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G D Sebastiani

Publications and source records attributed to G D Sebastiani.

34 records · Page 2Linked to original sources
PubMed1991

Prevalence and clinical associations of anticardiolipin antibodies in systemic lupus erythematosus: a prospective study.

The main objectives of the present study were to determine the prevalence of IgG and IgM anticardiolipin antibody (aCL) isotypes in systemic lupus erythematosus (SLE) in order to analyze their possible association with the clinical manifestations of the disease. Clinical features of 64 consecutive and unselected SLE patients were prospectively studied. Sera from the same patients taken during each clinical manifestation were tested for the presence of aCL. The prevalence of aCL was 43.75% for the IgG isotype and 9.4% for the IgM isotype. A strong linkage between the presence of these antibodies and the occurrence of both thrombosis and abortions was found: a weaker association with neurological events and thrombocytopenia was also demonstrated. The titre of aCL appeared to be linked with the probability of having the clinical manifestations associated with these autoantibodies. Our results suggest that thrombosis and abortion, and possibly thrombocytopenia and central nervous system involvement, may be associated with the presence of aCL at the time when these clinical events develop in SLE patients.

Adult↗
PubMed1990

[Pulmonary hypertrophic osteoarthropathy of Pierre-Marie-Bamberger. Discussion of 5 cases and review of the literature].

Hypertrophic osteoarthropathy (HOA) may be defined as a syndrome of chronic proliferative periostitis of the long bones, clubbing of the fingers and toes, arthralgia and or arthritis, oligo- or polysynovitis. It is often associated with primary pulmonary carcinoma, rarely with other intra- or extrathoracic disease processes. With the present report, the Authors would like to contribute some informations on the clinical aspects of pulmonary HOA and a review of literature.

Adult↗
PubMedDate not supplied

[The current outlook in the therapy of autoimmune diseases].

Recent advances in immunology and molecular biology have contributed to a much greater understanding of the pathogenetic mechanisms of the autoimmune diseases and thus to the development of new rationally-based therapies. Most of the immunosuppressive agents that have been tried in autoimmune disease patients nonspecifically suppress the immune response, often causing various side effects. Autoimmune diseases result from the activation of self-reactive T cells that recognize autoantigens or foreign antigens cross-reactive with an autoantigen coupled with major histocompatibility complex (MHC) products on an antigen presenting cell. It appears possible to modulate T cell activation by interfering with the interaction between T cell receptor and the peptide-MHC molecule complex. A number of sites are potential targets for immunologic intervention, such as MHC molecules, T cell receptor, CD4 and CD3 molecules, adhesion molecules, cytokines and cytokine receptors. In the present review the most important new therapeutic approaches to autoimmune conditions, which appear to be more selective in overcoming the limitations of non-specific treatments, are summarized. They include monoclonal antibodies, cytokines and cytokine-inhibitors, peptides interacting with MHC molecules and T cell vaccination.

Antibodies, Monoclonal↗
PubMedDate not supplied

Association of extrahepatic manifestations with HLA class II alleles and with virus genotype in HCV infected patients.

It has been postulated that host factors, such as the human leucocyte antigen (HLA) system, may play a predominant role in the pathogenesis of HCV-related extra-hepatic manifestations. This study was performed to investigate the role of HLA- DR and DQ alleles in a group of Italian patients, with HCV infection and associated extrahepatic manifestations and to test whether an association between HCV genotype, HLA locus and clinical or serological manifestations can be demonstrated. Thirty unrelated patients affected by HCV infection with extra-hepatic manifestations were consecutively included in the study. One hundred and sixty-three HCV patients without extrahepatic manifestations were tested as controls for the prevalence of HCV genotypes, and 283 healthy donors were used as controls for HLA class II alleles distribution. HCV-RNA was quantified by an reverse transcription-PCR. HLA class II alleles typing was performed using a standard microlymphocytotoxicity assay on B lymphocyte purified. HCV 2c genotype was found in 53.3% compared to 18.4% of controls (p=0.00001; OR=5.1). Cryoglobulins were detected in 72.7% DR6+ patients and in 31.6% DR6- patients (p=0.05; OR=3.21). Rheumatoid factor was found in 90.9% of DR6+ patients and in 42.1% DR6- patients (p=0.018; OR 13.7). Only two DR5+ patients (20%) had cryoglobulinemia, while 6 patients (30%) in the DR5- group had cryoglobulinemia (p=0.02; OR=0.07). Associations were found between DR7 and ANA (OR=1.74) and between DQ2 and ANA (OR=1.97). According to our findings HLA-DR6 might play an important role in developing extra-hepatic manifestations and genotype 2c could be considered as a risk factor for their onset.

