Nasljedni (hereditarni) angioedem je bolest karakterizirana periodičnim, nepredvidivim oticanjima mekih tkiva različitih lokalizacija i intenziteta s najčešće samoograničavajućim tijekom trajanja, te ...je u pravilu posredovan bradikininom. Bolest može uzrokovati blagi, estetski ili funkcionalni poremećaj, ali može dovesti i do smrtnog ishoda u slučaju zahvaćanja gornjih dišnih putova i gušenja. Postoje stanja koja oponašaju kliničku sliku hereditarnog angioedema, a mogu biti posredovana različitim molekulama (bradikininom, histaminom i sl.), odnosno uzrokovana drugim mehanizmima nastanka. Cilj je ovog članka na temelju ilustrativnih primjera upozoriti na najčešće oponašatelje kliničke slike hereditarnog angioedema s kojima se susreću liječnici različitih specijalnosti, posebice u radu u hitnoj službi.
Psoriatic arthritis is an inflammatory arthritis with heterogeneous disease presentation. The most affected clinical domain of the disease determines the therapeutic approach. We report the case of a ...34-year-old man with all six crucial domains of psoriatic arthritis (psoriasis, peripheral arthritis, axial skeletal manifestations, dactylitis, nail changes, and enthesitis) treated unsuccessfully with conventional synthetic DMARDs, NSAID’s, and steroids as well as topical treatment and phototherapy. With golimumab as the first line of bDMARD partial remission was achieved. After 24 months the treatment was switched to secukinumab due to secondary inefficacy. The psoriasis and psoriatic arthritis relapsed after 21 months of treatment with secukinumab. The patient was cycled to ixekizumab with an excellent result. IL-17A inhibitor cycling may be a successful treatment option in some difficult to treat psoriatic arthritis patients.
The involvement of ankles in systemic lupus erythematosus (SLE) has not been widely studied. The aim of our prospective study was to determine the characteristics of the ankle joint and tendon ...involvement in SLE using ultrasound (US) as an imaging modality. Sixty consecutive patients with SLE underwent a detailed clinical evaluation and US examination. Gray-scale and power Doppler US of the bilateral tibiotalar (TT) joints, subtalar (ST) joints, and ankle tendons were performed using a multiplanar scanning technique. Joint effusion, synovitis, tenosynovitis, enthesitis, and vascularization were assessed according to the OMERACT recommendations. The Total Ankle Ultrasound Score (TAUSS) was calculated as the sum of the grades of joint effusion and synovial hypertrophy for both TT and ST joints bilaterally (ranging from 0-24) and power Doppler activity was assessed separately. Finally, US findings were correlated with physical evaluation, laboratory parameters, and SLE activity scores. US ankle joint involvement was present in 32/60 (53.3%) patients. TT joints were affected in 26 (43.3%) and ST joints in 16 (26.7%) patients. Thirteen (21.7%) patients had US tendons and/or enthesal involvement. TT joint effusion was the most frequent finding, present in 55/240 (22.9%) examined joints, followed by synovial hypertrophy detected in 18/240 (7.5%) joints. The median (interquartile range; range) TAUSS of the US-affected joints was 1 (0-2; range 1-10). There were no significant correlations between US findings and inflammatory parameters or serological parameters of disease activity, but we found a weak positive correlation between TAUSS and the European Consensus Lupus Activity Measurement (r = 0.281, P = .029). This study revealed a high prevalence of pathological US ankle changes in patients with SLE and a positive correlation between ankle US involvement and disease activity score (European Consensus Lupus Activity Measurement).
Svrha ovog prikaza slučaja bila je opisati vrlo rijetku oralnu leziju kao posljedicu primarne plućne tuberkuloze. Prikaz slučaja: Opisan je bolesnik s refraktornom bezbolnom ulceracijom ventralne ...površine jezika. Pacijentu je uzeta detaljna medicinska anamneza te obavljen klinički pregled sluznice usne šupljine i palpacija regionalnih limfnih čvorova. Kliničkim pregledom ustanovljena je ulceracija na ventrolateralnoj površini jezika promjera oko dva centimetra. Palpacijom regionalnih limfnih čvorova nije otkriveno njihovo povećanje. Toluidinski test sumnjive lezije učinjen je tijekom svakoga kontrolnog pregleda. Biopsijski uzorci za histopatološku analizu uzeti su tri puta. Histopatološka analiza prvoga biopsijskog uzorka pokazala je nespecifičnu upalu, druga kazeoznu nekrozu bez pozitivnog bojenja prema Ziehl-Neelsenu, a treća granulomatoznu upalu visoko sumnjivu na sarkoidozu. Pacijent je tijekom hospitalizacije obavio cjelovit fizikalni pregled, te laboratorijsku i radiološku dijagnostiku. Fizikalni pregled prsnog koša pokazao je bilateralno prisutne grube krepitacije, a laboratorijski nalazi krvne slike normocitnu anemiju kronične bolesti. Na rendgenskoj snimci pluća uočeni su mali multipli čvorovi bilateralno, a nalaz direktnog sputuma bio je pozitivan. Zaključak: Iako je oralna tuberkuloza rijetko stanje, mora se uzeti u obzir u diferencijalnoj dijagnozi refraktornih bezbolnih oralnih ulkusa.
