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Biomedical subjects

C Salvarani

Publications and source records attributed to C Salvarani.

At least 127 records · Page 7Linked to original sources

RS3PE syndrome: an overview.

More than ten years ago McCarty et al. described the RS3PE syndrome based on their study of 23 patients. Numerous additional cases have since been reported. In addition to the isolated or "pure" type which probably forms part of the clinical spectrum of polymyalgia rheumatica, inflammatory swelling with pitting edema of the dorsum of the hands and/or feet can be observed in different inflammatory rheumatic diseases as well as in haematological and solid malignancies.

Diagnosis, Differential↗

Intercellular adhesion molecule 1 (ICAM-1) gene polymorphisms in Italian patients with rheumatoid arthritis.

AIMS: Rheumatoid arthritis (RA) has a wide range of clinical expressions which probably reflects different genetic backgrounds. Intercellular adhesion molecule-1 (ICAM-1) plays an important role in the inflammatory synovial activity in RA. The aim of this study was to examine the potential associations of ICAM-1 gene polymorphisms with RA and its severity. METHODS: Seventy-eight seropositive Italian RA patients with erosive disease entered the study. Radiographs of hands and feet 5 years after the diagnosis were available for 68 patients and were evaluated for the number of eroded joints. We obtained an erosive score for each patient by counting the number of joints with at least one erosion. Patients in the upper part of the distribution over the median were considered as fast eroders (FE) and the others as slow eroders (SE). Patients' records were also evaluated for the presence of extra-articular features. 228 healthy subjects of the same ethnic origin were selected as a control group. All of the RA patients and controls were genotyped by polymerase chain reaction and allele-specific oligonucleotide techniques for ICAM-1 polymorphisms G/R at codon 241 (exon 4) and E/K at codon 469 (exon 6). RESULTS: The carriage rate of allele R241 was significantly higher in RA patients than in healthy controls (12.8% versus 5.7%, p = 0.039; odds ratio: 2.4 [95% CI 1.02 to 5.79]). The allele frequencies and carriage rate of the E 469 gene did not differ significantly between RA patients and the control group. When we compared the control group with the patients with more or less severe disease (presence or absence of extra-articular features, SE and FE) we found that only the group of patients with the more favourable course maintained a significant difference in the carriage rate of R241 (16.7 vs 5.7%, p = 0.009 for patients without extra-articular features and 18.9 vs 5.7%, p = 0.004 for SE patients). CONCLUSION: Our preliminary findings show that G/R 241 polymorphism of ICAM-1 is associated with RA, and that this confers a reduced risk of extra-articular manifestations and is associated with a slow rate of joint destruction.

Aged↗

Absence of the association with CC chemokine receptor 5 polymorphism in polymyalgia rheumatica.

OBJECTIVE: Elevated RANTES serum levels are present in polymyalgia rheumatica (PMR) patients with active disease. Chemokines may contribute to the inflammatory PMR process through their binding to CC chemokine receptor 5 (CCR5). The aim of this study was to examine if the 32 base pair deletion allele in CCR5 (CCR5 delta 32 allele) might be associated with PMR susceptibility and influence the disease outcome. METHODS: We enrolled 88 consecutive patients with PMR residing in the Reggio Emilia area (Italy) who had a follow-up duration of at least one year. As a control group we used 86 healthy blood donors from the same geographic area. The CCR5 genotype of all PMR patients and controls was studied by polymerase chain reaction amplification of the region which includes the 32 deletion (CCR5 delta 32). RANTES serum levels were measured by commercial ELISA kits in CCR5 delta 32 heterozygous and CCR5 homozygous PMR patients at diagnosis before starting corticosteroid therapy and again after 6 months of therapy, as well as in 28 healthy subjects over 50 years of age. RESULTS: Frequencies of the CCR5 and CCR5 delta 32 alleles in patients and controls did not differ significantly. Homozygosity for CCR5 delta 32 was not detected in PMR patients and was detected in only one of the controls. No significant differences were observed between the patients carrying the CCR5 delta 32 allele and those homozygous for the normal CCR5 allele when we compared sex, presence of distal synovitis and systemic signs and/or symptoms, initial and cumulative prednisone dose, duration of therapy, ESR at diagnosis, frequency of relapse/recurrence and RANTES serum levels at diagnosis and after 6 months of corticosteroids. CONCLUSION: These results indicate that the frequency of the 32 deletion of the CCR5 receptor was not significantly different between PMR patients and healthy controls, and this genotype does not appear to be associated with the susceptibility to or severity of PMR.

Adrenal Cortex Hormones↗

Interleukin-1 cluster and tumor necrosis factor-alpha gene polymorphisms in polymyalgia rheumatica.

