Population mortality before and during the COVID-19 epidemic in two Sudanese settings: a key informant study.

BACKGROUND: Population mortality is an important metric that sums information from different public health risk factors into a single indicator of health. However, the impact of COVID-19 on population mortality in low-income and crisis-affected countries like Sudan remains difficult to measure. Using a community-led approach, we estimated excess mortality during the COVID-19 epidemic in two Sudanese communities. METHODS: Three sets of key informants in two study locations, identified by community-based research teams, were administered a standardised questionnaire to list all known decedents from January 2017 to February 2021. Based on key variables, we linked the records before analysing the data using a capture-recapture statistical technique that models the overlap among lists to estimate the true number of deaths. RESULTS: We estimated that deaths per day were 5.5 times higher between March 2020 and February 2021 compared to the pre-pandemic period in East Gezira, while in El Obeid City, the rate was 1.6 times higher. CONCLUSION: This study suggests that using a community-led capture-recapture methodology to measure excess mortality is a feasible approach in Sudan and similar settings. Deploying similar community-led estimation methodologies should be considered wherever crises and weak health infrastructure prevent an accurate and timely real-time understanding of epidemics' mortality impact in real-time.

Alcohol Addiction: One Entity or Different Entities? A DSM-4-Based Attempt Toward a Geographicization of Alcohol Addiction and Abuse.

AIM: To examine whether in Europe perceptions of 'alcoholism' differ in a discrete manner according to geographical area. METHOD: Secondary analysis of a data set from a European project carried out in 2013-2014 among 1767 patients treated in alcohol addiction units of nine countries/regions across Europe. The experience of all 11 DSM-4 criteria used for diagnosing 'alcohol dependence' and 'alcohol abuse' were assessed in patient interviews. The analysis was performed through Multiple Correspondence Analysis. RESULTS: The symptoms of 'alcohol dependence' and 'alcohol abuse', posited by DSM-IV, were distributed according to three discrete geographical patterns: a macro-area mainly centered on drinking beer and spirit, a culture traditionally oriented toward wine and a mixed intermediate alcoholic beverage situation. CONCLUSION: These patterns of perception seem to parallel the diverse drinking cultures of Europe.

Potentially inappropriate prescribing before and after nursing home admission: a retrospective observational study in a sample of Italian nursing homes.

OBJECTIVES: to assess the occurrence of potentially inappropriate prescribing (PIP) in residents of Tuscany nursing homes (NHs) and its variation before and after NH entry. DESIGN: retrospective observational study using data from the Regional Administrative Database of Tuscany. SETTING AND PARTICIPANTS: the study involved residents of 67 Tuscan NHs identified between 2011 and 2012. To estimate PIP prevalence before and after NH, a subset of 10 indicators of the Screening Tool of Older Person's Prescriptions (STOPP) criteria were selected. MAIN OUTCOME MEASURES: prevalence of PIP. RESULTS: considering 2,801 NH residents, the proportion of PIP ranged from 0.0% to 55.2% and from 0.0% to 33.9% before and after the NH admission, respectively. Overall, this study showed a decrease in the occurrence of PIP after the NH admission for most of the indicators, reaching statistical significance for indicator 3 (tricyclic antidepressants in combination with an opiate or calcium channel blockers), 7 (prescription of NSAIDs in heart failure patients), and 9 (warfarin in combination with NSAIDs). CONCLUSIONS: although the reduction of PIP after NH admission may suggest greater awareness about the appropriateness of drug use, more efforts still need to be made.

Multiple sclerosis spatial cluster in Tuscany.

Tuscany (Central Italy) is a high-risk area for multiple sclerosis (MS) with a prevalence of 188 cases per 100,000 at 2011, and it is characterized by a heterogeneous geographic distribution of this disease. Our objective was to update prevalence at 2013 and to evaluate the presence of spatial clusters in Tuscany. The MS prevalence was evaluated on 31 December 2013 using a validated case-finding algorithm, based on administrative data. To identify spatial clusters, we calculated standardized morbidity ratios (SMRs) for each Tuscan administrative municipality. In addition to the classical approach, we applied the hierarchical Bayesian model to overcome random variability due to the presence of small number of cases per municipality. We identified 7330 MS patients (2251 males and 5079 females) with an overall prevalence of 195.4/100,000. The SMR for each Tuscan municipality ranged from 0 to 271.4, but this approach produced an extremely non-homogeneous map. On the contrary, the Bayesian map was much smoother than the classical one. The posterior probability (PP) map showed prevalence clusters in some areas in the province of Massa-Carrara, Pistoia, and Arezzo, and in the municipalities of Siena, Florence, and Barberino Val d'Elsa. Our prevalence data confirmed that Tuscany is a high-risk area, and we observed an increasing trend during the time. Using the Bayesian method, we estimated area-specific prevalence in each municipality reducing the random variation and the effect of extreme prevalence values in small areas that affected the classical approach.

