Combined interventional sialendoscopy and intraductal steroid therapy for recurrent sialadenitis in Sjögren's syndrome: Results of a pilot monocentric trial.

OBJECTIVES: To evaluate the effectiveness of interventional sialendoscopy alone or combined with outpatient intraductal steroid irrigations in patients with sialadenitis due to Sjögren's syndrome (SS). DESIGN: A pilot therapeutic study. SETTING: ENT Clinics, Universities of Milan and Pavia. STUDY POPULATION: We included 22 patients with SS of whom 12 underwent interventional sialendoscopy followed by intraductal steroid irrigations (group A), and 10 interventional sialendoscopy alone (group B). OUTCOMES MEASURES: The following outcome measures were considered and recorded before and after the therapeutic intervention: (i) number of episodes of glandular swelling, (ii) cumulative prevalence of patients with glandular swelling assessed by the specific domain, the EULAR SS Disease Activity Index (ESSDAI), (iii) severity of pain by means of a 0-10 pain visual analogue scale (VAS), (iv) severity of xerostomia and other disease symptoms assessed by the EULAR SS Patient Reported Index (ESSPRI) and the Xerostomia Inventory questionnaire. RESULTS: The postoperative reduction in the mean number of episodes of glandular swelling was 87% (95% CI: 77-93) and 75% (95% CI: 47%-88%) in the groups A and B, respectively. The percentage of patients with glandular swelling decreased from 41.7% to 0.0% in the group A and from 30.0% to 0.0% in the group B, respectively. Most of the patients experienced a subjective clinical improvement documented by the statistically significant reductions in the postoperative mean pain VAS (group A P<.001; group B P=.004), Xerostomia Inventory (P<.001 and P=.003) and ESSPRI scores (P<.001 and P=.008). Interventional sialendoscopy followed by outpatient intraductal steroid irrigations was more effective than interventional sialendoscopy alone, when pain VAS, Xerostomia Inventory and ESSPRI scores before and after treatment were analysed together using the multivariate Hotelling T2 test (P=.0173). CONCLUSIONS: This pilot study confirms that interventional sialendoscopy with steroid duct irrigation significantly reduces the number of painful episodes of sialadenitis and improves the subjective sensation of oral dryness and other disease symptoms in patients with SS. The study results also suggest that the improvement is greater when interventional sialendoscopy is combined with a cycle of outpatient steroid ductal irrigations. Larger controlled randomised studies are certainly needed to confirm these preliminary data.

[Unusual presentation of bilateral bullous Pneumocystis jiroveci pneumonia complicated with pneumothorax]. / Presentación inhabitual de un caso de neumonía bulosa aguda bilateral por Pneumocystis jiroveci complicada con neumotórax.

In patients with human immunodeficiency virus infection, pneumothorax overcomes in advanced stages of the illness, associated with infections by Mycobacterium tuberculosis or Pneumocystis jiroveci, in smokers and intravenous drug users. We present a case with this unusual complication: homosexual man, 30 years of age, with a history of one month of cough, progressive dyspnea, weight loss and diarrhea. He was hospitalized with the diagnosis of atypical pneumonia, respiratory failure and a presumptive HIV infection. His clinical course was complicated by the presence of bullae and pneumothorax by day 15 of hospitalization. The existence of Pneumocystis jiroveci in sputum was confirmed by a direct immunofluorescence test. The patient was treated with sulpha-trimethoprim, steroids, oxygen, but he died 21 days after admittance. The strategies recommended for treatment of pneumothorax appeared during P. jiroveci pneumonia in HIV positive patients are summarized.

Autologous hematopoietic stem cell transplantation has better outcomes than conventional therapies in patients with rapidly progressive systemic sclerosis.

