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P F Gambari

Publications and source records attributed to P F Gambari.

At least 19 recordsLinked to original sources
PubMed2004

Health-related quality of life in Italian patients with systemic lupus erythematosus. I. Relationship between physical and mental dimension and impact of age.

OBJECTIVE: To examine health-related quality of life (HRQOL) in Italian patients with systemic lupus erythematosus (SLE) and compare it with that of healthy people, and to investigate relationships among different dimensions and subscales of a generic health status measure. METHODS: The Medical Outcomes Study Short Form-36 (SF-36) was applied in a cohort of 126 consecutive SLE patients and 96 healthy controls. At the time of HRQOL testing, all patients underwent clinical and laboratory evaluation. RESULTS: Both physical (PCS) and mental (MCS) component summary scores of the SF-36 were reduced in patients compared with controls. In SLE great variability in all the subscales was observed. Significant correlations between PCS and MCS and between many different subscales were observed in patients but not in controls. The PCS was higher than MCS more frequently in controls than in SLE patients (81 vs 48.4%, P<0.00001). In SLE, HRQOL tended to worsen with age. CONCLUSION: Both PCS and MCS contribute to the decrease in HRQOL in SLE patients. In SLE the mutual interaction between these two dimensions seems to be more relevant than in healthy people.

Adolescent↗
PubMed2004

Antinucleosome antibodies in SLE: a two-year follow-up study of 101 patients.

We prospectively analyzed the diagnostic sensitivity and specificity as well as the clinical relevance of antinucleosome antibodies in SLE. One hundred and one consecutive SLE patients were followed for 3 years. Three serial serum samples from each patient were tested for antinucleosome antibodies by ELISA (optimum cut-off value 10 U/ml), and for anti-dsDNA antibody (by ELISA and IIF on Crithidia luciliae), and anti-dsDNA avidity (by Scatchard plot analysis). Sera from 100 healthy individuals (HI), 35 patients with systemic sclerosis (SSc), 30 with primary Sjögren's syndrome (SS), 20 with rheumatoid arthritis (RA) and 48 with infectious diseases (ID), were assayed as controls. SLE activity and damage were evaluated using the ECLAM score and the SLICC/ACR index. At baseline, antinucleosome antibodies were found in 87 patients with SLE (86.1%), in 8 patients with SSc (22.8%), in 2 HI (2%), and in 1 ID (2.1%). The sensitivity and specificity of antinucleosome testing for SLE were 86.1% and 95.3%, respectively. The prevalence of antinucleosome antibodies in SLE was significantly higher than that of anti-dsDNA antibodies, with a correlation between them. No relevant relationship was found between antinucleosome antibodies and disease features, including renal involvement, disease activity, and disease damage. During follow-up, no significant variation was observed in antinucleosome level, nor in anti-dsDNA antibody level or avidity. We conclude that antinucleosome antibodies are commonly found in SLE. Low antibody levels can be detected in SSc, whereas medium/high levels are highly specific for SLE. Their clinical relevance during the disease course and utility for monitoring the individual patient seem to be poor.

Adult↗
PubMed2003

Risk factors for subclinical atherosclerosis in a prospective cohort of patients with systemic lupus erythematosus.

