A Scoping Review of Educator Proficiency Interventions in Pharmacy Education Illustrated by an Interdisciplinary Model Integrating Pedagogical Theories into Practice.

Pharmacy schools recognize the need for flexibility and comprehensive curricular transformation with a competency-based focus to effectively prepare for the evolving practitioner competencies and challenges of the pharmacy profession. The curricular implementation of evidence-based teaching and learning theories and practices demands educator proficiency through skills development with indispensable faculty leadership support. Our scoping review of online databases and pharmacy education-related journals aims to identify faculty development interventions or teaching proficiency programs that integrate educational and pedagogical theories. Original studies and reviews published between 2010 and 2022 were screened based on four inclusion criteria. Thirty-four manuscripts were eligible for full-text analysis, of which seven results referenced target faculty pedagogy knowledge development. Nine key messages, as Results Statements, synthesize and provide a framework for our results analysis. An ongoing Hungarian intervention model of comprehensive faculty development with strong interdisciplinary cooperation is discussed in our study to illustrate the applicability of the Results Statements through each stage of the process. Educator motivation and relatedness to students or awareness of the educator roles are intrinsic factors, which may not be easily detectable yet significantly impact teaching proficiency and student learning outcomes. The integration of evidence-based pedagogical knowledge and training in educator proficiency development contributes to the sustainability and cost-effectiveness of faculty interventions.

Characteristics of ScleroID highlighting musculoskeletal and internal organ implications in patients afflicted with systemic sclerosis.

BACKGROUND: Systemic sclerosis (SSc) is a multi-organ disease with impaired health-related quality of life (HRQoL). The EULAR SSc Impact of Disease (ScleroID) is a newly introduced SSc-specific patient-reported outcome to evaluate HRQoL in SSc. OBJECTIVE: To investigate the correlation between the ScleroID and the involvement of organ systems as well as disease activity/damage in a SSc cohort from a large tertiary care centre. PATIENTS AND METHODS: The ScleroID and clinical characteristics including internal organ involvement and hand function were investigated in 160 consecutive patients with SSc (median age 46 (43;56) years; diffuse cutaneous SSc 55%). RESULTS: A strong correlation was found between the ScleroID and articular disease activity scores (DAS28-CRP, DAS28-ESR, CDAI, SDAI), a hand function performance test, the Hand Anatomy Index and muscle strength tests. Additionally, a strong significant correlation was discovered using instruments representing hand function and musculoskeletal disability including the Cochin Hand Function Scale, the Quick Questionnaire of the Disability of the Hands, Arms and the Shoulders and the Health Assessment Questionnaire Disability Index. A significant negative correlation was found between the ScleroID score and the 6-min walking test (6MWT) (rho - 0.444, p < 0.001). Clinically mild lung/heart disease did not show increased ScleroID values. The Mouth Handicap in the Scleroderma Scale and the University of California Los Angeles Scleroderma Clinical Trials Consortium gastrointestinal tract 2.0 also showed significant positive correlations to the ScleroID score (rho: 0.626, p < 0.001; rho: 0.646, p < 0.001, respectively). Patients experiencing oesophageal difficulties bore a significantly higher score compared to individuals with a normal functioning oesophagus (3.2/1.5;4.5/ vs. 2.2/1.0;3.2/, p = 0.011). Moreover, the ScleroID showed a significant positive correlation to the revised EUSTAR disease activity index and modified activity index. CONCLUSION: In a large single-centre cohort, the previously described ScleroID-related findings were confirmed. Furthermore, several organ involvement-related functional and performance tests showed a good correlation to the ScleroID including the 6MWT and gastrointestinal-related complaints. Many aspects of musculoskeletal damage, overall disease activity, pain and fatigue were also well represented in the ScleroID, which efficiently reflects the impact of organ involvement, disease activity and functional damage.

Significance of autoantibody assays in systemic lupus erythematosus / Az autoantitest-vizsgálatok jelentosége szisztémás lupus erythematosusban

Systemic lupus erythematosus (SLE) is a systemic autoimmune disease characterized by pathological immune complexes and various autoantibodies. Detectable pathological autoantibodies produced by plasma cells differentiated from B cells play a significant role in the establishment of clinical diagnosis, classification, and differential diagnosis as well as assessing the disease activity during patients' follow-up. Autoantibody determination is very important in

Is present pharmacy education adapted to needs? Survey results from young practitioner views regarding pharmacy education outcome towards a national reform in Hungary.