Problematic drinking and health problems: an epidemiologic study through general practitioners in the Tuscany Region (Central Italy) and Friuli Venezia Giulia Region (Northern Italy). / Bere problematico e problemi di salute in Toscana e in Friuli Venezia Giulia: uno studio epidemiologico attraverso i medici di medicina generale.

"OBJECTIVES: to identify the differences among patients of general practictioners (GPs) in both Tuscany Region (Central Italy) and Friuli Venezia Giulia (FVG) Region (Northern Italy), which are different for drinking cultures, as to motivation of consultation, hazardous drinking and alcohol dependence, health problems, and use of health services. DESIGN: cross-sectional study by means of both a medical examination and a subsequent structured interview carried out with a questionnaire. Data were analysed using chi-square test, logistic regression and differences in prevalence. SETTING AND PARTICIPANTS: the study was implemented between July and November 2013 on a sample of 492 patients of 30 GPs in FVG, and 451 patients of 25 GPs in Tuscany. RESULTS: although patients in FVG were less likely to drink alcohol (66.7% vs. 70.9%), consumed lower amounts of alcohol on average per day per drinker (10.9 vs. 14.5 grams of alcohol), and were less likely to be hazardous drinkers (11.2% vs. 13.8%) compared to patients in Tuscany, they had a 3.6 to 4.7 times higher risk of alcohol dependence. In addition, the prevalence of diseases (in particular hepato-gastrointestinal diseases, hypertension, and psychiatric problems), smoking, and obesity/ overweighting was higher among clients of FVG, which exceed the Tuscan patients by 5-12 percentage points. Compared to Tuscany, FVG patients were more hospitalized and required more help to GPs or other people for their drinking problems. CONCLUSIONS: compared to Tuscan patients, GPs' patients in FVG has higher prevalence of alcohol addiction and other diseases, as well as of smoking and overweight/obesity, and higher need for health interventions as to their drinking problems."

[Impacts of geocoding quality in environmental epidemiology studies: two case-studies in Tuscany Region (Central Italy)]. / L'impatto della qualità della georeferenziazione negli studi di epidemiologia ambientale: due casi studio in Toscana.

OBJECTIVES: Geographical Information Systems (GIS) are widely used in environmental epidemiology studies to locate study population by geocoding addresses and to evaluate exposures and relationship with health outcomes. Despite this, Italian environmental epidemiologists poorly discuss quality of address geocoding results. DESIGN: two case-studies have been carried out in Tuscany Region (Central Italy): one in the mountain area in the Municipality of Piancastagnaio (Siena Province) and one in the urban area around the airport of Florence. Three geocoding systems have been compared: the geographical database produced by Tuscany Region and two commercial systems (Google and Bing-Microsoft); 1,549 addresses in Piancastagnaio and 2,946 addresses in Florence have been tested. RESULTS: Tuscan geographical database showed better performance than the two commercial systems, with bigger differences in Piancastagnaio. In this area, mean difference between regional system and Google service is more than 300 mt, with peaks of 7-8 km. Bing- Microsoft system does not provide any information on addresses in Piancastagnaio: all input addresses were geocoded in the centroid of the municipality or in the centre of a few principal streets. Lowest differences among the three methods were observed in the urban area of Florence: mean difference between Tuscany and Goggle systems was 150 mt, with less than 2 km peaks; between Tuscany and Bing-Microsoft mean difference was 100 mt with 3 km peaks. In both case-studies, but especially in Piancastagnaio area, these differences gave rise to great misclassification in the evaluation of individual exposure and health outcome. CONCLUSION: the study highlighted the impacts of address geocoding process in exposure assessment in environmental health research and pointed out the need of specifically evaluate the quality of cartographic data.

A cross-sectional survey to investigate the quality of care in Tuscan (Italy) nursing homes: the structural, process and outcome indicators of nutritional care.

