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E Mirapeix

Publications and source records attributed to E Mirapeix.

At least 19 recordsLinked to original sources
PubMed2005

[IgA nephropathy with crescentic glomerulonephritis and ANCA positive].

We present a case of IgA nephropathy diagnosed by renal biopsy that presents after 2 years of folow-up an impairment of the renal function associate histoligically to a crescentic glomerulonephritis. The immunologic determinations showed of high titers of antineutrophil cytoplamic antibodies (ANCA) (P-ANCA IgG antiMPO and P-ANCA IgA anti-MPO). The patient began treatment with haemodyalisis and one year later she received a cadaveric kidney transplantation with good result. Two years later she had normal urine sediment, negative proteinuria, normal renal function but high titers of IgG and IgA PANCA anti-MPO. In summary, we believe that the determination ANCA in acute renal failure due to IgA nephropathy can indicate the existence of a IgA crescentic glomerulonephritis superimposed or an associated small vessel vasculitis and it confers a worse prognosis. The positive maintenance of IgG P-ANCA anti-MPO IgG anti-MPO titers during the course suggests that the sequential determination of ANCA in this entity is not useful to the monitoring of the clinical activity.

Adolescent↗
PubMed2001

Circulating soluble adhesion molecules in ANCA-associated vasculitis.

BACKGROUND: To evaluate whether changes in concentrations of soluble (s) E-selectin, sP-selectin, sL-selectin, intercellular adhesion molecule 1 (sICAM-1), and vascular cell adhesion molecule 1 (sVCAM-1) reflect disease activity in patients with ANCA-associated vasculitis and whether serum levels of these adhesion molecules are related to the degree of renal failure in patients with chronic renal failure (CRF). SUBJECTS AND METHODS: A sandwich ELISA was used to measure these soluble adhesion molecules in (i) sera from 20 patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (10 patients with Wegener's granulomatosis (WG) and 10 patients with microscopic polyangiitis (MPA)), obtained at the time of diagnosis and during the remission period; (ii) sera from 40 patients with CRF not undergoing haemodialysis. RESULTS: At the time of diagnosis, serum levels of sE-selectin, sICAM-1 and sVCAM-1 (88+/-42 ng/ml, 437+/-184 ng/ml, 1720+/-1174 ng/ml respectively) were significantly higher in patients with ANCA-associated vasculitis than in healthy controls (P<0.0001, P=0.002 and P=0.001 respectively). Serum sP-selectin values did not differ from those obtained in normal donors. In contrast, sL-selectin levels (940+/-349 ng/ml) were significantly lower in patients than those recorded in healthy controls (P<0.0001). A significant decrease in concentrations of sE-selectin, sP-selectin, sICAM-1, and sVCAM-1 was observed between active and remission phases (P<0.0001, P=0.002, P=0.001 and P=0.001 respectively). No significant differences were observed in sL-selectin levels between active and remission phases. sL-selectin concentrations (802+/-306 ng/ml) during the remission phase remained lower than those observed in healthy controls (P<0.0001). No correlation was observed between serum creatinine and sE-selectin, sP-selectin, sICAM-1 and sVCAM-1 in patients of the CRF group. A slight negative correlation was established between creatinine and sL-selectin concentration. CONCLUSIONS: Increased serum levels of sE-selectin, sICAM-1, and sVCAM-1 and decreased levels of sL-selectin in active ANCA-associated vasculitis, and the normalization of sE-selectin, sICAM-1, and sVCAM-1 during the remission phase suggest that the concentration of soluble levels of these adhesion molecules reflects disease activity. The decrease in sP-selectin levels between active and inactive phases also suggest that this receptor may reflect clinical activity. The lack of correlation between serum levels of sE-selectin, sP-selectin, sICAM-1, and sVCAM-1 and the degree of renal failure in patients with CRF suggests that the mechanism of clearance of these molecules is not renal.

