2019 EULAR points to consider for the assessment of competences in rheumatology specialty training.

BACKGROUND AND AIM: Striving for harmonisation of specialty training and excellence of care in rheumatology, the European League Against Rheumatism (EULAR) established a task force to develop points to consider (PtCs) for the assessment of competences during rheumatology specialty training. METHODS: A systematic literature review on the performance of methods for the assessment of competences in rheumatology specialty training was conducted. This was followed by focus groups in five selected countries to gather information on assessment practices and priorities. Combining the collected evidence with expert opinion, the PtCs were formulated by the multidisciplinary task force, including rheumatologists, medical educationalists, and people with rheumatic and musculoskeletal diseases. The level of agreement (LoA) for each PtC was anonymously voted online. RESULTS: Four overarching principles and 10 PtCs were formulated. The overarching principles highlighted the importance of assessments being closely linked to the rheumatology training programme and protecting sufficient time and resources to ensure effective implementation. In the PtCs, two were related to overall assessment strategy (PtCs 1 and 5); three focused on formative assessment and portfolio (PtCs 2-4); three focused on the assessment of knowledge, skills or professionalism (PtCs 6-8); one focused on trainees at risk of failure (PtC 9); and one focused on training the trainers (PtC 10). The LoA (0-10) ranged from 8.75 to 9.9. CONCLUSION: These EULAR PtCs provide European guidance on assessment methods throughout rheumatology training programmes. These can be used to benchmark current practices and to develop future strategies, thereby fostering continuous improvement in rheumatology learning and, ultimately, in patient care.

2019 EULAR recommendations for the generic core competences of health professionals in rheumatology.

BACKGROUND/OBJECTIVES: To maintain and optimise the quality of care provided by health professionals in rheumatology (HPRs), adequate educational offerings are needed. This task force (TF) aimed to develop evidence-based recommendations for the generic core competences of HPRs, with specific reference to nurses, physical therapists (PTs) and occupational therapists (OTs) to serve as a basis for their postgraduate education. METHODS: The EULAR standardised operating procedures for the development of recommendations were followed. A TF including rheumatologists, nurses, PTs, OTs, patient-representatives, an educationalist, methodologists and researchers from 12 countries met twice. In the first TF meeting, 13 research questions were defined to support a systematic literature review (SLR). In the second meeting, the SLR evidence was discussed and recommendations formulated. Subsequently, level of evidence and strength of recommendation were assigned and level of agreement (LoA) determined (0-10 rating scale). RESULTS: Three overarching principles were identified and 10 recommendations were developed for the generic core competences of HPRs. The SLR included 79 full-text papers, 20 of which addressed the competences, knowledge, skills, attitudes and/or educational needs of HPRs from multiple professions. The average LoA for each recommendation ranged from 9.42 to 9.79. Consensus was reached both on a research and educational agenda. CONCLUSION: Evidence and expert opinion informed a set of recommendations providing guidance on the generic core competences of HPRs. Implementation of these recommendations in the postgraduate education of HPRs at the international and national level is advised, considering variation in healthcare systems and professional roles.

IL-1 receptor blockade skews inflammation towards Th2 in a mouse model of systemic sclerosis.

