Recent developments in the therapy of localized and systemic sclerosis.
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Humans↗
Biomedical subjects
S Bombardieri
Publications and source records attributed to S Bombardieri.
Decreased elastase activity in circulating granulocytes of systemic sclerosis patients: a possible pathogenetic role.
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Blotting, Western↗
[Pain patterns in Italian patients with osteoarthritis: preliminary results of the MI.D.A. Study (Misurazione del Dolore nell'Artrosi)].
OBJECTIVES: To evaluate the characteristics of pain in a cohort of Italian patients with osteoarthritis (OA) of the hip and knee. METHODS: The 657 general practitioners participating in the study were asked to enroll 10 consecutive patients with OA diagnosed according to the American College of Rheumatology (ACR) clinical criteria. A questionnaire evaluating demographic data, clinical characteristics of OA, including the "Questionario Semantico Reumatologico" (QSR) pain questionnaire, the Western Ontario and McMaster Universities Osteoarthritis Index (WOMAC) and the Lequesne indices, and information on previous diagnostic and therapeutic interventions was administered. RESULTS: A total of 4,109 patients were enrolled. Of them, 2356 were affected by knee OA and 1817 by hip OA. There were 2863 (69.7%) women and 1246 (30.3%) men. Median age was 68.2 years (range 50-103 years). Of the 4109 enrolled subjects, 3128 (76.1%) reported one or more medical comorbidities, mostly cardiovascular (52.7%), endocrinological (14.7%), gastrointestinal (13.4%), and respiratory (11.2%) disorders. The median pain visual analogue scale (VAS) score was 58.1+/-22.6 mm, higher in women (60.2+/-22.3 mm) than in men (53.3+/-22.6mm) (p<0.00001). OA pain was also higher in patients from Southern Italy (p<0.00001). NSAIDs were administered to nearly 70% of patients, COX-2 inhibitors to 55%, disease-modifying anti OA drugs to 19% and analgesics to 28.2%. Differences in drugs utilization were associated with OA localization and patient's geographical origin. Results of the WOMAC index were similar throughout groups. Responses to the QSR pain questionnaire showed differences, which are related to OA localization and geographical origin of the patients. CONCLUSIONS: The MI.D.A. study can help to better understand the patterns of pain in osteoarthritis and the associated treatment.
Age Factors↗
[Chronic inflammation and endothelial dysfunction: analysis of a cohort of patients with SLE and UCTD].
OBJECTIVE: Cardiovascular complications, mainly caused by an accelerated atherosclerosis, are one of the leading causes of death and disability in patients with systemic autoimmune diseases. Endothelial dysfunction is considered the earliest and reversible step of atherogenesis. Aim of the present study is to investigate endothelial function (EF) in patients with systemic lupus erythematosus (SLE), undifferentiated connective tissue diseases (UCTD) and correlate the results with clinical and laboratory variables. METHODS: EF was assessed on the peripheral microcirculation by the perfused forearm technique that can estimate both endothelium- dependent and endothelium- independent vasodilatation. The same evaluation has been repeated in two patients after the administration of 20 mg of 6-metilprednisolone. RESULTS: Twenty-three female patients with SLE or UCTD, with a follow up of at least 1 year have been studied and compared with 8 healthy controls matched for epidemiological variables and traditional risk factors for cardiovascular disease. A significant reduction both in endothelium dependent than endothelium independent vasodilatation was observed in both patients groups compared with controls. In addition, UCTD patients demonstrated a significant reduction in the nitric oxide pathway compared with controls and SLE patients. Finally, steroid administration induced an improvement of vascular reactivity. CONCLUSIONS: Despite the well documented side effects of chronic corticosteroid therapy, our data might suggest a role for antinflammatory and immunosuppressive therapy in the prevention of premature atherosclerosis in patients with systemic autoimmune diseases.
Adult↗
[Italian consensus on EULAR recommendations 2005 for the management of hip osteoarthritis].