Background: The pharmacist career is constantly adapting to societal and health care needs. The past decade has seen a growing demand for curricular development to align graduation outcome with workforce competencies. Objective: This study aims to identify expectations for both didactic and experiential components of a new curriculum based on young pharmacist practitioner views. Methods: An online survey questionnaire was used in 2019-2020 to evaluate the pharmacy curriculum to detect indicators or key areas which require comprehensive reform. Results: The predominant majority of the 205 study participants recommended reduction in credit hours for Natural Sciences (78.54%) and a similar increase in the Theoretical and Practical Expertise Module (77.9%). Pharmaceutical care, clinical therapeutics and clinical pharmacy competencies should also be more highlighted in the program. Findings indicate the current training does not prepare for problem-solving and daily workplace challenges (72.7%) or for extended pharmacist skills and competencies (71.71%). Results show inconsistency in practical training experience, as all respondents participated in practical training for drug manufacturing and analysis but 61.0% reported no hands-on skills training in a hospital-clinical simulation setting. Indications for practitioner involvement into the natural sciences and biomedical subjects (86.3%) confirm the obvious need for more practice-oriented education. Conclusions: Educational reforms seem to be inevitable to achieve measurable improvement in professional practice and skills competency. The country specific demand for a needs-based pharmacy education reflects global trends but may also provide useful insights for individual transitions to transform education through practice and improve practice through education.

Minimal Clinically Important Differences (MCID) for the Functional Assessment of Chronic Illness Therapy Fatigue Scale in Patients with Systemic Sclerosis.

(1) Background: Systemic sclerosis (SSc) is characterized by significant fatigue, causing diminished quality of life (QoL). The aim of this study was to examine fatigue levels and their associations with clinical factors and determine the minimal clinically important difference (MCID) value for the Functional Assessment of Chronic Illness Therapy Fatigue Scale (FACIT-FS). (2) Methods: A total of 160 SSc patients and 62 individuals without SSc were followed-up over a 12-month period by measuring the FACIT-FS and the Visual Analogue Scale and the Short Form 36 Vitality Score analyzing changes in exhaustion. (3) Results: Fatigue was strongly correlated with HRQoL, level of pain, emotional disorders, physical capability and functionality. The MCID values for FACIT-FS were calculated as -3 for deterioration and +4 for improvement after a 12-month follow-up. The predictors of improvement of fatigue from baseline parameters were the significant disease activity, the patients' poorer functionality and the short disease duration. Patients with scleroderma-related interstitial lung disease at baseline had approximately tripled risks for worsening fatigue. The independent influential factors regarding the changing of FACIT-FS were improving or worsening in the same direction in reference to physical condition, gastrointestinal and emotional factors. (4) Conclusions: Fatigue is a multi-dimensional symptom, which is strongly correlated to HRQoL. MCID values of FACIT-FS can be useful tools in monitoring the changes of HRQoL in clinical trials and in daily practice among patients with SSc.

Patients with Systemic Sclerosis with and without Overlap Syndrome Show Similar Microvascular Abnormalities.

INTRODUCTION: Nailfold video capillaroscopy (NVC) is a useful tool for measuring capillary density (CD) and capillary morphology parameters and is mainly used in systemic sclerosis (SSc). OBJECTIVE: We aimed to assess the prevalence of an SSc pattern and CD in different connective tissue diseases (CTDs). METHODS: NVC was performed on 268 patients with CTDs. Control groups consisted of 104 healthy volunteers (HVs) and 36 primary Raynaud's patients (PRPs). RESULTS: Decreased CD was more prevalent in SSc, systemic lupus erythematosus (SLE), inflammatory myopathies (IIM), and overlap CTD patients compared with both controls. Average CD, the prevalence of decreased CD, and the prevalence of an SSc pattern did not differ significantly between SSc patients with (n = 39) and without (n = 50) overlap syndrome. An SSc pattern was significantly more prevalent in SLE (23%), SSc (82%), IIM (35%), and rheumatoid arthritis (17%) compared with both control groups. The prevalence of an elevated microangiopathy evaluation score (MES) was significantly higher in SLE, SSc, and IIM than in the HVs. CONCLUSION: The presence of another CTD in SSc did not influence CD or morphology. An SSc pattern may also be present in CTDs other than SSc. The MES is a useful instrument to distinguish between patients with CTDs and controls.

