Biomedical subjects

M Ingelmo

Publications and source records attributed to M Ingelmo.

At least 91 records · Page 5Linked to original sources

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

Immunohistochemical characterization of inflammatory cells and immunologic activation markers in muscle and nerve biopsy specimens from patients with systemic polyarteritis nodosa.

OBJECTIVE: To investigate the phenotype of infiltrating cells in classic lesions of polyarteritis nodosa (PAN). METHODS: Twenty-one muscle and 10 sural nerve biopsy samples from 24 patients with systemic PAN were studied using avidin-biotin-peroxidase and alkaline phosphatase-anti-alkaline phosphatase immunohistochemical techniques. RESULTS: The inflammatory infiltrates consisted mainly of macrophages (41%) and T lymphocytes (41%), particularly of the CD4+ subset. Granulocytes were present in varying quantities (0-45%) and were more abundant in heavily infiltrated vessels and in those with fibrinoid necrosis. Dendritic cells could be identified in 4 samples. Proliferating and interleukin-2 receptor-expressing cells, present in 71% and 79% of the patients, respectively, were more frequent in untreated patients. CONCLUSION: T cell-mediated immune mechanisms may play a role in the development and perpetuation of PAN lesions.

Adult↗

PubMed1994

Responsiveness of T lymphocytes from systemic lupus erythematosus to signals provided through CD26 antigen.

To investigate whether the T cell defective capacity to proliferate observed in systemic lupus erythematosus (SLE) T cells is a possible consequence of an intrinsic T cell disorder, the integrity of the accessory activation pathway mediated through CD26 antigen in SLE T cells was studied. Hyporesponsiveness of peripheral blood mononuclear cells (PBMC) from SLE to PHA and CD26 Mab was observed and no differences were found when the responsiveness of highly purified T cells to IL-2, IL-2 plus CD26 Mab, phorbol 12-myristate 13-acetate (PMA), or when PMA plus CD26 Mab was analyzed. Findings suggest that signals induced by triggering CD26 are not intrinsically altered in SLE T cells. However, some alteration of the regulatory involvement of monocytes or B cell over T cell function may be involved.

Adult↗

PubMed1994

Comparison of the primary and secondary antiphospholipid syndrome: a European Multicenter Study of 114 patients.

PURPOSE: To determine whether the features of the antiphospholipid syndrome (APS) are in any way influenced by the presence or absence of systemic lupus erythematosus (SLE). We followed up patients with 'primary' APS (PAPS) and APS secondary to SLE (APS plus SLE) with the objective of comparing laboratory and clinical events and of determining whether patients with PAPS would have evolution to SLE. PATIENTS AND METHODS: A total of 114 patients from 3 European referral centers were included in this study. Fifty-six had APS plus SLE and 58 had PAPS. Laboratory and clinical data were collected during an average 2-year period. RESULTS: Patients with PAPS and patients with APS plus SLE had similar clinical and laboratory profiles, with the exceptions of autoimmune hemolytic anemia, endocardial valve disease, neutropenia, and low C4 levels, all of which occurred more frequently in patients with APS plus SLE (p values: < 0.05, < 0.005, < 0.01, and < 0.001, respectively). On follow-up, 10 thrombotic episodes occurred in 10 patients, 8 of whom were receiving anticoagulant therapy. No patient with PAPS had either anti-DNA or anti-extractable nuclear antigen antibodies, and these patients had a significantly lower prevalence of antinuclear antibodies (41%) than patients with APS plus SLE (89%). CONCLUSIONS: Patients with APS plus SLE and PAPS have similar clinical profiles, although heart valve disease, hemolytic anemia, low C4 levels, and neutropenia seem to be more common in patients with APS plus SLE. Patients with APS may develop further thrombotic events despite anticoagulation therapy.

Adult↗

PubMed1994

Antibodies to endothelial cells in Behçet's disease: cell-binding heterogeneity and association with clinical activity.

OBJECTIVES: To investigate the prevalence and characteristics of antibodies to endothelial cells (aEC) from large vessel and from microvasculature in a group of patients with Behçet's disease (BD) to determine the relationship of these antibodies with clinical and laboratory features of the disease. METHODS: Thirty patients with BD were prospectively and consecutively studied. The aEC were determined by enzyme-linked immunosorbent assay (ELISA) using endothelial cells derived from human umbilical vein (large vessel) as well as from retroperitoneal adipose tissue (microvasculature). RESULTS: Fifteen patients (50%) had aEC, either directed to large vessel [8(26%) patients] or microvascular [13(43%) patients] endothelial cells. The percentage of active patients was significantly higher in the aEC-positive group [12(80%) patients] compared with the aEC-negative group [5(33%) patients] (p < 0.05). CONCLUSIONS: Patients with BD have a high prevalence of aEC when microvascular endothelial cells are used in the assay. These antibodies seem to be a marker of disease activity in this condition, previously considered as negative for autoantibodies.