We retrospectively evaluated the efficacy of autologous hematopoietic stem cell transplantation (AHSCT) in 18 patients with rapidly progressive diffuse cutaneous systemic sclerosis (rp-dcSSc), and compared their disease outcomes with those of 36 demographically- and clinically-matched patients treated with conventional therapies. Cutaneous involvement, by performing modified Rodnan skin score (mRss), lung diffusion capacity, by measuring diffusing capacity of lung for carbon monoxide (DLCO), and disease activity, by applying the European Scleroderma Study Group (ESSG) scoring system, were the outcome variables measured at the baseline time and then every 12 months for the following 60 months in both the AHSCT-treated patients and the control group. In the AHSCT group, treatment-related mortality was 5.6%. In this group, both mRss and ESSG scores showed a significant reduction 1 year after AHSCT (P<0.002); and these results were maintained until the end of follow-up. Conversely, DLCO values remained stable during the whole period of follow-up. Survival rate of AHSCT group was much higher than that observed in the whole control group (P=0.0005). The probability that the ESSG score and mRss would remain at a high level, and DLCO could decrease, was significantly higher in the control group as a whole and in the subgroup of control patients treated with cyclophosphamide than in the AHSCT group. This study confirms that the AHSCT is effective in prolonging survival, as well as in inducing a rapid reduction of skin involvement and disease activity, and preserving lung function in patients with rp-dcSSc.

Chronic myeloid leukemia in Waldenström's macroglobulinemia.

Eighteen months after the occurrence of Waldenström's macroglobulinemia, Philadelphia (Ph1) chromosome-positive chronic myeloid leukemia developed in a 69-year-old woman. The coexistence of the two disorders was characterized by an initial reduction of paraproteinemia at the time that leukemia occurred, a long-lasting remission of the two disorders, and a final parallel increase of paraproteins and WBCs. Since leukemia occurred 15 months after the interruption of melphalan therapy, the potential mutagenic role of chemotherapy was considered irrelevant. Therefore, Waldenström's macroglobulinemia might favor the occurrence of chronic myeloid leukemia.

Role of triiodothyronine in down-regulation and recovery of lymphocyte beta-adrenoceptors in thyroidectomized patients.

Thyroid function modulates beta-adrenergic sensitivity through the regulation of beta-adrenoceptor density. In particular, thyroidectomy decreases and thyroid hormone injections increase beta-adrenoceptor density on human lymphocytes. In the present paper the relationship between receptor modifications and thyroid hormone levels has been studied in human lymphocytes from patients after thyroidectomy. The patterns of early changes as well as recovery trends have been investigated. Results show a statistically significant fall in receptor density, parallelled by a decrease in T3 levels, 1-3 days after thyroidectomy. Recovery of receptor density is observed 5-8 days after surgical intervention and is accompanied by increased T3 levels. There is a positive correlation between receptor density and T3 levels. On the contrary, no statistically significant correlation was observed for receptor density and T4 levels. The time course of disappearance and recovery of receptor density and that of T3 levels have also been analyzed with the aid of a mathematical model fitting experimental data. On this basis, the hypothesis that both down-regulation and subsequent recovery of beta-adrenoceptor density are driven by the fall and rise of T3 is suggested. Data are also discussed in relation to experimental results we have obtained in animal studies.

A simple method for the biochemical purification of Ro/SS-A antigen.

In the present study, Ro/SS-A antigen has been isolated from human spleen by a two-step procedure. In the first step most of the non-antigenic material was removed by means of ammonium sulphate precipitation and ion exchange chromatography. The final purification was obtained by passing the Ro/SS-A-containing fractions twice through a Mono Q ion exchange fast protein liquid chromatography (FPLC) column. The purified antigen showed identical immunoreactivity with crude material on CIE and was composed of two polypeptides with a molecular weight of approximately 60,000 and 55,000 respectively on SDS-PAGE, both reacting on Western blotting with a panel of anti-Ro/SS-A antisera. This system permits milligrams of highly purified antigen to be obtained from grams of human spleen.

Evaluation of S-adenosylmethionine in primary fibromyalgia. A double-blind crossover study.