The aim of this case report was to discuss an extremely rare oral lesion as a result of primary pulmonary tuberculosis.
In this case report, the patient with refractory painless ulceration at ventral ...surface of the tongue was described. Detailed medical history was taken followed by clinical examination of the oral mucosa and palpation of regional lymph nodes. Clinical examination revealed ulceration on the patient's ventro-lateral surface of the tongue, approximately two centimeters in diameter. Palpation of regional lymph nodes has not revealed enlargement. The toluidine blue test of the suspected lesion was performed at each control examination. Biopsy samples for histopathologic diagnosis were taken three times. The analysis of the first biopsy sample for histopathology revealed a non-specific inflammation, the second biopsy revealed a caseous necrosis without positive Ziehl-Neelsen staining and the third biopsy revealed a granulomatous inflammation which was highly suspicious of sarcoidosis. During hospitalization, the patient underwent a complete physical examination, and laboratory and radiological diagnostics. Physical chest examination revealed bilaterally coarse crepitations and laboratory findings of his complete blood count revealed normocytic anemia of chronic disease. Radiographic examination of lungs showed multiple small nodules bilaterally and positive direct sputum smear.
Although oral tuberculosis is a rare condition, it must be taken into account in differential diagnosis of refractory painless oral ulcers.
Sistemski eritemski lupus (SLE) je multisistemska autoimuna bolest sa raznim kliničkim simptomima. Gigantocelularni arteritis (GCA) je bolest kod koje dolazi do kronične upale krvnih žila, često u ...temporalnoj regiji. Prikazujemo slučaj starije pacijentice kojoj je dijagnosticiran SLE, ali zbog GCA ima glavobolje.
Background: Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with musculoskeletal involvement as one of the most common and earliest clinical manifestations which occur in 95% of ...patients. High-resolution ultrasound (US) already proved to be a useful diagnostic tool for the evaluation of pathological changes of the joints and tendons in the majority of inflammatory rheumatic diseases. There are no studies that evaluate the frequency of involvement of ankle joints in adult patients with SLE.
Objectives: Th e aim of this study is to asses the frequency of ankle joints and tendons involvement in SLE patients using US and correlate the findings with physical examination, laboratory tests, and disease activity scores. Here we will show preliminary results of the survey in the first 10 out of 60 included patients. Methods: Ten consecutive SLE patients were enrolled in the study and underwent clinical evaluation, laboratory tests and bilateral high-resolution US on the same day. Gray scale and power Doppler (PD) US were performed for imaging the talocrural (TC), subtalar joints (ST) and ankle tendons, then second and third MCP joints, second and third PIP joints, wrists and second and third MTP joints. Ankle inflammatory US score and global inflammatory US score were calculated.
Results: Preliminary results in 10 patients show the US detected inflammatory joint abnormalities in 7/10 (70%) patients and tendon involvement in 1/10 (10%). A total of 180 joints and 200 tendons were examined. Both of MTP and TC joints were affected in 60% patients, MCP joints in 50%, ST in 40%, wrists in 30% and PIP joints in 10% of patients. Th e most prevalent pathological US finding was joint effusion, less frequently synovial hypertrophy while positive PD signal was rarely presented. Only one patient had bony erosion detected. Effusion in TC joints was present in 60% patients, synovial hypertrophy in 40% and positive PD in 10%. As many as 62,5% of patients without inflammatory joint symptoms had pathological US findings in ankle joints. Th e global US inflammatory score had a mean value of 5,6, and ankle US inflammatory mean value score 2,9. Conclusions: Results of the preliminary study show a high prevalence of US verified inflammatory joint changes in SLE patients. Surprisingly, the foot and ankle joints were most commonly affected and a great number of asymptomatic patients had pathological US findings in ankle joints. 1. Iagnocco A, Ceccarelli F, Rizzo C, Truglia S, Massaro L, Spinelli FR, et al. Ultrasound evaluation of hand, wrist and foot joint synovitis in systemic lupus erythematosus. Rheumatology. 2014;53(3):465–72.
