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Uvod: Avaskularna nekroza kosti (AVN) rijetka je mišićno-koštana komplikacija u bolesnika sa sistemski eritemskim lupusom (SLE). Iako je loše vaskularizirana kost predisponirajući čimbenik nastanku osteomijelitisa, rijetko se opisuje istovremeni nastanak AVN i upale kosti u bolesnika sa SLE.. Prikazujemo djevojku u dobi od 17 godina koja je primljena u našu Kliniku u lipnju 2006 g. s anamnezom poliartralgije, vrućice, glavobolje i osipa na licu unazad 3 tjedna. Na temelju ACR kriterija postavljena je dijagnoza SLE i započeto liječenje metilprednizolonom (2 mg/kg). Budući da nije došlo do zadovoljavajućeg kliničkog i laboratorijskog odgovora, u terapiju su uvedeni i hydroxicloroquine i azathioprine. Doza metilprednizolona polagano je smanjena na 0, 5 mg/kg/d. Djevojka je ponovno je primljena nakon tri mjeseca zbog bolova u desnom koljenu i mišićima, opće slabosti, te povišenih upalnih laboratorijskih parametara. Imunološki tetsovi za SLE su bili u granicama normale. Četvrti dan boravka postaje visoko febrilna uz izraziti otok i bol desne potkoljenice. Učinjena je obrada, te je na MRI postavljena dijagnoza bilateralne tibijalne avaskularne nekroze i osteomijelitisa distalnog femura i proksimalne tibije desno. Učinjena je biopsija i drenaža distalnog dijela desnog femura i desnog koljena, a kulturom je potvrđen Staphylococcus aureus kao uzročnik upale. Zbog teških nuspojava na clindamycin i ciprofloxacin, terapija je promijenjena u rifampicin i cloxacillin intravenski, klavocin i rimfapicinwhich. Doza metilprednizolona je postupno smanjena na 0, 2 mg/kg, a azathioprine je ukinut. Trenutačno je SLE u potpunoj kliničkoj i laboratorijskoj remisiji, a također nema kliničkih znakova infekcije. Zaključak: Opisali smo tešku komplikaciju u bolesnika sa SLE, za koju se smatra da je posljedica visokih doza kortikosteroida, koji su doveli do infekcije osteoartikularnog tkiva sa slabim odgovorom na terapiju. U razdoblju od 1995 do 2006 god. na našem Odjelu dijagnosticirano je 54 bolesnika sa SLE-om, ali ovo je prvi slučaj AVN kosti s teškim multifokalnim osteomijelitisom. Svi bolesnici su liječeni visokim dozama kortikosteroida, no niti jedan nije razvio avaskularnu nekrozu kosti. Mišljenja smo da postoje i drugi čimbenici, kao npr. genski defekti ili neki drugi okolišni čimbenici, koji zajedno s oštećenjem funkcije T-limfocita u SLE mogu pridonijeti pojavi multifokalne avaskularne nekroze i osteomijelitisa kod takvih bolesnika.

Summary: Introduction: Juvenile idopathic arthritis (JIA) is a chronic inflammatory disease with incidence from 2 to 20 per 100, 000 population. It is one of the more frequent chronic illnesses of children and important cause of longterm disability, but may also remit with no or minor sequelae. The aim of our study was to identify risk factors for an unfavorable outcome in children with JIA. Patients and methods: One hundred ninety-eight children with JIA, first admitted to the Department of Paediatric Rheumatology, University Hospital Zagreb, Croatia, between January 1990 and November 1995 were examined after 7 to 12 years (median 8.4) of disease duration. Clinical examination, laboratory variables (ESR, platelets, hemoglobin, ANA, HLA-B27 and radiographs of affeceted joints were obtained. Results: At disease onset, from 198 children with JIA, 104 were female (52, 6%) and 94 male (47, 4%). One hundred twenty six patients (63, 6%) had oligoarticular type (96 (48, 4%) persistent and 30 (15, 2%) extended subtype), 52 patients (26, 3%) had polyarticular type (15 (7, 6%) RF-negative and 37 (18, 7%) RF-positive), and 20 patients (10, 1%) had systemic type (Table 1 and 2).Within a median of 8.4 years of disease duration 31 patients (39, 3%) had oligoarticular type (12 (15, 2%) persistent and 19 (24, 1%) extended subtype), 44 patients (55, 7%) had polyarticular type (15 (18, 9%) RF-negative and 29 (36, 8%) RF-positive), and 4 patients (5.0%) had systemic type (Tables 1, 2, 3 and 4). At follow up, 119 patients (60, 1%) were in remission, 47 (23, 7%) had developed joint erosions, and 32 (16, 2%) had different functional disabilities, which required orthopedic prostheses or in some cases operation. The remission rate was higher in the patients with persistent oligoarticular than in those with extended oligoarticular and RF positive polyarticular JIA (Table 2.). Conclusion: In our study early risk factors for an unfavorable outcome were young age at onset, female sex, symmetric arthritis (polyarthrtitis), early hip joint involvement, early joint erosions, elevated ESR, positive RF and HLA-B27. The presence of uveitis in oligoarticular onset JIA didn’ t show like unfavorable prognostic factor, like was suggested in some studies