The effect of S-adenosylmethionine (SAMe) and placebo was evaluated in a short-term crossover study of 17 patients with primary fibromyalgia. Eleven of 17 patients had a significant depressive state as assessed by either the Hamilton Depression Rating Scale or the Scala di Autovalutazione per la Depressione (SAD) rating scale. The number of trigger points plus painful anatomic sites decreased after administration of SAMe (p less than 0.02) but not after placebo treatment. In addition, scores on both the Hamilton and SAD rating scales improved after SAMe administration (p less than 0.05 and p less than 0.005, respectively), whereas they did not significantly change after placebo treatment. In all the patients, there was a good correlation between scores on the Hamilton rating scale and the number of trigger points. Thus, this preliminary study confirms the close relationship between primary fibromyalgia and psychologic disturbances, particularly with regards to a depressive state. SAMe treatment, by improving the depressive state and reducing the number of trigger points, seems to be an effective and safe therapy in the management of primary fibromyalgia.

Low-antigen-content diet in the treatment of patients with mixed cryoglobulinemia.

PURPOSE: The effects of a low-antigen-content diet (LAC diet) versus a standard normocaloric diet on the signs and symptoms of mixed cryoglobulinemia (MC) were compared in a crossover randomized study. PATIENTS AND METHODS: The study consisted of 24 outpatients with MC, and was carried out in a 48-week period. After 18 weeks of either the LAC or the placebo diet, patients returned to a totally unrestricted diet for 12 weeks (washout period) and crossed over to the second half of the study for the other 18 weeks. RESULTS: After three weeks of the restricted LAC diet, the cryocrit decreased from 3.5 +/- 3.4% (mean +/- SD) to 2.3 +/- 2.0% (p less than 0.01), and the circulating immune complex levels decreased from 48 +/- 30% to 39 +/- 34% (p less than 0.01). At the same time, the purpura score (p less than 0.05), glutamic pyruvic transaminase level (p less than 0.01), and gamma glutamyl transferase level (p less than 0.001) significantly improved. Splenic reticuloendothelial function, measured as the half-life of heat-damaged autologous red cells, decreased from 41 +/- 21 minutes to 21 +/- 10 minutes (p less than 0.005). In contrast, no significant parallel clinical, biochemical, and immunologic changes occurred in the same patients during the placebo (standard normocaloric) diet. CONCLUSION: These data show that an LAC diet decreases the amount of circulating immune complexes in MC and can modify certain signs and symptoms of the disease. These effects of the LAC diet may be explained by postulating a functional restoration of the mononuclear phagocytic system.

Autosomal dominant transmission of familial laterality defects.

Heterotaxy results from failure to establish normal left-right asymmetry during embryonic development. Most familial cases are thought to be autosomal recessive. We have identified a family in which 4 individuals from 3 generations manifest laterality defects. Twenty-five family members have been examined. Two have complete reversal of normal laterality (situs inversus) while 2 others have asplenia, midline liver, and complex cardiac malformations (situs ambiguus). Two additional obligate gene carriers are anatomically normal (situs solitus). Male-to-male transmission confirms autosomal inheritance. Identification of this family establishes an autosomal dominant form of laterality defect, suggesting that a portion of sporadic cases may be new-mutation dominant or unrecognized familial cases. The finding of all forms of laterality (solitus, ambiguus, and inversus) among obligate disease gene carriers within a single family may be relevant to genetic evaluation and counseling in apparently isolated patients with laterality disturbance.

Diagnostic criteria for Sjögren's syndrome: the state of the art.

The diagnostic approach to Sjögren's syndrome (SS) is devoted to the assessment of glandular and extraglandular features of the disorder. Although this syndrome has been attracting growing interest from many investigators in the last few decades, complete agreement on the diagnostic tools to be used for this syndrome has not yet been reached. Finding a rational diagnostic approach to Sjögren's syndrome is even further complicated by the fact that this disease may exist alone, or in association with a number of extraglandular systemic manifestations or a well-defined systemic autoimmune disease. In this paper the entire matter has been critically reviewed in order to evidentiate the differences among the various diagnostic procedures proposed by different authors and to underline the need for a uniform classification criteria for SS.