Adult↗
PubMed2001

Comparison of anti-PR3 capture and anti-PR3 direct ELISA for detection of antineutrophil cytoplasmic antibodies (ANCA) in long-term clinical follow-up of PR3-ANCA-associated vasculitis patients.

A total of 118 sera from 11 patients with anti-neutrophil cytoplasmic antibodies against proteinase-3- (PR3-ANCA) associated vasculitis were retrospectively screened by anti-PR3 capture and anti-PR3 direct ELISA tests. We studied the relationship between capture and direct ELISA scores and the clinical activity of PR3-ANCA-associated vasculitis patients during follow-up. We also studied the ability of the anti-PR3 capture ELISA to detect positive values of PR3-ANCA in clinical vasculitis relapses. Only capture ELISA presented a significant relationship (p < 0.05) with clinical activity of PR3-ANCA-associated vasculitis patients over time. Capture ELISA appears to be a reliable method for detecting clinical relapses in this group of patients. Our results indicate that the new capture ELISA test is more effective than direct ELISA in the follow-up of patients with PR3-ANCA-associated vasculitis and in the detection of relapses.

Adult↗
PubMed1999

Relationship between ANCA and disease activity in small vessel vasculitis patients with anti-MPO ANCA.

BACKGROUND: We analysed the usefulness of antineutrophil cytoplasmic antibodies (ANCA) as a marker of clinical activity in patients with small vessel vasculitis associated with anti-myeloperoxidase (MPO) ANCA. METHODS: We studied a group of 25 patients, 15 with microscopic polyangitis and 10 with renal limited vasculitis, so-called rapidly progressive glomerulonephritis type III. The clinical and serological follow-up was accomplished quarterly over an average of 2.79 +/- 2.08 years (range 0.25-6 years). ANCA was analysed by indirect immunofluorescence and enzyme-linked immunosorbent assays (ELISAs). RESULTS: At the time of diagnosis, all patients were ANCA positive (P-ANCA and anti-MPO). Following a standardized treatment, all patients except one achieved complete remission of vasculitis in <3 months. One patient suddenly died during the active phase (1 month of follow-up) and with positive ANCA. Seroconversion from positive to negative occurred in 24/25 patients (96%). Eighteen of these 24 patients (75%) achieved the seroconversion within the first 6 months. During the follow-up, two patients had four major relapses, all of them associated with positive ANCA. ANCA seroconversion from negative to positive was observed in one patient with microscopic polyangitis without clinical relapse of vasculitis. CONCLUSION: ANCA should be used in conjunction with other markers of disease activity in the management of microscopic polyangitis and renal limited vasculitis patients with anti-MPO ANCA.

Adult↗
PubMed1998

[Usefulness of anti-neutrophil cytoplasmic antibodies, anti-proteinase 3 and anti-myeloperoxidase in management of small vessel vasculitis].

BACKGROUND: Analysis of usefulness of antineutrophil cytoplasmic antibodies (AN-CA) as a marker of clinical activity in small vessel vasculitis. PATIENTS AND METHODS: 33 patients, 10 patients with Wegener's granulomatosis (WG) and 23 with microscopic polyangiitis (MPA) and rapidly progressive glomerulonephritis type III (RPGN III). The clinic and serologic follow-up was accomplished every 3 months during an average of 19 (SD, 24) months (range 3-52 months. The serologic follow-up included the determination of ANCA by indirect immunofluorescence (IFI) and ELISA, as well as the serum level of C reactive protein (RCP). RESULTS: At the time of diagnosis all patients were ANCA positive by IFI and ELISA. The seroconversion of ANCA from positive to negative was produced in 30/33 patients (90%). Twenty-six out of these 30 patients (87%) achieved the seroconversion within the first 6 months. During the follow-up 4 patients had a major relapse, all with positive ANCA. In 2 patients, one of each group, seroconversion from negative to positive was not associated with clinical relapse of vasculitis. CONCLUSION: ANCA should be used in conjunction with other indices of disease activity in patients with small vessel vasculitis.

