Overlap Syndrome at Rheumatology Department – Case Report

Systemic lupus erythematosus (SLE) is an autoimmune connective tissue disease, affecting mostly women of childbearing age. Dermatomyositis (DM) is a rare autoimmune inflammatory myopathy affecting both skin and muscles. Overlap syndrome is an autoimmune disease which shares features of at least two recognised connective tissue diseases. We present a case of an overlap syndrome refractory to treatment

Vrednovanje klasifikacijskih kriterija za sistemski eritemski lupus [Validation of classification criteria for systemic lupus erythematosus]

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with heterogenuous clinical presentations. Most widely used ACR classification from 1997 (ACR-97) shows high specificity and acceptable sensitivity in classifiying patients with established SLE, but its value declines in early stages and in milder cases. SLICC group has published new, revised (SLICC-12) criteria which have shown higher sensitivity, but lower specificity than the ACR-97 criteria. The objective of this study was to validate SLICC-12 and ACR-97 classifications on a patient cohort from UHC Zagreb. It comprised 308 patients with SLE (n=146) and SLE-allied conditions (n=162). Sensitivity and specificity, as well as sensitivity and specificity according to disease duration were compared between two classifications. Merit of every criterium to diagnosing SLE was calculated using logistic regression analysis. A clear distinction between SLICC-12 and ACR-97 criteria is observed. Sensitivity of SLICC-12 criteria is significantly higher with a tendency to rise with disase duration. ACR-97 criteria have shown higher specificity. Specificity of SLICC-12 criteria is low and declines with disease duration. Comparing the overall value of the new SLICC-12 classification to ACR-97 criteria, the new criteria show superiority in our patients. Although SLICC-12 criteria show superiority to ACR-97, and are more successful in diagnosing early SLE, specificity in our population is too low. Our results contribute to the current initiative for developing new criteria for SLE

Rare complications of Sjögren’s syndrome in a female patient

Sjögren’s syndrome (SjS) is a systemic autoimmune disease, most common in middle-aged women, characterized by chronic inflammation of exocrine glands. It can present by itself or alongside other autoimmune diseases. Main symptoms are dry eyes and mouth, but the disease can affect joints, lungs, kidneys, peripheral nervous system (PNS), and rarely central nervous system (CNS). SS-A antibodies in SjS can pass the fetoplacental barrier and cause congenital heart block. Patients with SjS are at higher risk of developing lymphoma

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

Primopredaja i prijelaz bolesnika [Patient handover and transition]

Patient handover is considered to be one of the most delicate medical procedures as well as the most preventable cause of medical error. It takes place at primary, secondary and tertiary healthcare, with specificities at every level of healthcare. Improvement in the quality of patient handover is therefore essential for good medical practice. Accurate and comprehensive communication between medical staff is required for patient safety and continuous adequate healthcare. In this article, we describe recommendations for successful and efficient patient handover and highlight communication errors during the process. Special attention is given to handover of rheumatological patients and transition from pediatric to adult healthcare. Also, we emphasize the necessity of education. Medical staff should be competent in sharing and exchanging relevant information when the patient transfers to another medical care provider. Teaching communication skills has been part of undergraduate and postgraduate curriculum in medical schools. At the University of Zagreb School of Medicine we have introduced a longitudinal six-year course Fundamentals of Medical Skills where special attention is given to communication skills. Nevertheless, medical specialty training still lacks educational contents specialized in patient handover and safe patient discharge

