathic systemic vasculitis. EC/BCR Project for ANCA Assay Standardization.

Anti-neutrophil cytoplasmic antibodies (ANCA) are widely used as diagnostic markers for Wegener's granulomatosis (WG), microscopic polyangiitis (MPA), Churg-Strauss syndrome (CSS) and idiopathic rapidly progressive glomerulonephritis (iRPGN). The objective of this study was to evaluate the diagnostic value of ANCA measurement by the indirect immunofluorescence (IIF) test, and by anti-PR3 and anti-MPO ELISA performed in different locations, in patients with idiopathic small vessel vasculitis. Fourteen centers participated in a standardization study of ANCA assays, and entered a total number of 169 newly diagnosed and 189 historical patients with idiopathic systemic vasculitis or iRPGN. Patients were classified according to a pre-defined diagnostic classification system. Results were compared with those of 184 disease controls and 740 healthy controls. The IIF test was performed according to standard methodology; ELISAs had been standardized among the participants in a previous phase of the study. The sensitivities of assays in patients were as follows. The sensitivity in WG was: cANCA 64%, pANCA 21%, anti-PR3 66%, anti-MPO 24%. In MPA the sensitivity was: cANCA 23%, pANCA 58%, anti-PR3 26%, anti-MPO 58%. Sensitivity in iRPGN was: cANCA 36%, pANCA 45%, anti-PR3 50%, anti-MPO 64%. The specificity of assays (related to disease controls) was: cANCA 95%, pANCA 81%, anti-PR3 87%, anti-MPO 91%. When the results of the IIF test were combined with those of the ELISAs (cANCA/anti-PR3 positive, pANCA/anti-MPO positive), the diagnostic specificity increased to 99%. The sensitivity of the combination of cANCA + anti-PR3 or pANCA + anti-MPO for WG, MPA or iRPGN was 73%, 67% and 82%, respectively. From this study we conclude that the value of the IIF test for ANCA detection can be greatly increased by the addition of a well standardized antigen-specific ELISA. In a significant number of patients with idiopathic small vessel vasculitis, however, the ANCA test results (either in IIF or ELISA) are negative.

Adolescent↗
PubMed1991

The role of antineutrophil cytoplasm antibodies, anticardiolipin antibodies, von Willebrand factor antigen, and fibronectin for the diagnosis of systemic vasculitis.

Autoantibodies directed against cytoplasmic components of neutrophil granulocytes and monocytes (c-ANCA) are a disease specific marker for Wegner's granulomatosis (WG). Autoantibodies against cardiolipin (aCl) are specific for a subgroup of autoimmune disorders, which can also be associated with systemic vasculitis. Fibronectin (Fn) and von Willebrand factor antigen (vWfAg) are produced by blood vessel endothelial cells in response to injury. We tested sera of 61 patients with various types of systemic vasculitides, sera of 13 patients with retinal vasculitis, and sera of 199 patients with rheumatic diseases for c-ANCA, aCl, Fn, and vWfAg. c-ANCA was positive in 14/17 patients with WG, and 2/4 with polyarteritis nodosa (PAN). No serum from healthy donors or patients with other vasculitic or rheumatic diseases was positive for c-ANCA. Moreover, we found aCl, Fn, and vWfAg significantly elevated in almost all patients with vasculitic syndromes. Therefore, we consider c-ANCA a marker, specific for the diagnosis of WG or PAN, whereas aCl, Fn, and vWfAg are nonspecific but sensitive markers of vascular damage.

Adult↗
PubMed2002

[Central nervous system vasculitis and of the peripheral nerves in the elderly].

Vasculitis of the nervous system are rare in the elderly. When present, they may constitute an urgent diagnosis and a therapeutic emergency. Clinical expression is rich and without specificity. Atypical signs (unusual course of dementia, systemic signs) or atypical laboratory results (inflammatory syndrome) may suggest the diagnosis of vasculitis. However, as multiple comorbidity is the rule in elderly subjects, searching for intercurrent factors (e.g. atrial fibrilation due to infectious disease causing embolic stroke) may be more contributive than searching for proof of a rare disease (vasculitis) with invasive procedures in this population. Giant cell (temporal) arteritis is the only vasculitis specifically related with age; the vital prognosis of vision may be compromised. Corticosterid therapy must be instituted without delay. Periartritis nodosa begins in 30% of cases after 60 years of age. The clinical features are the same as in younger subjects. Other vasculidis are rare in the elderly. In absence of specific studies in this population, therapeutic protocols are the same as in younger subjects but may have to be adjusted.