Sjögren's syndrome, mixed cryoglobulinaemia and the monoclonal gammopathies.

Sjögren's syndrome (SS) represents a pathological model of the evolution from polyclonal B lymphocyte activation to oligo-monoclonal B cell expansion, which may culminate in the development of a true lymphoproliferative disease. The different phases of this process are usually marked by the appearance of type II mixed cryoglobulins in which the monoclonal component is commonly represented by an IgM with rheumatoid factor activity. A similar mechanism exists in mixed cryoglobulinaemia (MC), a different pathological entity in which hepatitis C virus infection has been demonstrated to play an important etiopathogenetic role. Although there are significant differences between SS and MC, they also share many clinical and immunological characteristics, which suggest that common pathogenetic mechanisms may underlie both disorders.

Activity criteria in systemic lupus erythematosus.

Several clinical indices have been proposed to measure disease activity in systemic lupus erythematosus (SLE), a disorder characterised by alternate phases of flare and remission. Over the last 5 years a European multicenter study was carried out in order to reach a consensus on the definition of SLE activity. A new index, ECLAM (European Consensus Lupus Activity Measurement), was created and then validated in the first and in the second parts of the study, respectively. In addition, a comparison between ECLAM and the other most commonly used lupus activity indices was performed. ECLAM appeared to be the best activity index for classifying lupus patients, whether used as a single state index (i.e., to measure activity at a given moment in time) or as a transition index. (i.e., to measure variations in activity over time).

Evidence of neuromuscular hyperexcitability features in patients with primary fibromyalgia.

The presence of clinical and electromyographic (EMG) features of neuromuscular hyperexcitability (NMHE) and of the commonly associated neurovegetative disturbances (NVD) were investigated in 49 patients with primary fibromyalgia (PF) and in a control group of 33 patients with rheumatoid arthritis (RA). At least two clinical features of NMHE were present in 39%, and at least three NVD in 63% of PF patients. In contrast, only 1 RA control had two NMHE features (p greater than 0.005) and three NVD (p less than 0.001). Moreover, a significant post-ischemic spontaneous EMG hyperactivity was observed in 11 PF patients, and in only 1 control with RA (p less than 0.05). Finally, in patients with PF the number of tender points were correlated with psychological tests for depression (p less than 0.02), and the number of NVD. The present study shows that in patients with PF there is a large prevalence of NMHE complaints and NVD. The potential underlying pathogenetic mechanisms are also discussed.

International survey on the management of patients with SLE. I. General data on the participating centers and the results of a questionnaire regarding mucocutaneous involvement.

