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M Ingelmo

Publications and source records attributed to M Ingelmo.

At least 73 records · Page 4Linked to original sources
PubMed1997

[Primary Sjögren's syndrome: clinical and immunologic study of 80 patients].

BACKGROUND: To determine the clinical and immunologic characteristics of a large cohort of patients with primary Sjörgen's syndrome (SS) and to asses if the sex, the age at onset, the time of evolution and the immunologic pattern define different subsets with specific characteristics. PATIENTS AND METHODS: We included 80 patients (76 female and 4 male) that were prospectively studied at our Unit. All patients fulfilled the European Community criteria proposed in 1993 for the diagnosis of SS. RESULTS: Mean age of patients was 62 years with a mean disease duration of 8 years. The most frequently observed clinical manifestations were xerostomia (96%), xerophthalmia (94%) and parotidomegaly (46%). The main extraglandular manifestations were arthritis (45%), Raynaud's phenomenon (20%) and liver involvement (19%). The immunologic study showed antinuclear antibodies in 82% of patients, rheumatoid factor in 45%, anti-Ro/SS-A in 40% and anti-La/SS-B in 20%. In patients with an onset of disease before the age of 40 years, a higher prevalence of parotidomegaly, peripheral neuropathy, cutaneous vasculitis, rheumatoid factor, anti-Ro/SS-A and anti-La/SS-B antibodies was observed. A disease duration longer than 10 years was associated with a higher prevalence of pulmonary involvement and more focus of mononuclear cells in the minor salivary glands. Clinical manifestations associated to any one or more immunologic marker (rheumatoid factor, anti-Ro/SS-A and/or anti-La/SS-B) were Raynaud's phenomenon, arthritis, thyroid disease, cutaneous vasculitis and peripheral neuropathy. CONCLUSIONS: Primary SS is an autoimmune disease characterized by a marked heterogeneity in the clinical presentation and evolution, thus allowing the definition of several subsets of patients with their own clinical and immunological characteristics.

Adult↗
PubMed1997

Antibodies to thromboplastin in systemic lupus erythematosus: isotype distribution and clinical significance in a series of 92 patients.

We determined the prevalence and relationship with clinical manifestations of antibodies to thromboplastin (aTP) in 92 patients with systemic lupus erythematosus (SLE). Thirty-two (35%) patients had aTP: 13 (14%) were positive for IgG aTP, 13 (14%) for IgM aTP, and 6 (7%) for both. Patients with aTP had an increased incidence of thrombosis (p = 0.01), thrombocytopenia (p < 0.001), hemolytic anemia (p < 0.001), and fetal losses (p = 0.03). When the IgG and IgM aTP isotypes were analysed separately, the IgG aTP were found to be associated with thrombosis (p < 0.001), thrombocytopenia (p < 0.001), and fetal losses (p = 0.02). The IgM aTP were associated with hemolytic anemia (p < 0.001). A correlation was found between the titers of aTP and those of anticardiolipin antibodies, in both IgG (p < 0.01, r = 0.6) and IgM (p < 0.01, r = 0.64) isotypes, and between the titers of IgG aTP and the diluted Russell's viper venom time used to detect the lupus anticoagulant (p < 0.001, r = 0.42). This test is a reliable, reproducible and sensitive assay for the detection of antiphospholipid antibodies, specially in those patients under anticoagulant therapy.

Adolescent↗
PubMed1997

Optic neuropathy in the "primary" antiphospholipid syndrome: report of a case and review of the literature.

Several visual disturbances have been associated with the presence of antiphospholipid antibodies, including vaso-occlusive retinopathy and ischaemic optic neuropathy. The latter has been rarely described in patients with "primary" antiphospholipid syndrome, and, when present, is generally associated with neurological disorders. We describe the case of a patient presenting with optic neuropathy in whom a "primary" antiphospholipid syndrome was diagnosed, and review all previously reported similar cases. The pathogenesis and treatment are also discussed.

