[Case records of injuries from 1 July 1962 to 31 December 1968].
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Accidents, Home↗
Biomedical subjects
R Caporali
Publications and source records attributed to R Caporali.
Severe retinopathy in systemic lupus erythematosus associated with IgG anticardiolipin antibodies.
A six-year-old boy with systemic lupus erythematosus developed insidiously a monolateral serous retinal detachment leading to severe visual loss. Serial determinations of anticardiolipin antibodies showed the presence of IgG anticardiolipin antibodies only at the time of ocular involvement. This case underlines the need for regular ophthalmologic evaluation in children with active systemic lupus erythematosus and supports the previous hypothesis of a relationship between antiphospholipid antibodies and retinal involvement in systemic lupus erythematosus.
Antibodies, Anticardiolipin↗
Anticardiolipin antibodies in first-degree relatives of type 1, insulin-dependent, diabetic patients.
First-degree relatives (FDRs) of diabetic patients are at risk of IDDM, and frequently present several autoantibodies. We detected anticardiolipin antibodies (aCL) in 42 FDRs, aged 12.4 +/- 4.2 years and in 52 controls. aCL (IgG and IgM) were measured by ELISA and their results expressed in arbitrary units. All FDRs underwent islet cell antibodies (ICA) measurement, intravenous glucose tolerance test and HbA1c levels. HLA typing and HLA-DQ molecular analysis were performed in all FDRs. Positive levels of aCL-IgG were observed in 8/42 FDRs and no control subject (p = 0.04); aCL-IgM values were similar in FDRs and controls. No correlation was found between aCL levels and chronologic age or HbA1c levels. No association was observed between aCL frequency and immunologic (ICA), metabolic or genetic (HLA) parameters. No FDR showed any feature of antiphospholipid syndrome. aCL-IgG presence in FDRs is suggestive of a need to carry out a follow-up study to establish the significance of these antibodies.
Adolescent↗
[Early electroretinografic changes in elderly RA patients treated with hydroxychloroquine].
OBJECTIVE: To evaluate the effectiveness of fundoscopy, electrooculography, electroretinogram and visually evoked potentials in early detection of hydroxychloroquine retinal toxicity in RA patients and to evaluate the influence of patients' age, drug dosage, concomitant therapy (prednisone and methotrexate) and serum creatinine levels in the development of this side effect. METHODS: From september to december 1999, we have enrolled 32 RA patients (13 males, 19 females) starting hydroxychloroquine treatment. The patients underwent regular ophthalmological examination (fundoscopy, electro-oculography, electroretinogram and visually evoked potentials) every 4 months. Disease activity was evaluated every two months by clinical and routine serological examination. RESULTS: No patients developed retinopathy during 1 year's follow-up; fundoscopy, electrooculography, and visually evoked potentials did not vary from the baseline. On the other hand, electroretinogram showed early alterations of scotopic and photopic response; moreover a significant statistical correlation between patients' age (more than 65 years) and b1 photopic wave increase (p < 0,05) was observed. No correlation was found between the development of electro-retinographic alterations and hydroxychloroquine dosage, concomitant therapy and serum creatinine levels CONCLUSION: Our data show the inefficacy of fundoscopy, electrooculography and visually evoked potentials in early detection of hydroxychloroquine retinopathy. On the other hand electroretinogram allows early detection of retinal alterations during hydroxychloroquine treatment, in patients older than 65 years.
Adult↗
[TNF-alpha inhibition in anti-Ro/SSA positive patients with rheumatoid arthritis: clinical and immunologic effects].
OBJECTIVE: To analyse efficacy and safety of anti-TNFalpha treatment in 17 patients with rheumatoid arthritis (AR) and anti-Ro antibodies, in order to detect difference in clinical and immunological response. METHODS: 322 patients, affected by RA and treated with anti-TNFalpha drugs, were considered, searching every 6-12 months ANA, anti-dsDNA and anti-ENA antibodies. Seventeen were anti-Ro positive and 305 anti-Ro negative before starting treatment. RESULTS: Anti-Ro positive subjects showed active arthritis at baseline (mean DAS: 5), with frequent extra-articular features, such as ocular and oral sicca symptoms. They showed rapid and stable improvement during the treatment, with-out significant difference compared to anti-Ro negative group. A good clinical Eular response was shown in 46% of anti-Ro negative subjects, steady stable during time. On the contrary, fewer anti-Ro positive patients seem to be "good" responders. RA remission (DAS <1,6) was achieved in 9-25% of anti-Ro positive and 21-29% of anti-Ro negative, without significant difference. Antinuclear antibodies tend to increase in both groups, during the time. Anti-DNA increased to 40% of anti-Ro positive sera since 6th month, while they slightly increased in first 12 months in anti-Ro negative ones, then decreased to baseline value. No differences were shown about the frequency and reasons of anti-TNFalpha withdrawal, except for cutaneous lupus-like disease, more detected in anti-Ro positive group. CONCLUSIONS: Anti-TNFalpha drugs are effective in anti-Ro positive RA as well as other RA patients. Anti-DNA positivity and lupus-like disease were more frequently observed in anti-Ro positive group.