OBJECTIVE: To evaluate traditional and non-traditional risk factors for subclinical atherosclerosis in systemic lupus erythematosus (SLE). METHODS: A prospective cohort of 78 patients with SLE without overt atherosclerotic disease was studied. SLE clinical and laboratory parameters, disease activity and damage, treatment and traditional risk factors for atherosclerosis were evaluated. At baseline (T1) and after five years' follow up (T2), the serum levels of anti-oxidised palmitoyl arachidonoyl phosphocholine (oxPAPC), anti-heat shock protein 65, and anti-beta(2)-glycoprotein I antibodies and C reactive protein were tested. At T2, intima-media thickness (IMT) was measured using duplex carotid sonography. Thickened intima, plaque, mean IMT (m-IMT), and maximum IMT (M-IMT) were assessed. RESULTS: A thickened intima was seen in 22/78 (28%) patients and plaque in 13/78 (17%). M-IMT and m-IMT were (mean (SD)) 0.77 (0.34) mm and 0.55 (0.15) mm, respectively. Patients with carotid abnormalities were significantly older, had higher blood pressure and total serum cholesterol levels, and had taken a higher prednisone cumulative dosage than those without any lesions. The carotid abnormalities were associated with renal disease and ECLAM >2 at T1, and with azathioprine treatment. In multivariate analysis, age and cumulative prednisone dose were associated with carotid abnormalities; age, hypertension, and anti-oxPAPC at T2 were correlated with higher M-IMT and m-IMT. CONCLUSIONS: In patients with SLE some non-traditional risk factors for atherosclerosis were identified, the most important of which was the cumulative prednisone dose. The role of some traditional risk factors, such as age and hypertension, was also confirmed. The predictive value of the new immunological and inflammatory markers of atherosclerosis seems to be masked by some disease related features.

Adult↗
PubMed2002

Lack of multimer organization of von Willebrand factor in an acquired von Willebrand syndrome.

We report a case of acquired von Willebrand syndrome (AVWS) in a 20-year-old-woman with systemic lupus erythematosus, in whom severe bleeding complications followed kidney biopsy. Coagulation studies demonstrated undetectable levels of ristocetin-induced platelet aggregation (RIPA), von Willebrand factor antigen (VWF:Ag) and VWF ristocetin cofactor activity (VWF:RCo), associated with significantly prolonged bleeding time; unlike type 3 von Willebrand disease (VWD), platelet VWF was reduced but not undetectable. The plasma VWF multimer pattern was characterized by the presence of only two bands, one of low molecular weight (MW) running as the protomer of plasma VWF in normals, the other of abnormally high MW without detectable intermediate multimers; this pattern resembles that of VWF present in endothelial cells. A search for an anti-VWF antibody demonstrated the presence of an inhibitor at high titre. This anti-VWF antibody did not interfere in the interaction of VWF with platelet glycoprotein (GP) Ib through the A1 domain, and did not react with the A2 domain of VWF; instead, it seemed to modify the relative representation of high and low MW VWF multimers released by normal human umbilical vein endothelial cells (HUVEC). After Azathioprine and corticosteroid treatment, the anti-VWF antibody disappeared and the patient's haemostatic profile normalized, except for the platelet VWF content which still remained decreased. We suggest that the anti-VWF antibody present in the AVWS described compromised both circulating VWF levels and their multimeric organization, inducing the maintenance of the multimer structure that VWF normally has before or in the early phase after secretion from endothelial cells.

Adult↗
PubMed2002

Pregnancy outcome in 100 women with autoimmune diseases and anti-Ro/SSA antibodies: a prospective controlled study.

Anti-Ro/SSA antibodies are associated with neonatal lupus but are also considered a possible cause for unexplained pregnancy loss and adverse pregnancy outcome. In a large multicentres cohort study we have prospectively followed 100 anti-Ro/SSA positive women (53 systemic lupus erythematosus (SLE)) during their 122 pregnancies and 107 anti-Ro/SSA negative women (58 SLE) (140 pregnancies). Anti-Ro/SSA antibodies were tested by immunoblot and counterimunoelectrophoresis. Mean gestational age at delivery (38 vs 37.9 weeks), prevalence of pregnancy loss (9.9 vs 18.6%), preterm birth (21.3 vs 13.9%), cesarean sections (49.2 vs 53.4%), premature rupture of membranes (4.9 vs 8.1%), preeclampsia (6.6 vs 8%), intrauterine growth retardation (0 vs 2.3%)and newborns small for gestational age (11.5 vs 5.8%) were similar in anti-Ro/SSA positive and negative SLE mothers; findings were similar in non-SLE women. Two cases of congenital heart block were observed out of 100 anti-Ro/SSA positive women. In conclusion, anti-Ro/SSA antibodies are responsible for congenital heart block but do not affect other pregnancy outcomes, both in SLE and in non-SLE women. The general outcome of these pregnancies is now very good, ifprospectively followed by multidisciplinary teams with ample experience in this field.