Surgical solutions in multivisceral arterial occlusions / Sebészi megoldások krónikus multivisceralis arteriás elzáródások esetében

Introduction: Acute occlusions of the visceral arteries result in fulminant clinical consequences and without fast and appropriate treatment may lead to fatal outcome. If the obliterative disease has slow progression the huge capacity of the collateral circulation of the visceral arteries may remain free of symptoms at long run. By progression of the obliterative disease and extension to both, the renal arteries and the aortoiliac segment the clinical picture becomes more colourful. Symptoms mimicking other abdominal disorders may easily lead to misdiagnosis and/or unnecessary delay of adequate treatment ­ finally to organ or life threatening condition. In the present paper we deal with this infrequent, but severe disease in order to recognize it in time, to recommend proper diagnostic workup and propose adequate treatment.

Acari-caused human diseases with skin symptoms / Atkafélék okozta, bortünetekkel járó humán megbetegedések.

Összefoglaló. Az atkák alosztálya heterogén csoport. Az eltéro megjelenésu és életformájú atkák növényeken, állatokon és embereken is képesek különféle megbetegedéseket okozni, melyek csípés, táplálkozás, élosködés, vektorként terjesztett kórokozók által valósulhatnak meg. Az atkák okozta betegségek a lokális, spontán szuno csípéstol egészen a sokszervi elégtelenséget okozó fertozo betegségig széles skálán mozognak. Munkánk során az atkafélék által okozott humán megbetegedések elofordulását, klinikai megjelenését, differenciáldiagnosztikáját, kezelését és közegészségügyi jelentoségét mutatjuk be. Orv Hetil. 2021; 162(36): 1430-1437. Summary. The Acari subclass is a heterogeneous group. They have different appearance and lifestyle. They can cause different diseases on plants, in animals and in humans. These illnesses can be caused by their bites, nutrition, parasitism and by their endoparasites. The diseases move on a wide scale from a local, spontaneously disappearing bite to an infection caused multiorgan failure. We would like to summarize the Acari-caused human diseases' incidence, clinical symptoms, diagnostics, differential diagnostics, treatments and public health importance. Orv Hetil. 2021; 162(36): 1430-1437.

Analysis of PI3K Pathway Associated Molecules Reveals Dysregulated Innate and Adaptive Functions of B Cells in Early Diffuse Cutaneous Systemic Sclerosis.

B cell activation is an early event in the development of systemic sclerosis (SSc). The classical activation of B cells downstream of the B-cell receptor (BCR) involves the phosphatidylinositol-3 kinase (PI3K) pathway that integrates the effects of multiple co-stimulatory receptors. Our analysis of PI3K pathway associated molecules in peripheral blood B cells of early diffuse cutaneous SSc (dcSSc) patients showed altered mRNA expression of Toll-like receptor (TLR) homolog CD180, TLR4, complement component 3, IL-4 receptor and secreted phosphoprotein 1 (SPP1). Parallel to this, we found elevated basal SPP1 secretion in dcSSc B cells, but, with BCR + IL-4 receptor co-stimulation, we could not induce further secretion. CD180 stimulation alone resulted in NF-κB activation in more B cells than CD180 + BCR co-stimulation both in dcSSc and healthy control (HC), but the co-engagement increased the phosphorylation of NF-κB only in dcSSc B cells. Additionally, in contrast with HC B cells, the lower basal production of IL-10 by dcSSc B cells could not be elevated with CD180 stimulation. Furthermore, activation via CD180 increased the percentage of CD86+ switched memory (CD27+IgD-) B cells in dcSSc compared to HC. Our results suggest that alternative B cell activation and CD180 dysfunction cause imbalance of regulatory mechanisms in dcSSc B cells.

Treatment options for complex aortic aneurysms ­ own experiences / Complex aortaaneurysmák kezelési lehetoségei - saját tapasztalatok

Complex aortic aneurysms extend to more aortic segments, and/or include one or more orifices of highly important side branches. Meanwhile complex aneurysms need reconstructive solutions in one sitting or hybrid procedures timely close to each other, multiple aneurysms can be treated technically and timely separated. Previously, open surgery was the only opportunity to intervene, which was associated with significant surgical trauma and was not suitable for high risk patients when devastating complications were likely. Recently combination of lower risk surgery with endovascular treatment options ­ the so called hybrid techniques ­ resulted in that indications for treatment remarkably widened. In addition, permanent technical progress made available pure endovascuar solutions, so a wide range of surgical procedures provide number of options for treatment. In this paper we report on the treatment options of complex aortic aneurysms, and present our own relevant experience.

Sensitivity to change of joint count composite indices in 72 patients with systemic sclerosis.