Adipose Tissue↗

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↗

PubMed1993

Anti-neutrophil cytoplasmic autoantibodies (ANCA): antigenic specificities and clinical associations.

We have conducted a prospective study of 372 patients with well-defined forms of systemic vasculitis and connective tissue diseases to determine the prevalence, the antigenic specificities and the clinical associations of ANCA in such cases. These antibodies were detected by indirect immunofluorescence on ethanol-fixed neutrophils and also by enzyme-linked immunosorbent assay using myeloperoxidase (MPO) as a substrate. In our study, ANCA with a cytoplasmic immunostaining pattern were mainly found in patients with biopsy-proven Wegener's granulomatosis with or without renal involvement and pulmonary hemorrhage. Furthermore, MPO-ANCA strongly correlated with necrotizing glomerular and alveolar capillaritis, mostly in patients having a well-established diagnosis of polyarteritis nodosa.

Antibodies, Antineutrophil Cytoplasmic↗

PubMed1993

Anti-granulocyte perinuclear antibodies but not anti-neutrophil cytoplasmic antibodies (ANCA) in rheumatoid arthritis.

We studied 45 patients with rheumatoid arthritis for the presence of ANCA. These antibodies were determined by indirect immunofluorescence (IIF) and by enzyme-linked immunosorbent assays (ELISAs) using as a substrate purified myeloperoxidase and purified extract of azurophilic granules. By IIF, we found a characteristic perinuclear immunostaining pattern in 21 cases (47%). However, no patient had a positive result by the two ELISAs performed. Patients with a positive IIF result had significantly higher levels of anti-nuclear and anti-ds DNA antibodies than those with a negative IIF result. Therefore, these antibodies must correspond to the previously reported as granulocyte specific antinuclear antibodies (GS-ANA).

Adult↗

PubMed1993

Low-dose aspirin for prevention of pregnancy losses in women with primary antiphospholipid syndrome.

Pregnancy loss, often recurrent, is one of the most important clinical manifestations associated with the primary antiphospholipid syndrome. In these cases, pregnancy wastage is related to the presence of antiphospholipid antibodies, namely lupus anticoagulant and anticardiolipin antibodies, but patients do not have features of systemic lupus erythematosus or any other well-defined autoimmune disease. We report here on the outcome of 21 consecutive pregnancies in 18 patients with the syndrome who were treated with low-dose aspirin (100 mg/day) from 1 month before attempting conception and throughout the pregnancy. Low-dose prednisone (15-30 mg/day) was added for potentially non-obstetric (autoimmune-related) reasons in six pregnancies. Patients were monitored as having high-risk pregnancies. Prior to therapy, the rate of live-born babies was 6.1% (46 previous fetal losses and three live-born babies), and after therapy, it was 90.5% (21 pregnancies and 19 live-born babies). Pre-term delivery due to maternal or fetal indications was required in 15% (3/20) of the viable pregnancies. Except for prematurity (20% of viable pregnancies) and its potential associated complications, there were no significant adverse effects to either mothers or babies. Our treatment modality is advocated for prevention of pregnancy losses in patients with the 'obstetric' primary antiphospholipid syndrome.

Abortion, Habitual↗

PubMed1993

[Functional abnormality of the diaphragm in the "shrinking lung" in systemic lupus erythematosus].

Functional respiratory disorders in patients with systemic lupus erythematosus (SLE) have been previously reviewed. We report a patient with SLE who developed progressive dyspnea with a restrictive pattern in the functional respiratory tests, and diaphragmatic disfunction. Symptoms and functional tests improved after a treatment with steroids. We also review the pathophysiologic mechanisms of this disorder.

Diaphragm↗

PubMed1992

[Anti-endothelial cell antibodies in systemic lupus erythematosus: association with vascular and renal lesions].

BACKGROUND: The aim of this study was to know the prevalence and characteristics of endothelial anticellular antibodies (EAA) in systemic lupus erythematosus and its relation with the clinical and immunologic manifestations of this entity. METHODS: A prospective study of 50 patients (44 females and 6 males) diagnosed with SLE was carried out. EAA and anticardiolipin antibodies (ACA) were determined by the ELISA technique, lupic anticoagulant by coagulometric techniques, antinuclear antibodies by indirect immunofluorescence, anti-DNA antibodies by the Farr technique, anti-ENA by contra-immunoelectrophoresis and the complement values by radial immunodiffusion. The statistical study was carried out by chi-square test and Fisher test. RESULTS: Positive titers of EAA were observed in 29 (58%) of the patients with SLE. The patients with EAA presented greater prevalence of both, vascular lesions (31% vs 5%, P < 0.05) and kidney involvement (62% vs 29%, p < 0.05) than those without EAA. Patients with EAA also presented a higher prevalence of antiphospholipid antibodies than patients without EAA (59% vs 10%, p < 0.001). No association was found between EAA and the titers of antinuclear antibodies, anti-DNA, anti-ENA or complement levels. CONCLUSIONS: Endothelial anticellular antibodies appear with greater frequency in patients with systemic lupus erythematosus and are more often associated with vascular and kidney involvement and with the detection of antiphospholipid antibodies suggesting that they may play an etiopathogenic role in the production of these lesions.

