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J Font

Publications and source records attributed to J Font.

At least 181 records · Page 10Linked to original sources
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

Systemic lupus erythematosus evolving into systemic vasculitis: a report of five cases.

Five patients with systemic lupus erythematosus are described who developed the disease in their second or third decade. The pattern of their disease then changed markedly and they developed the clinical features of a systemic vasculitis. The possible explanations for this transition of one connective tissue disease to another is discussed.

Adolescent↗
PubMed1993

Amyotrophic lateral sclerosis in a patient with systemic lupus erythematosus.

We report the case of a 48-year-old woman diagnosed as having systemic lupus erythematosus who developed a neurological disorder that finally resulted in amyotrophic lateral sclerosis. Although neurological disturbances caused by systemic lupus erythematosus are protean, there are no reported cases of amyotrophic lateral sclerosis associating with lupus disease. This association and the suspected autoimmune mechanism of pathogenesis in amyotrophic lateral sclerosis suggest that this might not be a simple coincidence but perhaps a causal relationship.

Amyotrophic Lateral Sclerosis↗
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↗
PubMed1993

Esophageal motor function in primary Sjögren's syndrome.

To evaluate esophageal motor function in patients with primary Sjögren's syndrome (PSS) and its relation to the presence of dysphagia, 20 outpatients, who met the four criteria for PSS proposed by Fox et al. were prospectively studied by esophageal manometry after dry or wet swallows. Dysphagia was present in 15 (75%) patients, although the presence or severity of the symptom was not related to any specific motility pattern. Moreover, this study did not show any correlation between dysphagia and degree of inflammatory infiltrate of the labial minor glands, the parotid flow rate, or the presence of autoantibodies. In conclusion, although dysphagia is a common complaint in patients with PSS, esophageal motor studies have failed to show any consistent pattern.

Deglutition Disorders↗
PubMed1993

Systemic lupus erythematosus: clinical and immunologic patterns of disease expression in a cohort of 1,000 patients. The European Working Party on Systemic Lupus Erythematosus.

In the present study we have analyzed the prevalence and characteristics of the most relevant clinical and immunologic features in 1,000 patients with SLE. Several differences in the expression of the disease have been observed in relation to the patients' age at onset, sex, and autoantibody serology. The childhood-onset patients more often had malar rashes (55% vs 39%) and nephropathy (28% vs 15%) as presenting manifestations. During the evolution of the disease, these patients had an increased prevalence only of malar rash (79% vs 56%) and a lower prevalence of rheumatoid factor (6% vs 19%). The older-onset patients (age 50 or older) less often showed malar rash (21% vs 42%), arthritis (52% vs 71%), and nephropathy (3% vs 17%) as the first symptom. During the evolution of their disease, these patients had a decreased prevalence of malar rash (33% vs 60%), photosensitivity (29% vs 47%), arthritis (73% vs 85%), nephropathy (22% vs 41%), thrombosis (4% vs 15%), and anti-La antibodies (6% vs 20%), but an increased prevalence of sicca syndrome (33% vs 15%). Males more often had serositis (28% vs 16%) as a first symptom, but they presented with a lower prevalence of arthritis (74% vs 85%) during the evolution of the disease. The presence of ANA, a high titer of anti-dsDNA, rheumatoid factor, anti-ENA, and antiphospholipid antibodies also distinguished additional homogeneous SLE subsets of clinical significance.

Adolescent↗
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

Intracellular Ca2+ mobilization and not calcium influx promotes phorbol ester-stimulated thromboxane A2 synthesis in human platelets.

Phorbol esters, potent activators of protein kinase C (PKC), greatly enhance the release of arachidonic acid and its metabolites (TXA2, HETES, HHT) by Ca2+ ionophores in human platelets. In this paper, we report the relationship between intracellular Ca2+ mobilization and external calcium influx into platelets and the ability of PMA plus A23187 to promote thromboxane A2 (TXA2) synthesis. The enhanced levels of TXA2 due to the synergistic stimulation of the platelets with A23187 and phorbol esters are not affected significantly by the presence of external Ca2+ or the calcium-chelator EGTA. PKC inhibitors, staurosporine and sphingosine, abolished phorbol myristate acetate (PMA) potentiation of TXA2 production which strongly supports the role of PKC in the synergism. Platelet aggregation is more sensitive to PMA and external calcium than TXA2 formation. PMA increased TXA2 production as much as 4-fold at low ionophore concentrations. The A23187-induced rise in [Ca2+]i was reduced by pretreatment of human platelets with phorbol esters, both in the presence and absence of EGTA, and staurosporine reversed this inhibitory effect. These results indicate that the synergistic stimulation of TXA2 production by A23187 and phorbol esters is promoted by intracellular Ca2+ mobilization and not by external calcium influx. Our data also suggest that PKC is involved in the regulation of Ca2+ mobilization from some specific intracellular stores and that PKC may also stimulate the Ca(2+)-dependent phospholipase A2 at suboptimal Ca2+i concentrations.

Alkaloids↗
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

Development of Hodgkin's disease in the course of primary Sjögren's syndrome.

We report two patients with a definite diagnosis of primary Sjögren's syndrome who developed Hodgkin's disease. Clinical and laboratory features of this transformation comprised prolonged fever, the appearance of lymphadenopathy together with loss of serum autoantibodies and a reduction in serum gammaglobulin levels. We know of only one well documented case of such an association. From these observations, it seems reasonable to include Hodgkin's disease in the clinical spectrum of the lymphoproliferative disorders that may occur in the course of primary Sjögren's syndrome.

Adult↗
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↗