The recommendations for the management of osteoarthritis (OA) of the hip were proposed by EULAR in 2005. Among the most important objectives of the expert charged to provide these recommendations were their wide dissemination and implementation. Thus, the information generated can be used by each individual country to produce their own set of management guidelines and algorithms for treatment in primary care. According with that previously executed for the EU-LAR recommendation 2003 for the knee, the Italian Society of Rheumatology (SIR) has organised a Consensus on the EULAR recommendations 2005 for the management of hip OA. To obtain an acceptability as large as possible, the group of experts was composed by many physicians interested in the management of hip OA, including Orthopaedics, Rheumatologists, Physiatrists, and General Practitioners. Main aim of the Consensus was to analyse the acceptability and applicability of the recommendations according to own experience and local situations in the Italy. The results of this Consensus have demonstrated that a large majority of the EULAR recommendations are endorsed by the Italian experts. Furthermore, the final document of the Italian Consensus clearly indicated the need that the specialists involved in the management of hip OA strongly encourage the dissemination of the EULAR 2005 recommendations also in Italy.
European Union↗
[Antinflammatory therapy and cardiovascular risk: a consensus view].
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Anti-Inflammatory Agents, Non-Steroidal↗
Multicenter validation of recombinant, natural and synthetic antigens used in a single multiparameter assay for the detection of specific anti-nuclear autoantibodies in connective tissue disorders.
OBJECTIVES: We investigated the feasibility of using a single multi-parameter test based mainly on recombinant autoantigens for the detection of anti-nuclear autoantibodies, and analyzed the agreement between this test format and conventional techniques. METHODS: The presence of autoantibodies was determined by a line immunoassay (LIA) in 755 sera derived from patients with different autoimmune connective tissue disorders. All sera were previously tested by standard assays that are routinely used at the 8 participating European centers. RESULTS: The overall sensitivity and specificity of autoantibody detection by LIA was similar or higher as compared to combined conventional techniques (CCT). In particular, the detection of anti-Ro52 in systemic lupus erythematosus (SLE) sera (P = 0.004) and anti-LA in both SLE (P < 0.0009) and in Sjögren's syndrome (P < 0.0009) sera was significantly more sensitive when using LIA compared to CCT. By contrast, CCT was never more sensitive than LIA for any of the markers. CONCLUSION: The LIA is a reliable alternative to a combination of conventional techniques for the detection of specific anti-nuclear autoantibodies. The multi-parameter test also reveals autoantibody reactivities that may not be detected when only a limited number of conventional techniques are applied.
Antibodies, Antinuclear↗
Undifferentiated connective tissue diseases (UCTD): a review of the literature and a proposal for preliminary classification criteria.
The connective tissue diseases include a heterogeneous group of conditions characterized by a wide variety of signs and symptoms. There also exists, however, a group of systemic autoimmune disorders with signs and symptoms not sufficiently evolved to fulfill any of the accepted classification criteria for the defined connective tissue diseases. These conditions have been defined as undifferentiated connective tissue syndromes, latent lupus, incomplete lupus, and undifferentiated connective tissue diseases. After an analysis of the existing literature, we discuss the possible significance of the undifferentiated diseases among the already defined connective tissue diseases and make a proposal for preliminary classification criteria for these conditions.
Connective Tissue Diseases↗
Treatment of the idiopathic inflammatory myopathies: a retrospective analysis of 63 Caucasian patients longitudinally followed at a single center.