Anticoagulants↗
PubMed1997

Anti-beta 2-glycoprotein I antibodies: a useful marker for the antiphospholipid syndrome.

We studied anti-beta 2-glycoprotein I antibodies (a beta 2GPI) in autoimmune disease patients to evaluate their relationship to clinical findings. Seventy-nine systemic lupus erythematosus (SLE) patients [44 with antiphospholipid antibodies (aPL)], 21 with primary antiphospholipid syndrome (APS), eight asymptomatic individuals with aPL and 60 controls were studied. Sixteen SLE patients (14 with aPL and two without aPL) and six with primary APS had a beta 2GPI. A significant relationship was found between a beta 2GPI and aPL (P < 0.01). In SLE, a significant correlation was found between previous thrombosis or thrombocytopenia and a beta 2GPI or a beta 2GPI + aPL, but not between fetal losses and a beta 2GPI. These data suggest that a beta 2GPI may be useful in the study of APS.

Adult↗
PubMed1997

Chorea in the antiphospholipid syndrome. Clinical, radiologic, and immunologic characteristics of 50 patients from our clinics and the recent literature.

We analyzed the clinical, radiologic, and immunologic characteristics of 50 patients with chorea and the antiphospholipid syndrome (APS) (6 from our clinics and 44 from a MEDLINE computer-assisted review of the literature from 1985 through 1995). Forty-eight (96%) patients were female and 2 (4%) were male. Twenty-nine (58%) patients had defined systemic lupus erythematosus (SLE), 6 (12%) had "lupus-like" syndrome, and 15 (30%) patients had "primary" APS. Mean age of patients in this series was 23 +/- 12 years (range, 6-77 yr); mean age at presentation of chorea was 21 +/- 12 years (range, 6-77 yr). In 11 (22%) patients, the onset of chorea was in childhood (6-14 yr), and in 2 (4%) patients it presented at 60 years or more. Six (12%) patients developed chorea soon after they started taking estrogen-containing oral contraceptives, 3 (6%) developed chorea gravidarum, and 1 (2%) patient developed chorea shortly after delivery. Most patients (66%) presented only 1 episode of chorea. Chorea was bilateral in 55% of patients. Computed tomography and magnetic resonance imaging scans reported cerebral infarcts in 35% of patients. The following antibodies were detected: lupus anticoagulant (92%), anticardiolipin antibodies (91%), antinuclear antibodies (82%), anti-DNA (59%), anti-Ro (10%), anti-RNP (8%), anti-La (2%), and anti-Sm (2%). The chorea in these patients responded to a variety of medications, for example, steroids, haloperidol, antiaggregants, anticoagulants, or a combination of therapy, usually prescribed in the presence of other manifestations of APS or SLE. However, many patients responded well to haloperidol and to the discontinuation of oral contraceptives if this was the precipitating factor.

Adolescent↗
PubMed1997

Hepatitis C virus infection in 'primary' Sjögren's syndrome: prevalence and clinical significance in a series of 90 patients.

OBJECTIVES: To determine the prevalence and clinical significance of hepatitis C virus (HCV) infection in a large cohort of patients with "primary' Sjögren's syndrome (SS). METHODS: 90 consecutive patients (83 female and seven male) were included, with a mean age of 62 years (range 31-80) who prospectively visited our unit. All patients fulfilled the European Community criteria for SS and underwent a complete history, physical examination, as well as biochemical and immunological evaluation for liver disease. Serum from all patients was tested for antibodies to HCV by third generation enzyme linked immunoassay and positivity was confirmed by polymerase chain reaction. RESULTS: Antibodies to HCV were present in 13 (14%) patients with 'primary' SS. When compared with patients without HCV infection, patients with HCV infection presented a higher prevalence of hepatic involvement, (100% v 8%, p < 0.05). Transcutaneous liver biopsy was performed in five patients with HCV infection, and specimens obtained showed in all cases a chronic active hepatitis with varying degrees of portal inflammation. CONCLUSION: HCV infection is frequent in patients with "primary' SS and liver involvement is presented in all these patients. The possible pathogenic role of HCV infection in these patients is still unclear.