OBJECTIVES: We validated the responsiveness of joint count composite indices (JCCIs) in 72 patients with systemic sclerosis (SSc). METHODS: Changes in Disease Activity Score of 28 Joints using ESR and CRP (DAS28-ESR, DAS28-CRP), Simplified Disease Activity Index (SDAI) and the Clinical Disease Activity Index (CDAI) were evaluated in a one-year follow-up study. Charts of patients including swollen/tender joint counts, laboratory signs of inflammation, and visual analogue scales referring to disease activity, severity and pain were also blindly categorized by two rheumatologists as improved, unchanged or deteriorated. These categories were used as references for the determination of effect size (ES) and standardised response mean (SRM). RESULTS: Articular inflammation improved in 15, deteriorated in 12, and remained unchanged in 45 (63%) patients with SSc based on the concordant opinion of two clinical investigators. All four JCCIs were sensitive to changes (ES>1; SRM>1). The correlation between changes in JCCIs and the physicians' evaluation was high (r >0.68; p<0.001). Arthritis was predominantly prone to change in patients with high JCCIs, impaired functional status, anti-RNA polymerase III antibodies and patients on DMARD therapy. Synovitis was more prevalent in patients with early diffuse SSc, and tended to improve during the follow-up. CONCLUSIONS: All four JCCIs were sensitive to changes, if tender/swollen joints were present at baseline. Articular inflammation was most prone to change in patients with high JCCIs, impaired functional status and already decreased health-related quality of life at baseline.

Electrophysiological changes of the spinal cord during thoracoabdominal aortic clamping -- Experimental study / Thoracoabdominalis aortakirekesztés során fellépo elektrofiziológiai változások a gerincveloben

Severe neurological complications of the thoracoabdominal aortic clamping were published in numerous clinical and experimental studies. These hardly predictable, devastating consequences demanded to develop a monitoring system which might detect impending level of spinal cord ischemia in time ­ in order to introduce or enhance protective procedures and prevent permanent neurological deficit. The most widely used monitoring in clinical practice is the continuous surveillance of the motor evoked potentials (MEP) during and after thoracoabdominal aortic clamping. Much less used, but promising opportunity is to control the metabolic changes and cellular integrity utilizing specific markers like liquor lactate and neuron specific enolase (NSE) etc. In our earlier study we published data of our canine experiment related to coherencies between neurological outcome and specific perfusion of the spinal cord during and after one hour thoracoabdominal aortic clamping. In the present paper we investigate the behavior of motor evoked (MEP) and sensory evoked (SEP) potentials related to neurological changes. We conclude the behavior of SEP values hardly correlate with the neurologic outcome, meanwhile decrease of MEP amplitude provides reliable signal for developing spinal cord ischemia. We could not confirm a numeric correlation of these data and the level of the final neurologic outcome.

Surgical ischemia and reperfusion of the spinal cord ­ effect on specific perfusion and neurological outcome. Experimental study / A sebészi gerincvelo ischemia és reperfusio hatása a specifikus átáramlásra és a neurológiai következményekre

Clamping of the thoracoabdominal aorta reduces perfusion of the spinal cord significantly, which clinically may present as paraparesis or paraplegia ­ devastating and unpredictable complications of open thoracoabdominal aortic surgery. Introduction of monitoring of evoked potentials and/or biochemical markers, methods increasing distal arterial pressure, indirect procedures enhancing residual flow (like liquor drainage), drugs, and use of hypothermia contributed to achieve better outcome. Preconditioning of spinal cord circulation is also a promising method. New endovascular techniques for thoracoabdominal aortic aneurysms and dissections reduced surgical trauma significantly. Despite all these progressions spinal cord ischemic damage is still a significant risk. To address this problem we carried out an experimental work using a canine model focusing on the protective effect of distal arterial perfusion, spinal fluid drainage, and their combination in a one hour setting of thoracoabdominal aortic clamping. In this paper we publish our data of circulatory and specific perfusion parameters of the spinal cord during and after declamping in correlation of final neurologic outcome.

The presence of small joint contractures is a risk factor for survival in 439 patients with systemic sclerosis.