OBJECTIVE: To investigate whether polymorphisms in the interleukin (IL)-1 locus (human chrom. 2q13) and TNF-alpha gene are associated with susceptibility to or severity of polymyalgia rheumatica (PMR). METHODS: The study included 92 consecutive PMR patients diagnosed over a 5-year period who were prospectively followed-up for at least one year and 79 healthy controls over the age of 50 residing in the same area. All the patients and controls were Caucasians of Italian origin. We tested the allelic distribution of IL-1A (+4845), IL-B (-511), IL-B (+3954), IL-1RN Intron 2 VNTR and TNFA (-308). Frequencies were compared in the patient and control groups. RESULTS: A statistically significant association between PMR patients and the IL1RN*2 allele in the homozygous state was found [OR 8.46 (95% CI 1.05-68.31)]. The polymorphisms in the other genes of the IL-1 gene cluster did not reveal any association with PMR when compared with controls. A weak association between PMR patients and the TNF2 allele was also present [OR 2.09 (95% CI 1.0-4.17)]. None of the gene variants studied was associated with the disease severity of PMR. CONCLUSION: Our findings show that IL1RN*2 allele, particularly in the homozygous state, is associated with susceptibility to, but not with the severity of, PMR.

Aged↗

Polymyalgia manifestations in different conditions mimicking polymyalgia rheumatica.

Polymyalgia rheumatica (PMR) is a generally benign syndrome involving the neck, shoulder, and hip girdles in the elderly. However, none of the clinical and laboratory findings are specific for this syndrome. Different diseases may present with features suggesting PMR. The consideration of other conditions which in some cases resemble PMR is very important, as their therapy and prognosis differ completely from that of PMR. Four patients presenting with typical PMR manifestations, who were finally diagnosed as having conditions very different from PMR, are described. The importance of the differential diagnosis in patients presenting with polymyalgia symptoms is underlined.

Aged↗

Distal extremity swelling with pitting edema in psoriatic arthritis: a case-control study.

OBJECTIVE: To evaluate the frequency and the clinical characteristics of distal extremity swelling with pitting edema in patients with psoriatic arthritis (PsA). METHODS: This was a case-control study of consecutive outpatients with PsA (old and new diagnosis) observed over a 3-month period in three secondary referral centers in Italy. As controls we used the two consecutive rheumatic outpatients, excluding those with spondylarthropathies, observed after a PsA patient. The demographic and clinical features were assessed by clinical examination and review of the medical records. RESULTS: A total of 183 patients with PsA and 366 controls were evaluated. Distal extremity swelling with pitting edema was recorded in 39/183 (21%) PsA patients and in 18/366 (4.9%) controls (p < 0.0001). In 8/39 (20%) patients this feature presented as a first, isolated manifestation of PsA, and in 8 others it was associated with other features of PsA at diagnosis. The upper and lower extremities were affected, predominantly asymmetrically, in 40% and 60% of the cases respectively. In patients with pitting edema compared to those without this feature, the frequency of Achilles enthesitis and plantar fasciitis, calculated together, was higher (p < 0.05) and the duration of arthritis was significantly lower (p = 0.02). In 7 patients the clinical evidence of a predominant involvement of tenosynovial structures was confirmed by MRI. CONCLUSION: Upper or lower distal extremity swelling with pitting edema due to tenosynovitis, usually unilateral, is a common feature in PsA patients and may represent the first, isolated manifestation of the disease.

Adult↗

Disease-modifying antirheumatic drug therapy for psoriatic arthritis.

As erosive and deforming arthritis is present in 40% of patients with psoriatic arthritis (PsA), early and aggressive treatment with disease-modifying antirheumatic drugs (DMARDs) may be as effective in controlling the progression of the disease as it is for rheumatoid arthritis (RA). Methotrexate (MTX), sulfasalazine (SSZ), and cyclosporine (CsA) are the most widely used DMARDs in the treatment of PsA and are safe and effective in patients with active peripheral arthritis, although they do not appear to be effective on axial manifestations. No controlled study has evaluated the efficacy of these drugs on the progression of radiological damage. It has recently been demonstrated that leflunomide and anti-tumor necrosis factor (TNF) agents are effective in PsA and psoriasis. The symptomatic improvement has been important and sustained and side effects minimal. In particular, inhibitors of TNF appear to have excellent potential to treat PsA. These agents are able to slow joint damage in rheumatoid arthritis and they are effective on spinal symptoms in ankylosing spondylitis. Hopefully, these findings will prove true in PsA as well.

Antirheumatic Agents↗

Chronic neutrophilic leukemia (CNL) with karyotypic abnormalities associated with plasma cell dyscrasia: a case report.