Adult↗
PubMed1997

Shrinking lungs syndrome in systemic lupus erythematosus: improvement with inhaled beta-agonist therapy.

The pathogenesis and therapy of shrinking lungs syndrome in patients with systemic lupus erythematosus remains controversial. It has been previously reported that corticosteroid treatment can be effective. We report a patient with shrinking lungs syndrome who presented a good response to inhaled beta-agonist therapy. Therapeutic approach to this syndrome is discussed.

Administration, Inhalation↗
PubMed1997

Anti-endothelial cell antibodies in systemic autoimmune diseases: prevalence and clinical significance.

OBJECTIVE: To investigate the prevalence and characteristics of anti-endothelial cell antibodies (AECA) in a large cohort of patients with several well defined systemic autoimmune diseases, in order to determine their relationship with the clinical and laboratory features of these diseases. METHODS: Clinical and laboratory features of 216 consecutive Caucasian patients were prospectively studied. One hundred and seven patients had been diagnosed as having a primary systemic vasculitis-specifically, 39 had temporal arteritis (TA), 25 polyarteritis nodosa (PAN), 9 Wegener's granulomatosis (WG), and 34 Behcet's disease (BD)-, 90 patients had systemic lupus erythematosus (SLE), and 19 had a primary Sjogren's syndrome (SS). The AECA were determined by ELISA. RESULTS: One hundred and four (48%) patients with systemic autoimmune diseases were found to have a positive titre of AECA. Specifically, AECA were detected in 41 (38%) patients with a primary systemic vasculitis (13 (33%) with TA, 14 (56%) with PAN, 5 (56%) with WG and 9 (26%) with BD), in 58 (63%) patients with SLE, and in 5 (26%) patients with a primary SS. In patients with a primary systemic vasculitis, those with AECA were found to have an increased prevalence of disease activity (P < 0.05). In SLE patients, those with AECA were found to have an increased prevalence of vascular lesions (P < 0.05), lupus nephropathy (P < 0.05), and anticardiolipin antibodies (aCL) (P < 0.001). CONCLUSIONS: Patients with systemic autoimmune diseases have a high prevalence of AECA and they are associated with the presence of vascular lesions, nephropathy, and aCL in SLE, as well as with disease activity in several primary systemic vasculitis (TA, PAN, WG and BD).

Adult↗
PubMed1996

Elevated soluble CD27 levels in serum of patients with systemic lupus erythematosus.

The CD27 belongs to the NGF-R, a family of type I transmembrane receptors present in most T cells. The soluble form of CD27 can be found in body fluids, and elevation of serum levels is observed in T cell activation. Preliminary studies indicate that measurement of sCD27 levels might be of use in the evaluation of immune responses in vivo. Systemic lupus erythematosus (SLE) is a disease of unknown etiology. T cells from SLE patients have impaired responsiveness to various kinds of stimuli. However, the precise localization of the defect in the activation of SLE remains unclear. In this study we have analyzed the sCD27 serum levels in a SLE population and in a healthy group control. Seventy patients with SLE were prospectively studied. As controls, 20 healthy volunteer blood donors, matched by sex and age, were studied. We developed an enzyme-linked immunosorbent assay (ELISA) to measure sCD27. The mean level (+/-SD) of sCD27 was higher in the SLE patients (48.29 +/- 23.86 u) than that in the control group (36.13 +/- 7.48 u) (P = 0.02). In addition, patients with active SLE revealed higher serum concentration of sCD27 (58.20 +/- 31.06 u) than that of patients in remission (42.77 +/- 16.71 u) (P < 0.01). We found a positive correlation among sCD27 and sCD25 serum levels in SLE patients (r = 0.30, P = 0.01). No significant relation was found for other clinical symptoms or immunological parameters. In conclusion, sCD27 serum levels were increased in SLE patients and this increase was associated with the activity of the disease. Our data are consistent with the hypothesis that the CD27 antigen may constitute a marker of disease activity.