Adult↗
PubMed1998

[antineutrophil cytoplasmic autoantibodies in inflammatory bowel disease].

BACKGROUND: The aim of the present study was to determine the prevalence and diagnostic usefulness of antineutrophil cytoplasmic antibodies (ANCA) in a Spanish population of patients with inflammatory bowel disease from the province of Tarragona. PATIENTS AND METHODS: One hundred and fifty-six sera obtained from 116 patients with inflammatory bowel disease (75 ulcerative colitis and 41 Crohn's disease) and 40 healthy controls were tested using an indirect immunofluorescence assay. RESULTS: ANCA were detected in 65% of patients with ulcerative colitis but in only 12% of patients with Crohn's disease (p < 0.01), and 2.5% of control subjects (p < 0.01). The overall sensitivity of the test for the diagnosis of ulcerative colitis was 65% with a specificity of 88% and a positive predictive value of 91%. Among patients with ulcerative colitis there was no relationship between the presence or titre of ANCA and the duration, the clinical course, the extent, the disease activity or the need for medical treatment. CONCLUSIONS: In the population studied, ANCA occur more commonly in ulcerative colitis than in Crohn's disease, as reported in other populations. Their determination in patients with inflammatory bowel disease may be useful to differentiate ulcerative colitis from Crohn's disease.

Adult↗
PubMed1998

Antineutrophil cytoplasmic antibodies in primary Sjögren's syndrome: prevalence and clinical significance.

OBJECTIVE: To evaluate the prevalence of cytoplasmic (c) and perinuclear (p) antineutrophil cytoplasmic antibodies (ANCA) in patients with primary Sjogren's syndrome (SS), and to correlate the presence of ANCA with extraglandular and immunological manifestations related to SS. METHODS: In a cross-sectional study, we included 82 consecutive patients (75 female and seven male; mean age 61 yr; range 33-87 yr) attending our unit. All patients fulfilled four or more of the diagnostic criteria for SS proposed by the European Community Study Group in 1993. Extraglandular manifestations such as arthralgia and/or arthritis, Raynaud's phenomenon, autoimmune thyroiditis, peripheral neuropathy, renal involvement and cutaneous vasculitis were also recorded. Serum samples were examined by indirect immunofluorescence (IIF) and by ELISA using as substrates myeloperoxidase (MPO) and proteinase 3 (PR3). RESULTS: ANCA were detected in nine (11%) patients: seven had pANCA and two an atypical pattern. These two atypical ANCA became cANCA when paraformaldehyde fixation was applied. ELISA findings showed that two patients had antibodies against MPO, and no patient had antibodies to PR3. The most common extraglandular manifestations in the ANCA-positive patients were articular involvement in six (66%) patients, peripheral neuropathy in five (55%), Raynaud's phenomenon in four (44%) and cutaneous vasculitis in four (44%). Of the four patients with cutaneous vasculitis and ANCA, two had a mononuclear inflammatory vascular disease (MIVD) in the biopsy specimen. When compared with patients without ANCA, those with these antibodies had a higher prevalence of cutaneous vasculitis (44% vs 8%, P = 0.01), Raynaud's phenomenon (44% vs 8%, P = 0.01) and peripheral neuropathy (55% vs 7%, P < 0.001). CONCLUSION: ANCA positivity can be found in patients with primary SS and its detection is associated with the presence of clinical manifestations attributable to vascular involvement (cutaneous vasculitis, peripheral neuropathy and Raynaud's phenomenon).

Adult↗
PubMed1997

Relationship between ANCA and clinical activity in inflammatory bowel disease: variation in prevalence of ANCA and evidence of heterogeneity.