OBJECTIVE: The diagnosis and treatment of the mucocutaneous (MC), neuropsychiatric (NP), and renal (RN) manifestations of systemic lupus erythematosus (SLE) remain unsolved issues. To shed light on these issues, a questionnaire was prepared and sent to 153 lupus centres around the world, in order to determine the level of agreement between experts in their approach to these complex aspects of the disease. METHODS: The first section of the questionnaire was designed to collect information on the characteristics of the responding lupus centres. The second section was dedicated to MC manifestations, with questions focusing on: (i) the frequency of MC manifestations as a whole and of the single clinical MC entities; (ii) clinical features, outcome and therapy of subacute cutaneous lupus erythematosus (SCLE); (iii) the utility of the lupus band test (LBT); and (iv) the use of various therapeutic protocols to treat MC manifestations. RESULTS: Sixty-one questionnaires from 19 countries were analysed. Out of these, 37 were completed by Departments of Rheumatology, 21 by Departments of Internal Medicine or Clinical Immunology, and 3 by Departments of Nephrology. About 66% of these centres stated that they were currently following more than 100 lupus cases, 95% had an in-patient ward and 82% had their own laboratory. The American College of Rheumatology classification criteria and various scales for disease activity assessment were regularly used by 87% and 57% of centres respectively. The overall prevalence of MC manifestations was judged to be over 30% by 82% of the respondents (Rs), and over 60% by 36% of the Rs. Among the different MC manifestations, malar rash was reported to be the most frequent (40%), followed by alopecia (24.1%) and oral ulcers (18.6%). In reporting the prevalence of each MC manifestation, the Rs showed a low level of agreement, the coefficient of variation (CV) being > 0.75 for all of the manifestations listed with the exception of malar rash (CV = 0.54). Poor agreement among centers was also found for the reported association of various MC manifestations with SCLE (15 different answers), and on the prognostic factors for SCLE (17 different answers). There was agreement on the best procedure (up to 70% of the Rs preferred a non-UV exposed skin area) and on the utility of the LBT (83% using it only for diagnostic purpose). Hydroxychloroquine was the most popular therapeutic protocol, being used by 85% of the Rs for a wide variety of MC manifestations. Among other therapies, azathioprine was used by 59%, dapsone by 41%, and thalidomide by 35% of the Rs, all to treat a wide spectrum of MC manifestations. Pulse steroid, cyclosporin A and pulse cyclophosphamide were less commonly employed (by 27%, 22% and 13% of the Rs, respectively), and were reserved for the most severe MC manifestations, particularly vasculitis. CONCLUSION: The present survey indicates that, although most of the participating centres had extensive experience in the management of SLE, their approach to the MC manifestations was not homogeneous, and collaborative studies are clearly needed, particularly to optimise the therapeutic protocols.

International survey on the management of patients with SLE. II. The results of a questionnaire regarding neuropsychiatric manifestations.

OBJECTIVE: To determine the diagnostic and therapeutic approach used in clinical practice for the management of systemic lupus erythematosus (SLE) patients with primary SLE-mediated neuropsychiatric (NP) manifestations. METHODS: A questionnaire was drawn up to assess how clinicians manage various clinical manifestations of SLE. A portion of this questionnaire was designed to assess how clinicians diagnose and treat primary NP-SLE. Most of the questions in the NP-SLE section consisted of lists of different clinical manifestations and laboratory or radiological studies that participants were asked to rate on a scale of importance [from 1 (extremely important) to 5 (not important)] to the diagnosis of primary NP-SLE. The questionnaire also assessed how different NP manifestations are treated in clinical practice. The relative importance of each clinical manifestation was determined through its mean score, and the agreement among participants on each issue was determined using the coefficient of variation (CV). Fifty-nine lupus centers participated in the NP-SLE portion of the survey. RESULTS: The clinical manifestations which were considered to be of extreme or major importance for the diagnosis of primary NP-SLE were seizures, psychosis, transverse myelitis, stroke, transient ischemic attack (TIA) and aseptic meningitis. Among the radiological and laboratory studies, only brain magnetic resonance imaging (MRI) and antiphospholipid antibodies (aPL) achieved "extremely important" mean scores (between 1 and 2). aPL testing was used routinely in the majority of patients (mean 96.8%; CV = 0.1), while brain MRI was used less frequently (mean 56.5%; CV = 0.61). Only brain MRI and cerebral angiography were considered to be helpful in differentiating cerebral vasculopathy from multi-infarct (mean score = 1.6 and 1.9, respectively), whereas a prompt response to treatment with increased doses of steroids was considered helpful in differentiating SLE-related psychosis from steroid-induced psychosis (mean score = 1.58). The results of aPL testing, coagulation tests for the lupus anticoagulant, an brain MRI were considered to be of extreme or major importance in decisions involving treatment with anticoagulant or anti-platelet therapy. Symptomatic therapies, such as heparin, or anti-convulsant, anti-platelet, oral anticoagulant, and antipsychotic therapy were the most widely used. Corticosteroids were the most frequently used immunosuppressive therapy. The administration of other immunosuppressive agents as specific treatment for NP-SLE was uncommon. CONCLUSIONS: Our survey found that in clinical practice, the NP manifestations currently considered to be diagnostic of primary SLE-mediated CNS involvement are not limited to those included in the American Rheumatism Association (ARA) criteria, e.g. seizures and psychosis. Antiphospholipid antibodies appeared to be the laboratory parameter most frequently relied upon in the diagnosis of NP-SLE, and in decisions regarding treatment. Apart from that, only brain MRI and, in selected cases, cerebral angiography seemed to be of real help in diagnosis. The lack of consensus regarding the treatment of primary NP-SLE manifestations most probably reflects both the complex nature of neurological illness in SLE patients and the lack of clear diagnostic criteria.