Adolescent↗
PubMed1996

Antibodies to endothelial cells and to beta 2-glycoprotein I in the antiphospholipid syndrome: prevalence and isotype distribution.

The aim of this study was to analyse the prevalence and isotype distribution of antibodies to endothelial cells (aEC) and to beta 2-glycoprotein I (a beta 2GPI) in the antiphospholipid syndrome (APS). Fifteen patients with an APS [nine associated with systemic lupus erythematosus (SLE) and six "primary'] and 15 with SLE without an APS were prospectively studied. The aEC were determined by an enzyme-linked immunosorbent assay (ELISA) using endothelial cells derived from human umbilical vein and the a beta 2GPI by ELISA using highly purified beta 2GPI. A positive titre of aEC was detected in 20 out of 30 patients (67%), but in none of the control group. Ten patients had both IgG and IgM isotypes, five had IgG only and five had only IgM. Thirteen patients with the APS (87%) were found to have a positive titre of aEC, while only seven with SLE but without a history of APS (47%) had aEC (P < 0.05). Nine patients with the APS (60%) had a positive titre of a beta 2GPI (four had both IgG and IgM isotypes, one had IgG only and four had only IgM), while none of the patients without an APS (0%) had these antibodies (P < 0.001). A significant association was also found between the presence of aPL and aEC (P < 0.05), as well as between aPL and a beta 2GPI (P < 0.001). Both aEC and a beta 2GPI can be found in the APS. This reinforces the theory that APS represents a complex autoimmune disorder in which several autoantibodies co-exist with aPL.

Adult↗
PubMed1996

Von Willebrand factor in the outcome of temporal arteritis.

OBJECTIVE: To determine fluctuation in circulating von Willebrand factor (vWF) in the outcome of patients with temporal arteritis. METHODS: Plasma vWF antigen concentrations were measured in 65 patients with biopsy proven temporal arteritis at different disease activity stages, in 12 with isolated polymyalgia rheumatica, and in 16 controls. Fourteen temporal arteritis patients underwent serial determinations during the course of their disease. RESULTS: vWF concentrations were significantly raised in temporal arteritis (mean 220 [arbitrary units], range 96 to 720) and in polymyalgia rheumatica (mean 196, range 103 to 266) compared with healthy controls (mean 98, range 75 to 137) (P < 0.05). Although vWF values tended to be higher in temporal arteritis, no significant differences were found between temporal arteritis and polymyalgia rheumatica patients nor between temporal arteritis patients with ischaemic complications (mean 269, range 130 to 720) and those who presented with polymyalgia rheumatica or constitutional symptoms only (mean 179, range 140 to 220). The highest levels were obtained in patients with associated, mainly infectious, diseases (mean 631, range 240 to 1680). Raised vWF values found in active temporal arteritis patients (mean 220, range 96 to 720) persisted within the first two years after the beginning of treatment (mean 244, range 102 to 510) but tended to normalise in patients in long term remission (mean 143, range 50 to 260). CONCLUSIONS: Persistent elevation of vWF during early remission of temporal arteritis might represent an endothelial activation status induced by a remaining inflammatory microenvironment rather than a marker of endothelial cell injury. In long term remission, decreasing vWF concentrations might reflect progression of inflammatory lesions to a healing stage.

Acute Disease↗
PubMed1996

Hypercoagulable state in patients with antiphospholipid syndrome is related to high induced tissue factor expression on monocytes and to low free protein s.