OBJECTIVE: To analyze the therapies used over the past 20 years at a single center to treat patients with idiopathic inflammatory myopathies (IIM), and to compare their effectiveness. METHODS: Sixty-three Caucasian IIM patients were selected for this study based on the following parameters: (i) a definite diagnosis of IIM; (ii) a follow-up period of at least one year; and (iii) a complete record of clinical and serological data for the course of the follow-up. The following data were collected from the patients' records: the first choice and subsequent therapies, the patient's response to these treatments, the outcome at the end of the follow-up. RESULTS: Two therapeutic approaches were identified: steroids alone and the combination of steroids with immunosuppressive drugs. Of the 63 patients studied, 36 received steroids alone and 27 received steroids plus immunosuppressors. Sixteen patients did not respond to the initial therapy, 33 showed a stable response, and 14 experienced a relapse in disease activity during the follow-up. No statistically significant differences among these 3 groups of patients were observed with respect to sex, age at disease onset, diagnosis, CPK levels at disease onset, and therapeutic approach. CONCLUSION: Corticosteroids represent the mainstay of IIM therapy, both as the first choice treatment and as maintenance therapy. The use of immunosuppressive agents should be restricted to those patients with severe contraindications to steroid treatment.
Age of Onset↗
Reconstructing the pyramid in rheumatoid arthritis. An urgent need.
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Algorithms↗
Hepatitis B virus-related markers in secondary and in essential mixed cryoglobulinemias: a multicentric study of 596 cases. The Italian Group for the Study of Cryoglobulinemias (GISC).
The aim of this study was to assess the prevalence of HBV-related markers in cryoglobulinemias and the possible association between hepatitis B virus (HBV) and essential mixed cryoglobulinemia (EMC). A retrospective survey of the prevalence of HBV infection in cryoglobulinemic patients was carried out in 596 cases of cryoglobulinemia. On the basis of clinical and laboratory criteria the cases were grouped as secondary to connective tissue disease, to lymphoproliferative disorders, to chronic liver diseases or to chronic infectious diseases. The cases in which an associated disease was not evidenced were considered as essential mixed cryogobulinemias. Subjects in which liver dysfunction was first diagnosed simultaneously with cryoglobulinemia, were considered as a separate group. A greater prevalence of HBsAg and anti-HBc antibodies was found in cryoglobulinemias secondary to chronic liver disease (p < 0.0001) and in those associated with liver involvement at diagnosis (p < 0.05) than that found in EMCs. The prevalence of anti-HBs antibodies did not differ significantly among the groups. Proven contact with the virus, documented by at least one positive marker, was evidenced more frequently in cryoglobulinemias secondary to liver disease than in the other groups (p < 0.01). The prevalence of HBV related markers in EMCs and in hospitalized patients not suffering from diseases associated with cryoglobulin production were similar, and seems to reflect the epidemiological situation of HBV infection in Italy. In conclusion, our findings do not support an association of HBV with EMC.
Biomarkers↗
Disease activity in systemic lupus erythematosus: report of the Consensus Study Group of the European Workshop for Rheumatology Research. I. A descriptive analysis of 704 European lupus patients. European Consensus Study Group for Disease Activity in SLE.
Using a detailed questionnaire, the cumulative historical and current demographic, clinical and serological data on 704 SLE patients from 29 European centres and 14 countries have been assessed. Ninety-three percent of the patients were Caucasian and the female/male ratio was 10:1. Analysis of the cumulative incidence showed that arthralgia/arthritis (94%), rash (69%), Raynaud's phenomenon (49%), serositis (44%) and renal disease (38%) were the most frequent clinical manifestations. Virtually all the patients (98%) were antinuclear antibody positive, while anti-ds-DNA antibodies (76%), hypocomplementaemia (71%) and anti-Ro(SSA) antibodies (35%) were frequent serological abnormalities. Whilst much of this data is in line with previous reports, it is notable that renal, lung, and central nervous system involvement and the frequency of rheumatoid factor, anti-Sm and anti-RNP antibodies were much lower than in most comparable series in the United States. We assume that ethnic differences and the greater present awareness of lupus could explain this variations. Low dose corticosteroids, non-steroidal anti-inflammatory drugs and anti-malarials were used to treat over half of the patients, 75% of whom were between 15 and 55 years of age. This report offers a useful overview of lupus both clinically and serologically in Europe in the 1990's.