Antibodies, Antinuclear↗
Impaired diastolic function in active rheumatoid arthritis. Relationship with disease duration.
OBJECTIVE: Using digitized M-mode and Doppler echocardiography, we evaluated left ventricular (LV) function in 54 patients (43 women and 11 men; mean age 50 years) suffering from active rheumatoid arthritis (RA) without obvious cardiovascular disease, and compared them with 54 age- and sex-matched normal subjects. RESULTS: No differences were found in LV end-diastolic diameter, systolic function and parietal thickness between the patients and controls. However, a significant reduction in various indexes of LV diastolic function was found, including E/A (ratio of early to late filling waves of mitral inflow Doppler) and the peak lengthening rate of the LV diameter (an index of LV relaxation evaluated by M-mode echocardiography). The former was correlated with patient age and was independent of disease duration, while the latter was more markedly correlated with disease duration than with patient age. CONCLUSION: The relationship between diastolic impairment and disease duration in active RA may open new perspectives in the study of RA-associated cardiovascular disease.
Adult↗
Infliximab in the treatment of adult Still's disease refractory to conventional therapy.
In this study we evaluated the efficacy of Infliximab in the treatment of adult Still's disease (ASD) refractory to conventional therapy. Three patients with chronic and active ASD unresponsive to corticosteroids and methotrexate were given intravenous Infliximab infusions at a dosage of 3 mg/kg at weeks 0, 2, 6 and then once every 8 weeks. Methotrexate was maintained in all cases at a dosage of 15 mg/week, whereas the prednisone dose was modified according to disease activity. The follow-up lasted 50 weeks and disease activity improved in all cases during Infliximab therapy. Two patients presented arthralgias and sore throat at 20 and 28 weeks, that was rapidly controlled by Infliximab reinfusion every 4 weeks. One patient relapsed at 18 weeks and dropped out at 22 weeks due to an urticarioid rash after the beginning of the fifth infusion. Infliximab may be effective in the treatment of relapse of ASD refractory to conventional therapy and requiring continuous high dose corticosteroid medication. Further studies are needed to evaluate the long-term safety, efficacy and the optimal schedule of infusion.
Adult↗
Undifferentiated connective tissue disease with antibodies to Ro/SSa: clinical features and follow-up of 148 patients.
OBJECTIVE: To evaluate the clinical and serologic profile, the rate of progression to well defined CTD and the possible predictors of disease evolution in patients affected by UCTD with antibodies anti-RoISSA. METHODS: 148 patients diagnosed as UCTD were retrospectively evaluated. Antibodies to SSA/Ro were determined by counter-immunoelectrophoresis and ELISA. RESULTS: Thirty-six patients (24.3%) developed a well-defined CTD after a mean follow-up of 4.5 years. Most patients developed primary Sjögren's syndrome (SS) (50%) or systemic lupus erythematosus (SLE) (30.5%). Leukopenia and xerophthalmia developed more frequently in the group of patients evolving to defined CTDs (p < 0.0032 and p < 0.0063). Leukopenia independently predicted the evolution in CTD by multivariate regression analysis (p < 0.019). Anti-dsDNA predicted the evolution in SLE (p < 0.0207), while the presence of additional anti-ENA specificity to anti-Ro/SSA was not associated with the outcome. CONCLUSION: 24.3% of patients with UCTD and antibodies to Ro/SSA can progress in a relatively short period of time to well-defined CTDs. The development of primary SS could be predicted by xerophthalmia and SLE by the appearance of anti-dsDNA antibodies.
Adolescent↗
Salivary gland lymphocytic infiltrates and Helicobacter pylori serology in anti-SSA/Ro positive patients in Italy.
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Antibodies, Antinuclear↗
Autoantibodies to RNA-polymerases in Italian patients with systemic sclerosis.