Antiphospholipid antibodies (aPLs) are associated with thrombosis, but the mechanisms of this thrombotic tendency are unknown. We studied 56 patients 612 with systemic lupus erythematosus [SLE] and aPLs and previous thrombosis, 12 with SLE and aPLs but no thrombosis, 15 with SLE without aPLs or thrombosis, 11 with primary antiphospholipid syndrome with thrombosis, and 6 asymptomatic subjects with aPLs) to investigate the ability of aPLs to induce tissue factor (TF) expression on human normal monocytes. A double direct immunofluorescence technique (anti-CD14 and anti-TF) was used, and procoagulant activity in viable and disrupted cells was measured after plasma incubation for 6 hours at 37 degrees C with normal mononuclear cells. Hemostasis regulatory proteins, prothrombin fragment 1 + 2, and thrombin-antithrombin III complex levels were determined. Increased TF expression and procoagulant activity were observed using plasma samples from SLE patients with aPLs and thrombosis (P < .01) and from primary antiphospholipid syndrome patients (P < .01) but not from patients with SLE and aPLs but no thrombosis, patients with SLE without aPLs, or asymptomatic patients with aPLs. Purified aPL immunoglobulins from one primary antiphospholipid syndrome and two SLE patients added to normal plasma showed a significant increase in both TF expression and procoagulant activity (P < .05) compared with purified aPL from two SLE patients without thrombosis. The addition of nonspecific IgG from three SLE patients without aPLs and from three control subjects did not increase TF expression. Low free protein S was seen in eight patients. Increased TF expression and low free protein S correlated with thrombosis (P < .01) and with higher prothrombin fragment 1 + 2 and thrombin-antithrombin III values (P < .01). These observations may contribute to a further understanding of the thrombotic risk in aPL patients.

Antiphospholipid Syndrome↗
PubMed1995

The effect of systemic lupus erythematosus and long-term steroid therapy on bone mass in pre-menopausal women.

The aim of our study was to assess bone mineral density (BMD) in pre-menopausal women with systemic lupus erythematosus (SLE) and the influence of disease activity and use of corticosteroids. Lumbar and femoral BMD were measured in 43 patients with SLE (28 on regular steroid therapy, 15 with recent onset, non-treated) and compared with 43 healthy women matched for age. In addition, 21 SLE patients treated with corticosteroids were followed-up with a mean of 36.6 +/- 12.7 months. BMD was significantly lower in SLE patients with prednisone doses > or = 7.5 mg/day than those of recent onset (lumbar: 1.07 +/- 0.11 vs 1.15 +/- 0.13, P = 0.039; femur: 0.85 +/- 0.13 vs 0.98 +/- 0.21, P = 0.034) and the control group (lumbar: 1.07 +/- 0.11 vs 1.13 +/- 0.10, P = 0.040; femur: 0.85 +/- 0.13 vs 0.93 +/- 0.10, P = 0.028). In addition, 18% of SLE steroid users had osteoporosis. Lumbar and femoral BMD were inversely correlated with the time of treatment and the cumulative doses of prednisone. There were no significant BMD changes during the 3-yr follow-up period. In conclusion, in SLE pre-menopausal patients lumbar and femoral BMD is decreased and related to long-term corticosteroid therapy.

Adult↗
PubMed1995

Lack of relationship between human immunodeficiency virus infection and systemic lupus erythematosus.

The objective of this work was to determine whether HIV-1 and HIV-2 could be involved in the pathogenesis of systemic lupus erythematosus (SLE). Seventy-five consecutive Caucasian patients with SLE presenting at one institution over a 2-year period were studied. Serum samples were surveyed for anti-HIV-1 antibodies by a commercial ELISA coated with HIV-1-p24. For confirmation, conventional immunoblots were performed with the following antigens: HIV-1-gp41, p31, p24 and p17 (recombinant) and HIV-2-gp36 (synthetic peptide). Additionally, Western blots with HIV-1-gp160, gp120, gp41, p65, p51, p24 and p18 bands were applied. Seventeen (23%) patients exhibited reactivity with HIV-1-p24 in the ELISA, but in the immunoblots and Western blots these sera samples were negative. Patients with SLE may exhibit a reactivity with HIV-1-p24 in the ELISA for HIV infection screening but not in the confirmatory blots. This false-positive reactivity is probably due to molecular mimicry between autoantigens and retroviruses or a contaminant or artefacts in the antigen preparation procedure.