Adult↗
PubMed2001

Risk of congenital complete heart block in newborns of mothers with anti-Ro/SSA antibodies detected by counterimmunoelectrophoresis: a prospective study of 100 women.

OBJECTIVE: To assess the true prevalence of congenital complete heart block (CCHB) in infants of anti-Ro/SSA-positive women known to have connective tissue disease (CTD) and, secondarily, to evaluate the prevalence of other electrocardiographic abnormalities in these newborns at birth. METHODS: A prospective study was conducted in 4 referral hospitals. One hundred anti-Ro/SSAA-positive mothers were followed up before they became pregnant and during the index pregnancy. Counterimmunoelectrophoresis and immunoblotting were used to test for antibodies to extractable nuclear antigens. RESULTS: Of the 100 women with anti-Ro/SSA antibodies, 2 had infants who developed CCHB in utero (2%). The CCHB was detected at 22 weeks and 20 weeks, respectively. One of the 2 mothers had primary Sjögren's syndrome (SS), and the other had undifferentiated CTD (UCTD). No case of CCHB occurred among the infants of 53 mothers with systemic lupus erythematosus (SLE). No fetal death occurred due to CCHB. In 2 centers, electrocardiography was recorded in 24 unselected newborns, and 4 were found to have sinus bradycardia. CONCLUSION: The prevalence of CCHB in newborns of prospectively followed up women already known to be anti-Ro/SSA positive and with known CTD was 2%. This finding is useful with regard to preconception counseling of these women. The risk of delivering an infant with CCHB may be higher in mothers with primary SS or UCTD than in those with SLE. Additional electrocardiographic abnormalities such as sinus bradycardia and prolongation of the QT interval may be present in their children.

Antibodies, Antinuclear↗
PubMed2000

The use of Tween 20 in immunoblotting assays for the detection of autoantibodies in connective tissue diseases.

Autoantibodies directed against intracellular antigens can be detected by immunoblotting (IB). Due to its high sensitivity this technique has many advantages, but it can give misleading results when the specific bands are weak or blurred against the background staining. To decrease background staining, non-ionic detergents (Tween 20, Triton X-100, Nonidet P-40) are generally used as blocking agents. Moreover, these agents appear to have a renaturating action towards proteins and antigens. Tween 20 has a more pronounced renaturating effect on proteins than other detergents and thereby improves antigen-antibody binding. To evaluate the effect of Tween 20 on specific autoantibody detection by IB, we tested the sera of 162 patients with connective tissue diseases (CTDs) by adding this detergent at certain steps of the IB assay. We found that the use of Tween 20 in the IB procedure significantly improved the binding of autoantibodies to Jo-1, Scl70, (U1)RNP 68 kDa and C, Sm B/B' and D. Moreover, it increased the sensitivity for the detection of anti-Sm D peptide in systemic lupus erythematosus (SLE) sera with no decrease in specificity. In contrast, the addition of Tween 20 significantly decreased the binding of autoantibodies specific for ribosomal P proteins, La/SSB, Ro/SSA, but not the overall sensitivity and specificity of the method. We conclude that the addition of Tween 20 to standard IB is advantageous for anti-nuclear antigen antibody detection and improves the sensitivity of the method in revealing anti-Sm-positive sera in SLE. However, Tween 20 is not recommended for the detection of anti-cytoplasmic antibodies.

Autoantibodies↗
PubMed2000

Psoriatic arthritis exacerbated by Salmonella infection.

Psoriatic arthritis (PsA) is an inflammatory joint disease in which environmental factors, particularly trauma and infections, are thought to play an important role. The authors describe the case of a patient with a mild and long-untreated form of PsA which was severely exacerbated by Salmonella typhimurium infection. This case confirms the importance of infectious agents in the occurrence and course of PsA.

Adult↗
PubMed2000

Anti-ribosomal P protein antibodies detected by immunoblotting in patients with connective tissue diseases: their specificity for SLE and association with IgG anticardiolipin antibodies.