Antineutrophil cytoplasmic antibodes (ANCA) are markers of necrotizing vasculitis. ANCA have been recently detected in the two forms of inflammatory bowel disease (IBD), ulcerative colitis (UC) and Crohn's disease (CD). To assess the possible role of ANCA in the diagnosis and management of IBD we studied the prevalence of ANCA at diagnosis and during follow-up in a group of 89 IBD patients. The relationship between ANCA and clinical features of IBD was investigated. ANCA assayed by indirect immunofluorescence were detected in 38/52 (73%) of the UC patients but only 6/37 (16.6%) of the CD patients (P<0.005) and in none of the controls. In the UC group, but not in the CD group, there was a positive correlation between ANCA and disease activity. The sensitivity and specificity of ANCA for the diagnosis of UC were 73 and 83.7% respectively. The most commonly observed pattern of ANCA in IBD patients was perinuclear: in 84% of the UC and 66.6% of the CD patients positive for ANCA, respectively. However, careful comparison of IFL patterns revealed some distinct features of IBD-associated ANCA when compared to vasculitis-associated ANCA. In addition, most ANCA positive sera from IBD patients were negative for antibodies to proteinase 3 and myeloperoxidase by ELISA. These results suggest that the autoantigens recognized by ANCA are different in patients with IBD from those with necrotising vasculitis.

Adolescent↗
PubMed1997

Circulating soluble adhesion molecules in patients with classical polyarteritis nodosa.

The objective was to evaluate whether changes in circulating soluble adhesion molecule levels reflect disease activity in patients with systemic polyarteritis nodosa (PAN). A sandwich ELISA was used to measure soluble (s) intercellular adhesion molecule 1 (sICAM-1), vascular cell adhesion molecule 1 (sVCAM-1), E-selectin, L-selectin and P-selectin in sera and plasma from 22 patients with active PAN, in sera from 13 of these patients taken serially during follow-up, and in sera from 13 healthy controls. At the time of diagnosis, sICAM-1, sVCAM-1 and sE-selectin levels (488.5 +/- 201.3, 1176.5 +/- 514.1 and 60.6 +/- 27 ng/ml, respectively) were significantly higher in patients than in controls (P < 0.0001, P = 0.001 and P = 0.003, respectively). In contrast, sL-selectin levels tended to be lower in patients than in controls. Within the first 7 days after starting treatment, there was a significant increase in sICAM-1 concentrations, which fell thereafter, but did not completely reach normal levels when patients achieved clinical remission. sE-selectin also remained elevated during follow-up. Only sVCAM-1 decreased, tending to reach normal values in inactive disease. Increased levels of sICAM-1, sVCAM-1 and sE-selectin, and decreased levels of sL-selectin, in active PAN suggest immune and endothelial stimulation during disease activity. Abnormal levels of soluble adhesion molecules in clinically inactive patients might reflect persistence of immune activation and/or endothelial cell exposure to a remaining inflammatory microenvironment.

Adult↗
PubMed1996

Development and standardization of solid phase assays for the detection of anti-neutrophil cytoplasmic antibodies (ANCA). A report on the second phase of an international cooperative study on the standardization of ANCA assays.

Anti-neutrophil cytoplasmic antibodies (ANCA) are diagnostic markers for systemic vasculitis. They are classically detected by an indirect immunofluorescence test using normal donor neutrophils as substrate. This assay lacks antigenic specificity and is not quantitative. The 'EC/BCR Project for ANCA Assay Standardization' is an international collaboration study with the aim to develop and standardize solid phase assays for ANCA detection. In this part of the study the isolation and characterization of proteinase-3 and myeloperoxidase, the two main target molecules for ANCA, and the development and standardization of ELISAs with these antigens are described. Six laboratories successfully isolated purified proteinase-3 preparations that could be used. Three of these preparations, together with one myeloperoxidase preparation, were subsequently used for ANCA testing by ELISA. The ELISA technique was standardized in two rounds of testing in the 14 participating laboratories. The coefficient of variation of these new assays decreased from values of approx. 50% in the first round to approx. 20% in the second round. We conclude that purified proteinase-3 and myeloperoxidase can be used in standardized ELISAs for ANCA detection. Whether such procedures offer advantages over the IIF test will be determined in a prospective clinical study.