Acquired Immunodeficiency Syndrome↗
PubMed1995

Effect of plasma from patients with primary antiphospholipid syndrome on platelet function in a collagen rich perfusion system.

The effect on platelet function of plasma from 9 patients with primary antiphospholipid syndrome (PAS) with previous thrombotic episodes was investigated under flow conditions. Five asymptomatic individuals with antiphospholipid antibodies (aPL) (A-aPL) and 14 normal controls were also studied. Patients and controls plasmas were added (1:20 v/v) to anticoagulated blood and perfused through annular chambers containing collagen rich vessel segments. The interaction of platelets with vessel subendothelium was morphometrically evaluated in thin sections. An increase in both covered surface and thrombi formation was observed in perfusions in the presence of PAS-plasma (mean +/- SD: 34.2% +/- 9.6% and 23.2% +/- 10.0% respectively) compared with control plasmas (21.4% +/- 7.3% and 10.1% +/- 7.7%, p < 0.01). Affinity purified anticardiolipin antibodies from one PAS patient showed a similar effect when added to normal blood. In contrast, A-aPL plasma had no effect on platelet-subendothelium interaction. In parallel studies, the same plasmas were incubated with isolated normal platelets before and after activation with ADP or collagen and the binding of immunoglobulins (Ig) was determined by flow cytometry. A significantly increased binding of Ig was observed in 8 out of 9 plasmas from PAS patients when platelets had been activated with collagen but not when resting or ADP activated platelets were used. No increased Ig binding to platelets was seen using A-aPL individuals plasma. These observations might help to explain the pathophysiology of the thrombotic events occurring in patients with PAS.

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

[Primary antiphospholipid syndrome: clinical and biological study of 36 cases].

BACKGROUND: The aim of this study was to analyze the clinical and biological characteristics and follow up in a series of patients with the primary antiphospholipid syndrome. METHODS: Thirty-six patients were prospectively studied. The antiphospholipid antibodies were determined with the following techniques: IgG and IgM anticardiolipin antibodies by ELISA, lupic anticoagulant by coagulometric tests and serology of syphilis by a reaginic test. RESULTS: The mean age of the patients was of 29.9 years with a female/male relation of 4.1 to 1. The most frequently observed clinical manifestations were miscarriage and fetal death in a total of 22 patients (61%). Thrombotic phenomena were observed in 15 patients (42%) with predominance of venous thrombosis of the deep venous system of the limbs and pulmonary embolism. Thrombocytopenia was detected in 9 cases (25%), autoimmune hemolytic anemia in 3 (8%) and low or moderate titres of antinuclear antibodies in 12 (33%). Other infrequent clinical manifestations were cardiac valve lesions, livedo reticularis and epilepsy. Three patients (8%) had relatives with systemic lupus erythematosus. The antiphospholipid antibody study demonstrated the presence of anticardiolipin antibodies in 28 patients (78%), lupic anticoagulant in 22 (67%) (not determined in 3 patients) and falsely positive test for syphilis in 5 (14%). Patients with thrombosis received anticoagulant therapy, with all evolving favorably except two who died (multiple pulmonary thromboembolism in one case and cerebral hemorrhage in the other) mortality of the series thus being 5.6%. Fourteen women wished to become pregnant following fetal loss and were treated with acetylsalicylic acid in 3 cases together with prednisone (15 to 30 mg/day) with successful pregnancies being achieved in 12 cases (86%). CONCLUSIONS: The primary antiphospholipid syndrome is a well differentiated clinical entity and the presence of antiphospholipid antibodies should be investigated in young people presenting thrombosis or fetal loss for no apparent reason.

Abortion, Spontaneous↗