OBJECTIVE: To assess the prevalence and clinical and serological associations of anti-ribosomal P protein antibodies (anti-P antibodies) in patients with connective tissue diseases (CTDs) and investigate the immunobiological nature of autoantibody clustering in which anti-P antibodies play a part. METHODS: IgG anti-P antibodies in the sera of 267 patients with CTDs and 31 healthy subjects were analysed by immunoblotting performed on cytoplasmic extract of Raji cells. 60 patients with systemic lupus erythematosus (SLE), 32 systemic sclerosis, 46 primary Sjögren's syndrome, 16 poly/dermatomyositis, 11 rheumatoid arthritis, 8 undifferentiated CTD, 72 overlap CTD, and 22 primary antiphospholipid syndrome were studied. Anti-P antibodies were affinity purified by elution from nitrocellulose bound antigen and tested by ELISA for their binding activity to cardiolipin. RESULTS: Anti-P antibodies were detected in 16 (6%) patients and in none of the controls: 12/60 SLE (20%) and 4/80 undifferentiated/overlap patients with CTD (5%). A close association of IgG antibodies with P proteins and with cardiolipin was seen in lupus sera (p=0.0009, odds ratio 18.33). Anti-P antibodies from 9 of 12 anti-P lupus serum samples could be affinity purified and none of the affinity purified fractions cross reacted with ELISA plate coated cardiolipin. CONCLUSIONS: Anti-P immunoreactivity is a specific marker of SLE and lupus-like disease and its detection is recommended as a powerful diagnostic tool. Anti-P antibodies are strongly clustered with IgG anticardiolipin antibodies in lupus sera, even if they are independently elicited. This suggests that their cognate autoantigens play a part in a common pathogenetic pathway in SLE.

Antibodies, Anticardiolipin↗
PubMed1999

Clinical and laboratory manifestations of elderly onset psoriatic arthritis: a comparison with younger onset disease.

OBJECTIVE: Although the influence of age on clinical and laboratory features has been widely demonstrated in many arthropathies, studies on elderly onset (> 60 years) psoriatic arthritis (EOPsA) are rare. This study compares manifestations at onset and two year outcome of EOPsA with those of younger onset PsA (YOPsA). PATIENTS AND METHODS: Sixty-six consecutive PsA patients with disease duration < 1 year, 16 EOPsA (> 60 years) and 50 YOPsA (< or = 60 years) were admitted to a prospective study. Clinical, laboratory, and radiographic assessment were carried out at admission and after two years. HLA class I and bone scintigraphy were also recorded. In 10 patients with EOPsA and 24 with YOPsA it was possible to obtain synovial fluid, which was subsequently analysed for local inflammatory indices, including interleukin (IL) 1 beta, IL6, and IL8. RESULTS: Presenting manifestations of EOPsA differed from YOPsA in number of active joints (mean (SD)) (12.2 (6.3) v 6.7 (4.6), p < 0.001), foot bone erosions (2.7 (1.2) v 1.1 (1.1), p < 0.001), erythrocyte sedimentation rate (64.2 (35.3) v 30.5 (30.0) mm 1st h, p < 0.001), C reactive protein (3.9 (2.0) v 1.3 (1.3) mg/dl, p < 0.001) and synovial fluid IL1 beta (8.0 (4.7) v 3.0 (3.0) pg/ml, p < 0.001) and IL6 (828.2 (492.6) v 469.3 (201.4) pg/ml, p < 0.005). No differences were found in the number of subjects with dactylitis, pitting oedema, HLA-B27, or signs of sacroiliac and sternoclavicular joint involvement at bone scintigraphy. After two years, progression was more evident in EOPsA than in YOPsA, as the number of new erosions in the hands and also the C reactive protein were higher in EOPsA patients. CONCLUSION: PsA has a more severe onset and a more destructive outcome in elderly people (onset > 60 years) than in younger subjects. This behaviour may be influenced by immune changes associated with aging, as suggested by the higher concentrations of IL1 beta and IL6 found in the synovial fluid of EOPsA than in YOPsA.