Aged↗
PubMed2005

Mycophenolate mofetil in anti-neutrophil cytoplasm antibodies-associated systemic vasculitis.

BACKGROUND: Mycophenolate mofetil (MMF) is an immune suppressive initially introduced for the prevention of solid organ allograft rejection that is increasingly used in autoimmune conditions, including vasculitis. METHODS: This retrospective study evaluated the efficacy and tolerability of MMF in 51 sequential patients with anti-neutrophil cytoplasm antibodies-associated systemic vasculitis (AASV) treated in a single centre between 2001 and 2004. RESULTS: The mean age was 54 years and median disease duration was 36 months. A mean of 3.5 systems were involved and the previous median exposure to cyclophosphamide was 9 g. MMF was administered either as remission maintenance therapy (29/51, 56.9%) or as treatment for active disease (22/51, 43.1%). The mean duration of MMF therapy was 20 months and the mean MMF dose during the first year was 1.6 g/day. 14/29 (48.3%) of those receiving MMF for remission maintenance therapy eventually relapsed with a mean time to relapse of 14 months. Of those receiving MMF for relapsing disease, 3 failed to respond to therapy while the rest achieved remission by 3.9 months. However, 9 of these subsequently flared; mean time to disease flare was also 14 months. MMF was withdrawn in 28 patients (54.9%) because of treatment inefficacy in 21, severe adverse events in 5 and intolerance in 2. Of the 51 treated, 36 (70.6%) experienced at least 1 side effect, namely infections in 24, gastrointestinal side effects in 12 and psychological events in 6 patients. CONCLUSIONS: We have observed varying efficacy of MMF in AASV, with over 50% of patients with relapsing disease achieving remission and marked falls in concomitant steroid doses. However, longer follow-up indicates a subsequent relapse rate of over 50% that may be associated with low MMF dosing.

Antibodies, Antineutrophil Cytoplasmic↗
PubMed1995

Histopathologic specificity of systemic vasculitis.

A definitive diagnosis of vasculitis almost always requires histologic documentation of a true inflammatory disease of the blood vessels. Although each major type of systemic vasculitis may have its own characteristic and unique features, variability and overlaps still exist, and histopathologic specificity is rarely an absolute discriminator. The correct interpretation of biopsy specimens for the diagnosis of vasculitis remains more an art than a science; it is subject to such variables as the examining pathologist's interest and expertise, tissue selection and sample size, the age of the disease from onset to the time of biopsy, and whether there was drug treatment prior to the biopsy.

Churg-Strauss Syndrome↗
PubMedDate not supplied

Obliterative fibrotic middle ear disease in systemic vasculitis.

A case of bilateral oliterative fibrotic middle ear disease occurring in a patient suffering from leukocytoclastic angiitis is presented. Following a brief discussion of middle ear involvement in systemic vasculitis the pathologic picture and differential diagnosis are considered. Biopsy of unexplained obliterative fibrotic middle ear tissue is recommended.

Diagnosis, Differential↗
PubMed1988

[Sternocostoclavicular hyperostosis. Association with pustular psoriasis and systemic vasculitis].

A female patient with sternocostoclavicular hyperostosis has been followed for 14 years. The case merits special attention because of its association with psoriasis and with an episode of systemic vasculitis. Although patients with sternocostoclavicular hyperostosis usually lack the tissue type antigen HLA-B27, there are grounds for suspicion that the disease is related to the group of seronegative spondylo-arthritides.

Female↗
PubMed1990

[Megadosage of 6-methylprednisolone in systemic vasculitis with renal involvement. A prospective study].

A prospective, open and uncontrolled study of effectiveness and side effects of intravenous 6-methylprednisolone megadoses was carried out in 20 patients with systemic vasculitis with rapidly progressive renal failure. The results showed a favorable response of renal function in 80% of patients and a control of pulmonary hemorrhage in the patients in which it was present. Oliguria and the need for hemodialysis during the acute phase of the disease significantly reduced the response to treatment. Extracapillary proliferation in more than 80% of glomeruli resulted in a lesser degree of recovery of renal function. Severe side effects developed in 2 patients (10%), but they could be exclusively attributed to the 6-methylprednisolone megadose in 1 (5%). It was concluded that 6-methylprednisolone megadoses may be an effective therapeutic alternative for the treatment of these patients.

Biopsy↗
PubMed1984

Monocyte and macrophage Fc-receptor function in systemic vasculitis with renal involvement.