Adolescent↗
Disease activity in systemic lupus erythematosus: report of the Consensus Study Group of the European Workshop for Rheumatology Research. II. Identification of the variables indicative of disease activity and their use in the development of an activity score. The European Consensus Study Group for Disease Activity in SLE.
A European Consensus Group study, involving 29 centres from 14 countries, was performed in order to reach agreement on the definition of disease activity in systemic lupus erythematosus (SLE) and to construct a new disease index. Data on 704 lupus patients were collected and analysed, using univariate and multivariate statistical procedures, to select those clinical and laboratory features of the disorder which best correlate with the global assessment of disease activity assigned to the patients by the physician of each participating centre. A combination of 15 clinical and laboratory variables was shown to be the best predictor of disease activity in SLE. A European Consensus Lupus Activity Measurement (ECLAM) was then formulated. This index included the 15 selected variables, weighted (with some adjustments) according to their respective regression coefficients in the multivariate model. ECLAM appears to be an effective instrument for scoring patients with different degrees of disease activity. This is the first SLE disease activity index based on data from a very large number of lupus patients followed at a large number of lupus centres in different countries. It might therefore very well serve as a standardised measure for future European clinical studies. Final assessment of the validity, reliability and sensitivity of this index is now underway.
Europe↗
Disease activity in systemic lupus erythematosus: report of the Consensus Study Group of the European Workshop for Rheumatology Research. III. Development of a computerised clinical chart and its application to the comparison of different indices of disease activity. The European Consensus Study Group for Disease Activity in SLE.
In the first phase of this study, a data-base containing clinical and laboratory findings of 704 patients with systemic lupus erythematosus (SLE), originating from 29 centres and 14 countries, was used to assess the validity of 4 common indices of disease activity, SLAM, BILAG, SLEDAI and SIS. The physician's judgement of activity was assumed as the unique reference criterion (gold standard). Computer programmes were developed to calculate automatically the 4 activity indices; this computation appeared to correspond with manual computations in a sample of 60 appropriately selected cases. All 4 indices were closely correlated with each other (r in the range of 0.716 to 0.872), and with the physician's score (r in the range of 0.620 to 0.719). In the second phase of the study the activity index developed in part I (ECLAM) was prospectively validated, and its performance compared to that of the other scales, both as a single state index and as a transition index (i.e., its ability to assess disease activity at a single point in time and to detect variations in consecutive readings). A computer-assisted clinical chart was prepared for this purpose. This chart allowed us to calculate automatically all the indices. Two consecutive observation times (time 0, and time 1 three months later) were included in the study protocol. Data on 75 patients from 19 centres were collected, and each patient was observed twice. All the computed indices were closely correlated, both at time 0 (r ranging from 0.725 to 0.884), and at time 1 (r ranging from 0.607 to 0.833).(ABSTRACT TRUNCATED AT 250 WORDS)
Diagnosis, Computer-Assisted↗
Sjögren's syndrome: a retrospective review of the cutaneous features of 93 patients by the Italian Group of Immunodermatology.
OBJECTIVE: Various cutaneous manifestations have been described (xerosis, eyelid dermatitis, angular cheilitis, cutaneous vasculitis and annular erythema) in Sjögren's syndrome (SS), but so far only three studies on large numbers of SS patients have been carried out. The frequency of cutaneous manifestations and their association with specific clinical or immunological parameters have never been studied. The aim of the Italian Group of Immunodermatology was to evaluate these associations in a large number of SS patients. METHODS: A retrospective review was performed on 93 patients with SS followed over a ten-year period (1990-2000) at 6 Italian dermatological centers. They were subdivided into two groups, 62 with primary SS and 31 with secondary SS, and the frequency of cutaneous manifestations and specific antibodies was determined and compared between them. RESULTS: We found significantly higher levels of xerosis (p = 0.009) (56.4% versus 25.8%) and angular cheilitis (p = 0.017) (38.7% versus 16.1%) in primary SS patients than in those with secondary SS. A significant association of xerosis with anti-SSA + SSB (p = 0.033) antibodies was also demonstrated. Eyelid dermatitis and pruritus were common but less specific cutaneous symptoms. Annular erythema was found more often in primary (6.45%) than in secondary (3.2%) SS and was associated with SSA + SSB antibodies in 75% of the cases. Cutaneous vasculitis was present in 30.6% of primary SS (manifesting as palpable purpura in 84%) and in 29.3% of secondary SS cases. CONCLUSION: Xerosis is the most frequent and characteristic cutaneous manifestation of primary SS. It is not linked to decreased sebaceous or sweat gland secretion, but more probably to a specific alteration of the protective function of the stratum corneum. Angular cheilitis is a common but less specific skin lesion in SS and is associated with xerosis and xerostomia.
