Search

SLE je složena i rijetka bolest, tipični predstavnik kroničnih multisistemskih autoimunih bolesti. U ovoj studiji prikazujemo model populacijskog monitoriranja SLE u Hrvatskoj. Utvrdili smo da su standardizirani indeksi prevalencije i mortaliteta nesenzitivni u detekciji regionalnih razlika u morbiditetu i mortalitetu od SLE. Postojeći rutinski prikupljani podaci u HZJZ se predlažu kao osnovni set podataka za daljnju analizu. U Hrvatskoj su od 2002. do 2007. godine registrirane 2372 hospitalizacije 1067 bolesnika s otpusnim dijagnozama SLE (MKB X kodovi M32.1 do M32.9) uz omjer žena i muškaraca 6:1 u incidentnim hospitalizacijama. 148 podataka o hospitalizacijama je izostavljeno zbog neadekvatnosti ili nedostatnosti podataka ili nehrvatskog državljanstva. U istom je razdoblju zabilježeno 78 smrti povezanih sa SLE, od čega 65 žena i 13 muškaraca. Zabilježene stope bolničkog pobola i smrtnosti su direktno standardizirane na Europsku standardnu populaciju. DSMR za SLE ukazuje na nešto viši mortalitet u Panonskoj i Mediteranskoj regiji Hrvatske. DSHR za SLE ukazuje na učestalije hospitalizacije zbog SLE u Sjeverozapadnoj regiji Hrvatske. Hospitalizacije bolesnika u Sjeverozapadnoj regiji Hrvatske su u prosijeku kraće nego u bolesnika u Panonskoj i Mediteranskoj regiji. Podaci o smrtima i hospitalizacijama su vizualizirani na koroplet mapama relativnog rizika baziranih na razini općina. Prostorno skeniranje podataka o hospitalizacijama s programom SatScan temeljeno na diskretnom Poissonovom modelu upućuje na postojanje dva statistički značajna klastera, u središnjoj Dalmaciji te u istočnoj Slavoniji. Istom metodom nisu detektirani statistički značajni klasteri povećanog rizika smrtnosti od SLE, vjerojatno zbog malog broja umrlih. DSMR i DSHR su izračunate i na razini županija koje su pritom rangirane, a rangovi zbrajani kako bi se dobio sintetski rang kojim se procjenjuje ukupni „populacijski rizik“ od SLE. Kombiniranim rangom se ističe Osječko Baranjska županija, moguće i zbog povećanog opterećenja bolešću u regiji. Izravno standardizirane stope hospitalizacija i smrtnosti vezane uz SLE, vizualna analiza koroplet mapa relativnog rizika smrtnosti i hospitalizacija od SLE na rezoluciji razine općina, rangiranje na županijskoj razini prema navedenim stopama, kumulativno rangiranje županija, usporedba regija prema DSMR i DSHR uz analizu preživljenja i Poissonovu regresijsku analizu te konačno spacijalna analiza rizika prostornim skeniranjem čine sastavnice jeftinog, jednostavnog i učinkovitog sustava monitoriranja koji može kumulativno detektirati regionalne varijacije u prevalenciji, težini i prezentaciji bolesti, ali također detektira razlike vezane uz javno zdravstvena pitanja., SLE is a rare flagship chronic systemic autoimmune disease. We present a new model of population based SLE monitoring in Croatia. We found prevalence and mortality to be to insensitive to detect regional differences in disease related morbidity and mortality. Existing administrative, routinely colected mortality and morbidity data in Croatian Institute of Public Health were proposed as a basic dataset for further analysis. 2372 hospitalizations of 1067 patients with discharge diagnoses of SLE (ICD codes M32.1-32.9) were registered in Croatia from 2002-2007 with female to male ratio 6,1 (incident hospitalizations). 148 hospitalizations had to be omitted from further analysis due to missing or inadequate data, or non-Croatian citizenship. 78 SLE relatd death cases (65 female and 13 male) were noted in the same period. Observed disease specific mortality and hospitalization rates (DSMR and DSHR) were directly standardized using European standard 100000 population. DSMR suggest higher mortality rates in Panonian and Mediterranean regions of Croatia. DSHR suggest higher hospitalization rates in northwestern Croatia. Hospitalizations in patients from northwestern Croatia were shorter than in patients living in Mediterranean and Panonian region. Visualisation of mortality and hospitalization data were presented using coropleth maps of municipality based relative risk for SLE related hospitalizations and mortality. Space scaning of municipality based hospitalization data with SatScan using discrete Poissons method indicate that there are two significant clusters with increased risk of SLE related hospitalizations in Croatia. Same method has not been able to detect significant clusters of SLE related mortality probably due to the low total counts. DSMR and DSHR were also calculated on the county level. Counties were ranked according to both rates and the ranks were combined giving synthetic rank estimating total „population risk“ of SLE. The highest combined rank has been noted in Osiječko Baranjska county. DSMR and DSHR , visual analysis of mapped data of relative risk for SLE related death or hospitalization, county based ranking acording to the DSMR and DSHR, cumulative county ranks, regionaly based differences in DSMR and DSHR coupled with survival analysis and Poissons regression and finaly spacial scan analysis present components of the simple, cheap and effective monitoring system able to detect differences and regional variations in disease prevalence, presentation and severity which could be of important regional public health issues.