BACKGROUND: Previous studies have investigated process and structure indicators of nutritional care as well as their use in nursing homes (NHs), but the relative weight of these indicators in predicting the risk of malnutrition remains unclear. Aims of the present study are to describe the quality indicators of nutritional care in older residents in a sample of NHs in Tuscany, Italy, and to evaluate the predictors of protein-energy malnutrition risk. METHODS: A cross-sectional survey was conducted in 67 NHs. Information was collected to evaluate quality indicators of nutritional care and the individual risk factors for malnutrition, which was assessed using the Malnutrition Universal Screening Tool. A multilevel model was used to analyse the association between risk and predictors. RESULTS: Out of 2395 participants, 23.7 % were at high, 11 % at medium, and 65.3 % at low risk for malnutrition. Forty-two percent of the NHs had only a personal scale to weigh residents; 88 % did not routinely use a screening test/tool for malnutrition; 60 % used some standardized approach for weight measurement; 43 % did not assess the severity of dysphagia; 12 % were not staffed with dietitians. Patients living in NHs where a chair or platform scale was available had a significantly lower risk of malnutrition (OR = 0.73; 95 % CI = 0.56-0.94). None of the other structural or process quality indicators showed a statistically significant association with malnutrition risk. CONCLUSIONS: Of all the process and structural indicators considered, only the absence of an adequate scale to weigh residents predicted the risk of malnutrition, after adjusting for case mix. These findings prompt the conduction of further investigations on the effectiveness of structural and process indicators that are used to describe quality of nutritional care in NHs.

In vivo confocal scanning laser microscopy in patients with primary Sjögren's syndrome: A monocentric experience.

AIMS: (i) To analyze the in vivo corneal structure and sub-basal plexus nerves in patients with primary Sjögren's syndrome (pSS) and no-SS dry eye by confocal scanning laser microscopy (CSLM) and (ii) to correlate CSLM findings with tear function tests and with patients' subjective dryness. METHODS: Seventeen patients with pSS, 16 no-SS dry eye, and 20 healthy volunteers were included. CSLM parameters taken into consideration included: basal epithelial integrity, corneal thickness, epithelial cellular density, keratocyte activation, and sub-basal plexus morphology. Statistical analysis was carried out using SPSS-13 (Chicago IL, USA). RESULTS: CSLM pachymetric data and the superficial epithelium cell density were significantly lower in pSS versus no-SS dry eye (p < 0.0001); keratocyte activation and sub-basal nerve abnormalities were also more frequent in pSS patients (p < 0.0001). CSLM findings well correlated with both the ocular test results and the patients' perception of ocular dryness at the baseline and over the follow-up. CONCLUSION: CSLM might be a useful novel tool in the assessment of the involvement of the lachrymal functional unit in pSS.

Primary Sjogren's syndrome as a multi-organ disease: impact of the serological profile on the clinical presentation of the disease in a large cohort of Italian patients.

OBJECTIVE: The aims of this study were to describe the clinical presentation of primary SS (pSS) in a large cohort of patients by assessing the prevalence of the patient subgroups at high risk for severe extraglandular manifestations and to explore the influence of the patients' serological profile on disease severity and on immunosuppressive drug utilization. METHODS: Cumulative demographic, clinical, serological, histological and therapeutic data of 1115 pSS patients were retrospectively evaluated. Independent serological markers for glandular and extraglandular disease manifestations were identified by logistic regression. RESULTS: The cohort included 1115 (1067 female, 48 male) pSS patients. Severe extraglandular manifestations were detectable in 15% of the patients and were represented by active synovitis (11%), axonal sensory-motor neuropathy (2%), severe leucocytopenia (14%), cutaneous vasculitis (6%) and non-Hodgkin's lymphoma (4.5%). We found that low C3/C4, hypergammaglobulinaemia, RF and cryoglobulinaemia were markers of severity for pSS. According to the number of serological variables, the patients were subdivided into three distinct groups: favourable (no serological markers), intermediate (one serological marker) and poor (two or more serological markers). In comparison with the other two patient groups, pSS patients presenting with two or more adverse determinants had a higher frequency of severe visceral disease complications and required more aggressive therapeutic interventions. CONCLUSION: This study confirmed that the prevalence of the pSS high-risk subset for severe systemic manifestations is ∼15%. Serological markers might help in the early identification of patients who are candidates to receive more aggressive treatments.

Vitamin D in "early" primary Sjögren's syndrome: does it play a role in influencing disease phenotypes?