The interleukin (IL)-1 family of cytokines is strongly associated with systemic sclerosis (SSc) and pulmonary involvement, but the molecular mechanisms are poorly understood. The aim of this study was to assess the role of IL-1α and IL-1ß in pulmonary vascular and interstitial remodelling in a mouse model of SSc.IL-1α and IL-1ß were localised in lungs of SSc patients and in the fos-related antigen-2 (Fra-2) transgenic (TG) mouse model of SSc. Lung function, haemodynamic parameters and pulmonary inflammation were measured in Fra-2 TG mice with or without 8 weeks of treatment with the IL-1 receptor antagonist anakinra (25 mg·kg-1·day-1). Direct effects of IL-1 on pulmonary arterial smooth muscle cells (PASMCs) and parenchymal fibroblasts were investigated in vitroFra-2 TG mice exhibited increased collagen deposition in the lung, restrictive lung function and enhanced muscularisation of the vasculature with concomitant pulmonary hypertension reminiscent of the changes in SSc patients. Immunoreactivity of IL-1α and IL-1ß was increased in Fra-2 TG mice and in patients with SSc. IL-1 stimulation reduced collagen expression in PASMCs and parenchymal fibroblasts via distinct signalling pathways. Blocking IL-1 signalling in Fra-2 TG worsened pulmonary fibrosis and restriction, enhanced T-helper cell type 2 (Th2) inflammation, and increased the number of pro-fibrotic, alternatively activated macrophages.Our data suggest that blocking IL-1 signalling as currently investigated in several clinical studies might aggravate pulmonary fibrosis in specific patient subsets due to Th2 skewing of immune responses and formation of alternatively activated pro-fibrogenic macrophages.

Validation of the new classification criteria for systemic lupus erythematosus on a patient cohort from a national referral center: a retrospective study.

AIM: To validate Systemic Lupus International Collaborating Clinics (SLICC)-12 and American College of Rheumatology (ACR)-97 classification criteria on a patient cohort from the University Hospital Center Zagreb. METHODS: This retrospective study, conducted from 2014 to 2016, involved 308 patients with systemic lupus erythematosus (SLE) (n=146) and SLE-allied conditions (n=162). Patients' medical charts were evaluated by an expert rheumatologist to confirm the clinical diagnosis, regardless of the number of the ACR-97 criteria met. Overall sensitivity and specificity, as well as the sensitivity and specificity according to disease duration, were compared between ACR-97 and SLICC-12 classifications. Predictive value for SLE for both classifications was assessed using logistic regression and receiver operating characteristic (ROC) curves. RESULTS: The SLICC-12 criteria had significantly higher sensitivity in early disase, which increased with disease duration. The ACR-97 criteria had higher specificity. The specificity of the SLICC-12 criteria was low and decreased with disease duration. Regression analysis demonstrated the superiority of the SLICC-12 classification criteria over the ACR-97 criteria, with areas under the ROC curve of 0.801 and 0.780, respectively. CONCLUSION: Although the SLICC-12 criteria were superior to the ACR-97 and were more sensitive for diagnosing early SLE, their specificity in our population was too low. The sensitivity of the SLICC-12 classification is increased by better defined clinical features within each criterion. Our results contribute to the current initiative for developing new criteria for SLE.

[Pregnant patient with rheumatoid arthritis treated with methotrexate and infliximab].

Tumor necrosis factor-alpha inhibitors have become an established therapeutic regimen for patients with rheumatoid arthritis. Regarding their harmful potential they are classified as category B medications. Animal reproduction studies have failed to demonstrate a risk to the fetus and there are no adequate and well-controlled studies in pregnant women. Disease-modifying antirheumatic drugs (DMARDs) are often used in combination with biological therapy and treatment with methotrexate has shown good results. This antimetabolite is classified as a category X drug and its teratogenic effect is well known. The incidence of inflammatory rheumatic diseases is significantly higher in women. There are many reports on pregnant patients treated with biological therapy, oft en in combination with DMARDs. The effects of such a therapy on reproductive health is a theme of debate, with controversial views on the matter. We present a patient with rheumatoid arthritis whose pregnancy was discovered at 31 weeks of gestation. During that period she had been treated with methotrexate and infliximab, with no adverse effects.

Differences and similarities in rheumatology specialty training programmes across European countries.