OBJECTIVE: To assess the frequency and clinical correlates of the systemic sclerosis-related autoantibodies to RNA polymerases in Italian patients. METHODS: Sera from 115 patients with systemic sclerosis (SSc) and 10 patients with systemic sclerosis-overlap syndromes recruited from a single center in northern Italy were investigated for antibodies to RNA polymerase I, II, and III by means of immunoprecipitation using 35S-labeled HeLa cell antigen extract. Twenty-five normal volunteers and 91 patients with different connective tissue diseases were studied as a control group. RESULTS: Antibodies to RNA-polymerases were found in 14/115 SSc patients (12.1%). None of the normal controls and none of the patients with other connective tissue diseases, including overlap syndromes, were positive. Antibodies reacting with RNA-polymerase I and III (+/- RNA-polymerase II) were found in 9/115 patients (7.8%) and were mutually exclusive with respect to other scleroderma-related autoantibodies. Isolated anti-RNA polymerase II reactivity was found in 5 patients and was associated with anti-topoisomerase I antibodies in 4 cases. Anti-RNA-polymerase I and III antibodies were associated with diffuse cutaneous involvement and male gender. Only two patients from our series had scleroderma renal crisis, and one of them had anti-RNA polymerase antibodies. CONCLUSIONS: Anti-RNA-polymerase antibodies appear to be less frequent in Italian patients than in Caucasian patients from the United Kingdom or USA. This might be associated with the lower frequency of scleroderma renal crisis.
Adult↗
Role of chemokines and chemokine receptors in regulating specific leukocyte trafficking in the immune/inflammatory response.
Antigen recognition, lymphocyte priming and effector responses in inflamed tissues depend on a coordinated and sequential series of events that take place in different anatomical compartments. The integration of these processes is favoured by the dynamic capacity of leukocytes to recirculate between the bloodstream and specific organs and to navigate inside the tissues in a programmed fashion, regulated by a complex interaction of cell adhesion molecules and soluble chemoattractants, in particular chemokines. In this review we discuss the role of chemokines and chemokine receptors in regulating leukocyte trafficking in different anatomical sites in the context of distinct functional phases of the immune/inflammatory response.
Autoimmune Diseases↗
Direct and indirect costs of osteoarthritis of the knee.
BACKGROUND: Rheumatic diseases have an economic impact of 1-2.5% of GDP in industrialized countries and osteoarthritis is the most common joint disorder. Osteoarthritis of the knee is especially common and is a major cause of disability requiring extensive utilization of health care resources. OBJECTIVE: To estimate the burden of osteoarthritis of the knee in Italy, we studied retrospectively a cohort of 254 patients over a period of 12 months in 2000-2001. METHODS: Twenty-nine rheumatology institutes took part in the study. A bottom-up approach was used, analytically measuring pro capita consumption. We considered medical (hospitalization, diagnosis, and therapies) and non-medical costs (transport, temporary caregivers, and auxiliary devices) as direct costs. We calculated losses of productivity borne by patients and caregivers, and informal care provided by caregivers as indirect costs. RESULTS: Direct costs came to Euros 934 per patient per year: Euros 233 were spent on hospitalization, Euros 209 on diagnostic procedures (56% on visits and 44% on instrumental and laboratory tests), Euros 146 on therapy (58% on physiotherapy and 42% on drugs), and Euros 346 on non-medical costs (73% on salaries to temporary caregivers, 14% on transport, and 13% on auxiliary devices). It is interesting to point out that at least 37% of costs were charged directly to patients. Indirect costs were almost 30% higher and came to Euros 1236 per patient per year: 31% was due to loss of productivity of patients, 60% due to informal care provided by primary caregivers, and 9% by other caregivers. CONCLUSIONS: This study confirms that the direct and indirect costs attributable to osteoarthritis of the knee are substantial.
Adult↗
Antibodies to hn-RNP protein A1 in systemic lupus erythematosus: clinical association with Raynaud's phenomenon and esophageal dysmotility.
Antibodies to recombinant hn-RNP protein A1 were found by ELISA in sera from 26 out of 67 unselected patients with systemic lupus erythematosus. A higher number of anti-A1 positive patients had Raynaud's phenomenon (50% vs 7%) and esophageal dysmotility (42% vs 5%) than the anti-A1 negative patients. All 8 patients with both Raynaud's phenomenon and esophageal dysmotility had a positive anti-A1 assay. No association was found with other clinical findings, nor with disease activity and treatment regimes. Anti-A1 antibodies did not correlate with anti-RNP and anti-Sm antibodies, which were present in 30% and 12% of the anti-A1 positive cases and in 22% and 7% of the anti-A1 negative cases, respectively. Our results indicate that antibodies to hn-RNP protein A1 may be associated with a subset of SLE patients with clinical features overlapping those of progressive systemic sclerosis and quite distinct from the group identified by anti-RNP antibodies.