The term spondyloarthropathy is used for a group of rheumatic diseases that affect the joints of the axial skeleton as well as peripheral joints and that differ from juvenile idioapthic arthritis in many ways. Juvenile spondyloarthropathies (JSpA) include: juvenile ankylosing spondylitis, juvenile psoriatic arthritis, the arthritis of inflammatory bowel disease, reactive arthritis and undifferentiated forms of spondyloarthropathies which are predominantly present during childhood and adolescence. The cause of JspA is still unknown. The strong association with HLA-B27 suggests that genetically determined mechanisam is central in the pathogenesis. The aim of our study was to determine: 1) the incidence of JSpA in Croatian population in the last 5 years in relation to other rheumatic diseases in children, and 2) the presence of HLA-B27 in children with JSpA. All patients aged from 1 to 18 years and diagnosed during the period 1997-2002 at the Department of Paediatric Rheumatology, University Hospital Zagreb, Croatia, as having JSpA were entered into study, following strict criteria of European Spondyloarthropathy Study Group. Using hospital and outpatient records, we identified 665 newly diagnosed patients with rheumatic diseases, out of which 65 patients were identified as having JSpA (9, 8%) amongst 7, 983 patients with rheumatic disorders examined in the period between 1997 and 2002. Sex ratio was 18 females against 47 males, mean age 13, 5 years. Undifferentiated spondyloarthropathy had 53 patients (81, 5%), 4 patients had Reiter’ s syndrome (6, 2%), 6 had arthritis of inlammatory bowel disease-Mb. Crohn (9, 3%), one patient had psoriatic arthritis (1, 5%) and only one had ankylosing spondilitis (1, 5%). Ocular complications were present in 23 patients (35, 4%), enthesitis in 50 (76, 9%), sacroileitis in 59 (90, 8%) and peripheral arthritis in all 65 patients (100%). HLA-B27 was present in 48 patients (73, 9%). Our epidemilogical study showed similar incidence of JSpA among other rheumatic diseases in childhood and adolescencets (9, 8%) compared to the results of American, Canadian and British studies in which this incidence is 7, 9-9, 8%. However, the presence of HLA-B27 antigene is lower (73, 9%) in relation to other studies (86-100%), which is probably due to a greater number of patients with undifferentiated spondyloarthropathies, who could later in some cases correlate with the evolution of idiopathic SEA syndrome

Chronic recurrent multifocal osteomyelitis (CRMO), first described by Giedion in 1972, is the disease of unknown etiology with clinicaly features similiar to those of septic osteomyelitis. It is characterised by multifocal bone pain, often accompanied by fever. We present two patients who meet the criteria for CRMO. The first patient is a 4-year old girl who was admitted to our hospital after bone biopsy, which showed chronic reactive inflammatory changes in distal tibia. First symptoms appeared four months before hospitalisation with impaired walking, pain in distal part of right tibia and methatarsal bones, followed by swelling. She also had recurrent fever. Bone scintigraphy revealed multifocal increased radionuclide uptake (right mandibule, distal part of right tibia and right methatarsus). Radiographs showed lytic lesions of distal tibia and third methatarsal bone. All bacteriological specimens were negative. She was treated with wide-spectrum antibiotics, but unsuccessfully. After we established the diagnosis of CRMO, the therapy with NSAIDs and azithromycin (because of its anti-inflammatory effect) was introduced. After two months the patient was painless, walked normally and radiographs showed improvement with regression of osteolytic lesions. The second patient is a 3-year old girl who initially was admitted to Department of Paediatric Neurology because she was suspected to have neuromuscular disorder. First symptoms were present two years ago, when she started walking. She had muscle weakness and often cried painfully. In the last several months she refused to walk, her arms and legs were swollen, she avoided manipulation and had periodic fever. Bone scintigraphy and radiographs showed multifocal osteolytic lesions (vertebra, ribbs, clavicles, long bones of both arms and legs). Neurological disfunction appeared probably as the result of changes in the spine. EMNG revealed myopathic pattern in distal muscles, and severe chronic neurogenic lesion in proximal muscles. When the diagnosis of CRMO was established, NSAIDs were introduced in the therapy and certain clinical improvement was noticed, but as the process was so widely spread we decided to give glucocorticoids. After one month of this therapy the child started walking, she had no pains, and radiographs showed improvement of osteolytic lesions, but not complete regression. Conclusion: CRMO is not an infective disease, so antibiotic therapy is unnecessary. The cause is still unknown. Good response to NSAID and steroid therapy suggests that autoimmune process might play the major role in the pathogenesis of CRMO

