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R Cimaz

Publications and source records attributed to R Cimaz.

At least 37 records · Page 2Linked to original sources
PubMed2002

Prevalence and clinical significance of anti-cyclic citrullinated peptide antibodies in juvenile idiopathic arthritis.

BACKGROUND: Antibodies against cyclic citrullinated peptide (anti-CCP) are considered to be specific for rheumatoid arthritis (RA). OBJECTIVE: To assess the clinical significance of anti-CCP in a cohort of patients with juvenile idiopathic arthritis (JIA). METHODS: Anti-CCP were tested by an enzyme linked immunosorbent assay (ELISA) in serum samples from 109 patients with JIA (30 boys, 79 girls), with a mean age of 8.7 years (range 0.6-20.3) and mean disease duration of 3.6 years (range 3 months to 15.6 years). As control groups, anti-CCP were also tested in sera of 30 healthy children, 25 patients with juvenile onset systemic lupus erythematosus (SLE), and 50 adult patients (30 with RA, 20 with SLE). RESULTS: Positive anti-CCP values were found in sera of two patients with JIA (2%), one with polyarthritis, and one with oligoarthritis. Statistical analysis showed that anti-CCP were not associated with the presence of antinuclear antibodies, raised erythrocyte sedimentation rate, or erosions. In the control groups, none of the patients with juvenile onset SLE and only one of 20 adults with SLE were positive for anti-CCP, but 19/30 (63%) adults with RA showed anti-CCP positivity. CONCLUSIONS: Anti-CCP can be detected in children with JIA, but are less frequently present than in adults with RA.

Adolescent↗
PubMed2002

Recent advances in antiphospholipid antibodies and antiphospholipid syndromes in pediatric populations.

In recent years, antiphospholipid antibodies (aPL) and their associated clinical features have been recognized increasingly in various pediatric autoimmune and non-autoimmune diseases. Pathogenic mechanisms involved in pediatric antiphospholipid syndrome (APS) appear to be the same as in adults. However, since pediatric patients do not have prothrombotic risk factors present in adults, there clearly are differences in the spectrum of clinical findings. The frequency of aPL-related thrombotic events is generally low in pediatric populations. On the other hand, various commonly acquired infections are likely to be responsible for higher percentage of non-pathogenic and transient aPL in childhood. Such points have to be considered in clinical judgment of elevated aPL in children. In this review we summarize the recent data on the prevalence and clinical significance of aPL in neonates, children and adolescents.

Adult↗
PubMed2002

Ultraviolet light exposure is not a requirement for the development of cutaneous neonatal lupus.

Cutaneous neonatal lupus erythematosus (NLE) is a rare disorder, linked to the presence of transplacentally acquired maternal autoantibodies (anti-ENA). NLE skin lesions frequently appear in the second or third month of life, and ultraviolet exposure is thought to be an initiating factor since it can externalize intranuclear autoantigens at the cell surface. We report a baby who was born already with an extensive NLE rash, suggesting that sun exposure is not a requirement for the development of NLE skin lesions. A 31-year-old woman affected with mixed connective tissue disease gave birth to a female after 38 weeks of gestation. Pregnancy was uneventful and no perinatal complications were seen. The mother was positive for anti-RNP, but negative for anti-SSA/Ro and SSB/La autoantibodies. Already at birth, an extensive scarring rash with a few erythematosus lesions was present on the baby's face and scalp; this progressed over the following months, and subsequently stabilized. Anti-RNP were present in the baby's serum. Due to the unusual features of the disease expression, a skin biopsy was performed at age 5 months; results were consistent with the diagnosis of NLE, showing mononuclear cell infiltration and immunoglobulin deposition. No other features of NLE were detected. This observation is unusual for: (1) the presence of an NLE rash in the absence of anti-SSA/Ro; (2) the scarring and atrophic characteristics of the lesions; and (3) the development already in utero. This latter finding argues against sun exposure being necessary for lesion induction.

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

Unexpected healing of cutaneous ulcers in a short child.