Dopuna preporuka HRD-a iz 2017.

Sjögren’s syndrome (SS) is a chronic, systemic autoimmune disease of unknown aetiology that manifests with various clinical manifestations. It is characterized by dense lymphocytic infiltration of exocrine glands which leads to functional impairment. Involvement of the salivary and lacrimal glands, and the consequent dryness of the eyes and mouth, are among the most common clinical manifestations of the disease. SS may occur as a primary disease or secondary to another organ-specific autoimmune disease or overlap with other rheumatic conditions. The disease is named after the Swedish ophthalmologist Henrik Sjögren. The diagnosis of SS is made based on clinical and laboratory indicators and objective findings of involvement of the salivary glands and lacrimal glands. In clinical practice, classification criteria often help in making a diagnosis, although they were initially developed and validated to standardize a cohort of patients for inclusion in the clinical trials and studies. Over the years, more than ten different classification criteria for SS have been proposed. In 1993, the first multicentric preliminary European classification criteria for SS were proposed. These criteria were subsequently revised by the American-European Consensus Group (AECG ) in 2002. In 2012, the new modified classification criteria of the American College of Rheumatology (ACR ) for SS were published, which did not provide any significant diagnostic changes. The 2016 criteria of the ACR and the European Alliance of Associations for Rheumatology (EULAR ) are currently used for the classification of the disease. A significant shift with the new criteria is that symptoms of dry eyes or mouth are not necessary for classification, and it is sufficient to have only one of the systemic manifestations of the disease., Sjögrenov sindrom (SS) je kronična, sustavna autoimunosna bolest nepoznate etiologije koja se manifestira širokim rasponom kliničkih očitovanja. Histološki je karakterizirana gustom limfocitnom infiltracijom egzokrinih žlijezda, što pridonosi oštećenju njihove funkcije. Zahvaćanje žlijezda slinovnica i suznih žlijezda te posljedična suhoća očiju i usta spadaju u najčešće kliničke manifestacije bolesti. SS se može pojaviti kao primarna bolest ili sekundarno uz drugu organ-specifičnu autoimunu bolest te u preklapanju s drugim reumatološkim stanjima. Bolest je ime dobila po švedskom oftalmologu Henriku Sjögrenu. Dijagnoza SS-a postavlja se na temelju kliničkih i laboratorijskih pokazatelja te objektivnih nalaza zahvaćenosti žlijezda slinovnica i suznih žlijezda. Najčešće u kliničkoj praksi za postavljanje dijagnoze pomažu klasifikacijski kriteriji, iako su oni prvotno razvijeni i validirani u svrhu standardiziranja kohorti bolesnika za uključivanje u klinička ispitivanja i studije. Tijekom godina je predloženo više od deset različitih klasifikacijskih kriterija za SS. Godine 1993. predloženi su prvi multicentrični preliminarni europski klasifikacijski kriteriji za SS. Te kriterije naknadno je revidirala 2002. godine Američko-europska grupa stručnjaka (AECG – prema engl. American- European Consensus Group). Godine 2012. objavljeni su novi, dosta izmijenjeni klasifikacijski kriteriji Američkoga reumatološkog društva za SS koji nisu donijeli velikih dijagnostičkih pomaka. Za klasifikaciju bolesti trenutno se koriste kriteriji iz 2016. godine Američkog reumatološkog društva (ACR – prema engl. American College of Rheumatology) i Europskog saveza udruga za reumatologiju (EULAR – prema engl. European Alliance of Associations for Rheumatology). Značajna je izmjena u novim kriterijima da za klasifikaciju nije nužna prisutnost simptoma suhoće očiju ili usta, već je dovoljno imati neku od sustavnih manifestacija bolesti.