Aged↗
PubMedDate not supplied

Autoinflammatory syndromes.

The autoinflammatory disorders are a new and expanding classification of inflammatory diseases characterized by recurrent episodes of systemic inflammation in the absence of pathogens, autoantibodies or antigen specific T cells. These disorders are caused by primary dysfunction of the innate immune system, without evidence of adaptive immune dysregulation. Innate immune abnormalities include aberrant responses to pathogen associated molecular patterns (PAMPs) like lipopolysaccharide and peptidoglycan, prominent neutrophilia in blood and tissues, and dysregulation of inflammatory cytokines (IL-1beta, TNF-alpha) or their receptors. The autoinflammatory diseases comprise both hereditary (Familial Mediterranean Fever, FMF; Mevalonate Kinase Deficiency, MKD; TNF Receptor Associated Periodic Syndrome, TRAPS; Cryopyrin Associated Periodic Syndrome, CAPS; Blau syndrome; Pyogenic sterile Arthritis, Pyoderma gangrenosum and Acne syndrome, PAPA; Chronic Recurrent Multifocal Osteomyelitis, CRMO) and multifactorial (Crohn's and Behçet's diseases) disorders. Mutations responsible for FMF, TRAPS, CAPS, PAPA are in proteins involved in modulation of inflammation and apoptosis.

Autoimmune Diseases↗
PubMedDate not supplied

[Diffuse idiopathic skeletal hyperostosis of the cervical spine in a patient with ankylosing spondylitis. Description of a personal case].

Diffuse idiopathic skeletal hyperostosis (DISH) is a well-described disorder of middle-aged people, with a unique spinal pathology characterized by calcification and ossification of the antero-lateral aspect of at least four contiguous vertebral bodies, with the sparing of intervertebral spaces and sacroiliac joints. DISH has rarely been reported associated with ankylosing spondylitis (AS), a chronic inflammatory articular disease most commonly involving the spine and sacroiliac joints. A 63-year-old man with clinical and radiological findings of DISH with associated AS is described here. The authors conclude that these two diseases may, albeit rarely, coexist.

Cervical Vertebrae↗
PubMedDate not supplied

HLA antigens in Italian patients with systemic lupus erythematosus: evidence for the association of DQw2 with the autoantibody response to extractable nuclear antigens.

In order to verify the hypothesis that Italian patients with systemic lupus erythematosus (SLE) may be immunogenetically distinct from SLE patients born in other regions, we investigated the HLA class I and II antigens and their relation with the various autoantibodies characteristic of the disease in an Italian SLE population. Forty-four SLE patients were typed for HLA-A, -B, -C, -DR and -DQ antigens; sera from the same patients were tested for the presence of antibodies to the nuclear or cytoplasmic antigens Ro/SSA, La/SSB, Sm and RNP (ENA). Results of HLA typing showed that the frequencies of DR3 and DQw2 were increased in patients compared with controls. Analysis of the correlations between HLA antigens and anti-ENA antibodies showed that both DQw2 and DR3 were increased in patients with anti-Ro and/or antiLa antibodies, while in patients with anti-Sm and/or antiRNP antibodies the DQw2 and DR4 were found to be increased. Only DQw2 was found to be significantly increased in anti-ENA positive patients. These results might suggest that Italian patients with SLE are, at least in part, different from lupus patients living in other geographical areas and suggest the association of DQw2 with the autoantibody response to ENA in SLE.

Adult↗
PubMedDate not supplied

[Clinical manifestations correlated with anticardiolipin antibodies in systemic lupus erythematosus: preliminary results of a prospective study].

Antiphospholipid antibodies (aPL) have been linked to various clinical manifestations in systemic lupus erythematosus (SLE), mainly thrombosis, repeated abortions and thrombocytopenia. Despite the large number of studies which have been published in the recent years, there is still some debate on this matter, and no firm conclusion has been reached as yet. Among the various aPL, anticardiolipin antibodies (aCL) have received more attention, mostly because they can be easily detected by means of immunoenzymatic assays. The main objective of the present study was to determine the prevalence of IgG and IgM aCL isotypes in SLE in order to compare their possible association with the clinical manifestations of the disease. Clinical features of 40 consecutive and unselected SLE patients (35 female and 5 male) were prospectively studied. Sera from the same patients were tested for the presence of aCL, using a standardised ELISA assay, and the presence of aCL was correlated with the various clinical events. Results showed that the prevalence of aCL was 42.5% for the IgG isotype and 10% for the IgM isotype. Regarding the clinical associations of aCL, we found a strong linkage between the presence of these antibodies and the occurrence of both thrombosis and abortions; a weaker association with neurological events was also demonstrated. These results, if confirmed on larger series, suggest that aCL should be searched in patients with SLE in order to identify those who are at greater risk of developing some severe clinical problems and could benefit by prophylactic treatment.

