ObjectiveRelapsing polychondritis (RP) is a rare autoimmune inflammatory disease that attacks mainly cartilaginous structures or causes serious damage in proteoglycan-rich structures (the eyes, heart, blood vessels, inner ear). This study shows results regarding the epidemiology, progression, and associations of this highly variable disease by collecting all cases from a 124-million-person-year Central European nationwide cohort.MethodsWe used the Hungarian Health Care Database to identify all persons with possible RP infection. We followed patients who had International Classification of Diseases 10th edition code M94.1 at least once in their inpatient or outpatient records between January 1, 2002 and December 31, 2013 in Hungary. We classified these patients into disease severity groups by their drug consumption patterns between January 1, 2010 and December 31, 2013. We analyzed the regional distribution of RP incidences as well. Overall maps of comorbidity are presented with network layouts.ResultsWe identified 256 patients with RP among cumulatively 11.5 million registered inhabitants. We classified these patients into four severity classes as “extremely mild” (n=144), “mild” (n=22), “moderate” (n=41), and “severe” (n=4). Two additional groups were defined for patients without available drug data as “suspected only” (n=23) and “confirmed but unknown treatment” (n=22). The age and sex distributions of patients were similar to worldwide statistics. Indeed, the overall survival was good (95% confidence interval for 5 years was 83.6%–92.9% and for 10 years was 75.0%–88.3% which corresponds to the overall survival of the general population in Hungary), and the associations with other autoimmune disorders were high (56%) in Hungary. Almost any disease can occur with RP; however, the symptoms of chromosomal abnormalities are only incidental. Spondylosis can be a sign of the activation of RP, while Sjögren syndrome is the most frequent autoimmune association. Regional distribution of incidences suggests arsenic drinking water and sunlight exposure as possible triggering factors.ConclusionThe good survival rate of RP in Hungary is probably associated with the early diagnosis of the disease.

According to this whole population based study from Hungary, the incidence of lower limb major amputation is high with no change over the past 9 years. An explanation for this remains to be determined, as the traditional risk factors in Hungary do not account for it. The characteristics of major amputation (the rate of primary amputation, the ratio of below to above knee amputation and the age of the affected population) underline the importance of screening, early detection, improved vascular care and an optimal revascularization policy. Standardization and validation of amputation detection methods and reporting is essential.

WHAT THIS PAPER ADDSThis study is the first long period report from the Central Eastern European region on lower limb amputations and vascular procedures. These two complementary indicators of vascular care show a 10 year lag compared with international trends (decline in amputations and change from open to endovascular procedures). Also, the volume of procedures is at least twofold higher for major amputations and close to half for vascular procedures, in comparison with findings from more developed countries. This report calls attention to the importance of obtaining data from across Europe to explore inequalities in vascular care.Objective: The aim of this study was to assess the long term trends of lower limb amputation and revascularisation in Hungary over 14 years. Methods: This was a retrospective cohort study that included all patients who underwent lower limb amputation or revascularisation over a 14 year period (2004e2017) in Hungary. Inpatient administrative data claims covering the entire beneficiary population were incorporated. Lower limb amputations (both minor and major) and revascularisation procedures (both open and endovascular) were identified in the claims files. Incidence rates were calculated and time trends were assessed via a generalised additive model. Results: From 2004 to 2017, a total of 121 351 lower limb amputations (61 154 minor; 60 197 major) and 149 355 revascularisation procedures (89 243 open; 60 112 endovascular) were detected in 140 581 patients.The number of minor amputations decreased moderately in the last few years of the study period, while major amputations showed a slight decline (15%) beginning after 2013, which was more marked (22%) following adjustment for age. While the crude incidence of open vascular surgery procedures decreased by 31% (from 74.5/10 5 to 51.4/10 5 ), endovascular procedures showed growth by 79% (from 33.7/10 5 to 60.4/10 5 ) over the whole observation period. Conclusion: Observed amputation and revascularisation trends in Hungary are similar to the international experience. The major difference is a more than one decade lag in the starting point of the decline of amputations and in the move towards endovascular procedures. The number of amputations is more than twofold higher and the number of revascularisations is close to half that reported internationally. This comprehensive report of two vascular care performance indicators reveals an east/west vascular health divide in Europe and indicates the need to improve amputation prevention.

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Summary. Although more and more data on lower limb amputations are becoming available by leveraging the widening access to health care administrative databases, the applicability of these data for public health decisions is still limited. Problems can be traced back to methodological issues, how data are generated and to conceptual issues, namely, how data are interpreted in a multidimensional environment. The present review summarised all of the steps from converting the claims data of administrative databases into the analytical data and reviewed the wide array of sources of potential biases in the analysis of such data. The origins of uncertainty of administrative data analysis include uncontrolled confounding due to a lack of clinical data, the left- and right-censored nature of data collection, the non-standardized diagnosis/procedure-based data extraction methods (i.e., numerator/denominator problems) and additional methodological problems associated with temporal and spatial analyses. The existence of these methodological challenges in the administrative data-based analysis should not deter the analysts from using these data as a powerful tool in the armamentarium of clinical research. However, it must be done with caution and a thorough understanding and respect of the methodological limitations. In addition to this requirement, there is a profound need for pursuing further research on methodology and widening the search for other indicators (structural, process or outcome) that allow a deeper insight how the quality of vascular care may be assessed. Effective research using administrative data is based on strong collaboration in three domains, namely expertise in claims data handling and processing, the clinical field, and statistical analysis. The final interpretations of results and the countermeasures on the level of vascular care ought to be grounded on the integrity of research, open discussions and institutionalized mechanisms of science arbitration and honest brokering.