Longitudinalni ekstenzivni transverzalni mijelitis (LETM) je iznimno rijetka, životno ugrožavajuća komplikacija sistemskog eritemskog lupusa (SLE). Riječ je o upalnoj leziji leđne moždine koja zahvaća najmanje tri susjedna vertebralna segmenta. Klinički se prezentira paraparezom ili tetraparezom, senzornim deficitom i vegetativnom disfunkcijom, a u teškim slučajevima uzrokuje respiratornu insuficijenciju. Prikazana je bolesnica u dobi od 28 godina koja se posljednjih 11 godina prati zbog SLE-a, a kod koje se bolest komplicirala razvojem LETM-a sa zahvaćanjem cervikalne i torakalne medule. LETM je nastupio nakon razdoblja stabilne remisije bolesti, a bio je praćen kliničkom, a prethodno i serološkom aktivacijom SLE-a. U sklopu LETM-a bolesnica je razvila tetraparezu i visoku hipesteziju, a zbog pareze respiratorne muskulature i posljedične respiratorne insuficijencije bila je potrebna mehanička ventilacija. Budući da obradom nije dokazana intratekalna infekcija, provedena je kombinirana imunosupresivna terapija kojom je postignut postupni i djelomični neurološki oporavak i poboljšanje neuroradiološkog nalaza. LETM je rijetka, ali ozbiljna komplikacija SLE-a, koja često ostavlja teške neurološke sekvele ili pak dovodi do letalnog ishoda. Uz prikaz bolesnice u pregledu literature navedena su dosadašnja saznanja o patogenezi, dijagnozi i terapijskom pristupu LETM-u u kontekstu neuropsihijatrijskog lupusa., Longitudinal extensive transverse myelitis (LETM) is rather rare life threatening complication of systemic lupus erythematosus (SLE). It is defined as an inflammatory spinal cord lesion that extends over three or more adjacent vertebral segments. Clinical presentation can consist of paraparesis or tetraparesis, sensory disturbances and vegetative dysfunction, and respiratory failure may follow in severe cases. A female patient aged 28 years, diagnosed with SLE 11 years ago, has been herein presented, whose disease was complicated by LETM affecting cervical and thoracic spinal cord. LETM occurred after the period of stable remission, and was followed by clinical, as well as previously serologically active disease. In the context of LETM the patient developed tetraparesis and high hypoesthesia, while respiratory muscle paralysis and consecutive respiratory failure required mechanical ventilation. After the exclusion of underlying intracranial infection, combined immunosuppressive therapy has been used resulting in a gradual but partial neurological recovery, and an overall neuroradiological improvement. LETM is an uncommon but severe complication of SLE leading to serious neurological sequelae, and often resulting even in fatal otcome. Along with herein presented case report, current available findings on pathogenesis, diagnosis and therapheutic approach in the context of neuropsychiatric lupus have been depicted through the review of the literature.

Dopuna prijedloga preporuka HRD-a iz 2017.

This abstract elaborates the importance of early intervention in patients with inflammatory rheumatic diseases such as rheumatoid arthritis and systemic lupus erythematosus.

Common variable immunodeficiency (CVID) is a form of primary immunodeficiency that results from impaired B-cell secretion of immunoglobulins. Patients with CVID have an increased susceptibility to recurrent and chronic infections and also an increased incidence of autoimmune and lymphoproliferative disorders and cancer. A 33- year old male patient has been followed-up with the diagnosis of CVID and from early childhood he experienced recurrent respiratory infections. In January 2010, patient was admitted in GH with the symptoms of bronchopneumonia and during disease evaluation he was diagnosed with CVID. According to the guidelines, patient was treated with substitute IV immunoglobulins. During follow-up in day care and stationary UHC departments (2011 - 2020), patient experienced in total 14 respiratory (7 pneumonias, 5 acute sinusitides, 2 acute exacerbations of chronic bronchitis) and 2 digestive system (chronic gastritis, colitis) complications of CVID. Lastly, he developed noninfectious pulmonary complication - granulomatous-lymphocytic interstitial lung disease (GLILD). In 2015, CVID treatment was modified in order to accomplish satisfactory IG levels. Therefore, patient was transferred from IV to conventional IGSC therapy. Due to easier application and greater volume of SC inserted IG, patient was switched to new generation IGSC therapy in 2019. Besides that, corticosteroides and mesalazine were also administered and with current treatment patient is stable. Although respiratory infections are common CVID complications it is also necessary to consider the autoimmune and neoplastic manifestations of the disease. Multidisciplinary approach, regular follow-up and application of immunoglobulins are the key factors in decreasing the disability and mortality in patients with CVID.

PubMed: 30852552, Objectives To determine whether progressive skin fibrosis is associated with visceral organ progression and mortality during follow-up in patients with diffuse cutaneous systemic sclerosis (dcSSc). Methods We evaluated patients from the European Scleroderma Trials and Research database with dcSSc, baseline modified Rodnan skin score (mRSS) ?7, valid mRSS at 12±3 months after baseline and ?1 annual follow-up visit. Progressive skin fibrosis was defined as an increase in mRSS >5 and ?25% from baseline to 12±3 months. Outcomes were pulmonary, cardiovascular and renal progression, and all-cause death. Associations between skin progression and outcomes were evaluated by Kaplan-Meier survival analysis and multivariable Cox regression. Results Of 1021 included patients, 78 (7.6%) had progressive skin fibrosis (skin progressors). Median follow-up was 3.4 years. Survival analyses indicated that skin progressors had a significantly higher probability of FVC decline ?10% (53.6% vs 34.4%; p, Bayer Bayer, 1Department of Rheumatology, University Hospital Zurich, Zurich, switzerland 2Graf Biostatistics, Winterthur, switzerland 3Clinical Development Pulmonology, Bayer Us llC, Whippany, new Jersey, Usa 4Data science and analytics, Bayer plc, Reading, UK 5Rheumatology a Department, Paris Descartes University, inseRM U1016, sorbonne, Paris Cité, Cochin Hospital, Paris, France 6Division of Rheumatology, University of Florence, Florence, italy 7Department of Medicine, Division of Rheumatology, University of Western Ontario, st. Joseph’s Health Care, london, Western Ontario, Canada 8Department of Rheumatology, Royal Free Hospital, University College london, london, UK 9scleroderma Program, Department of internal Medicine, Division of Rheumatology, University of Michigan, ann arbor, Michigan, Usa Acknowledgements The authors thank nicole schneider for excellent administration and data entry into the eUsTaR cohort. Medical writing assistance was provided by adelphi Communications ltd (Bollington, UK), funded by Bayer aG (Berlin, Germany)., This study was supported by a grant from Bayer aG.