Adult↗
Undifferentiated connective tissue diseases in 2004.
The existence of patients with signs and symptoms suggestive of a systemic autoimmune disease but not fulfilling the classification criteria for defined diseases is a common experience in clinical practice. The first description of these diseases dates back to 1980, when LeRoy proposed the term "Undifferentiated Connective Tissue Syndromes" (UCTS) to define the early phases of connective tissue diseases that at their onset are undefined, as opposed to mixed or overlapping syndromes in which the disease appears well defined. LeRoy also raised the fundamental question as to whether a proportion of patients with an undifferentiated profile may see their condition remain undifferentiated or even experience a remission of all pathologic features, rather than evolving to a definite connective tissue disease (CTD). Many studies conducted on undifferentiated diseases have shown that up to 75% of patients will not develop a defined CTD and that these conditions exhibit typical clinical and serological manifestations and a good prognosis. We and others have defined these conditions as Undifferentiated Connective Tissue Diseases (UCTD) and it is our opinion that the UCTD represent distinct clinical entites. UCTD could offer an ideal clinical model for the study of single autoantibody specificities, the effects of various factors (such as pregnancy) on the disease course, and the general pathogenesis of autoimmune conditions. Before they can be so used, more must be discovered about their nature and characteristics, and studies will be necessary to improve the sensitivity and specificity of the existing preliminary classification criteria.
Autoantibodies↗
Acronyms for the rheumatology literature.
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Abbreviations as Topic↗
Comparison of masticatory dysfunction in temporomandibular disorders and fibromyalgia.
AIM: Many theories have been proposed as to the relationship between fibromyalgia (FM) and temporomandibular disorders (TMD). The aim of this study was to investigate the clinical features of the involvement of the stomatognathic system in patients with fibromyalgia, and to compare signs and symptoms of masticatory dysfunction between TMD and FM patients. METHODS: The study sample consisted of 30 subjects with fibromyalgia recruited at the Rheumatology Disease Department, University of Pisa, Italy, and of 30 with temporomandibular disorders at the Section of Prosthetic Dentistry, Department of Neurosciences, University of Pisa, Italy. Masticatory dysfunction has been compared between the 2 groups by means of a clinical assessment conducted according to the Research Diagnostic Criteria for Temporomandibular Disorders (RDC/TMD). Patients of the TMD group underwent a rheumatologic examination to investigate for the presence of previously undiagnosed fibromyalgia. RESULTS: As regards specific clinical variables and RDC/TMD diagnoses, differences between the 2 groups appear to be not relevant, with the exception of the values of mouth opening, both voluntary and passive, and the presence of trigger points. FM patients showed a decrease in both maximum voluntary and passive mouth opening which was about 2 mm worse than that recorded in TMD patients. FM patients also showed a significantly higher number of trigger points, even though muscle palpation evoked tenderness in the same number of sites in the 2 groups. CONCLUSIONS: This study showed that most patients with fibromyalgia (86.7%) report signs and symptoms localized at the stomatognathic system; by contrast, only a minority of patients with temporomandibular disorders (10%) are actually affected by fibromyalgia.
Adult↗