Antibodies, Antineutrophil Cytoplasmic↗
PubMed1996

Antineutrophil cytoplasmic antibodies in relatives of patients with inflammatory bowel disease.

BACKGROUND: The occurrence of antineutrophil cytoplasmic antibodies (ANCA) has been reported more frequently than expected in healthy first-degree relatives of patients with ulcerative colitis, suggesting that these antibodies may represent a subclinical marker of genetic disease susceptibility. AIM: To determine the prevalence of ANCA in unaffected first-degree relatives of inflammatory bowel disease patients in a Spanish population. METHODS: Three hundred and seventy sera obtained from 80 patients with inflammatory bowel disease (55 ulcerative colitis, 25 Crohn's disease), 217 unaffected first-degree relatives (157 from ulcerative colitis and 60 from Crohn's disease patients), 62 healthy controls, and 11 celiac disease patients were tested using an indirect immunofluorescence assay. RESULTS: Antibodies were detected in 64% of patients with ulcerative colitis but in only 12.5% of patients with Crohn's disease. ANCA were seldom present in their unaffected first-degree relatives (4.6%), control subjects (1.6%), and celiac disease patients (0%). CONCLUSIONS: In the Spanish population studied, antineutrophil cytoplasmic antibodies occur more commonly in ulcerative colitis than in Crohn's disease, as reported in other Caucasian populations. Moreover, their presence is not increased in their first-degree relatives. These findings indicate that ANCA are not a subclinical marker of genetic susceptibility to inflammatory bowel disease in this population.

Adult↗
PubMed1995

Occurrence of antineutrophil cytoplasmic and antineutrophil (peri)nuclear antibodies in rheumatoid arthritis.

OBJECTIVE: To elucidate whether sera from patients with rheumatoid arthritis (RA) contain antineutrophil cytoplasmic antibodies (ANCA) or granulocyte specific antinuclear antibodies (GS ANA), or both, and to analyze possible correlations with different clinical and laboratory data. METHODS: Forty-seven consecutive outpatients with RA were included. Control sera were obtained from patients with well defined rheumatic diseases and from healthy individuals. Serum samples were examined by indirect immunofluorescence (IIF) on both ethanol and paraformaldehyde fixed neutrophils and by ELISA using as substrates myeloperoxidase (MPO), proteinase 3, and a purified extract of alpha-granules. ANA were detected by IIF using cultured HEp-2 cells. RESULTS: Twenty-three patients (49%) had a perinuclear pattern (p-ANCA) by ethanol fixation, of which only 2 became cytoplasmic on paraformaldehyde fixed cells. These 2 patients also had a positive myeloperoxidase ELISA, while none of the remaining 45 had a positive result from the 3 ELISA performed. All 21 patients (45%) with a p-ANCA pattern that was not modified by paraformaldehyde fixation had a specific immunostaining upon examination at high power magnification; we termed this GS ANA specific pattern. The specificity of this pattern was further confirmed by a doubled blind test performed by 2 independent observers. In our study, all GS ANA pattern positive sera fulfilled the previously known definition of these antibodies. We found no relationship between GS ANA and variables such as disease duration and activity, rheumatoid factor, and vasculitis. Notably, 2 RA patients with "true" ANCA (anti-MPO antibodies) had an associated pulmonary-renal syndrome (microscopic polyangiitis). CONCLUSION: Most p-ANCA in our series of patients with RA did not seem to correspond to "true" ANCA but to antibodies directed against nuclear or perinuclear antigenic constituents of the neutrophils (GS ANA). The observation of their distinctive and specific immunostaining pattern, when screening patients for the presence of ANCA by IIF, may alert us to the possible presence of RA.

Adult↗
PubMed1994

[Neutrophil anticytoplasmic antibodies: their diagnostic utility in vasculitis and glomerulonephritis].