OBJECTIVE: Systemic sclerosis (SSc) is a heterogeneous connective tissue disease that is typically subdivided into limited cutaneous SSc (lcSSc) and diffuse cutaneous SSc (dcSSc) depending on the extent of skin involvement. This subclassification may not capture the entire variability of clinical phenotypes. The European Scleroderma Trials and Research (EUSTAR) database includes data on a prospective cohort of SSc patients from 122 European referral centers. This study was undertaken to perform a cluster analysis of EUSTAR data to distinguish and characterize homogeneous phenotypes without any a priori assumptions, and to examine survival among the clusters obtained. METHODS: A total of 11,318 patients were registered in the EUSTAR database, and 6,927 were included in the study. Twenty-four clinical and serologic variables were used for clustering. RESULTS: Clustering analyses provided a first delineation of 2 clusters showing moderate stability. In an exploratory attempt, we further characterized 6 homogeneous groups that differed with regard to their clinical features, autoantibody profile, and mortality. Some groups resembled usual dcSSc or lcSSc prototypes, but others exhibited unique features, such as a majority of lcSSc patients with a high rate of visceral damage and antitopoisomerase antibodies. Prognosis varied among groups and the presence of organ damage markedly impacted survival regardless of cutaneous involvement. CONCLUSION: Our findings suggest that restricting subsets of SSc patients to only those based on cutaneous involvement may not capture the complete heterogeneity of the disease. Organ damage and antibody profile should be taken into consideration when individuating homogeneous groups of patients with a distinct prognosis. ispartof: ARTHRITIS & RHEUMATOLOGY vol:71 issue:9 pages:1553-1570 ispartof: location:United States status: published

Biološka se terapija primjenjuje u liječenju upalnih reumatskih bolesti posljednjih desetak godina čime se znatno unaprijedila kontrola aktivnosti bolesti. Sigurnost primjene biološke terapije u pretkoncepcijskom razdoblju te tijekom trudnoće i laktacije veoma je važna pri planiranju liječenja mladih bolesnica. Ni Američka agencija za hranu i lijekove ni Europska agencija za lijekove do danas nisu nijedan biološki lijek proglasile sigurnim u trudnoći. Europska liga protiv reumatizma i Britansko reumatološko društvo predložili su smjernice za liječenje reumatoloških bolesnica tijekom trudnoće i laktacije, dok Američko reumatološko društvo trenutačno izrađuje smjernice za terapijski pristup u trudnoći. S druge strane, objavljeni su mnogi radovi o ishodima trudnoća u bolesnica liječenih inhibitorima faktora tumorske nekroze i drugom biološkom terapijom, s malim brojem neželjenih ishoda. Cilj je ovog rada bila analiza primjene biološke terapije u bolesnica koje su planirano ili neplanirano zanijele za vrijeme liječenja u našoj ustanovi te ishoda tih trudnoća. Naši se rezultati ne razlikuju od dosad objavljene literature. Petnaest prikazanih bolesnica primalo je jedan od blokatora tumorske nekroze-α, a jedna bolesnica blokator interleukina- 6 tijekom trudnoće ili neposredno prije začeća. Samo su dvije bolesnice poštovale preporučeni minimalni period od obustave terapije do začeća. Od navedenih 16 bolesnica 3 su imale medicinski indiciran prekid trudnoće: jedna zbog teške malformacije ploda, a dvije zbog terapije konvencionalnim lijekovima koji modificiraju bolest, a kontraindicirani su u trudnoći., Treatment of inflammatory rheumatic diseases and control of disease activity have considerably improved after the introduction of biologic therapy over a decade ago. The safety profile of biologic therapy in the preconception period as well as during pregnancy and lactation is neccessary to consider when planning the treatment of young female patients. Neither the Food and Drug Administration Agency nor the European Medicines Agency have declared biologic therapy safe during pregnancy. Both the European League Against Rheumatism and the British Society for Rheumatology proposed guidelines for the treatment of female rheumatology patients during pregnancy and lactation, and the American College of Rheumatology is currently developing guidelines for the therapeutic approach during pregnancy. On the other hand, there are numerous publications of pregnancy outcomes in patients treated with biologic therapy, with a small number of adverse effects. We analyzed the modalities of biologic therapy and pregnancy outcomes in patients treated at our Department, during planned and unplanned pregnancies. Our results do not differ from the literature published to date. Among the patients described here, 15 were treated with an inhibitor of tumor necrosis factor-α and one with an IL-6 inhibitor during or just before pregnancy. Only 2 patients stopped biologic therapy in compliance with the guidelines. Of the 16 aforementioned patients, 3 had to undergo medically-induced abortion; one patient because of a severe fetal malformation and the other two patients because they had bee.