Adult↗
PubMed1996

Blotting patterns of IgG anti-(U1)RNP antibodies in mixed connective tissue disease.

Serum reactivities towards individual U1 snRNP proteins were determined by immunoblotting in 32 patients with mixed connective tissue disease (MCTD). Time persistence of immunoblot profiles and clinical significance of anti-(U1)RNP antibody specificities were also investigated. IgG anti-(U1)RNP antibodies were found in the sera of 29 out of 32 patients (90.6%): 21 (65.6%) reacted with the 70-kD protein, 25 (78.1%) with A, 23 (71.9%) with C and 20 (62.5%) with B/B' proteins. None were reactive with the Sm-D peptide. Seventy kilodalton antibody specificity was strongly associated with a higher antinuclear antibody titre (> 160) and slightly associated with disease activity; anti-B/B' specificity was associated with lymphadenopathy. Anti-A, -C and -B/B' antibodies were negatively associated with systemic lupus erythematosus (SLE) skin rashes. Two types of anti-(U1)RNP blotting patterns were selected: "full spectrum" (53.1% of cases) and a "partially/no reactive" one (46.9%). Such patterns were unchanged over time in 14 out of 16 cases prospectively examined (87.5%), while the pattern shifted from "full spectrum" to "partially/no reactive" in 2 cases (12.5%): in 1 after a prolonged clinical remission (> or = 4 years) and in the other following immunosuppressive therapy. The anti-(U1)RNP antibody immunoblot profile in MCTD patients consisted of various reactivities and remained unchanged over time in most cases. Antibody reactivity against the 70-kD protein represented the major U1 snRNP specificity. The various anti-(U1)RNP specific reactivities demonstrated poor clinical significance within MCTD. Thus, MCTD seems to be characterized by a longstanding serological heterogeneity whose reactivities do not apparently correspond to distinct features within the broad clinical spectrum of MCTD.

Adult↗
PubMed1996

Photosensitivity in systemic lupus erythematosus: laboratory testing of ARA/ACR definition.

OBJECTIVE: The aim of our study was to evaluate the prevalence of photosensitivity in SLE as defined by either clinical or laboratory assessment, the concordance of findings obtained by two methods, and the relationship between photosensitivity and clinical and immunological parameters. METHODS: Forty-four SLE patients and 31 healthy subjects were included. Patients and controls underwent a standard questionnaire testing and the minimal erythemal dose (MED) measurement performed by Dermalight-Blue Point. The standard questionnaire was designed in order to meet, as near as possible, the definition of photosensitivity included in the ARA/ACR criteria for classification of SLE. RESULTS: The prevalence of photosensitivity was (patients vs controls): 57% vs 45% according to questionnaire; 79.5% vs 51.6% (P = 0.02) according to MED. The agreement between questionnaire and phototest was absent in SLE (kappa 0.01) and poor in controls (kappa 0.36). Discoid rash was significantly associated with questionnaire positive (P = 0.01) and renal involvement with questionnaire negative results (P = 0.02), serositis with MED abnormality (P = 0.03), malar rash and anti-Sm antibody with MED normal values (P = 0.03 and P = 0.01), respectively). Moreover, by multivariate analysis, malar rash and anti-Sm antibody significantly predicted MED-defined photosensitivity, with probability ranging from 42% (presence of both) to 92% (lack of both). CONCLUSIONS: Photosensitivity is frequently observed in SLE patients as well as in healthy subjects. Its prevalence is significantly higher in SLE than in controls only when it is detected using the laboratory method. However, due to the difficulty in objectively defining such manifestation, the disagreement between questionnaire and MED results was high and its clinical meaning appears ambiguous. Thus, the use of photosensitivity as a classification criterion for SLE remains questionable, at least when it is assessed according to the ARA/ACR definition.

Adolescent↗
PubMed1996

Pancreatic exocrine involvement in primary Sjögren's syndrome.