The Fc-receptor function of macrophages and monocytes was studied in 10 healthy subjects and 10 patients (5 in phase of clinical activity) affected by systemic vasculitis with histological evidence of renal involvement. Macrophage function was detected by measuring the clearance in vivo of IgG-sensitized 51Cr-labelled autologous red blood cells (RBC). In vitro immune phagocytosis of monocytes was analysed as a kinetic phenomenon by incubating IgG-coated RBC with nearly pure suspensions of peripheral monocytes. All 5 patients studied in phase of clinical activity showed a defective Fc-receptor function in both the assays, as compared with normal subjects. Since a good correlation was found between the in vitro and the in vivo methods, the use of this non-invasive in vitro procedure is proposed as a substitute for the radioactive in vivo techniques in monitoring the course of the above disease.

Antigen-Antibody Complex↗
PubMed1993

ANCA anti-idiotype antibodies and the treatment of systemic vasculitis with intravenous immunoglobulin.

The presence of anti-idiotype antibodies (anti-id) to anti-neutrophil cytoplasm autoantibodies (ANCA) in intravenous immunoglobulin (IVIg) and remission sera from patients with systemic vasculitis, and the use of IVIg as an alternative therapeutic agent in open studies, has suggested a role for idiotypic regulation in the normal control of these disorders. Clinical benefit with IVIg has been reported in 15/16 patients, with sustained remission in eight. The ability of IVIg to produce lasting remission has been associated with a fall in ANCA levels and stimulation of endogenous immunoglobulin production. IVIg has the potential to influence the pathogenetic process in patients with vasculitis at several stages, and an influence on the idiotypic regulation of ANCA may explain the observed clinical responses and point to possible targets for more specific immunotherapy in the future.

Animals↗
PubMed1997

Agranulocytosis, arthritis and systemic vasculitis in a patient receiving the oral iron chelator L1 (deferiprone).

A thalassaemic girl presented with agranulocytosis, arthritis of both ankles and clinical and laboratory features consistent with the diagnosis of systemic vasculitis, during oral iron chelator L1 (deferiprone) treatment. Changes in the humoral and cell-mediated immune function. including antinuclear antibodies (ANA), anti-DNA and extractable-nuclear antigens (ENA) antibodies positivity, increased immunoglobulin values, decreased T suppressor and the presence of circulating immune complexes, suggest a cause-and-effect relationship with the observed clinical manifestations. A careful monitoring of the immune function is recommended in patients who are receiving the oral iron chelator L1.

Adult↗
PubMed1993

Further characterization of an animal model of systemic vasculitis.

The demonstration of vasculitis and anti-myeloperoxidase antibodies in the mercuric chloride treated Brown Norway rat provides a useful, though limited, animal model of systemic vasculitis. We describe some preliminary experiments on the effect of transfer of serum from mercuric chloride treated rats and of two forms of immunotherapy: intravenous immunoglobulin and an anti-CD4 antibody. Transfer of serum did not lead to tissue injury and neither of the two forms of therapy proved beneficial in this model.

Animals↗
PubMedDate not supplied

[A rare systemic vasculitis with asthmatic syndrome].

The Authors describe the case of a multisystem disease affecting a young asthmatic patient. The history and the course of the disease suggested the diagnosis of a primary systemic vasculitis, which had a pathologic picture (both bioptic and autoptic) typical of an allergic angiitis and granulomatosis, also known as Churg-Strauss syndrome.

Adult↗
PubMed2001

Anti-neutrophil cytoplasmic antibodies (ANCA) from patients with systemic vasculitis recognize restricted epitopes of proteinase 3 involving the catalytic site.

ANCA with specificity for proteinase 3 (PR3), a neutrophil primary granule enzyme, are of diagnostic value in Wegener's granulomatosis (WG) and certain other forms of systemic vasculitis. There is evidence to suggest that they play a pathogenic role in disease, and that the interaction of ANCA with PR3 is likely to be important. We showed, using a resonant mirror biosensor, that C-ANCA from different patients recognized the same or closely related epitopes on PR3. Studies using linear peptides in the SPOT system confirmed the highly restricted nature of this interaction and identified five linear epitopes. Fluid-phase inhibition studies, using a different set of peptides, validated the sequences involved. Using a computer-generated model of the structure of PR3, four of five epitopes were shown to be intimately linked with the catalytic site. The restricted number of epitopes, and their location at the catalytic site, has important implications for the role of C-ANCA in the pathogenesis of vasculitis.

Amino Acid Sequence↗