Pod nazivom juvenilne spondiloartropatije obuhvaćena je skupina bolesti kod kojih su upalom zahvaćeni zglobovi kralježnice, ali i periferni zglobovi, međutim koja se po mnogočemu razlikuje od juvenilnog idiopatskog artritisa. U juvenilne spondiloartropatije ubrajaju se: juvenilni ankilozantni spondilitis, juvenilni psorijatični artritis, enteropatski artritis, reaktivni artritis i nediferencirane spondiloartropatije, koje su najčešći oblik spondiloartropatija u djetinjstvu i adolescenciji. Ova skupina bolesti pokazuje značajnu povezanost s antigenom tkivne histokompatibilnosti HLA-B27, pa prema tome najvjerojatnije postoji genetska predispozicija. Cilj rada bio je odrediti pojavnost juvenilnih spondiloartropatija u našoj populaciji u zadnjih 5 godina u odonosu na druge reumatološke bolesti u djece, te odrediti učestalost antigena HLA-B27 u bolesnika oboljelih od juvenilnih spondiloartropatija U razdoblju od 1997. do 2001. god. od 7, 863 ukupno pregledane djece u Reumatološkoj ambulanti i na Reumatološkom odjelu Klinike za pedijatriju KBC Zagreb, bilo je 652 novootkrivenih reumatoloških bolesnika, od čega je prema kriterijima European Spondyloarthropathy Study Group bilo 62 bolesnika (9, 5%) s juvenilnim spondiloartropatijama (51 bolesnik s nediferenciranom spondiloartropatijom, 4 s Reiterovim sindromom-reaktivnim artritisom, 6 s enteropatskim artritisom i 1 s psorijatičnim artritisom). HLA-B27 antigen prisutan je u 46 tih bolesnika (46/62, 74, 2%). Iz opisanog epidemiološkog istraživanja i usporedbe s rezultatima iz američkih, kanadskih i britanskih epidemioloških studija o učestalosti juvenilnih spondiloartropatija u odnosu na druge reumatološke bolesti u dječjoj dobi možemo zaključiti da je učestalost juvenilnih spondiloartropatija u našoj populacija (9, 5%) približno jednaka onima u drugim zemljama (7, 9-9, 8%). Prisutnost HLA-B27 antigena je manja (74, 2%) u odnosu na studije iz drugih zemalja (približno 90%).