Exocrine pancreatic involvement of primary Sjögren's syndrome (SS) was studied. Pancreatic enzyme levels (total amylase, pancreatic isoamylase and immunoreactive trypsin) along with anti-ductuli antibodies (Ab) were studied in 77 patients with primary SS. In 10 patients with normal and 10 with abnormal enzyme levels pancreatic CT scans were also obtained. All enzyme levels were significantly increased in comparison to the control group. Immunoreactive trypsin was found to be the most frequently increased enzyme (35.3% of pSS patients). Anti-ductuli Ab were not found in any patient. Pancreatic CT scans were normal in all subjects with enzymatic increase, whereas 2 abnormal scans were demonstrated in patients without enzyme changes. Our study suggests that exocrine pancreatic involvement is frequent when measured by enzyme levels. Nevertheless, we were unable to demonstrate any morphological lesion by CT scan.

Abdominal Pain↗
PubMed1996

Thyroid gland disorders in primary Sjögren's syndrome.

OBJECTIVES: To evaluate the frequency of thyroid disorders in primary Sjögen's syndrome. PATIENTS AND METHODS: 121 consecutive patients meeting Vitali's criteria for primary Sjögren's syndrome and 74 with rheumatoid arthritis underwent thyroid hormone assays, tests for antimicrosomal and antithyroglobulin antibodies, tests for antinuclear antibodies and antibodies to extractable nuclear antigens. Antimicrosomal and antithyroglobulin antibodies were also assayed in 404 controls. RESULTS: frequencies were calculated separately in males and females, and data in females were subjected to statistical analysis. As compared with controls, Sjögren's syndrome patients were more likely to have antimicrosomal antibodies (9% versus 17.6%, P < 0.05) and both Sjögren's syndrome and rheumatoid arthritis patients were more likely to have antithyroglobulin antibodies (1% versus 13.4% and 10.9%, respectively, P < 0.0001). Hypothyroidism was more common among Sjögren's syndrome patients (13.4%) than rheumatoid arthritis patients (3.1%) (P < 0.05). Sjögren's syndrome patients with thyroid disorders were less likely to have antinuclear antibodies, rheumatoid factors or a Chisholm's stage 3 or 4 lip biopsy. CONCLUSIONS: our data confirm that thyroid disorders are more common in primary Sjögren's syndrome than in rheumatoid arthritis and controls. Production of autoantibodies and severe histologic lesions were less common in Sjögren's syndrome patients with than without thyroid disorders.

Adult↗
PubMed1995

Fibrinolysis and coagulation abnormalities in systemic lupus erythematosus. Relationship with Raynaud's phenomenon, disease activity, inflammatory indices, anticardiolipin antibodies and corticosteroid therapy.

Endothelial cell damage in systemic lupus erythematosus (SLE) was evaluated by measuring fibrinolytic activity and von Willebrand factor levels. Tissue-type plasminogen activator (t-PA) antigen, plasminogen activator inhibitor (PAI) activity, and von Willebrand factor antigen (vWF:Ag) and activity (vWF:RCof) were measured in 21 SLE patients (12 of whom were therapy free) and 22 controls. In addition, the relationship between such parameters and Raynaud's phenomenon, disease activity [according to personal criteria, Systemic Lupus Activity Measure (SLAM) and European Consensus Lupus Activity Measurement (ECLAM) scores] inflammatory indices [ESR, C-reactive protein (CRP), alpha 2-globulin], anticardiolipin antibodies and corticosteroid therapy was investigated. Lower levels of t-PA antigen (P = 0.003) and higher levels of vWF:Ag (P = 0.001) were found in SLE patients in comparison with controls. Moreover, t-PA antigen was lower (P = 0.02) in steroid-free patients in comparison with those taking steroids. No relationship was found between fibrinolysis and coagulation abnormalities and Raynaud's phenomenon, disease activity, inflammatory indices and anticardiolipin antibodies. Endothelial cell damage is probably a common feature in SLE patients; nevertheless, we were unable to clarify the nature of such abnormality. It is worth noting that low doses of steroids seem to be effective in improving endothelial cell function in SLE patients.

Adolescent↗