OBJECTIVES: To analyse the similarities and discrepancies between the official rheumatology specialty training programmes across Europe. METHODS: A steering committee defined the main aspects of training to be assessed. In 2013, the rheumatology official training programmes were reviewed for each of the European League Against Rheumatism (EULAR) countries and two local physicians independently extracted data on the structure of training, included competencies and assessments performed. Analyses were descriptive. RESULTS: 41 of the 45 EULAR countries currently provide specialist training in rheumatology; in the remaining four rheumatologists are trained abroad. 36 (88%) had a single national curriculum, one country had two national curricula and four had only local or university-specific curricula. The mean length of training programmes in rheumatology was 45 (SD 19) months, ranging between 3 and 72 months. General internal medicine training was mandatory in 40 (98%) countries, and was performed prior to and/or during the rheumatology training programme (mean length: 33 (19) months). 33 (80%) countries had a formal final examination. CONCLUSIONS: Most European countries provide training in rheumatology, but the length, structure, contents and assessments of these training programmes are quite heterogeneous. In order to promote excellence in standards of care and to support physicians' mobility, a certain degree of harmonisation should be encouraged.

[A patient with rheumatoid arthritis and primary biliary cirrhosis successfully treated with adalimumab]. / Uspjesna primjena adalimumaba u bolesnice oboljele od reumatoidnog artritisa i primarne bilijarne ciroze.

Rheumatoid arthritis and primary biliary cirrhosis coexist in up to 6% of cases. Tumor necrosis factor alpha seems to have an important role in the pathogenesis of both diseases. Tumor necrosis factor alpha inhibitors have become an established therapeutic regimen for patients with rheumatoid arthritis. The only approved drug for primary biliary cirrhosis is ursodeoxycholic acid. We describe the case of a female patient with both rheumatoid arthritis and primary biliary cirrhosis in a long term remission of both diseases induced with adalimumab. This case report is an important addendum to a few published similar reports.

[RHEUMATOLOGY IN EUROPE IN 2015]. / REUMATOLOGIJA U EUROPI 2015.

One of the main concerns of people with chronic conditions, particularly rheumatic and musculoskeletal diseases, is the availability of quality health care, which is being analyzed. There are differences between European countries regarding the access to health care. The pressure of the financial crisis has been recognized in making barriers more evident. Representatives of the European Parliament together with stakeholder organizations create policy documents for optimizing access to health care at both the EU and national levels. Great care is taken with the education of medical doctors and other professionals in rheumatology on all educational levels. Based on a recent study, there are similarities and discrepancies in the implementation of the specialty training programs (Chapter 6 of the UEMS Charter of Specialty Training Programmes) across Europe. In the meantime, the UEMS Council has endorsed the new Training Requirements for the Specialty of Rheumatology--European Standards for Postgraduate Medical Specialist Training. The training program is competency based, stressing the important role of professional behaviour. The document is being forwarded to the national societies in order to be implemented in the European countries. A strategy of assessment methods in the specialty training program is being developed. Work on the e-portfolio for European trainees as well as the European Specialty Board Examination is in progress.

[The proposal of the Croatian Society for Rheumatology for the treatment of adult rheumatoid arthritis patients with biologics, 2013]. / Prijedlog Hrvatskog reumatoloskog drustva HLZ-a za lijecenje reumatoidnog artritisa odraslih bolesnika bioloskim lijekovima, 2013.

Standardized approach to the patients with rheumatoid arthritis (RA) is one of the requirements of good clinical practice. Croatian Society for Rheumatology (HRD) of Croatian Medical Association (HLZ) updated the Proposed treatment of rheumatoid arthritis (RA) with biologic agents in line with recent findings in rheumatology for the last 3 years. By complying with the agreed standards of treatment we can avoid malpractice and irrational consumption, and to the most patients provide a greater chance for a favorable outcome.

[The proposal of Croatian Society for Rheumatology for anti-TNF-alpha therapy in adult patients with spondyloarthritides, 2013]. / Prijedlog hrvatskog reumatoloskog drustva HLZ-a za primjenu inhibitora TNF-alpha u odraslih bolesnika sa spondiloartritisima, 2013.