Adult↗
PubMedDate not supplied

[Prognostic factors in Raynaud's phenomenon: usefulness of antinuclear antibodies and of periungual capillaroscopy].

Antinuclear antibodies (ANA) and nailfold capillary microscopy have been evaluated as prognostic markers in patients with apparently idiopathic Raynaud's phenomenon. Results have shown that either ANA and peculiar alterations in nailfold capillaries are important risk factors as regard to the evolution into a connective tissue disease; furthermore, when both these markers are present in a patient with Raynaud's phenomenon, the risk of evolution is even greater. When sensibility and specificity have been compared, ANA appeared to be a more sensible test but nailfold capillary microscopy seemed to be more specific. Our data suggest that ANA and capillary microscopy are important tests in patients with Raynaud's phenomenon; they should be performed in order to identify those patients who are at greater risk of evolving to overt connective tissue disease.

Adolescent↗
PubMedDate not supplied

Anti-neutrophil cytoplasmic antibodies in 566 European patients with systemic lupus erythematosus: prevalence, clinical associations and correlation with other autoantibodies. European Concerted Action on the Immunogenetics of SLE.

OBJECTIVES: To evaluate, in a cohort of 566 patients with systemic lupus erythematosus (SLE) drawn from 11 European centres: (i) the prevalence of ANCAs and their subspecificities in a large series of European SLE patients; (ii) the possible associations of ANCA with the most common clinical manifestations of the disease; and (iii) whether ANCAs correlate with some of the autoantibodies commonly found in SLE. METHODS: ANCA detection was performed by indirect immunofluorescence (IIF), and by ELISA for lactoferrin (LF), myeloperoxydase (MPO), proteinase3 (PR3) and lysozyme (LZ) subspecificities. RESULTS: The prevalence of ANCA was 16.4% (IIF). The prevalence of LF was 14.3%, LZ 4.6%, MPO 9.3%, and PR3 1.7%. Our results show that ANCA is associated with certain clinical manifestations of SLE. In particular, positive correlations were found between IIF ANCA and serositis (p = 0.026), livedo reticularis (p = 0.01), venous thrombosis (p = 0.03) and arthritis (p = 0.04), while anti-LF antibodies were associated with serositis (p = 0.05) and livedo reticularis (p < 10(-3). Nevertheless, multivariate analysis demonstrated that other autoantibodies, such as aCL and SSA/Ro, are more closely correlated than ANCA with some of the aforementioned clinical features. CONCLUSION: Our results demonstrate that ANCA are detectable in SLE sera and that some of them are associated with particular clinical manifestations. Whether ANCA plays a direct pathogenetic role in the vascular damage of SLE or only represents an epiphenomenon or a marker of disease activity remains to be elucidated.

Adolescent↗
PubMedDate not supplied

Association of 16/6 and SA1 anti-DNA idiotypes with anticardiolipin antibodies and clinical manifestations in a large cohort of SLE patients. European Concerted Action on the Immunogenetics of SLE.

OBJECTIVE: The SA1 and 16/6 idiotypes can be found in some patients with systemic lupus erythematosus (SLE) or anti-phospholipid syndrome (APS). The aim of this study was to ascertain the prevalence of these idiotypes in a large cohort of SLE patients, and to determine whether their presence is correlated with the anticardiolipin (aCL) and anti-dsDNA antibodies or with the clinical manifestations of SLE. METHODS: 492 SLE patients were evaluated for clinical manifestations of SLE and were assigned a disease severity score. ds-DNA autoantibodies, aCL autoantibodies of the IgM, IgG and IgA isotypes, and SA1 and 16/6 idiotypes were also determined in these patients. RESULTS: The prevalence of the SA1 and 16/6 idiotypes in the 492 SLE patients was found to be 11% and 5.1%, respectively, and these idiotypes were significantly more prevalent in the patients who had aCL antibodies of either the IgG, IgM or IgA isotypes. Moreover, while the 16/6 idiotype was not associated with the clinical manifestation of either SLE or APS, the SA1 idiotype was found significantly more frequently in patients who had vascular events Raynaud's phenomenon or hemolytic anemia (p = 0.016, 0.01, 0.01, respectively). CONCLUSION: SLE patients with the SA1 idiotype may run a higher risk of developing vascular events, Raynaud's phenomenon or hemolytic anemia. These clinical manifestations can be attributed to both SLE and secondary APS when aCL autoantibodies are also found. These results indicate that the possible pathogenicity of certain idiotypes in SLE cannot be excluded.

Adolescent↗