International survey on the management of patients with SLE. III. The results of a questionnaire regarding renal involvement.

OBJECTIVE: To assess the practice patterns in the management of lupus nephritis (LN) of physicians dealing with systemic lupus erythematosus. METHODS: A multiple choice questionnaire was sent to 135 lupus centers, mainly in Europe. It was divided into 4 sections, one of which regarded LN. Sixty-one centers (40%) sent the questionnaire back before the meeting; however two of them did not fill out the LN section. Therefore, 59 valid LN questionnaires were collected and analyzed. Statistical evaluation was performed using frequency analysis and the chi-square test. RESULTS: In 50 centers (85%), renal biopsy is performed in all patients with clinically evident renal involvement, and in most of them it is repeated in cases of relapse and/or ineffectiveness of treatment. Oral steroid alone is the therapy preferred by 67% of responding physicians in patients with WHO class II LN. Multi-drug therapy is favoured by 57% in patients with class III LN, by 79% for mild-to-moderate forms of class IV LN (IVm), by 84% for moderate-to-severe forms of class IV LN (IVs), by 47% for mild-to-moderate forms of class V LN (Vm), and by 65% for moderate-to-severe forms of class V LN (Vs). Steroids plus cyclophosphamide (CYPH) is the association most commonly used for class III, IVm and IVs LN, having been indicated by 70%, 80% and 88% of the centers, respectively. Furthermore, pulse CYPH is largely preferred to oral CYPH by the majority of centers. It is worth noting that 41 centers (70%, p < 0.01) utilise the same drugs in the treatment of both WHO class IVm and IVs LN. No clear trends in the use of multi-drug associations were identifiable in the treatment of class V LN. Moreover, most of the centers (64%) said that they rely on histologic parameters in order to define renal prognosis and that they consider the chronicity index to be the best predictor of poor renal outcome (74% of the centers). CONCLUSIONS: It was possible to identify some clear trends in the behaviour of physicians who are "expert" in lupus patients: (i) they perform a renal biopsy in order to charaterize the LN and repeat it when they are faced with relapse or ineffective therapy; (ii) they treat WHO class II LN with oral steroids alone and class III and IV LN with steroids associated with CYPH (CYPH, generally in a pulse regimen); and (iii) they define renal prognosis by means of histologic predictors, especially the chronicity index. However, no trend seemed to exist for the treatment of class V LN, particularly Vm.

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)

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.

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.

Parotid sialography and lip biopsy in the evaluation of oral component in Sjögren's syndrome.

In order to evaluate their sensitivity and specificity, parotid sialography (PS) and labial salivary gland biopsy were performed in 150 putative patients with Sjögren's syndrome (SS) (74 with SS alone and 76 with SS associated with other well-defined disorders). Although PS and LSGB strongly correlated to each other (Kendall's Tau 0.47, z = 7.78, p less than 0.0001), PS confirmed the diagnosis of SS in a significantly higher prevalence of patients with respect to LSGB (63% vs 37%, chi 2 = 18.25, p less than 0.001). When the sensitivity and specificity of PS and LSGB were calculated, PS showed a better sensitivity, and an only slightly lower specificity than LSGB (86% vs 70% and 78% vs 86% respectively). In conclusion, our study indicates that PS has been unduly overlooked in the past decades as a diagnostic tool for the salivary component of SS, and should be reconsidered as a potentially useful and relatively non-invasive procedure in the approach of patients with this disease.