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J Font

Publications and source records attributed to J Font.

At least 199 records · Page 11Linked to original sources
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↗
PubMed1992

Multiorgan thrombotic disorders in systemic lupus erythematosus: a common link?

Multiorgan thrombotic disorders have been described in a variety of conditions including systemic lupus erythematosus. Among these are the 'catastrophic' antiphospholipid syndrome, disseminated intravascular coagulation and the thrombotic thrombocytopenic purpura-haemolytic uraemic syndrome. In this review we briefly analyse some specific clinical and haematological characteristics of these conditions and attempt to uncover common links between them.

Antiphospholipid Syndrome↗
PubMed1992

Lack of association between anticardiolipin antibodies and migraine in systemic lupus erythematosus.

We carried out a prospective study of the prevalence and characteristics of anticardiolipin antibodies (aCL) and migraine on 103 consecutive patients with systemic lupus erythematosus (SLE) derived from three European referral university centers. We also included a control group of 58 patients with migraine not associated with SLE. We interviewed all patients using a data collection form designed to provide a complete record of the clinical characteristics of headache. Fifty-four (52%) patients with SLE suffered recurrent headache. We diagnosed migraine in 32 patients, tension headache in 21, and cluster headache in one. Thirty (29%) patients with SLE had aCL. Migraine was present in five patients with aCL and in 27 patients without aCL (17% versus 37%, p = 0.07). No migraine control patient had aCL. Our study highlights the frequency of headache in SLE, but does not support an association between the presence of aCL and migraine in this disease.

Adolescent↗
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

Lupus anticoagulant as a marker of autoimmunity in recurrent pregnancy loss; a case report.

We present a patient with primary antiphospholipid syndrome, as defined by nine pregnancy losses, the presence of lupus anticoagulant (LAC), and the absence of clinical signs and symptoms of autoimmunity. A successful pregnancy was achieved by treatment with low-dose prednisone (15 mg daily) and aspirin (100 mg daily). The patient was followed-up throughout her two last pregnancies and a 6 months postpartum period. Our data indicate that LAC serves as a marker of disease in women with previous pregnancy wastages, and that aspirin-prednisone therapy is beneficial in carefully selected patients.

Abortion, Habitual↗
PubMed1991

Immunogenetic findings in cutaneous lupus erythematosus.

The serologic features and histocompatibility antigen associations of 93 patients with cutaneous lupus erythematosus are reported. Of them, 37 had chronic cutaneous lupus erythematosus and 56 had subacute cutaneous lupus erythematosus. Subacute cutaneous lupus erythematosus with an annular pattern occurred in 32 patients; 24 had a papulosquamous morphology. Sixty percent of patients with annular subacute cutaneous lupus erythematosus had anti-Ro antibodies. The incidence of this antibody in the other two groups was lower. In addition, patients with annular skin lesions had significant antigen association with HLA-B8 (37.5%; relative risk, 4.6) and HLA-DR3 (59.4%; relative risk, 4.3). There were no significant differences among chronic cutaneous lupus erythematosus and subacute cutaneous lupus erythematosus of the papulosquamous type and the control population for any HLA loci. Thus only patients with annular skin lesions represent a homogeneous subgroup in which there is a striking concordance of anti-Ro antibodies and the HLA-B8 and HLA-DR3 phenotypes.

Acute Disease↗
PubMed1991

Systemic lupus erythematosus in the elderly: clinical and immunological characteristics.

Systemic lupus erythematosus (SLE) predominantly affects young women in their 20s. In 40 out of 250 (16%) patients with SLE seen in our hospital disease onset occurred after the age of 50. The interval between the time of onset and diagnosis was five years in this older group compared with three years in the younger group. Arthritis, as a first symptom, was less common in the older onset group. During the follow up a lower incidence of arthritis, malar rash, photosensitivity, and nephropathy was found in the older onset group. In contrast, this group showed an increased incidence of myositis. High titres of anti-dsDNA tended to occur less often and the incidence of anti-Ro antibodies was lower in the older onset group. These features seem to distinguish patients with older onset SLE as a particular subset.

Adolescent↗
PubMed1991

High prevalence of significant heart valve lesions in patients with the 'primary' antiphospholipid syndrome.