PubMed: 31227488, Objectives Mortality and worsening of organ function are desirable endpoints for clinical trials in systemic sclerosis (SSc). The aim of this study was to identify factors that allow enrichment of patients with these endpoints, in a population of patients from the European Scleroderma Trials and Research group database. Methods Inclusion criteria were diagnosis of diffuse SSc and follow-up over 12±3 months. Disease worsening/organ progression was fulfilled if any of the following events occurred: new renal crisis; decrease of lung or heart function; new echocardiography-suspected pulmonary hypertension or death. In total, 42 clinical parameters were chosen as predictors for the analysis by using (1) imputation of missing data on the basis of multivariate imputation and (2) least absolute shrinkage and selection operator regression. Results Of 1451 patients meeting the inclusion criteria, 706 had complete data on outcome parameters and were included in the analysis. Of the 42 outcome predictors, eight remained in the final regression model. There was substantial evidence for a strong association between disease progression and age, active digital ulcer (DU), lung fibrosis, muscle weakness and elevated C-reactive protein (CRP) level. Active DU, CRP elevation, lung fibrosis and muscle weakness were also associated with a significantly shorter time to disease progression. A bootstrap validation step with 10 000 repetitions successfully validated the model. Conclusions The use of the predictive factors presented here could enable cohort enrichment with patients at risk for overall disease worsening in SSc clinical trials. © Author(s) (or their employer(s)) 2019. Re-use permitted under CC BY. Published by BMJ., Bayer Bayer, United Kingdom Université Paris Descartes Li Ka Shing Foundation, LKSF University of Michigan, U-M, 1Department of Rheumatology and the Centre of experimental Rheumatology, University Hospital Zurich, Zurich, switzerland 2Graf Biostatistics, Winterthur, switzerland 3Big Data institute, li Ka shing Centre for Health information and Discovery, nuffield Department of Medicine, University of Oxford, Oxford, UK 4Rheumatology a Department, Paris Descartes University, sorbonne Paris Cité, Cochin Hospital, Paris, France 5Data science and analytics, Bayer plc, Reading, UK 6UCl Division of Medicine, Royal Free Campus, london, UK 7Division of Rheumatology, Department of internal Medicine, University of Michigan scleroderma Program, University of Michigan, ann arbor, Michigan, Usa 8Department of experimental and Clinical Medicine, University of Florence, Florence, italy 9Bayer Us llC, Whippany, new Jersey, Usa 10Department of Medicine, Division of Rheumatology, University of Western Ontario, st. Joseph’s Health Care, london, Ontario, Canada Acknowledgements The R-code for the linear Mi-lassO was received from Qixuan Chen.21 Medical writing assistance was provided by adelphi Communications ltd (Bollington, UK), funded by Bayer aG (Berlin, Germany)., Contributors study conception and design, acquisition of data, analysis and interpretation of data and drafting and revising the article: OD and MB; analysis and interpretation of data: OD, MB, Rs and nG. all authors have critically reviewed and approved the final submitted version to be published. Funding This study was supported by a grant from Bayer aG. Bayer employees are coauthors of this paper and supported the study design and interpretation of the data, but otherwise Bayer had no influence on the study., Competing interests MOB declares no conflict of interest. OD has had consultancy relationships with actelion, Bayer, Biogen idec, Boehringer ingelheim, Chemomab, espeRare foundation, Genentech/Roche, GsK, inventiva, italfarmaco, lilly, medac, Medimmune, Mitsubishi Tanabe Pharma, Pharmacyclics, novartis, Pfizer, sanofi, sinoxa and UCB in the area of potential treatments of scleroderma and its complications. OD has received research funding from actelion, Bayer, Boehringer ingelheim, Mitsubishi Tanabe Pharma and Roche in the area of potential treatments of scleroderma and its complications. OD has a patent for mir-29 licensed for the treatment of systemic sclerosis. DK has consultancy relationships and/or has received grant/research support from Bayer, Bristol-Myers squibb, Boehringer ingelheim, Genentech/Roche, niH, Pfizer, sanofi-aventis Pharmaceuticals, actelion Pharmaceuticals Us, Chemomab, Corbus, Covis, Cytori, eicos, eMD serono, Gilead, GlaxosmithKline, and UCB Pharma. He is a shareholder of eicos. CPD has consultancy relationships with and/or has received speakers’ bureau fees from actelion Pharmaceuticals Us, Bayer aG, GlaxosmithKline, Csl Behring, Merck serono, Roche Pharmaceuticals, Genentech and Biogen iDeC inc., inventiva, sanofi-aventis Pharmaceuticals and Boehringer ingelheim. JeP has consultancy relationships with and/or has received grant/research support from actelion, Bayer aG, Bristol-Myers squibb, Merck, Pfizer inc. and Roche. MM-C has consultancy relationships and/ or has received grant/research support from Pfizer, Bristol-Myers squibb, actelion, UCB Pharma, Bayer, Chemomab, Genentech/Roche, inventiva and lilly. Ya has consultancy relationships with and/or has received grant/research support from actelion, Pharmaceuticals Us, Bayer aG, Bristol-Myers squibb, inventiva, Medac, Pfizer inc., Roche Pharmaceuticals, Genentech and Biogen iDeC inc., sanofi-aventis Pharmaceuticals and servier. JdOP and JC are employees of Bayer. nTG has nothing to disclose.