Adult↗

PubMed1992

Anti-myeloperoxidase autoantibodies in patients with necrotizing glomerular and alveolar capillaritis.

We conducted a prospective study of 651 Mediterranean patients from Catalonia (Spain) with well-defined forms of systemic vasculitis, connective tissue diseases, and renal and pulmonary disorders to determine the prevalence and clinical value of antineutrophil cytoplasmic autoantibodies (ANCA) with myeloperoxidase (MPO) specificity (MPO-ANCA). ANCA were first tested by indirect immunofluorescence on ethanol-fixed neutrophils. When a positive result was obtained, then MPO-ANCA were identified by performing the immunofluorescence assay again on neutrophils from a voluntary donor known to have a complete and selective deficiency of MPO. This disorder was detected by automated flow cytochemistry with the Technicon system and was further verified by cytochemical and biochemical studies. We detected MPO-ANCA in 61 of 70 (87%) patients with a perinuclear pattern (p-ANCA), but in none of 25 with a cytoplasmic pattern (c-ANCA). These results were corroborated by enzyme-linked immunosorbent assay (ELISA) using human purified MPO as a substrate. On immunofluorescence microscopy, all patients with MPO-ANCA were found to have a typical and restrictive immunostaining pattern. In our study, while c-ANCA were mainly found in patients with biopsy-proven Wegener's granulomatosis, MPO-ANCA identified those with idiopathic and polyarteritis nodosa-associated necrotizing and crescentic glomerulonephritis. In addition, pulmonary hemorrhage with necrotizing alveolar capillaritis as the main morphologic substrate occurred frequently among patients with MPO-ANCA, including three affected by polyarteritis nodosa and three who had pulmonary hemorrhage as the only clinical finding. On the other hand, these antibodies could be also detected in 30% of patients with a proven diagnosis of anti-glomerular basement membrane (GBM) disease.(ABSTRACT TRUNCATED AT 250 WORDS)

Antibodies, Antineutrophil Cytoplasmic↗

PubMed1992

Cardiac disease in systemic lupus erythematosus: prospective study of 70 patients.

A prospective two dimensional and Doppler echocardiographic study of 70 consecutive patients with systemic lupus erythematosus (SLE) and 40 controls was carried out. Forty patients (57%) were found to have echocardiographic disturbance. Valvular abnormalities were detected in 31 patients (44%) and in only two controls (5%). Mitral valve abnormalities were the most common findings (23/70 (33%)) with mild or moderate regurgitation the most frequent lesion (16% and 9% respectively). Three patients (4%) had a morphological echocardiographic pattern suggestive of non-infective verrucous vegetations affecting the mitral valve. No patient had haemodynamically significant clinical valve disease. Pericardial effusion was identified in 19 patients (27%), of whom 14 had mild and clinically silent disease. Myocardial abnormalities were found in 14 patients (20%), but clinical features of myocardial dysfunction were present in only one. Patients with antiphospholipid antibodies were found to have an increased prevalence of endocardial lesions, mainly valvular regurgitation. It is concluded that the inclusion of echocardiography in a study protocol of patients with SLE can identify an important subset of patients with cardiac abnormalities, many of which are clinically silent. In addition, the association of antiphospholipid antibodies with endocardial lesions suggests that these antibodies may have a prominent role in the pathogenetic mechanisms of heart valve disease in SLE.

Adolescent↗

PubMed1992

Systemic lupus erythematosus in men: clinical and immunological characteristics.

Although systemic lupus erythematosus (SLE) has traditionally been considered a disease of women, men may also be affected. Thirty of 261 patients (12%) with SLE seen in this hospital were men. Arthritis was less common as a first symptom in the men, although this group of patients had discoid lesions and serositis more often than the women. During the follow up a lower incidence of arthritis and malar rash and a higher incidence of other skin complications including discoid lesions and subcutaneous lupus erythematosus was found in the men. The incidence of nephropathy, neurological disease, thrombocytopenia, vasculitis, and serositis, was similar in the two groups. No significant immunological differences were found between men and women. These features indicate that several gender associated clinical differences may be present in patients with SLE.