OBJECTIVES: Analysis of risk factors and mortality of 439 patients with systemic sclerosis (SSc) in a tertiary care centre. METHODS: The mean follow up time was 8.4±5.6 years. Lost to follow up rate was 6.4%. Female to male ratio was 366 to 73. Two hundred sixty patients had limited and 179 diffuse cutaneous SSc (dcSSc). A standard protocol including musculoskeletal examinations was used for the assessment of patients. RESULTS: By Kaplan-Meier analysis the overall 5-, 10- and 15 year survival were 88.2%, 79.9% and 73.6%, respectively. Univariate analysis showed that dcSSc, male gender, presence of small joint contractures, pulmonary interstitial, cardiac, oesophageal involvement, scleroderma renal crisis, arterial hypertension, anti-topoisomerase antibody, anemia, hypalbuminemia, coexistent malignancies and elevated erythrocyte sedimentation were associated with poor survival. Lack of giant capillaries, avascular zones or neo-angiogenesis on capillaroscopy, and presence of anti-centromere antibodies were associated with favourable outcome. Multivariate regression analysis showed presence of small joint contractures, history of arterial hypertension, male gender, diffusing capacity of carbon monoxide <50%, right ventricular pressure >40 mmHg on echocardiography, less than 50% ejection fraction, anti-topoisomerase I positivity, anemia, and serum albumin concentration < 35 g/l as well as current or history of coexistent malignancy were independent poor prognostic factors. CONCLUSIONS: In addition to well-known factors predicting poor outcome in SSc, the presence of small joint contractures was a newly identi ed independent risk factor of mortality. Our data also confirmed a recent finding showing that history of arterial hypertension was also a poor prognostic factor.

Application of SeDeM expert systems in preformulation studies of pediatric ibuprofen ODT tablets.

Pediatric, ibuprofen containing orodispersible tablets (ODTs) were prepared using the SeDeM expert system methodology. In order to facilitate formulation, directly compressible ibuprofen was employed (Ibuprofen DC 8TM) and characterized using its SeDeM profile. The mannitol based superdisintegrant Ludiflash® was characterized by the SeDeM-ODT expert system, which also allowed calculation of the optimal excipient concentration in order to obtain suitable tablet hardness and disintegration time. After adding a sweetener and a standard combination of lubricants, the optimized formulation was directly compressed into tablets and evaluated in terms of tablet hardness, friability, disintegration time and dissolution profile. The SeDeM method was applied to determine the amount of corrective excipient (Ludiflash®) required for the compression of Ibuprofen DC 85TM in order to achieve suitable ODTs. Adequate tablet hardness, disintegration time, friability and dissolution profiles were found during tablet evaluation.

Malignancies in Patients with Anti-RNA Polymerase III Antibodies and Systemic Sclerosis: Analysis of the EULAR Scleroderma Trials and Research Cohort and Possible Recommendations for Screening.

OBJECTIVE: To analyze the characteristics of anti-RNA polymerase III antibodies (anti-RNAP3)- positive patients with systemic sclerosis (SSc) in the European League Against Rheumatism Scleroderma Trials and Research group (EUSTAR) registry with a focus on the risk of cancer and the characteristics of malignancies, and the aim to provide guidelines about potential cancer screening in these patients. METHODS: (1) Analysis of the EUSTAR database: 4986 patients with information on their anti-RNAP3 status were included. (2) Case-control study: additional retrospective data, including malignancy history, were queried in 13 participating EUSTAR centers; 158 anti-RNAP3+ cases were compared with 199 local anti-RNAP3- controls, matched for sex, cutaneous subset, disease duration, and age at SSc onset. (3) A Delphi exercise was performed by 82 experts to reach consensus for cancer screening in anti-RNAP3+ patients. RESULTS: In the EUSTAR registry, anti-RNAP3 were associated in multivariable analysis with renal crisis and diffuse cutaneous involvement. In the case-control study, anti-RNAP3 were associated with gastric antral vascular ectasia, rapid progression of skin involvement, and malignancies concomitant to SSc onset (OR 7.38, 95% CI 1.61-33.8). When compared with other anti-RNAP3+ patients, those with concomitant malignancies had older age (p < 0.001) and more frequent diffuse cutaneous involvement (p = 0.008). The Delphi exercise highlighted the need for malignancy screening at the time of diagnosis for anti-RNAP3+ patients and tight followup in the following years. CONCLUSION: Anti-RNAP3+ patients with SSc have a high risk of concomitant malignancy. These results have implications for clinical practice and suggest regular screening for cancer in anti-RNAP3+ patients.

Galectin-3 is an independent predictor of survival in systemic sclerosis.