Background Renal damage (RD) is one of the most important contributors to morbidity and mortality in patients with systemic lupus erythematosus (SLE). Objectives We aimed to assess features associated with RD in a group of 90 deceased SLE patients routinely followed-up at our institution, which serves as a national referral center for SLE. Methods We retrospectively analyzed 90 SLE patients (68 females) deceased from 2002 to 2011. All patients were ≥18 years of age at death, fulfilling ≥4/11 classification criteria of the American College of Rheumatology (ACR). We identified patients with RD, as defined by the Systemic Lupus International Collaborating Clinics (SLICC)/ACR index. An extensive set of variables was compared between patients with and without RD (RD and RD-N, respectively): demographics, ACR criteria at diagnosis and cumulatively at death, total damage and its components one year following diagnosis and non-renal damage and its components cumulatively at death, as well as components of the metabolic syndrome, smoking, sicca and Hughes syndrome. Frequencies were compared using the chi- square and Fisher’s exact test, and continuous variables using the t-test and Mann-Whitney U test. Variables associated with RD were analyzed using multivariate logistic regression. Results We identified 25/90 patients who accrued RD over the course of their disease. In the univariate analysis, we found no difference between RD and RD-N patients in any of the following parameters: demographics, total count of ACR criteria at diagnosis and death, as well as damage at one year after diagnosis and cumulative non-renal damage at death. Compared to RD-N patients, RD patients had a higher proportion of malar rash at diagnosis (11/25 vs. 13/65, p=0.021) and a higher cumulative proportion of renal disorder (19/25 vs. 30/65, p=0.011), including proteinuria and urinary casts (17/25 vs. 23/65, p=0.005, for both). RD patients also had a higher proportion of myocardial infarction as an item of cumulative damage (7/25 vs. 6/65, p=0.023) and were more frequently obese (11/25 vs. 15/65, p=0.049). Conversely, hematological disorder and leukopenia at diagnosis were less frequent in RD compared to RD-N patients (4/25 vs. 30/65, p=0.008 and 1/25 vs. 21/65, respectively). In the final multivariate model (adjusted for gender, age at diagnosis and disease duration), malar rash at diagnosis and the cumulative presence of renal disorder (classification criteria of the ACR) were positively associated with RD. Conversely, leukopenia at diagnosis was inversely associated with RD (Figure 1). Conclusion More than a quarter of deceased patients accrued RD. While malar rash at diagnosis may be associated with a higher likelihood of developing RD, early leukopenia may be associated with its lower likelihood in deceased patients.

Pismo uredniku

Background: Causes of death (CODs) and survival serve as indicators of overall care of SLE patients. While most of the available data on CODs and survival originate from highly developed healthcare settings and dedicated lupus cohorts, data from Croatia and neighboring countries are still lacking. Objectives: Retrospective analysis of disease features and CODs of SLE patients deceased from 2002 to 2011; assessment of survival of patients diagnosed over the same period. Methods: We analyzed features of 90 patients followed-up at our center, who deceased over the 2002–2011 period. Early death (ED) was defined as death occuring within 5 (10) years following diagnosis, while late death (LD) was defined as death occuring thereaft er. An extensive set of variables was compared between the ED and LD groups: demographics, ACR classification criteria, damage and causes of death. We also analyzed survival in a retrospective cohort of 213 patients. Results: Among 90 deceased patients (68 females), mean age at death was 58±15 years. Th e most frequent classification criteria were antinuclear antibodies (96%), immunological (92%) and hematological disorder (83%), with no difference between the ED and LD groups. 85/90 (94%) patients accrued organ damage, most frequently in the musculoskeletal (59%), cardiovascular (51%) and neuropsychiatric (NP) (48%) domains. Th e most frequent CODs were cardiovascular diseases (40%), followed by infections (33%), active SLE (29%) and malignancies (17%). Th ere was no difference between the frequencies of CODs, except for stroke, which caused death exclusively ≥10 years aft er diagnosis. SLE was recorded in death certificates of 41/90 patients. Five- and ten-year survival in the retrospective cohort (185 non-deceased, 28 deceased patients) was 91% and 80.5%, respectively. NP and renal disorder, serositis and later-onset disease were identified as predictors of death. Conclusions: Five-year survival >90% is in line with survival rates observed in developed countries, while tenyear survival is lower. Th e contribution of SLE to death seems to be underrecognized in SLE patients’ death certificates. References: 1. Padjen I et al. Croat Med J. 2018;59:3–12. 2. Calvo-Alen J et al. Rheumatology 2005;44:1186–9.

