EARLY DIAGNOSIS OF GIANT CELL ARTERITIS – DOES IT MATTER?

Purpose: If untreated, giant cell arteritis can lead to blindness and stroke. The study objectives were to assess diagnostic procedures and treatment in early interventional clinic in University Clinical Centre Maribor in patients with temporal arteritis. Methods: Retrospective study (from 2012 to 2017) of patients diagnosed with temporal arteritis. We assessed epidemiological data, delay of diagnosis, and diagnostic procedures. Results were assessed with statistical methods (SPSS 22.0). The main goal was to determinate the delay in days between symptom onset and admission to the interventional rheumatology clinic and to assess the causes of delay. Results: Fift y-three GCA (66 % female) patients with mean age 76.25 (from 63 – 89 years) years were included. Mean time duration of symptoms before admission to our early interventional clinic was 33.74 (0–180) days. The diagnostic procedure was completed in mean time of 2.04 days from the presentation at our interventional rheumatology clinic. Th e median time to the temporal artery biopsy (TAB) performed in 52 /53 patients was 2 days, with the median 2 days to the preliminary histological results from admission. TAB was positive in 43 (81.1%) of cases. The median time from admittance to colour Doppler sonography (CDS) of aortic arch branches was 2 days and it was positive in all 19 (35.8%) performed cases. 16 (30.2%) patients had polymyalgia rheumatica, 35 (66%) patients had visual disturbances, permanent one eye blindness occurred in 12 (22.64%) patients, and 2 (2.8%) patients experienced permanent blindness on both eyes. Seventeen patients (32.1%) were initially treated with intravenous methylprednisolone pulse. Th e mean initial dose of oral methylprednisolone was 45.55 (+/– 15.54) mg. All patients received low dose Aspirin. Conclusions: Early diagnosis and treatment of giant cell arteritis are very important as miss- or non-diagnose GCA can lead to permanent blindness of the patient. With better education and public awareness, better access and better professional education of primary care physicians, and early admission to secondary interventional clinics we might spare these patients from the devastating consequences of the GCA

Mnenje posvetovalnega sestanka revmatologov in pulmologov glede obravnave bolnika s prizadetostjo pljučnega intersticija v sklopu vnetnih revmatičnih bolezni

Vnetne revmatične bolezni so heterogena skupina bolezni, ki lahko prizadenejo tudi pljučni intersticij. Tako kot je pogostnost intersticijske pljučne bolezni (IPB) v okviru vnetnih revmatičnih bolezni variabilna, je tudi klinična slika zelo raznolika: od omejene nenapredujoče prizadetosti pljučnega intersticija do fulminantno potekajoče življenje ogrožajoče bolezni. Podatki za diagnosticiranje in zdravljenje IPB v sklopu vnetnih revmatičnih bolezni so razmeroma skopi. Temeljno zdravljenje praviloma predstavljajo imunomodulacijska zdravila. Nedavno so se jim pri progresivni pljučni fibrozi pridružili še protifibrotiki. Odločitev pri kom, kdaj in kako intenzivno zdraviti IPB pogosto predstavlja izziv. V prispevku podajamo sklepe multidisciplinarnega posvetovalnega sestanka slovenskih revmatologov in pulmologov, ki po mnenju avtorjev glede na dostopne podatke pomenijo dobro prakso pri obravnavi tovrstnih bolnikov. Osredinjamo se na bolnike s sistemskimi vezivnotkivnimi boleznimi in revmatoidnim artritisom