Pod nazivom juvenilne spondiloartropatije obuhvaćena je skupina bolesti kodkojih s upalom zahvaćeni zglobovi kralješnice ali i periferni zglobovi, međutim koje se po mnogočemu razlikuje odjuvenilnog idiopatskog artritisa. U juvenilne spondiloartropatije ubrajaju se: Juvenilni ankilozantni spondilitis, juvenilni psorijatični artritis, enteropatski artritis, reaktivni artritis i nediferencirane spondiloartropatije koje su najčešći oblik spondiloartropatija u djetinjstvu i adolescenciji. Ova skupina bolesti pokazuje značajnu povezanost s antigenom HLA B27 pa prema tomenajvjerojatnije postoji genska predispozicija. Cilj rada bio je odrediti pojavnost juvenilnih spondiloartropatija u našoj populaciji u zadnjih 5 godina u odnosu na druge reumatološkebolesti u djece te odrediti učestalost antigena HLA B 27 u bolesnika oboljelih od juvenilnih spondiloartropatija. U razdoblju od 1997-2001. godine od 7863 ukupno pregledane djece u Reumatološkoj ambulanti i na Reumatološkom odjelu Klinike za pedijatriju KBC Zagreb, bilo je 652 novotkrivenih reumatoloških bolesnika, od čega je prema kriterijima European Spondiloarthropathy Study Group bilo 62 bolesnika(9.5%) s juvenilnim spondiloartropatijama. HLA B27 antigen prisutan je u 46 (74.2%) tih bolesnika. Iz opisanog epidemiološkog istraživanja i usporedbe s rezultatima iz američkih, kanadskih i britanskih epidemioloških studija o učestalosti juvenilnih spondiloartropatija u odnosu na druge reumatološke bolesti u dječjoj dobi možemo zaključiti da je učestalos juvenilnih spondiloartropatija u našoj populaciji (9.5%) približno jednaka onima u drugim zemljama (7.9-9.8%). Prisutnost HLA B27 antigena je manja (74.2%) u odnosu na studijedrugih zemalja (približno 90%)

Kronični rekurentni multifokalni osteomijelitis (CRMO) prvi puta je opisao Giedion 1972. godine kao "subakutni i kronični simetrični osteomijelitis". Etiologija bolesti nije poznata. Većinom oboljevaju djeca i adolescenti, i to dva duta češće djevojčice. Najčešće su zahvaćene duge kosti (proksimalne i distalne metafize tibije i femura). Tijek bolesti sastoji se od lokalnih relapsa ili pojave novih lokusa između kojih su periodi remisije. Dijagnoza CRMO postavlja se ako su zadovoljeni ovi kriteriji: 1. bolest traje najmanje tri mjeseca, 2. nemogućnost izolacije mikroorganizma i 3. dokaz kronične upalne reakcije kosti histološkom analizom bioptata uz isključivanje drugih mogućih bolesti. Prikazujemo djevojčicu u dobi od 4 godine s CRMO distalne polovice desne potkoljenice i III metatarzalne kosti desnog stopala. Prvi simptomi bolesti započeli su 4 mjeseca prije hospitalizacije nepravilnim hodom djevojčice te su ordinirani ortopedski ulošci. Nakon dva mjeseca dolazi do otoka i jake boli dorzuma desnog stopala i distalnog dijela desne potkoljenice. Djevojčica više ne može stati na desnu nogu. Učini se RTG snimka potkoljenice i stopala, te scintigrafija s 99mTc. Na osnovi nalaza posumnja se da se radi o tumorskom procesu te se indicira biopsija. Histološki nalaz govori u prilog kroničnih reaktivnih upalnih promjena bez elemenata tumorskog tkiva. Djevočica se pod dijagnozom kroničnog osteomijelitisa prima na našu Kliniku. Hemokulture i bakteriološki nalaz bioptata bili su negativni. Ponovna RTG snimka i CT pokazuju progresiju ostelitičkih promjena zahvaćenih kostiju. Na osnovi kliničke slike, histološkog nalaza bioptata, negativne hemokulture, rentgenskih nalaza i CT-a postavi se dijagnoza CRMO. Do postavljanja dijagnoze liječena je antibioticima no bez značajnijeg učinka na tijek bolesti. Po postavljanju dijagnoze u terapiju su uvedeni su nesteroidni antiflogistik i azitromicin. Kontrolna RTG snimka kostiju učinjena nakon 3 tjedna liječenja pokazala je znatno poboljšanje. Zaključak: Nove smjernice za liječenje CRMO razlikuju se od prijašnjih, budući da se u ovom obliku osteomijelitisa ne može dokazati infektivni uzročnik bolesti. Zbog toga je napušteno dugotrajno liječenje antibioticima, a prednost se daje nesteroidnim protuupalnim lijekovima, rijetko i kortikosteroidima. Neke studije preporučuju i alternativno davanje azitromicina kroz više mjeseci (ne toliko koliko zbog njegovog antibiotskog, koliko zbog protuupalnog učinka), dok druge studije smatraju takvo liječenje nepotrebnim.

The role of wide-field nailfols capillaroscopy and hand X-rays in the investigation of patients with undifferentiated connective tissue disease.

Naša iskustva s magnetskom rezonancijom kod kroničnog juvenilnog artritisa