Purpose Neuropsychiatric damage (ND) is a major determinant of morbidity in SLE. We analysed ND in a group of deceased SLE patients and identified features associated with ND. Methods We retrospectively analysed 90 patients (68 females) deceased during 2002–2011. All patients fulfilled at least 4 classification criteria of the ACR. We identified patients with ND, as defined by the SLICC/ACR damage index, and its components: cognitive impairment/major psychosis (CIMP), seizures, cerebrovascular accident (CA), cranial/peripheral neuropathy and transverse myelitis (TM). Following variables were compared between patients with and without ND (ND +and ND­, respectively): demographics, ACR criteria at diagnosis and cumulatively at death (available at diagnosis for 73 patients), damage and its components one year following diagnosis and at death, disease activity (ECLAM) at diagnosis, components of the metabolic syndrome, smoking and immunosuppressive treatment. Frequencies were compared using the chi-square and Fisher´s exact test, and continuous variables using the t-test and Mann-Whitney U-test. Variables associated with ND were included in a multivariate logistic regression model. Results We identified 44/90 ND +patients: 17/44 with CIMP, 6/44 with seizures, 21/44 with CA, 20/44 with neuropathy, none with TM. ND +patients had a higher cumulative count of ACR criteria compared to their ND- counterparts (6.02±1.23 vs 5.39±1.26). They had a higher proportion of neurologic disorder (NRL-D) (6/37 vs 0/36) and a lower proportion of serositis (4/37 vs 11/36) at diagnosis, as well as higher cumulative proportions of NRL-D (9/44 vs 2/46), hematologic disorder (41/44 vs 34/46) and lymphopenia (34/44 vs 24/46) (p

Background Loss to follow-up in the year prior to death may lead to underrecognition and underreporting of systemic lupus erythematosus (SLE) as a cause of death of lupus patients. Objectives We aimed to assess the extent and features associated with loss to follow-up in the year prior to death in a group of 90 deceased SLE patients from our tertiary centre. Methods We retrospectively analysed 90 SLE patients (68 females) followed-up at our centre, deceased from 2002 to 2011. Patients were ≥18 years of age at death and fulfilled ≥4 classification criteria of the American College of Rheumatology (ACR). The cause and place of death were identified by matching patient data from our department’s SLE registry with data from the National Death Database. Patients were considered lost to follow- up in the year prior to death (LTF) if the time span between the last visit to our centre and death exceeded 1 year. Other patients were considered to be under regular follow-up (RGF). An extensive set of parameters was compared between the LTF and RGF groups: demographics, ACR classification criteria, cumulative damage according to the Systemic Lupus International Collaborative Clinics (SLICC)/ACR index, as well as causes of death. Frequencies were compared using the chi-square and Fisher’s exact test, and continuous variables using the t-test and Mann- Whitney U-test. Results We identified 35/90 patients in the LTF group (29 females). The time span between the last visit to our centre and death of LTF patients ranged from >1 to 3 years. Compared to the RGF group, LTF patients were diagnosed at a later age (mean ±SD: 54±15 vs. 44±17 years, p=0.006), while there was no difference in disease duration (median of 11 years, IQR of 5–15 years in the RGF group vs. median of 7 years, IQR of 5–15 years in the LTF group, p=0.285). The LTF and RGF groups did not differ in the count of ACR criteria (median of 5, IQR of 4–6 vs. median of 6, IQR of 5–7, p=0.053) and cumulative damage (median damage of 3, IQR of 2–5 vs. median of 5, IQR of 3–8, p=0.068). Compared to the RGF group, LTF patients had a lower cumulative proportion of pericarditis (1/35 vs.16/55), proteinuria (10/35 vs. 30/55), hemolytic anaemia (1/35 vs. 10/55), thrombocytopenia (5/35 vs. 21/55) and Hughes syndrome (2/35 vs. 13/55) (p

Causes of death and morbidity of patients with systemic lupus erythematosus (SLE) include the systemic disease itself and a number of comorbidities, most frequently cardiovascular diseases, infections and malignancies. Given the complex relationship between SLE and comorbidities, it is not always possible to ascribe a single cause of death to deceased patients with SLE. In this review we present the most important causes of morbidity and the way they contribute to death in patients with SLE. Furthermore, we analyze the problem of (non)recording of SLE in death certificates of SLE patients. Matching data from at least two different sources – medical charts from a lupus center and a vital statistics database – may improve the recognition of SLE as a cause of death in the general population.