Beyond its well-established role in the maintenance of mineral homeostasis, 25-OH-vitamin D deficiency seems to be involved in the development and severity of several autoimmune diseases. To date, contrasting data have been reported regarding the presence of hypovitaminosis D in primary Sjögren's syndrome (pSS). To assess the prevalence of hypovitaminosis D in pSS at an early stage of the disease and to evaluate its impact on pSS clinical manifestations and disease activity, unselected consecutive subjects with recent onset dry mouth and/or dry eyes who underwent a comprehensive diagnostic algorithm for pSS (AECG criteria) were prospectively included in the study. The levels of 25[OH]-D3 were measured by monoclonal antibody immunoradiometric assay. Conditions of 25[OH]-D3 severe deficiency, deficiency, and insufficiency were defined as levels <10, <20, and 20-30 ng/ml, respectively, and their frequencies were investigated in pSS patients and controls. The levels of 25[OH]-D3 were also correlated with patients' demographic, clinical, and serologic features. Seventy-six consecutive females were included: 30/76 patients fulfilled the AECG criteria for pSS. The remaining 46/76 patients represented the control group. No statistical differences were found in the serum levels of 25[OH]-D3 between pSS patients [median levels = 20 ng/ml (IQR 9.3-26)] and controls [median levels = 22.5 ng/ml (IQR 15.6-33)]. In particular, the frequency of 25[OH]-D3 severe deficiency was not significantly different in patients with pSS when compared to controls (23 vs. 17.4 %, p value = 0.24). We found a significant correlation between serum 25[OH]-D3 levels and white blood cells count (r = 0.29, p = 0.01). More specifically, leukocytopenia was significantly associated with 25[OH]-D3 severe deficiency, being documented in 40 % of the subjects with a 25[OH]-D3 severe deficiency and in 11 % of the subjects without a severe vitamin D deficiency (p = 0.02). We did not observe any further association or correlation between hypovitaminosis D and pSS glandular and extra-glandular features. Although the role of hypovitaminosis D in pSS pathogenesis remains controversial, the results of this study encourage the assessment of vitamin D in specific pSS subsets that could mostly benefit from a supplementation.

Cancer-associated myositis: a 35-year retrospective study of a monocentric cohort.

This study is aimed at retrospectively studying cancer-associated inflammatory myopathies (CAM) in a cohort of patients with inflammatory myopathies. CAM were diagnosed if the tumor was diagnosed 2 years before or after disease onset. One hundred and sixty-two patients were included, 27 (17 %) had CAM. A significant association was observed between CAM and dermatomyositis (DM), older age and dysphagia at disease onset. CAM have lower creatine kinase (CK) levels at onset and a low prevalence of autoantibodies. In conclusion, the association of male sex, older age, DM, dysphagia at onset, lower CK, and autoantibodies negativity carries a high suspicion of CAM.

Changing trends in European alcoholic beverage drinking: selected social, demographic, economic factors, drinking's related harms, and prevention control policies between the 1960s and 2000s.

This study confirms that during the decades following WW II there was a tendency towards closure of consumption of alcoholic beverages among the European countries. The Northern countries, which during the 1960s manifested the lowest rates of alcohol consumption, ended up with greater consumption rates than the Southern countries, manifested the opposite trend; greater amounts of consumptions in the 1960s and lower consumptions in the 2000s. During the same some period, social, demographic and economic indicators--urbanization, rate of elderly males, Income, female education, female employment and mother's age at their childbirths, tended to increase, while the alcoholic beverage control policy strategies showed differences according to the country. Liver disease-related mortality, decreased in most countries. Study limitations are noted.

Contextual determinants and alcohol control policies in the United Kingdom.

In the United Kingdom, between 1960 and the 2000s, there were many sociodemographic and economic factors that played a part in the changing picture of alcohol consumption and its related harm. This paper describes some of these variables along with the political measures that were identified as correlated with changes in consumption and harm. The resulting picture is unclear. No consistent pattern was identified among the variables analyzed. Beverage choice changed over time with a reduction in beer consumption and an increase in wines and spirits. Nevertheless, the overall picture showed an increase in total alcohol consumption and resulting harm.

Trends in consumption of alcoholic beverages and policy interventions in Europe: an uncertainty "associated" perspective.

Having qualitatively investigated, both the temporal curves of alcoholic beverage consumption trends and the introduction of preventive alcohol policy measures in six European countries during the 1960s-2000s, drinking control policy measures often appeared to operate as co-factors of change, while during some periods of time they were not even present even if effective consumption changes were occurring. Study limitations are noted.

Italy between drinking culture and control policies for alcoholic beverages.