A prospective echocardiographic study was carried out on 55 patients with the recently described 'primary' antiphospholipid syndrome derived from three university medical centres. The prevalence of valvular lesions in patients with this syndrome was 38% compared with 4% in a control group of 55 healthy volunteers (P < 0.001). Mean age of patients with valve abnormalities was 42 +/- 12 years and of those without, 30 +/- 10 years (P < 0.05). One patient had a morphologic echocardiographic pattern suggestive of non-infective verrucous mitral endocarditis. Twenty patients had a two-dimensional or Doppler echocardiographic pattern of significant valvular dysfunction--either regurgitation or stenosis--without evidence of vegetations. Mitral and aortic regurgitation were the most common lesions in these patients. During follow-up of patients with valvular disease, haemodynamically significant clinical valve disease developed in four and surgery was required in one. Eleven patients had cerebrovascular occlusions. Thus, valvular heart disease, particularly affecting the mitral and aortic valves, is common in patients with the 'primary' antiphospholipid syndrome, especially in those over 40 years old.

Adult↗
PubMed1991

Antiendothelial cell antibodies in patients with the antiphospholipid syndrome.

Antiendothelial cell antibodies (AECA) have been detected in 20 out of 30 patients (67%) with thrombosis associated with antiphospholipid antibodies. Seven patients had systemic lupus erythematosus and 13 had the "primary" antiphospholipid syndrome. Seven patients had both IgG and IgM AECA, 9 had IgG AECA only, and 4 had only IgM AECA. None of 30 control patients with thrombotic events not related to antiphospholipid antibodies had a positive titre of AECA (P less than 0.001). No correlation between AECA, antinuclear antibodies, anti-dsDNA antibodies, anti-neutrophil cytoplasm antibodies, precipitating antibodies to soluble nuclear and cytoplasmic antigens or complement components was found. The possible role of these AECA in the pathogenesis of thrombotic events is discussed.

Adult↗
PubMed1991

The 'primary' antiphospholipid syndrome: antiphospholipid antibody pattern and clinical features of a series of 23 patients.

Twenty-three patients with the 'primary' antiphospholipid syndrome were studied over 2-6 years. Twenty-two (96%) had antiphospholipid antibodies detected by ELISA (87% had antibodies to thromboplastin and 70% to cardiolipin), and 18 out of the 21 tested patients (86%) had lupus anticoagulant activity by coagulative assays. Mean age of the cohort was 29.9 years and the sex ratio (female:male) 4.75:1. Eleven patients presented 18 venous and/or arterial thrombosis and 13 had 25 foetal losses (84% occurred during the second and third trimester). Other clinical features were migraine, livedo reticularis, and epilepsy. Three patients had relatives with systemic lupus erythematosus. Thrombocytopaenia was seen in 33%, antinuclear antibodies in low or moderate titre in 30%, and haemolytic anaemia in 13%. During the follow-up, two patients presented recurrent thrombosis despite anticoagulant therapy, one of them dying because of recurrent pulmonary thromboembolism. Four patients achieved successful term pregnancies after treatment with aspirin and a further patient after treatment with aspirin and low dose prednisolone. No patient developed systemic lupus erythematosus or any other definable connective tissue disease. The 'primary' antiphospholipid syndrome may exist as a distinct clinical entity and all younger patients presenting with thrombotic events, foetal losses and/or thrombocytopaenia, without any evidence of a well defined disease, should be tested for antiphospholipid antibodies in order to rule out this syndrome.

Adult↗
PubMed1991

Prognostic implication of anti-neutrophil cytoplasmic autoantibodies with myeloperoxidase specificity in anti-glomerular basement membrane disease.

Anti-neutrophil cytoplasmic autoantibodies (ANCA) were detected in 12 out of 37 (32%) serum samples from patients with anti-glomerular basement membrane (GMB) disease by an indirect immunofluorescence assay. In 11 cases, ANCA were directed against myeloperoxidase, as revealed employing neutrophils devoid of this enzyme as the test substrate. Patients having both ANCA and anti-GBM antibodies (AGBMA) were considerably older (mean age 59 years) than patients with AGBMA alone (mean age 33 years). In addition, patients with both antibodies had some clinical and pathologic data that suggested an associated systemic vasculitis. This was supported by the fact that among these patients, those with highest ANCA titres recovered renal function despite being initially on hemodialysis, as opposed to those with lowest ANCA titres or AGBMA alone. In patients with both antibodies, there was an inverse relationship between AGBMA and ANCA values (p = 0.02). Moreover, the mean AGBMA level tended to be higher for patients with AGBMA alone than for those with both ANCA and AGBMA. These results suggest that, at least in some cases, there may be a contribution of an ANCA-related mechanism in the pathogenesis of anti-GBM disease. Although the exact role of ANCA in this and other diseases remains to be clarified, there is important clinical evidence that in anti-GBM disease ANCA may represent a serologic marker of good prognosis identifying a subset of patients who may recover renal function.

Adolescent↗