A case of chronic neutrophilic leukemia (CNL), a rare myeloproliferative syndrome associated with monoclonal gammopathy of uncertain significance (MGUS-Type IgGk), is reported. Karyotypic study, carried out on bone marrow, excluded Philadelphia-pos. chronic myeloid leukemia (CML) and showed Y loss (45 XO). Only a few cases of CNL with paraproteinemia have been reported, but no case of associated karyotypic abnormalities and paraproteinemia has so far been described.

Aged↗

The role of interleukin 1, erythropoietin and red cell bound immunoglobulins in the anaemia of rheumatoid arthritis.

Since interleukin 1 (IL-1) and erythropoietin (Epo) are believed to play a role in the pathogenesis of rheumatoid arthritis (RA) anaemia we measured IL-1 alpha and Epo concentrations in 10 RA patients with chronic disease anaemia (CDA) and in 14 RA patients without anaemia. Anaemic RA patients had significantly higher IL-1 alpha concentrations than patients without anaemia. IL-1 alpha correlated negatively with haemoglobin and correlated positively with ESR. The results of a multivariate analysis showed that the best predictors of the presence and absence of anaemia were IL-1 alpha and ESR. No clinical parameters permitted a distinction between these two groups of patients. Epo levels were not different in anaemic and non-anaemic RA patients. No correlation was found between Hb and Epo, indicating the presence of an impaired Epo response in RA patients with CDA. We completed our study with the determination of the mean red cell lifespan and with the quantification of IgG and IgM bound to the surfaces of red blood cells (RBC-IgG and RBC-IgM) using a sensitive ELISA method. We observed a modest reduction in red cell survival in anaemic RA patients compared to normal controls. We did not find any correlation between Hb and red cell lifespan and between Hb and RBC-IgG. RBC-IgG and RBC-IgM were not found to be more elevated in anaemic RA than in non-anaemic patients.

Adult↗

Clinical subgroups and HLA antigens in Italian patients with psoriatic arthritis.

The frequencies of HLA antigens were studied in 101 Italian patients with psoriatic arthritis. The total group showed a significant increase in frequency of A1 and B38, and a reduction of B5 when compared to healthy controls. No association between DR and/or DQw antigens and PA were demonstrated. The comparisons between the clinical subgroups and normal controls revealed a significant association of B38 with asymmetric peripheral arthritis, B27 and B39 with spondylitis (with or without peripheral involvement). When intergroup comparison were made, the patients with spondylitis had an increase in frequency of B27 and DQw3 as compared to those with symmetric and asymmetric peripheral disease. DR4 and DRw53 were associated with earlier age of onset of arthritis. There were also significant associations between DQw3 and severe disease, and between A9, B5 and presence of erosions and joint space narrowing. No association with DR4 was showed in a subgroup of patients with symmetric polyarthritis without DIP involvement.

Adult↗

Bartter's syndrome and chondrocalcinosis: a possible role for hypomagnesemia in the deposition of calcium pyrophosphate dihydrate (CPPD) crystals.

A new case of association between Bartter's syndrome and chondrocalcinosis is reported. The patient was shown to have marked hypomagnesemia. Indomethacin and magnesium therapy was started and resulted in increased magnesemia, even if it did not reach normal levels. There was complete remission of articular symptoms and no progression on the radiological picture after 2 years of continuous magnesium and indomethacin therapy. The 7 available family members were studied to assess the possible presence of a familial form of chondrocalcinosis and/or hypomagnesemia. The literature is reviewed and reports of previously described associations between Bartter's syndrome and chondrocalcinosis are summarized. The possible role of hypomagnesemia in predisposing to deposition of calcium pyrophosphate dihydrate crystal in cartilagine is also discussed.

Adult↗

Polymyalgia rheumatica and giant cell arteritis: a 5-year epidemiologic and clinical study in Reggio Emilia, Italy.

Among the population of Reggio Emilia, Italy, 56 patients with polymyalgia rheumatica (PR) and giant cell arteritis (GCA) were identified during the 5-year period 1981-85. The average annual incidence rates of PR and GCA were 12.8 and 8.8 respectively per 100,000 population aged 50 years or older. Forty-nine patients were followed up and the mean duration of follow-up was 32 months. All the patients received steroid therapy. We have evaluated the cumulative probability of requiring continued steroid therapy between patients with PR only, GCA only, and PR associated with GCA using life-table methods with permanent discontinuation of therapy as an end point. The different duration of steroid therapy between these 3 groups did not achieve statistical significance by the method of Lee and Desu. We identified a 5 variable discriminant function that correctly predicted whether the duration of therapy would be longer or shorter than 16 months (median duration of therapy) in 80% of our patients followed up for at least 24 months. The presence of synovitis in PR is also discussed.

Aged↗