Adolescent↗
Cyclosporine in addition to infliximab and methotrexate in refractory rheumatoid arthritis.
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Adult↗
Diagnostic value of procalcitonin measurement in febrile patients with systemic autoimmune diseases.
OBJECTIVE: To determine the usefulness of plasma procalcitonin (PCT) measurement to suspect infectious etiology in febrile patients with systemic autoimmune disease. METHODS: PCT, C-Reactive protein (CRP), erythrocyte sedimentation rate (ESR) and white blood cell count (WBC) were measured in 44 consecutive inpatients with a diagnosis of systemic autoimmune disease and fever >38 masculine C. After careful microbiologic screening no obvious infection was demonstrated in 24 patients (Group A) while an infectious bacterial complication was diagnosed in 20 cases (Group B). RESULTS: Median PCT levels were significantly higher in the group B (1.11 vs 0.24 ng/ml; p = 0.0007), whereas the differences for CRP, WBC and ESR did not reach statistical significance. PCT also exhibited a good sensitivity and specificity (75%) in differentiating patients with infection from those with disease flare. With respect to positive and negative predictive values (71.4% and 78.2%), PCT markedly exceeded the other variables. By analyzing PCT values by disease we identified a false positive subgroup of patients suffering from adult onset Still's disease (AOSD), showing markedly elevated PCT levels in absence of infection. By excluding these patients, PCT showed a very good sensitivity and specificity (73.6% and 89.4%) and the area under receiver operating characteristics (ROC) curve rose from 0.801 to 0.904. CONCLUSION: Our data indicate that elevated PCT concentrations offer good sensitivity and specificity for the diagnosis of systemic bacterial infection in febrile patients with systemic autoimmune diseases. However, in fever associated with AOSD PCT may be elevated even in the absence of infectious complication.
Autoimmune Diseases↗
Purpura fulminans as clinical manifestation of atypical SLE with antiphospholipid antibodies: a case report.
Purpura Fulminans and DIC were the main clinical manifestations of the antiphospholipid syndrome observed in a 62-year-old man. The patient was well until 44 years of age when he began to suffer from recurrent thrombophlebitis, without other symptoms suggestive of immune disease. At the time of hospital admission the pt. appeared acutely ill, showing high fever, severe anemia, massive urinary blood loss, multiple purpuric patches evolving to hemorrhagic bullae and gangrene rapidly spreading over about 30% of the total body area. No signs of neurological involvement or of visceral thrombotic occlusions were present. Clotting tests were consistent with a diagnosis of DIC, further confirmed by skin biopsy showing the presence of thrombi in dermal arterioles. The autoantibody research was positive as follows: Waaler-Rose 1:40, Anti-DNA 1:80; ANF 1:640, aCA IgG 100 GPL. LA was diagnosed according to standard criteria: prolonged KCT and RVVT not corrected by a mixture of normal plasma and abnormal TTI. Plasma exchange in association with heparin and prednisone was effective in arresting the progression of the skin lesion; nevertheless the patient died ten days after hospital admission for sepsi and acute renal failure.
Autoantibodies↗
Anticardiolipin syndrome in childhood: a report of two cases.
We describe two children with an otherwise unexplained deep vein thrombosis associated with high titer anticardiolipin antibodies (ACA) of the IgG class and circulating lupus anticoagulant (LAC). One of these patients had persistent proteinuria but neither had systemic lupus erythematosus. Our observation suggests that ACA and LAC assays should be performed in children with thromboembolic disease even when no underlying autoimmune disease can be found.
Adolescent↗
Autoantibodies in juvenile dermatomyositis.
Fourteen patients with juvenile dermatomyositis (JDM) have been investigated for the presence of several serum autoantibodies: antinuclear (ANA), anti-single-stranded and double-stranded DNA, anti-histones, anti-Sm, anti-ribonucleoprotein, anti-SSA/SSB, anti-PM-1, anti-Jo-1, anti-mitochondrial, anti-smooth muscle, anti-gastric parietal cells, anti-cardiolipin (ACA) antibodies and rheumatoid factor. Patients were negative for all autoantibodies except for ANA and ACA. ANA were detected in 50% of the patients when tested on rat liver, but the percentage of positivity rose to 86% when HEp-2 cells were used as substrate. This finding suggests that HEp-2 cells represent a more sensitive substrate than rat liver for the detection of ANA in JDM. Three patients were positive for ACA; two of these presented vascular complications, thus suggesting a possible relationship between ACA and vascular involvement in JDM.
Antibodies, Antinuclear↗