Adolescent↗

PubMed1992

Plasma from systemic lupus erythematosus patients with antiphospholipid antibodies promotes platelet aggregation. Studies in a perfusion system.

The possible platelet-aggregating effect of plasma from systemic lupus erythematosus (SLE) patients (n = 19) was investigated under flow conditions. Aliquots of the SLE plasmas with (n = 10) or without (n = 9) anticardiolipin antibodies (ACAs) were added to anticoagulated blood (1:20, vol/vol). Plasma from normal donors was used as a control. Blood was incubated for 15 minutes at 37 degrees C and then perfused through annular chambers containing denuded arterial segments. Perfusions were performed for 10 minutes at a shear rate of 800 sec-1. The interaction of platelets with vessel subendothelium (SE) was morphometrically evaluated in thin sections. In control experiments, the percentage of the SE covered with platelets was 23.6 +/- 4.3% (mean +/- SD). Large aggregates (more than 5 microns in height) covering 11.8 +/- 5.7% of the exposed SE were noted. The deposition of platelets was statistically increased (38.5 +/- 7.6%, p less than 0.01 versus control) in the presence of SLE plasmas with demonstrated antiphospholipid antibodies (APAs). The formation of large aggregates was also augmented (30.3 +/- 5.9%, p less than 0.01 versus control). A similar response was obtained after addition of affinity-purified immunoglobulin G and immunoglobulin M fractions from two patients with ACAs. SLE plasmas with no detectable APAs did not influence the morphometric parameters studied. Results of the present study indicate that the presence of APAs in SLE plasma promotes platelet aggregation under flow conditions. These observations may help to explain the pathophysiology of the thrombotic events occurring in patients with APAs.

Adolescent↗

PubMed1992

Antimyeloperoxidase autoantibody-associated necrotizing alveolar capillaritis.

In this report, we describe 3 patients who had pauci-immune necrotizing alveolar capillaritis-related pulmonary hemorrhage and who never developed other organic involvement, as revealed by clinical and laboratory data and also by autopsy examination in 1 case. Serum samples from these patients disclosed antimyeloperoxidase autoantibodies with initial immunofluorescence titers ranging from 1:1,600 to 1:3,200. Rapid institution of immunosuppressive therapy, as well as plasma exchange, led to prompt clinical improvement in 2 patients who were receiving mechanical ventilation support. We conclude that antimyeloperoxidase autoantibodies become new clues to support an underlying alveolar capillary vasculitis in patients with idiopathic isolated pulmonary hemorrhage, thus facilitating therapeutic decisions in this life-threatening condition.

Adult↗

PubMed1991

[Neutrophil anticytoplasmic antibodies in a patient with Wegener's granulomatosis: therapeutic implications of its detection and relation to clinical activity].

The case of a patient with a multisystemic process characterized by polyarthritis, hemoptysis, leucocytoclastic vasculitis, renal failure and ulcerated lesions in the palate and nasal bone is reported. The existence of antineutrophil anticytoplasmic antibodies (cytoplasmatic pattern) was proven by indirect immunofluorescence with an initial serum titration of 1:1.600. Detection of these antibodies permitted the establishment of immunosuppressive treatment when the clinical situation of the patient was considered serious (pulmonary hemorrhage with progressive diminution of the hematocrit). Four days after the initiation of treatment the histopathological results of the palate and nasal mucous biopsies were received and were compatible with Wegener's granulomatosis. Serial determination of the titers of these antibodies demonstrated a close correlation with the clinical biological activity of the process. Indeed, 3 days after initiation of the immunosuppressive treatment the concentration of the same had reduced to half, something which has not been previously reported. It is concluded that high specificity and sensitivity of antineutrophil anticytoplasmic antibodies with a cytoplasmatic pattern for Wegener's granulomatosis may contribute to the improvement, not only of the diagnosis but also to the prognosis, in permitting the immediate initiation of therapeutic measures when the clinical situation of the patient thus requires.

Adult↗

PubMed1991

[IgM myeloma: a report of 2 cases].

The IgM myeloma is a rare type of multiple myeloma (MM) with some features which differentiate it from other immunologic types of myeloma and from Waldenström macroglobulinemia (WM). Two patients with IgM myeloma in whom the only clinical feature of the disease was the development of bone lesions and general deterioration are reported. In none of the cases an M component was detected in proteinogram. A bone marrow proliferation of plasma cells was discovered in both, which it was accompanied with dura mater infiltration in one of them. The type of secreted immunoglobulin was IgM lambda in the first case and kappa in the second. Both patients had a poor clinical course after the diagnosis and died due to infective complications. Emphasis is made on the need to differentiate this condition from WM due to their different prognosis and therapy.

Aged↗