Patient handover and transition

Primopredaja bolesnika smatra se jednim od najdelikatnijih postupaka u medicini i najvažnijim sprječivim uzrokom medicinske pogreške. Ovaj se proces zbiva na svim razinama zdravstvene zaštite: primarnoj, sekundarnoj i tercijarnoj, od kojih svaka ima svoje specifičnosti. Unaprjeđenje kvalitete primopredaje na svakoj razini izrazito je važno. Prikladan i precizan prijenos informacija između medicinskog osoblja nuždan je za sigurnost bolesnika i kontinuiranu zdravstvenu zaštitu. U ovom su članku opisane preporuke za uspješnu i kvalitetnu primopredaju bolesnika te su istaknute pogreške pri komunikaciji i postupku prijelaza u skrb drugom liječniku. Poseban je osvrt dan na primopredaju reumatološkog bolesnika i na prijelaz iz pedijatrijske skrbi u adultnu reumatološku skrb. Osobito je naglašena potreba poučavanja pri komunikaciji i prenošenju podataka tijekom prijelaza bolesnika od jednog liječnika drugomu, iz ustanove u ustanovu. Poučavanje komunikacijskih vještina danas se provodi na medicinskim fakultetima na diplomskoj i poslijediplomskoj razini. Na Medicinskom fakultetu Sveučilišta u Zagrebu uveli smo longitudinalni 6-godišnji predmet Temelji liječničkog umijeća u kojem studente poučavamo komunikaciji. Ipak, ističemo nedovoljan broj nastavnih sadržaja specijalističkog usavršavanja koji obuhvaćaju izobrazbu o primopredaji bolesnika i vještinama otpusta bolesnika.Patient handover is considered to be one of the most delicate medical procedures as well as the most preventable cause of medical error. It takes place at primary, secondary and tertiary healthcare, with specificities at every level of healthcare. Improvement in the quality of patient handover is therefore essential for good medical practice. Accurate and comprehensive communication between medical staff is required for patient safety and continuous adequate healthcare. In this article, we describe recommendations for successful and efficient patient handover and highlight communication errors during the process. Special attention is given to handover of rheumatological patients and transition from pediatric to adult healthcare. Also, we emphasize the necessity of education. Medical staff should be competent in sharing and exchanging relevant information when the patient transfers to another medical care provider. Teaching communication skills has been part of undergraduate and postgraduate curriculum in medical schools. At the Universitiy of Zagreb School of Medicine we have introduced a longitudinal six-year course Fundamentals of Medical Skills where special attention is given to communication skills. Nevertheless, medical specialty training still lacks educational contents specialized in patient handover and safe patient discharge

Povezanost biološke terapije i malignih bolesti u upalnim reumatskim bolestima

Inflammatory rheumatic diseases are chronic, progressive autoimmune diseases which affect the musculoskeletal system and other organ systems. Nowadays, a large number of patients is treated with biological therapy. Although biological drugs selectively affect specific molecules of the immune system, they weaken the overall immune system of the body. Therefore, the patients are more susceptible to infections and other diseases such as lymphomas, breast and skin cancers and melanomas. Chronic inflammation which occurs due to autoimmune disease is also a risk factor for malignant development. So far, studies have not proven direct correlation between biological therapy and solid or haematologic tumours. On the other hand, the increased risk for developing skin cancer in patients on tumour necrosis factor alpha inhibitors has been described. In this review paper we analysed the available medical literature on the risks for malignant disease development in patients with rheumatic diseases who are on biological disease – modifying anti-rheumatic drugs.Sistemske upalne reumatske bolesti jesu kronične, progresivne autoimunosne bolesti koje zahvaćaju lokomotorni sustav i druge organske sustave. Danas je sve više bolesnika liječeno biološkom terapijom. Iako biološki lijekovi selektivno djeluju na specifične molekule imunosnog sustava, oni smanjuju opću obrambenu funkciju organizma, zbog čega su bolesnici podložniji infekcijama, ali i nekim malignim bolestima poput limfoma, karcinoma kože, dojke ili melanoma. Također, sama kronična upala u sklopu autoimunosne bolesti jest rizični čimbenik za razvoj tumorske bolesti. Prema do sada objavljenim studijama, nije dokazana jednoznačna povezanost primjene bioloških lijekova s razvojem solidnih i hematoloških tumora. Suprotno tomu, istraživanja su pokazala povezanost primjene inhibitora tumorske nekroze alfa i razvoja tumora kože. U ovom preglednom radu analizirana je dostupna medicinska literatura o rizicima za razvoj malignih bolesti u bolesnika s reumatološkim bolestima koji su liječeni biološkim antireumatskim lijekovima koji mijenjaju tijek bolesti

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 disease, 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

A PATIENT WITH RHEUMATOID ARTHRITIS AND PRIMARY BILIARY CIRRHOSIS SUCCESSFULLY TREATED WITH ADALIMUMAB

Bolesnici oboljeli od reumatoidnog artritisa u oko 6% slučajeva mogu istodobno bolovati i od primarne bilijarne ciroze. Čini se da faktor tumorske nekroze alfa (TNF-a) ima važnu ulogu u patogenezi obiju bolesti. Inhibitori TNF-a registrirani su za liječenje reumatoidnog artritisa. Jedini odobreni lijek za liječenje primarne bilijarne ciroze jest ursodeoksikolna kiselina. Prikazan je slučaj bolesnice oboljele od reumatoidnog artritisa i primarne bilijarne ciroze, koja je uz terapiju adalimumabom postigla dugotrajnu remisiju obiju bolesti. Ovaj prikaz bolesnice važan je dodatak uz nekoliko do sada objavljenih sličnih slučajeva.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