A boy aged 10 years was referred to the Paediatric Department of Milan University Hospital, Milan, Italy, with a long history of pain in the lower limbs, alleviated only by exposure to cold. His legs were swollen, with multiple cutaneous ulcers. He had severe painful crises, and was totally incapacitated. After the diagnosis of erythermalgia was made, numerous treatments were tried, but none were successful. After finding growth hormone (GH) deficiency, we started treatment with recombinant GH. He had immediate relief of pain and complete healing of ulcers. We postulate that the healing of the ulcers can be attributed to the GH-promoting effect on dermal connective tissue.

Child↗
PubMed2001

Safety and immunogenicity of a conjugate vaccine against Haemophilus influenzae type b in splenectomized and nonsplenectomized patients with Cooley anemia.

Patients with thalassemia are at increased risk for infections, especially after undergoing splenectomy. Vaccinations and antimicrobial prophylaxis are recommended in these patients, but the optimal immunization schedule for Haemophilus influenzae type b (Hib) vaccine is unknown. The immunogenicity of a conjugate Hib vaccine was investigated in 57 patients with thalassemia, 32 of whom had undergone splenectomy. Anti-capsular antibodies to Hib (anti-polyribosylribitol phosphate) were measured before vaccination and 2, 6, 12, 24, and 36 months after vaccination. Immunization was well tolerated. All patients achieved protective (>1 microg/mL) antibody levels. Antibody titers declined after the initial postvaccination increase, becoming undetectable in 4 patients and decreasing to concentrations of 0.15-1 microg/mL in another 2 patients when tested 2-3 years after vaccination. Hib conjugate vaccine is safe and immunogenic in patients with thalassemia major; however, additional studies are needed to assess the need and timing of booster vaccination to maintain long-term immunity.

Adolescent↗
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↗
PubMed2001

Osteoporosis.

Bone mass is determined primarily by genetic influences, but exogenous factors may also play a major role. The prevention of osteoporosis can start at childhood. Optimal achievement of peak bone mass during childhood and adolescence is important to minimize future fracture risk. Chronic inflammatory diseases can have a detrimental effect on bone mass through a variety of mechanisms. Different diagnostic methods for detecting osteoporosis (eg, dual x-ray absorptiometry, quantitative computed tomography, ultrasounds) are in use or under investigation. New treatment options are available; among these, the use of bisphosphonates seems to be the more promising approach.

Bone Density↗
PubMed2001

Efficacy and safety profile of cyclosporin A in the treatment of juvenile chronic (idiopathic) arthritis. Results of a 10-year prospective study.

OBJECTIVE: This open prospective trial was performed in order to assess the efficacy and safety of cyclosporin A in the treatment of patients with juvenile chronic arthritis (JCA). METHODS: Thirty-four of the patients enrolled were affected by systemic-onset disease and seven by chronic anterior uveitis associated with JCA. The cyclosporin dose was usually 3-5 mg/kg per day. The average duration of therapy was 1.4 yr, with a maximum of 7.2 yr. RESULTS: The efficacy of treatment was mainly evident in terms of control of fever and reduction of steroid therapy. The benefits with respect to arthritis, laboratory parameters and uveitis seemed to be less clear-cut. Side-effects were frequent but usually mild or reversible. Sixty-six per cent of the study population withdrew from therapy because of inefficacy or side-effects. Eight systemic patients withdrew from therapy owing to complete remission. CONCLUSION: Cyclosporin can be used in the treatment of JCA, its main benefits being the control of fever and a steroid-sparing effect.

Adolescent↗
PubMed2001

Evidence for immune activation against oxidized lipoproteins in inactive phases of juvenile chronic arthritis.

