[Antibodies to proteinase-3 and myeloperoxidase in systemic vasculitis]. / Antitela k proteinaze-3 i mieloperoksidaze pri sistemnykh vaskulitakh.

AIM: To investigate occurrence and diagnostic significance of antibodies to proteinase-3 (aPR-3) and myeloperoxidase (aMPO) in systemic vasculitis (SV). MATERIAL AND METHODS: A total of 98 patients with different forms of SV were examined: nonspecific aortoarteritis (NAA, n = 18), nodular polyarteritis (NP, n = 18), Wegener granulomatosis (WG, n = 20), obliterating thrombangiitis (OT, n = 21), and hemorrhagic vasculitis (HV, n = 21). Eight patients with primary antiphospholipid syndrome (PAPS) and 20 donors comprised a control group. aPR-3 and aMPO were detected by solid-phase enzyme immunoassay using kits ORGenTec Diagnostica GmbH. RESULTS: aPR-3 were detected in 1 (5.6%) patient with NP and in 3 (14.3%) patients with HV. aPR-3 were detected in 13 (65%) of 20 patients with WG being significantly more frequent not only vs controls (0%) but in some forms of SV and PAPS (p < 0.05). Mean aPR-3 level in 13 WG patients was significantly higher than in 4 patients (1 with NP and 3 with HV) the sera of whom also contained aPR-3. 84.6% patients with WG had higher concentrations of aPR-3, this is significantly more frequently than in the comparison group. In NP and HV these autoantibodies were encountered in the serum only in moderate or low concentrations in patients with high clinicolaboratory activity of the disease. In WG patients there was no correlation between aPR-3 presence, form of the disease and basic clinical manifestations, but mean values of index of clinical activity of vasculitis were significantly higher in patients with aPR-3 than in those free of them. Concentration of aPR-3 in an active phase of the disease was significantly higher than in patients in remission. Moreover, aPR-3 were detected in 83.3% cases in active vasculitis and in 37.5% patients without it. Detection of aPR-3 in WG group was associated with mean sensitivity and good specificity. In examination of the patients in an active phase specificity rose but sensitivity fell. Optimal results were obtained in estimation of aPR-3 level. Thus, in moderate or high concentration, aPR-3 have good sensitivity and high specificity for diagnosis of WG, in a high titer (> 15 U/ml) they are highly sensitive and specific for this vasculitis. aMPO were detected in 1 of 18 patients with NP, in 1 of 21--with OT, in 3 of 21--with HV and in 2 of 21--with NAA. None patients with WG or PAPS had aMPO. aMPO were detected in NP and HV in high activity of inflammation. Part of the patients had affected kidneys. CONCLUSION: Thus, WG is characterized by the presence and high concentration of aPR-3. In the latter case aPR-3 have high (100%) sensitivity and specificity for diagnosis of WG. Detection of aPR-3 can be used as an additional laboratory test for diagnosis of WG and estimation of its activity.

[Clinical significance of myeloperoxydase and proteinase 2 antibodies in patients with systemic lupus erythematosus]. / Klinicheskoe znachenie antitel k mieloperoksidaze i proteinaze-3 u bol'nykh sistemnoi krasnoi volchankoi.

Content of antibodies to neutrophil cytoplasma--myeloperoxidase (MPO)--and proteinase-3 (PR-3) was measured in the sera of 65 patients with SLE and 20 donors. Antibodies to MPO (a-MPO) and proteinase-3 (a-PR-3) significantly outnumbered those of the control. The number of a-MPO appeared elevated in 13, lowered in 7, moderate in 6 cases and directly correlated with anemia, pulmonary lesions, a-PR-3 level, inversely correlated with cerebrovasculitis and polyneuritis. The number of a-PR-3 was elevated in 14 cases (10 low titers and 4 moderate titers). High levels of both a-PR-3 and a-MPO were recorded in 8 sera. The content of a-PR-3 correlated directly with age of SLE onset but inversely with leukocyte count. Neither course of the disease nor inflammation activity were related to level of neutrophil antibodies. Factor analysis has identified groups of elements influencing the value of a-MPO and a-PR-3.

[Antibodies to phospholipids and the vascular endothelium in nodular polyarteritis]. / Antitela k fosfolipidam i éndoteliiu sosudov pri uzelkomov poliarteriite.

Clinical significance of antibodies to phospholipids (aPL) and vascular endothelium (aVE) was evaluated in 20 patients (9 women and 11 men aged 36 +/- 10.8 years) with nodular polyarteritis (NP) corresponding to classification criteria of the USA Rheumatology College. Antibodies to cardiolipin (aCL) (IgG and IgM) and to beta 2-glycoprotein (beta 2-GP1) (IgG) were titered by solid-phase enzyme immunoassay. Total serum level of aVE (IgG + IgM + IgA) was measured by solid-phase enzyme immunoassay using Eahy. 926 endothelial hybrydoma cell culture. Anticardiolipin antibodies were detected in 11 (55%) of 20 patients, 3 of these had IgG aCL, 4 IgM aCL, and 4 both antibody isotypes. Serum titers of all aCL were moderate in all cases. No antibodies to beta 2-GP1 were detected in any of the patients. Total serum endothelial activity varied from 0 to 89.7% in patients with NP. Mean aVE level was 24.45 +/- 21.2%, which was significantly higher than in donors (p < 0.001). In 4 (26.7%) of 15 patients with NP total level of aVE surpassed the upper threshold normal value. The presence of aCL directly correlated with the presence of reticular livedo (r = 0.54, p < 0.05), but not with any other clinical laboratory manifestations of the disease, including thrombotic complications (deep thrombosis of lower limb veins, stroke, myocardial infarction), renal involvement, increased erythrocyte sedimentation rate, increased concentrations of von Willebrand factor antigen and C-reactive protein, or angiitis activity. Vascular endothelial antibodies directly correlated with renal involvement (r = 1.00, p < 0.01), distal gangrene of the limb (r = 0.83, p < 0.01), and angiitis activity (r = 0.78, p < 0.001), with high level of von Willebrand factor antigen and increased erythrocyte sedimentation rate (r = 0.66 and r = 0.64, respectively; p < 0.01), but not with aCL (r = 0.43, p > 0.05) of any isotype (aCL IgG r = -0.01; r = 0.34; p < 0.05). All patients with aVE had aCL in the serum (aCL IgG in 1, aCL IgG and IgM in 1, and aCL IgM in 2 patients). The results indicate different significance of a CL and aVE in NP; the mechanisms of realization of their pathogenetic potential are still to be investigated.