Croatian Society for Rheumatology of Croatian Medical Association updated the proposal for the application of TNF-alpha inhibitors in adult patients with spondyloartritides (SpA) in accordance with the new classification of SpA and european recommendations for the treatment of SpA with biologic agents. In this way a standardized method of diagnosis, targeted treatment, monitoring and evaluating outcomes are proposed.

Prevalence of the American College of Rheumatology classification criteria in a group of 162 systemic lupus erythematosus patients from Croatia.

AIM: To identify systemic lupus erythematosus (SLE) patients diagnosed and treated at the outpatient clinic of our Division fulfilling at least four American College of Rheumatology (ACR) classification criteria at the time of the study, to determine the prevalence of each of the criteria at three different time points, and to compare the data with similar studies. METHODS: We performed retrospective and descriptive analysis of medical records of 162 patients fulfilling at least 4 ACR criteria. Classification criteria were counted and the frequency of each criterion was identified at three different time points: disease onset, time of diagnosis, and the time when the study was conducted. RESULTS: At diagnosis and at the time when the study was conducted there were 3.8 and 5.4 fulfilled classification criteria, respectively. The most common criterion at the time of the disease onset was arthritis (52.6%); at the time of diagnosis it was positive antinuclear antibody (ANA) titer (88.0%); and at the time when the study was conducted it was positive ANA titer (95.7%), immunologic disorder (89.5%), arthritis (71.0%), hematologic disorder (70.4%), malar rash (61.7%), and photosensitivity (51.9%). CONCLUSION: The prevalence of ACR criteria in our patients is similar to that in other studies, especially those involving Caucasian patients. Our results confirm the value of the ACR criteria in patients with an already established diagnosis. This is the first study on the prevalence of disease manifestations among Croatian patients with SLE.

[Rare myositides and myopathies]. / Rijetki miozitisi i miopatije.

Inflammatory myopathies comprise dermatomyositis, polymyositis, inclusion body myositis, secondary myopathies associated with connective tissue diseases, malignancies, sarcoidosis and Behçet's disease. In the article the inclusion body myositis is presented. Differential diagnosis ofproximal muscle weakness also includes certain non-inflammatory myopathies The statin myopathy, as an example of the drug-induced myotoxicity, is elaborated. The infectious myopathies are described with the emphasis on parasitic myopathy. The endocrine myopathies are overviewed.

Low back pain as the presenting sign in a patient with primary extradural melanoma of the thoracic spine--a metastatic disease 17 years after complete surgical resection.

Primary spinal melanomas are extremely rare lesions. In 1906, Hirschberg reported the first primary spinal melanoma, and since then only 40 new cases have been reported. A 47-year-old man was admitted suffering from low back pain, fatigue and loss of body weight persisting for three months. He had a 17-year-old history of an operated primary spinal melanoma from T7-T9, which had remained stable for these 17 years. Routine laboratory findings and clinical symptoms aroused suspicion of a metastatic disease. Multislice computed tomography and magnetic resonance imaging revealed stage-IV melanoma with thoracic, abdominal and skeletal metastases without the recurrence of the primary process. Transiliac crest core bone biopsy confirmed the diagnosis of metastatic melanoma. It is important to know that in all cases of back ore skeletal pain and unexplained weight loss, malignancy must always be considered in the differential diagnosis, especially in the subjects with a positive medical history. Patients who have back, skeletal, or joint pain that is unresponsive to a few weeks of conservative treatment or have known risk factors with or without serious etiology, are candidates for imaging studies. The present case demonstrates that complete surgical resection alone may result in a favourable outcome, but regular medical follow-up for an extended period, with the purpose of an early detection of a metastatic disease, is highly recommended.

[Heart involvement and pulmonary arterial hypertension in patients with systemic sclerosis]. / Ostecenje srca i plucna arterijska hipertenzija u bolesnika sa sistemskom sklerozom.