This paper focuses on whether the on-going dramatic decrease in alcohol consumption in Italy, especially of wine, during 1961-2008, was associated with which parallel sociodemographic and economic changes and with alcohol control policies. The study, using both time series (TS) and artificial neural network (ANN)-based analyses documents that its selected sociodemographic and economic factors, and particularly urbanization, had a definite connection with wine consumption decrease, spirits decrease, and the increase in beer consumption over time. On the other hand, control policies showed no effect on the decline in alcohol consumption, since no alcohol control policy existed in Italy between 1960 and 1987. A few policies introduced since 1988 (BAC and sale restrictions during mass events) may have contributed to reducing or to maintaining the on-going reduction. Study limitations are noted and future needed research is suggested.

Europe. An analysis of changes in the consumption of alcoholic beverages: the interaction among consumption, related harms, contextual factors and alcoholic beverage control policies.

This AMPHORA study's aim was to investigate selected factors potentially affecting changes in consumption of alcoholic beverages in 12 European countries during the 1960s-2008 (an average increase in beer, decreases in wine and spirits, total alcohol drinking decrease). Both time series and artificial neural networks-based analyses were used. Results indicated that selected socio-demographic and economic factors showed an overall major impact on consumption changes; particularly urbanization, increased income, and older mothers' age at their childbirths were significantly associated with consumption increase or decrease, depending on the country. Alcoholic beverage control policies showed an overall minor impact on consumption changes: among them, permissive availability measures were significantly associated with consumption increases, while drinking and driving limits and availability restrictions were correlated with consumption decreases, and alcohol taxation and prices of the alcoholic beverages were not significantly correlated with consumption. Population ageing, older mother's age at childbirths, increased income and increases in female employment, as well as drink driving limitations were associated with the decrease of transport mortality. Study's limitations are noted.

The relationships between the impact of alcoholic beverage control policies, selected contextual determinants, and alcohol drinking in Spain.

Alcohol prevention policies alone neither cause nor explain changes in alcohol consumption, nor in related harm. Alcohol consumption in Spain throughout the period 1962-2008 was analyzed considering selected contextual factors and alcohol policies. Increased urbanization was found to be associated with higher consumption, especially of beer. Restrictive policies regulating purchase age, advertising, and licensing premises to sell alcohol were associated with decreased alcohol consumption, while lower blood alcohol concentration limits were followed by an increase. Study limitations are noted. Changes in the evolution of socioeconomic, sociodemographic, and cultural factors should be carefully analyzed to inform alcohol policy planning and evaluation.

Multifocal contact lens success predictability.

PURPOSE: The current multi-study analysis combined data from three studies to quantify the relationship between the initial reaction to soft multifocal contact lens (MFCL) design types at dispensing and evaluate the predictability of overall vision satisfaction (OVS) and intention to purchase (ITP) after 1 week of wear. METHOD: Three prospective studies tested MFCLs over 1-week of wear following the same protocol, using a range of potentially predictive ratings at dispensing, and both OVS and ITP at 1-week as an indicator acceptance level. In each study, two of MyDay® multifocal, clariti® 1 day multifocal, Biofinity® multifocal (worn as a daily disposable lens) or 1 DAY ACUVUE® MOIST MULTIFOCAL were dispensed for 1-week of daily wear. OVS was recorded on a 100-point VAS and ITP on a 5-point LIKERT scale. Fourteen possible predictors were entered in the statistical model, and predictability was assessed using Chi-square Automatic Interaction Detector (CHAID) statistical test. RESULTS: A total of 210 participants (152 female & 58 male; 53.9 ± 6.5 years, range 41-71 years), representing 420 MFCL fits, equally distributed between emergent n = 65, established n = 70 and advanced n = 75 presbyopes, completed the studies. OVS on dispensing was the predictor of both OVS (p < 0.001) and ITP (p < 0.001) at 1-week. For OVS predictability, 70.8 % with OVS at dispensing > 91 points reported good OVS at 1-week and 73.4 % with OVS on dispensing ≤ 80 points reported poor OVS at 1-week. For ITP predictability, 74.6 % with OVS at dispensing > 94 points reported a positive ITP at 1-week and 65.9 % with OVS on dispensing ≤ 63 points reported negative ITP at 1-week. CONCLUSION: Overall vision satisfaction at the time of dispensing MFCLs is a powerful indicator of both OVS and ITP after 1-week of wear. Initial patient subjective assessments provide a clinically useful indicator of the likeliness of success.

Alteration of microcirculation is a hallmark of very early systemic sclerosis patients: a laser speckle contrast analysis.