BACKGROUND: Galectin-3 is a beta-galactoside-binding lectin that may be related to tissue sclerosis or aberrant activation of angiogenesis in systemic sclerosis (SSc). The aim of our study was to determine the associations between galectin-3 levels and patient characteristics, as well as to investigate the long term prognostic value of galectin-3 in a large cohort of SSc patients. METHODS: 152 patients with SSc (55±11years, 138 female) were included in our follow-up study. Blood samples and clinical data were collected at baseline. Primary and secondary outcomes were all-cause and cardiovascular mortality, respectively. RESULTSS: Galectin-3 levels showed positive correlation with the grade of left ventricular diastolic function (r=0.193; p=0.026), erythrocyte sedimentation rate (r=0.172; p=0.036) and serum level of C-reactive protein (r=0.200; p=0.015) while negative correlation with diffusing capacity for carbon monoxide (r=-0.228; p=0.006), in age, gender and BSA adjusted analyses. During the follow-up of 7.2±2.3years, 35 SSc patients (23%) died. In multivariate Cox regression analyses adjusted for age, gender, BSA, creatinine and NT-proBNP levels, galectin-3 was an independent predictor both of the all-cause mortality (HR: 2.780, 95% CI: 1.320-5.858, p=0.007) and cardiovascular mortality (HR: 3.346, 95% CI: 1.118-10.012, p=0.031). Using receiver-operating characteristic analysis, galectin-3>10.25ng/ml was found to be the best predictor of the all-cause mortality. CONCLUSIONS: Our results suggest that galectin-3 is an independent predictor of all-cause and cardiovascular mortality in SSc. Validation studies are required to establish whether galectin-3 may be proposed as simple biomarker for identifying patients with high mortality risk in SSc.

[Surgical treatment of atypical aortic coarctation -- long-term results]. / Atípusos aortacoarctatiók sebészi kezelésének hosszú távú eredményei.

INTRODUCTION: Aortic coarctation is a frequent congenital cardiovascular disorder representing 5-8% of all cases. It is typically localized in the isthmic region. However, in about 1% of cases coarctation may develop in atypical sites of the aorta and it is frequently complicated with severe hypertension. AIM: The aim of the authors was to present diagnostic and surgical methods used in 27 patients with atypical aortic coarctation during the last 35 years with special interest on long-term results. METHOD: There was a great advance in diagnostic and surgical treatment methods during the time period analyzed in this study. Nowadays morphologic diagnosis is most commonly obtained using computed tomography angiography and magnetic resonance angiography. Some cases were treated with endovascular techniques, but the authors used also a wide variety of surgical approaches in these patients with atypical aortic coarctation. RESULTS: No patient died after surgery and hypertension was reduced in all patients, too. Reintervention was necessary in patients operated in childhood due to change of body measures. CONCLUSIONS: Atypical aortic coarctation can be treated surgically with good early and late outcomes. Somatic growth of children may indicate surgical revision.

Autopsy versus clinical findings in patients with systemic sclerosis in a case series from patients of the EUSTAR database.

OBJECTIVES: Subclinical organ pathology occurs regularly in systemic sclerosis (SSc) and affects correct prognosis as well as treatment choices. We aimed to evaluate autopsy data for organ involvement with subsequent correlation to clinical data in order to assess discrepancies in pathological and clinical findings in SSc. METHODS: A standardised autopsy questionnaire from diseased patients registered in the European Scleroderma Trials and Research group (EUSTAR) cohort was analysed on cause of death and various manifestations in different organ systems. Clinical data obtained from the EUSTAR database of the corresponding patients including cause of death and disease manifestations of lung, heart, kidney, gastrointestinal, skin or musculoskeletal organ involvement were retrospectively analysed and compared to autopsy data. RESULTS: 11 patients (6 women, 5 male) aged between 23 and 84 were included. Cause of death defined by pathologist and clinician were identical in 9/11 cases. In 8 individuals, cause of death was related to heart and lung pathologies. Heart and lung involvement (both 10/11) were the most frequently detected organ involvement at autopsy. Here, myocardial fibrosis occurred in 66% and lung fibrosis in 50% of the patients. Clinically, diastolic function abnormalities (6/11), conduction block (4/11), reduced DCLO (6/11) and dyspnea (8/11) were the most prevalent cardiopulmonary findings. For heart and renal involvement we found higher prevalence in autopsy than by clinical diagnosis. Especially myocardial fibrosis and renal arteriosclerosis were only obtained by autopsy in several individuals. CONCLUSIONS: Clinical diagnostic procedures are limited in detection of end-organ damage, especially for cardiac involvement. All the more post mortem examinations are needed for quality verification of clinical diagnosis and might help as to better understand the disease processes as well as to improve patient care.