In patients with systemic sclerosis heart involvement is often manifest, associated with poor prognosis and increased mortality. Cardiac involvement may be manifested by myocardial disease, arrhhythmias, conduction system disturbances, pericardial abnormalities and right heart failure, which develops as a complication of pulmonary arterial hypertension and significantly influences the disease progression. Pulmonary arterial hypertension is a severe and progressive disease with significant morbidity and mortality, being more often diagnosed. Diagnostic methods in evaluation of heart involvement as well as pulmonary arterial hypertension are presented. Today's treatment modalities are discussed.

[Neuropsychiatric manifestations of systemic lupus erythematosus]. / Neuropsihijatrijska ocitovanja sistemskog eritemskog lupusa.

Neurologic and psychiatric disorder is reported in up to 70% of patients with systemic lupus erythematosus (SLE). New classification criteria for neuropsychiatric SLE define 19 syndromes with characteristic clinical features, laboratory findings and imaging. Better diagnostic possibilities, particularly imaging methods reveal new data on some conditions: demyelinating disorders in patients with SLE, neuropsychiatric disorders associated with antiphospholipid syndrome. Based on neuropsychological examination the diagnosis of cognitive dysfunction, most common psychiatric impairment, is more often established.

Non-MS autoimmune demyelination.

Connective tissue diseases can be characterised by central nervous system (CNS) involvement, in some patients manifested by demyelination areas in the white matter of the brain and spinal cord, which are difficult to differentiate from multiple sclerosis (MS) and other demyelinating processes, such as transverse myelitis and optic neuritis. Demyelinating process may be the feature of nervous impairment in systemic lupus erythematosus, Behcet's disease (BD), Sjoegren's syndrome (SS), systemic sclerosis (SSc) or very rarely other systemic autoimmune diseases. An acute isolated neurological syndrome, as the most common symptom of MS can sometimes be the only feature or even first manifestation of nervous impairment in connective tissue disease, hence presenting the diagnostic problem. Although the white matter abnormalities seen by magnetic resonance imaging may be similar in non-MS autoimmune demyelination and MS, it is the most important diagnostic tool in the differential diagnosis of the mentioned conditions. Investigating the presence of various autoantibodies potentially involved in the pathogenesis of demyelinating lesions as well as cerebrospinal fluid (CSF) analysis can be helpful.

[Cryptococcal meningitis as a diagnostic problem in a patient with SLE--case report]. / Kriptokokni meningitis kao dijagnosticki problem u bolesnika sa sustavnim eritemskim lupusom- prikaz bolesnika.

Systemic erythematosus lupus (SLE) is a disease with wide range of clinical manifestations, signs and symptoms. Disease outcome depends mostly on the affection of kidneys and central nervous system by the disease. Very important cause of death in patients with SLE is infection. Infections are very common among these patients due to aggressive immunosuppressive treatment that is needed for the disease inflammatory activity control. In this case report we have presented a patient with SLE who initially had severe renal affection, but also complications of immunosuppressive therapy that was administered. Even though the disease was accidentally diagnosed, it had a severe clinical progress. Because of lupus nephropathy, in the early phase of the disease we administered aggressive immunosuppressive therapy (combined parenteral therapy of glucocorticoides and cyclophosphamide). As an outcome of the combined effect of disease and immunosuppressive agents used in the treatment of the disease, the patient had increased infective diathesis (repeated infections caused by S. enteritidis--urinary infections and sepsis). During one of the disease flares the patient was hospitalized an opportunistic infection developed. It was meningitis caused by C. neoformans. This opportunistic mycosis infection presented with clinically totally nonspecific signs and symptoms of CNS affection. Therefore, we suspected affection of CNS with SLE. Even though all diagnostic procedures were made on time and that adequate antifungal and supportive agents were applied very early after the infection onset, the outcome was fatal. Because of infective diathesis in patients with SLE, which present with common and opportunistic infections, and due to high mortality rates caused by these infections, we have tried to emphasise the importance of taking adequate specimens early after infection outcome for these rare infective agents like C. neophormans. In recent medical literature are dominant cases reported in Asia. Reports from Europe are very rare, and this case is the one of that kind in Croatia.