BACKGROUND: The prevalence and diagnostic usefulness of antineutrophil cytoplasmic antibodies (ANCA) were analyzed in a series of patients with different types of vasculitis, connective tissue diseases, nephropathies and lung diseases. METHODS: Six hundred seventy-six consecutive patients with systemic vasculitis, connective tissue diseases, nephropathies and different lung disturbances were included in the study. The ANCA were determined by indirect immunofluorescence (IIF) on neutrophils fixed in ethanol. For the detection of antimyeloperoxidase (MPO) antibodies (Ab) a new technique was developed consisting in the performance of IIF on neutrophils with a deficiency in MPO. The serologic samples were also analyzed by enzyme-linked immunosorbent assays with MPO and purified proteinase 3 (PR3). RESULTS: ANCA were demonstrated in 95 patients. A cytoplasmic pattern (c-ANCA) was detected in 25 cases (26%) and in 70 (74%) a perinuclear pattern (p-ANCA) was observed. Twenty-one of the 25 cases (84%) of c-ANCA corresponded to anti-PR3 Ab and 61 of the 70 (87%) of p-ANCA corresponded to anti-MPO Ab. The anti-PR3 Ab identified Wegener's granulomatosis, with a sensitivity and specificity for the generalized and clinically active forms being 65 and 88%, respectively. The anti-MPO Ab were principally detected in patients with rapidly progressive glomerulonephritis (RPGN) and hemorrhagic alveolar capillaritis. The sensitivity and specificity of the anti-MPO Ab for the RPGN and alveolar capillaritis were 75% and 98%. CONCLUSIONS: Two principal subtypes of ANCA may be detected, which also recognize two fundamental clinicopathologic entities. The high diagnostic sensitivity and specificity of these antibodies for each of the processes to which they are associated can justify the adoption of therapeutic measures in determined cases.

Antibodies, Antineutrophil Cytoplasmic↗
PubMed1994

Antineutrophil cytoplasmic autoantibody-associated alveolar capillaritis in patients presenting with pulmonary hemorrhage.

The objective of this study was to determine the significance of the antineutrophil cytoplasmic autoantibodies (ANCAs) from the clinicopathologic viewpoint of pulmonary hemorrhage occurring as a prominent event of disease. Forty-three consecutive patients with both pulmonary hemorrhage as a prominent clinical manifestation and a positive test for antineutrophil cytoplasmic autoantibodies were studied. Thirty-six patients underwent open lung biopsy, including histologic, tissue immunofluorescence, and microbiologic studies. Immunoassays were performed to investigate the antigenic specificities of antineutrophil cytoplasmic autoantibodies in the patients studied. All patients with lung biopsy confirmation had pauci-immune hemorrhagic alveolar capillaritis as the main morphologic substrate. In addition, renal involvement in the form of pauci-immune crescentic glomerulonephritis was a common finding. Serum samples from the 43 study patients contained antibodies that were monospecific for proteinase 3 (n = 13) or myeloperoxidase (n = 30). In our study, whereas anti-proteinase 3 antibodies were mainly detected in patients with alveolar capillaritis and a well-established diagnosis of Wegener's granulomatosis, antimyeloperoxidase antibodies were principally found in those patients who had alveolar capillaritis and polyarteritis nodosa not only as a primary finding but also accompanying other diseases. However, a significant number of patients with alveolar capillaritis and antimyeloperoxidase antibodies showed no evidence of polyarteritis nodosa (idiopathic pulmonary-renal syndrome and isolated forms of pulmonary hemorrhage). We conclude that in patients presenting with pulmonary hemorrhage as a prominent event of disease, antineutrophil cytoplasmic autoantibodies are a new clue strongly supportive of a pulmonary capillary vasculitis, irrespective of the primary underlying disease. Moreover, the antigenic subtype of antineutrophil cytoplasmic autoantibodies helps in recognizing the type of vasculitic disorder involved.

Antibodies↗