OBJECTIVES: To investigate blood flow and microvascular reactivity by laser speckle perfusion imager (Perimed, Jarfalla) in consecutive patients affected by Raynaud's phenomenon at baseline and following dynamic stimulations. METHODS: Skin blood flow in the dorsum of the hand was measured at baseline and after cold test and post-occlusive hyperemia test in 56 consecutive subjects affected by Raynaud's phenomenon (RP), 20 primary (PRP) and 36 secondary to systemic sclerosis (SSc). Twenty healthy subjects (HS) were studied as controls. RESULTS: After cold test, SSc had a significant reduction of blood flow (-58%) as compared to HS (-19%) (p=0.01). Recovery time was significantly higher in SSc (58 minutes) as compared to HS (18 minutes) and PRP (19 minutes) (p=0.006 and 0.0016, respectively). Peak flow after ischaemic test was significantly reduced in SSc (+237%) as compared to PRP (+485%) (p=0.0068). Post-ischaemic hyperemic area under the curve (AUC) was blunted in SSc (79U/sec) compared to PRP (167 U/sec) (p=0.0126). Proximal distal gradient was noticed in 74% of HS, 45% of PRP and 36% of SSc (p=0.01). Homogeneous pattern of flux distribution was significantly different between HS (95%), PRP (80%), and SSc (16%) (p<0.0001). Among SSc patients, a significant difference in ischaemic challenge was shown between patients with early-SSc versus patients with definite-SSc. CONCLUSIONS: Our preliminary results indicate a clearcut alteration of the dynamic of microcirculation in SSc-RP as compared to PRP and HS. Among SSc patients, early-SSc is a separate entity as compared to established disease.

Overlap of ACA-positive systemic sclerosis and Sjögren's syndrome: a distinct clinical entity with mild organ involvement but at high risk of lymphoma.

OBJECTIVES: We aimed to assess the prevalence of patients with either primary Sjögren's syndrome (pSS) and positive anticentromere antibodies (ACA) and secondary Sjögren's syndrome (sSS) and limited cutaneous ACA positive-systemic sclerosis (SSc) in two large cohorts of patients with pSS and SSc¸ and also to compare the clinical features of these two subsets with those of patients affected by 'ACA-positive SSc without sicca symptoms' and 'pSS'. METHODS: In this retrospective monocentric study, the case records of 'overlap' patients fulfilling both the classification criteria for SS and the LeRoy criteria for early SSc were identified from two datasets of patients with limited cutaneous ACA positive SSc (209 subjects) and with pSS (402 subjects) who attended our Rheumatology Unit in the years between 1989 and 2011. Control groups were represented by SSc subjects without sicca symptoms ('SSc group') and ACA negative Pss patients ('pSS group'). SSc patients with sicca symptoms ('Sicca-SSc group') who did not complete the diagnostic algorithm for SS were excluded from the analysis. Demographic, clinical and immunological data of the patients enrolled were collected cumulatively over the entire follow up period. Statistical analysis was performed using SPSS 13 (SPSS Inc., Chicago IL, USA). RESULTS: Out of the two datasets 41 'overlap' patients were selected. The control groups were represented by 102/209 SSc subjects without sicca symptoms ('SSc group') and 387/402 pSS patients ('pSS group'). Eighty-one 'sicca-SSc' with an incomplete work-up for SS were excluded from the analysis. The prevalence of ACA positive pSS patients among pSS was 3.7% (15/402), while the frequency of patients with definite sSS in the SSc cohort was 20% (26/128). No differences were detected between 'overlap' patients and control groups, relatively to demographic characteristics. 'Overlap patients' were characterised by a milder SSc disease (i.e. lower frequency of sclerodactily, negative evolution of the capillaroscopy pattern or absence of severe systemic involvement) whereas, as far as the SS-related manifestations were concerned, although often lacking in specific autoantibodies (i.e. rheumatoid factor, anti-Ro/SSA, anti-La/SSB), the 'overlap patients' displayed a full blown SS phenotype with recurrent salivary gland enlargement, purpura, fatigue, arthralgias, and leukocytopenia. It is noteworthy that the prevalence of non-Hodgkin's lymphoma in the 'overlap patients' was higher than in pSS. CONCLUSIONS: Taken together, the results of our work emphasise the existence of a novel distinct clinical entity which might tentatively be called 'ACA-positive limited scleroderma/SS overlap syndrome' characterised by a benign SSc clinical course but at a high risk of non-Hodgkin's lymphoma.