EULAR 'points to consider' for the conduction of workforce requirement studies in rheumatology.

OBJECTIVE: Current methods used for forecasting workforce requirements in rheumatology are disparate, as are the parameters incorporated into workforce projection studies. The objective of these European League Against Rheumatism (EULAR points to consider (PTC) is to guide future workforce studies in adult rheumatology in order to produce valid and reliable manpower estimates. METHODS: The EULAR Standardised Operating Procedures were followed. A multidisciplinary task force with experts including patients with rheumatic diseases from 11 EULAR countries and the USA was assembled. A systematic literature review (SLR) was conducted to retrieve workforce models in rheumatology and other medical fields. PTC were based on expert opinion informed by the SLR, followed by group discussions with consensus obtained through informal voting. The level of agreement with the PTC was voted anonymously. RESULTS: A total of 10 PTC were formulated. The task force recommends models integrating supply (=workforce available in rheumatology), demand (=health services requested by the population) and need (=health services that are considered appropriate to serve the population). In general, projections of workforce requirements should consider all factors relevant for current and future workload in rheumatology inside and outside of direct patient care. Forecasts of workforce supply should consider demography and attrition of rheumatologists, as well as the effects of new developments in healthcare. Predictions of future need/demand should take demographic, sociocultural and epidemiological development of the population into account. CONCLUSION: These EULAR-endorsed PTC will provide guidance on the methodology and the parameters to be applied in future national and international workforce requirement studies in rheumatology.

Workforce requirements in rheumatology: a systematic literature review informing the development of a workforce prediction risk of bias tool and the EULAR points to consider.

OBJECTIVE: To summarise the available information on physician workforce modelling, to develop a rheumatology workforce prediction risk of bias tool and to apply it to existing studies in rheumatology. METHODS: A systematic literature review (SLR) was performed in key electronic databases (1946-2017) comprising an update of an SLR in rheumatology and a hierarchical SLR in other medical fields. Data on the type of workforce prediction models and the factors considered in the models were extracted. Key general as well as specific need/demand and supply factors for workforce calculation in rheumatology were identified. The workforce prediction risk of bias tool was developed and applied to existing workforce studies in rheumatology. RESULTS: In total, 14 studies in rheumatology and 10 studies in other medical fields were included. Studies used a variety of prediction models based on a heterogeneous set of need/demand and/or supply factors. Only two studies attempted empirical validation of the prediction quality of the model. Based on evidence and consensus, the newly developed risk of bias tool includes 21 factors (general, need/demand and supply). The majority of studies revealed high or moderate risk of bias for most of the factors. CONCLUSIONS: The existing evidence on workforce prediction in rheumatology is scarce, heterogeneous and at moderate or high risk of bias. The new risk of bias tool should enable future evaluation of workforce prediction studies. This review informs the European League Against Rheumatism points to consider for the conduction of workforce requirement studies in rheumatology.

[Specialty preferences among final-year medical students at Zagreb University Medical School]. / Sto zele specijalizirati studenti seste godine Medicinskog akulteta u Zagrebu.

The aim of this study was to investigate the sixth-year medical students' specialty preferences, at the Zagreb University Medical School. A total of 603 students were recruited during a three-year period (2003-2005), with a response rate of 83.3%. One third had a preferred specialty upon enrolment at the Medical School. Three most desirable specialties few months prior to graduation were internal medicine (12.6%), paediatrics (10.2%), and surgery (8.7%), while one fourth of students (24.4%) reported one of the controllable life-style specialties as their specialty choice. Students who were interested in internal medicine reported the best academic performance, while those who were interested in the family medicine (general practice) reported the poorest academic performance. The study indicates that family medicine, anaesthesiology, and radiology were the least commonly reported as specialties of the students' choice, compared to percentage of specialists employed in Croatian health care service. The most wanted specialties were ear-nose-throat and orthopaedics.