2. Delle Sedie A, Riente L, Scire CA, Iagnocco A, Filippucci E, Meenagh G, et al. Ultrasound imaging for the rheumatologist XXIV. Sonographic evaluation of wrist and hand joint and tendon involvement in systemic lupus erythematosus. Clin Exp Rheumatol. 2009;27(6):897–901.
3. Iagnocco A, Epis O, Delle Sedie A, Meenagh G, Filippucci E, Riente L, et al. Ultrasound imaging for the rheumatologist. XVII. Role of colour Doppler and power Doppler. Clin Exp Rheumatol. 2008 Oct;26(5):759 62.
4. Porta F, Radunovic G, Vlad V, Micu MC, Nestorova R, Petranova T, et al. Th e role of Doppler ultrasound in rheumatic diseases. Rheumatology. 2012;51(6):976–82.
Background: Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with musculoskeletal involvement as one of the most common and earliest clinical manifestations which occur in 95% of ...patients. High-resolution ultrasound (US) already proved to be a useful diagnostic tool for the evaluation of pathological changes of the joints and tendons in the majority of inflammatory rheumatic diseases. There are no studies that evaluate the frequency of involvement of ankle joints in adult patients with SLE.
Objectives: The aim of this study is to asses the frequency of ankle joints and tendons involvement in SLE patients using US and correlate the findings with physical examination, laboratory tests, and disease activity scores. Here we will show preliminary results of the survey in the first 10 out of 60 included patients. Methods: Ten consecutive SLE patients were enrolled in the study and underwent clinical evaluation, laboratory tests and bilateral high-resolution US on the same day. Gray-scale and power Doppler (PD) US were performed for imaging the talocrural (TC), subtalar joints (ST) and ankle tendons, then second and third MCP joints, second and third PIP joints, wrists and second and third MTP joints. Ankle inflammatory US score and global inflammatory US score were calculated.
Results: Preliminary results in 10 patients show the US detected inflammatory joint abnormalities in 7/10 (70%) patients and tendon involvement in 1/10 (10%). A total of 180 joints and 200 tendons were examined. Both of MTP and TC joints were affected in 60% patients, MCP joints in 50%, ST in 40%, wrists in 30% and PIP joints in 10% of patients. Th e most prevalent pathological US finding was joint effusion, less frequently synovial hypertrophy while positive PD signal was rarely presented. Only one patient had bony erosion detected. Effusion in TC joints was present in 60% patients, synovial hypertrophy in 40% and positive PD in 10%. As many as 62,5% of patients without inflammatory joint symptoms had pathological US findings in ankle joints. Th e global US inflammatory score had a mean value of 5,6, and ankle US inflammatory mean value score 2,9. Conclusions: Results of the preliminary study show a high prevalence of US verified inflammatory joint changes in SLE patients. Surprisingly, the foot and ankle joints were most commonly affected and a great number of asymptomatic patients had pathological US findings in ankle joints. References:
1. Iagnocco A, Ceccarelli F, Rizzo C, Truglia S, Massaro L, Spinelli FR, et al. Ultrasound evaluation of hand, wrist and foot joint synovitis
in systemic lupus erythematosus. Rheumatology. 2014;53(3):465–72.
2. Delle Sedie A, Riente L, Scire CA, Iagnocco A, Filippucci E, Meenagh G, et al. Ultrasound imaging for the rheumatologist XXIV.
Sonographic evaluation of wrist and hand joint and tendon involvement in systemic lupus erythematosus. Clin Exp Rheumatol.
2009;27(6):897–901.
3. Iagnocco A, Epis O, Delle Sedie A, Meenagh G, Filippucci E, Riente L, et al. Ultrasound imaging for the rheumatologist. XVII. Role of
colour Doppler and power Doppler. Clin Exp Rheumatol. 2008 Oct;26(5):759–62.
4. Porta F, Radunovic G, Vlad V, Micu MC, Nestorova R, Petranova T, et al. Th e role of Doppler ultrasound in rheumatic diseases. Rheumatology.
2012;51(6):976–82.