OBJECTIVE: Oxidative stress contributes to joint inflammation and damage in rheumatoid arthritis. In a mobile inflamed joint, exercise induced multiple cycles of hypoxia-reperfusion injury may lead to the creation of a redox environment in which oxido-reductase systems, by NADPH mechanisms, produce highly reactive chemical species (i.e., oxygen free radicals). We investigated 2 endproducts of lipid peroxidation, malonildialdehyde (MDA) and diene conjugates (DC), and the formation of antibodies against oxidized low density lipoproteins (Ab oxLDL) in juvenile chronic arthritis (JCA), and assessed the role of oxidative phenomena in different phases and subsets of this disease. METHODS: To assess the role of oxidative stress in JCA, we measured the endproducts of lipid peroxidation, MDA and DC, by the increase of absorbance at 586 nm and 234 nm, respectively, and the levels of Ab oxLDL by ELISA in the sera of 58 patients with JCA and 21 healthy controls. Due to crossreactivity between Ab oxLDL and anticardiolipin antibodies (aCL), the sera were also tested by a standard ELISA for IgG-aCL. The patients were divided into 3 subsets: 29 with pauciarticular (pauci), 15 with polyarticular (poly), and 14 with systemic (sys) onset disease, and then were subdivided, according to different variables appropriate to each subset, reflecting active and inactive disease, into 30 active (14 pauci, 8 poly, 8 sys) and 28 inactive (15 pauci, 7 poly, 6 sys). RESULTS: Levels of Ab oxLDL were significantly increased in the whole group of patients (566.6 +/- 263.0 vs 206.6 +/- 136.3 mU/ml; p < 0.001) and in each of the type of onset (pauci 660.8 +/- 272.1, p < 0.001; poly 341.3 +/- 134.7, p < 0.01; sys 497.8 +/- 114.8, p < 0.001) compared to controls. Ab oxLDL were higher in the inactive than in the active group (743.5 +/- 231.9 and 404.4 +/- 169.9; p < 0.001). MDA and DC levels were not increased significantly in patients' sera. No patient was positive for IgG-aCL. CONCLUSION: These findings suggest that MDA and DC cannot be considered major markers of oxidative stress in JCA and that the Ab oxLDL may represent a delayed sign of oxidative stress previously induced by the inflammatory process in patients with JCA.

Anti-Inflammatory Agents, Non-Steroidal↗
PubMed2000

QT interval prolongation in asymptomatic anti-SSA/Ro-positive infants without congenital heart block.

OBJECTIVE: To analyze the electrocardiograms (EKGs) of infants born to mothers with anti-SSA/Ro antibodies in order to evaluate the QT interval (the time from the beginning of the QRS complex to the end of the T wave). METHODS: Sera from mothers and children were analyzed for anti-Ro and anti-SSB/La antibodies by enzyme-linked immunosorbent assay (ELISA) and by Western blot analysis. Fine specificity of anti-Ro antibodies was evaluated by solid-phase ELISA against recombinant 52- and 60-kd proteins and by Western blot. A retrospective chart review was conducted for EKG analysis. Twenty-eight EKG tracings (21 from anti-Ro-positive and 7 from anti-Ro-negative infants born to mothers with autoimmune diseases) were analyzed by a single investigator who was blinded to the infant's antibody status. The QT interval was measured and corrected for heart rate according to Bazett's formula. RESULTS: The mean QT interval was significantly longer in anti-Ro-positive than in anti-Ro-negative infants, also after correction for heart rate (QTc) (P = 0.001). Nine of 21 anti-Ro-positive infants and 0 of 7 anti-Ro-negative infants had QTc values above the upper normal limit (440 msec). A 24-hour EKG recording was performed on 5 patients and confirmed the QT prolongation. These infants were subsequently treated with a beta-blocker in order to prevent arrhythmias. CONCLUSION: Infants born to mothers who carry anti-Ro autoantibodies may show QT interval prolongation and should be monitored with EKG during the first months of life.

Adult↗
PubMed2000

Efficacy and safety of alendronate for the treatment of osteoporosis in diffuse connective tissue diseases in children: a prospective multicenter study.