Reumatoidni artritis sustavna je upalna reumatska bolest koja, osim zglobova, zahvaća i unutarnje organe, kožu, osjetilni i živčani sustav, a znatno povisuje i rizik od kardiovaskularnih bolesti. Posljednjih godina znatno se promijenio pristup u dijagnostici i liječenju bolesnika s reumatoidnim artritisom. Rana dijagnoza i pravodobno započinjanje liječenja sintetskim i biološkim lijekovima iznimno su važni da bi se spriječile ireverzibilne posljedice ove teške bolesti. Hrvatsko reumatološko društvo Hrvatskoga liječničkog zbora u skladu s novijim spoznajama u reumatologiji obnovilo je preporuke za liječenje reumatoidnog artritisa.

Spondiloartritisi (SpA) grupa su upalnih reumatskih bolesti koje dijele zajednička etiopatogenetska, radiološka i klinička obilježja. Te su bolesti znatan teret za bolesnika i za društvo. Posljednjih godina dogodile su se velike novosti u klasifi kaciji, dijagnostici i liječenju SpA. Stoga je Hrvatsko reumatološko društvo Hrvatskoga liječničkog zbora ažuriralo prijedlog primjene bioloških lijekova (referentnih ili biosličnih) i novouvedenih ciljanih sintetskih molekula u algoritam liječenja odraslih bolesnika sa SpA. Prijedlog preporuka usklađen je s preporukama uglednih europskih i drugih međunarodnih organizacija iz ovog područja. Nadamo se da će standardizirani, sveobuhvatni i aktualni pristup u ispunjavanju kriterija, odabiru lijekova, praćenju i evaluaciji ishoda koji smo primijenili u cijelom spektru bolesnika sa SpA pomoći kliničarima i drugim zainteresiranima u postizanju optimalnih rezultata liječenja ovih bolesnika

Introduction: Transition for patients with chronic diseases is defined as the purposeful, planned movement of adolescents and young adults from child-centered to adult oriented health care system.1 Rheumatic diseases of childhood extend to adulthood as active diseases in 30-70%, which is the reason for requiring rheumatologic care into adulthood. First coordinated transition of pediatric rheumatologic patients in Croatia was organized in 2012 as developmental type of transition, where patient is gradually prepared for the transition by a pediatrician rheumatologist throughout years preceding transfer. The moment of final transfer to adult care is organized in the pediatric rheumatology clinic, where patients and their parents are meeting adult rheumatologist in known environment (pediatric rheumatology ambulance). At the transfer, patient's history is being presented to the adult rheumatologist and afterwards patient is examined by both of the specialists. At the end of the examination, patient is given next checkup date at adult rheumatologist clinic, where the patient will continue with regular follow up visits. Objectives: Our objective is to verify successfulness of organized transition of pediatric rheumatologic patients to adult care. Methods: We have conducted a retrospective research of patients’ databases at Departments of Pediatric Rheumatology and correlated them with data from adult Clinical Immunology and Rheumatology Department in order to verify number of pediatric patients that have continued with regular follow up in adult care after coordinated transition. Results: During 5 year period (2012.-2017.) 78 patients have been transferred to adult rheumatologist. There were 50 JIA patients, 18 SLE patients, and 10 patients with other rheumatic diseases (8 patients with mixed connective tissue disease, 1 with Wegener's granulomatosis, 1 with fever of unknown origin). Median age at the time of transfer was 19, 6 years (14, 6-26, 6). Patients with SLE were older at the time of transfer than two other groups of patients (SLE 21, 29 ± Pediatric Rheumatology 2017, 15(Suppl 2):64 Page 71 of 191 2, 99 vs. JIA 18, 83 ± 1, 22 vs. other diagnosis 19, 42 ± 2, 8, p < 0, 01). Total of 63 patients (80, 8%) have continued with regular follow up at adult rheumatologist and 15 patients never showed up at adult rheumatologist clinic, probably due to remission of the disease or possible follow up by other chosen adult rheumatologist. Conclusion: Young adults with rheumatic diseases have complex medical and psychological needs and transition to adult care is a critical component of care. In order to maintain adequate level of follow up and prevent additional flares in turbulent adolescent period, continuity of rheumatologic care is essential. By organizing coordinated transition to adult rheumatologist, a most of the pediatric patients with rheumatic disease (80, 8%) are continuing with regular follow up in the adult care. Each transition should be an individually tailored process according to needs and psychological maturity of patients.