Sustavni eritemski lupus (SLE) kronična je autoimunosna bolest s afekcijom muskuloskeletnog sustava kao jednom od najčešćih manifestacija. Ultrazvuk visoke rezolucije (UZ) dokazao se kao korisno ...dijagnostičko sredstvo pri evaluaciji zglobnih i tetivnih promjena u većini upalnih reumatskih bolesti. Cilj je ove studije odrediti učestalost zahvaćanja zglobova i tetiva gležnja u bolesnika sa SLE-om koristeći se
UZ-om te korelirati rezultate s fizikalnim pregledom, laboratorijskim nalazima i mjerama aktivnosti bolesti. Prikazali smo preliminarne rezultate studije na prvih 10 od ukupno 60 bolesnika. Uključeno je 10 uzastopnih pacijenata sa SLE-om koji su istog dana podvrgnuti kliničkoj evaluaciji, laboratorijskom ispitivanju i ultrazvučnom pregledu. Ultrazvučno su obostrano pregledani talokruralni (TC) i suptalarni (ST) zglobovi, tetive gležnja, drugi i treći metakarpofalangealni (MCP) zglobovi, drugi i treći proksimalni interfalangealni (PIP) zglobovi, drugi i treći metatarzofalangealni (MTP) zglobovi te ručni zglobovi. Ukupno je pregledano 180 zglobova i 200 tetiva. Preliminarni rezultati pokazuju ultrazvučno detektirane upalne zglobne promjene u 7/10 (70%) bolesnika i zahvaćenost tetiva u 1/10 (10%). MTP i TC zglobovi bili su zahvaćeni u 60% bolesnika, MCP zglobovi u njih 50%, ST u 40%, ručni zglobovi u 30%, dok su PIP zglobovi bili zahvaćeni u 10% bolesnika. Najčešći patološki ultrazvučni nalaz bio je zglobni izljev, nešto rjeđe sinovijalna hipertrofija, dok je pozitivan PD signal rijetko bio prisutan. Izljev u TC zglobovima detektiran je u 60% bolesnika, sinovijalna hipertrofija u njih 40%, a pozitivan PD u 10% bolesnika. Čak 62,5% asimptomatskih bolesnika imalo je patološki nalaz na UZ-u gležnjeva.Rezultati pokazuju veliku prevalenciju ultrazvučno verificiranih upalnih promjena zglobova u pacijenata sa SLE-om. Iznenađuje da su najčešće bili zahvaćeni zglobovi stopala i gležnja. Također, bitno je naglasiti da je velik broj asimptomatskih bolesnika imao patološki ultrazvučni nalaz zglobova gležnja.
Svrha ovog prikaza slucaja bila je opisati vrlo rijetku oralnu leziju kao posljedicu primarne plucne tuberkuloze. Prikaz slucaja: Opisan je bolesnik s refraktornom bezbolnom ulceracijom ventralne ...povrine jezika. Pacijentu je uzeta detaljna medicinska anamneza te obavljen klinicki pregled sluznice usne upljine i palpacija regionalnih limfnih cvorova. Klinickim pregledom ustanovljena je ulceracija na ventrolateralnoj povrini jezika promjera oko dva centimetra. Palpacijom regionalnih limfnih cvorova nije otkriveno njihovo povecanje. Toluidinski test sumnjive lezije ucinjen je tijekom svakoga kontrolnog pregleda. Biopsijski uzorci za histopatoloku analizu uzeti su tri puta. Histopatoloka analiza prvoga biopsijskog uzorka pokazala je nespecificnu upalu, druga kazeoznu nekrozu bez pozitivnog bojenja prema Ziehl-Neelsenu, a treca granulomatoznu upalu visoko sumnjivu na sarkoidozu. Pacijent je tijekom hospitalizacije obavio cjelovit fizikalni pregled, te laboratorijsku i radioloku dijagnostiku. Fizikalni pregled prsnog koa pokazao je bilateralno prisutne grube krepitacije, a laboratorijski nalazi krvne slike normocitnu anemiju kronicne bolesti. Na rendgenskoj snimci pluca uoceni su mali multipli cvorovi bilateralno, a nalaz direktnog sputuma bio je pozitivan. Zakljucak: Iako je oralna tuberkuloza rijetko stanje, mora se uzeti u obzir u diferencijalnoj dijagnozi refraktornih bezbolnih oralnih ulkusa. Zaprimljen: 2. svibnja 2017. Prihvacen: 2. studenoga 2017. Adresa za dopisivanje doc. dr. sc. Dragana Gabric Sveucilite u Zagrebu Stomatoloki fakultet Zavod za oralnu kirurgiju, Klinicki bolnicki centar Zagreb HR-10 000 Zagreb, Gunduliceva 5 tel:+38514802138 faks: +38514802159 dgabric@sfzg.hr Kljucne rijeci oralna tuberkuloza; jezik; oralni ulkus; dijagnosticke pogreke, tuberkuloza pluca