OBJECTIVE: Osteopenia/osteoporosis is being increasingly reported as a complication of many chronic diseases, even in children. In this preliminary study, we evaluated the effect of an oral bisphosphonate (alendronate) on bone mass in children with diffuse connective tissue diseases. METHODS: Thirty-eight children with low bone mass were treated with alendronate for 1 year; 38 children who had the same primary disorders as the study patients but in a less severe form served as untreated control patients. We were also able to evaluate changes in bone mass (before and after alendronate) in 16 of the treated patients whose bone mineral density (BMD) had been routinely measured before the present study was initiated. RESULTS: BMD increased by a mean +/- SD of 14.9 +/- 9.8% (P < 0.002 versus baseline) in the treated patients (reaching the normal range in 13 patients), while the BMD was 2.6 +/- 5% (not significant versus baseline) in the control group (15 had a decrease). Most interestingly, there was a large increase in BMD (15.3 +/-9.9%) after alendronate therapy in the 16 children who had their BMD followed up in the year before the study, during which time they had shown little increase in BMD (1.03 +/- 6.3%), and often a decrease. Considering their condition, increases in the height of all patients was satisfactory. No new fractures were observed after alendronate therapy was initiated. CONCLUSION: Bisphosphonates can be considered essential components of the treatment of secondary osteoporosis, not only in adults, but also in pediatric patients. Alendronate has a positive effect on secondary osteopenia/osteoporosis in children with connective tissue diseases.

Adolescent↗
PubMed2000

Comparison of quantitative calcaneal ultrasound and dual energy X-ray absorptiometry in the evaluation of osteoporotic risk in children with chronic rheumatic diseases.

Osteoporosis is a common complication in children with chronic rheumatic diseases (CRD). Although dual energy X-ray absorptiometry (DXA) is increasingly being used to determine bone mineral density (BMD) in children, it exposes the subject to ionizing radiation and does not provide a measure of true bone density; in fact, in growing bones the increase in BMD is mainly caused by the increase in bone size. In recent years, quantitative ultrasound techniques (QUS) have been used in radiation-free assessment of bone density and "bone quality" by measurement of the ultrasound waves attenuation by bone (BUA). In the present study we made a direct comparison of BUA in the calcaneum, determined by the pediatric contact ultrasound bone analyzer (CUBA) with lumbar BMD measured by DXA, in a group of 6-18-year-old patients with CRD. The study group consisted of 53 patients affected with juvenile rheumatoid arthritis (n = 29), systemic lupus erythematosus (n = 13), and juvenile dermatomyositis (n = 11). Mean age was 13.02 +/- 2.69 years. In 22 patients (19 girls, 3 boys) both DXA and CUBA were repeated after 1 year in order to assess the mean percentage rate of BMD and BUA change over this time. Both lumbar spine BMD and calcaneal BUA measurements were lower in the CRD patients compared with a control group (P < 0.001). Calcaneal BUA was significantly correlated (r = 0.83, P < 0.001) with lumbar spine BMD. Age and sex correction (Z-score) did not change the relationship between BUA and BMD (r = 0.80, P < 0.001). A significant correlation between the mean percentage of variation (delta%) of BMD and BUA (r = 0.76, P < 0.001) was also demonstrated in the 22 patients who were evaluated prospectively. Portability, ease of use, lower cost, and absence of radiation make CUBA a promising means of evaluating BMD in children.

Absorptiometry, Photon↗
PubMed2000

Juvenile rheumatoid arthritis.

A satisfactory classification of arthritis in childhood has still to be defined, and a system that can facilitate communication among physicians has been proposed by an international committee. The immunopathogenesis of the diseases that are encompassed by the term juvenile rheumatoid arthritis is complex, and many studies have investigated the role of genetic and cytokine balance abnormalities. HLA associations have been confirmed in multiple series, and there is evidence that non-major histocompatibility complex genes might also contribute to disease pathogenesis. Recent studies have added knowledge to the diverse clinical features of the disease, including joint distribution at presentation, association with Turner syndrome, presence of arthritis mutilans, and alterations in mandibulofacial development. Medical treatment remains a challenge. For cases recalcitrant to conventional therapy autologous hemopoietic stem-cell transplantation has been attempted; the risks of this procedure must however be carefully balanced in individual patients.

Antigens, Differentiation↗