Avtoimunske bolezni: diagnostični izziv

Imunski sistem nas varuje pred številnimi dejavniki okolja, ki lahko poškodujejo človeška tkiva. Deluje usklajeno in je sposoben razlikovati med človeku lastnimi in tujimi strukturami. Kadar prepoznava lastna tkiva kot tuja, se sprožijo vnetni mehanizmi proti lastnim tkivnim in celičnim strukturam. Te strukture imenujemo avtoantigeni, nanje se tvorijo protitelesa. Kadar prizadene avtoimunsko vnetje pomembne organske sisteme, govorimo o avtoimunskih boleznih. Te so lahko omejene, in sicer le na en organski sistem, ali sistemske, kjer je prizadetih več organskih sistemov. V prispevku obravnavamo najpomembnejše mehanizme, ki privedejo do avtoimunskega vnetja, v nadaljevanju pa tudi diagnostiko avtoimunskih bolezni, ki temelji predvsem na klinični sliki. V veliko pomoč pri diagnozi je tudi določevanje nekaterih specifičnih protiteles na avtoantigene. Navedene so najpogostejše laboratorijske preiskave in tisti imunološki testi, ki so pomembni za diagnozo posameznih avtoimunskih bolezni ter sodijo tudi med diagnostične kriterije. Poudarjeno je, da je zlasti v družinski medicini potrebno biti pozoren na klinične znake avtoimunskega vnetja, poznati pa je potrebno tudi imunološke laboratorijske teste, saj je pravočasna diagnostika bistvena za zdravljenje avtoimunskih boleznih.The immune system defends humans against foreign factors that could damage healthy tissue. It detects and distinguishes a wide variety of substances from the organismʼs own structures. Failing to recognize the organismʼs own structures as selfstructures causes autoimmune disorders. These self-structures are called ŽautoantigensŽ. Autoimmune disorders are divided into those directly damaging only a single organ system, and those in which many important organs are damaged (systemic). This article deals with the most important autoimmune mechanisms inducing autoimmune inflammation. It discusses the diagnoses of autoimmune disorders, which are based mainly on the clinical picture, and the most frequently used laboratory and immunological tests crucial for diagnosing particular autoimmune diseases. The purpose of this article is to emphasize the importance of the early clinical picture and laboratory testing of autoimmune diseases

Henoch-Schönlein purpura in adulthood

Background: Henoch-Schonlein purpura (HSP) is a systemic inflammation of small-vessel walls that predominantly affects children. In adults, on the other hand, it appears rarely, and can be more severe. The disease is characterised by a tetrad of clinical signs which manifest the skin, joint, gastrointestinal, and kidney involvement. HSP is diagnosed using internationally-accepted criteria. At present, there is no effective specific therapy for children and adults. However, in cases of severe organ involvement, aggressive treatment may stabilise the disease. Case report: We present a case of a 59-year-old immunologically uncom- promised patient with an extremely severe form of HSP. Epidemiology, etiopathogenesis, clinical features, diagnostic procedures, differential diagnosis, treatment, and prognosis of the disease are discussed. Conclusions: The patient was treated with corticosteroids, human polyspecific immunoglobulins, and immunosuppressive agents. A satisfactory clinical remission has been achieved.Izhodišča: Henoch-Schonleinova purpura (HSP) je sistemsko vnetje sten malih žil, ki najpogosteje prizadene otroke. Odrasli obolijo redko, potek bolezni pa je pri njih lahko življenjsko ogrožujoč. Za HSP je značilna tetrada kliničnih znakov, ki zrcali okvaro kože, sklepov, prebavnega trakta in ledvic. Pri postavitvi diagnoze je potrebno upoštevati zastavljena diagnostična merila. Učinkovito specifično zdravljenje bolezni pri otrocih in odraslih še ne obstaja. Kljub temu pa je pri težjem poteku z agresivnejšim terapevtskim pristopom možno doseči stabilno klinično sliko. Prikaz primera: Predstavljen je primer 59-letnega imunsko neprizadetega bolnika, pri katerem je bila potrjena izjemno težka oblika HSP. Razpravljamo o epidemiologiji, etiopatogenezi, klinični sliki, diagnostičnih postopkih, diferencialni diagnozi, zdravljenju in prognozi te bolezni. Zaključki: Pri bolniku je bilo uvedeno zdravljenje s kortikosteroidi, humanimi polispecifičnimi imunoglobulini ter citostatiki. Doseženo je bilo zadovoljivo začasno izboljšanje bolezni

Opportunities and challenges in rheumatology research in Central Europe

The Central European Congress of Rheumatology (CECR) has been organized by seven Central European countries: Austria, Croatia, Czech Republic, Hungary, Poland, Slovakia, and Slovenia. These countries have lots of similarities, but also differences, with respect to rheumatology research. In this paper, based on questionnaires, we wish to demonstrate achievements and difficulties in rheumatology research performed in our region