Objective
This study was performed to identify a possible association of the clinical parameters of systemic sclerosis (SSc) and the socioeconomic status (SES) with oral health-related quality of ...life (OHrQoL) as measured by the Oral Health Impact Profile 49 (OHIP 49), taking into account the effect of educational level (as a proxy of SES) on oral health.
Methods
Subjects were recruited from the Croatian SSc Center of Excellence cohort. Detailed dental and clinical examinations were performed according to standardized protocols. The associations of OHrQoL with disease characteristics and socioeconomic status were examined.
Results
Thirty-one consecutive patients with SSc were enrolled (29 women; mean age, 56.45 ± 13.60 years). OHIP 49 scores were significantly correlated with disease activity and severity. Furthermore, OHrQoL was positively correlated with skin involvement as evaluated by the modified Rodnan skin score. Impaired OHrQoL was positively correlated with the severity of general, skin, gastrointestinal, and joint/tendon involvement. The OHIP 49 score differed between patients who were positive and negative for anti-topoisomerase I antibody. Higher OHIP 49 scores were detected in patients with lower SES (primary school educational level).
Conclusion
Collaboration between rheumatologists and dental professionals is required to improve dental care and oral health outcomes of SSc.
Summary
Aim
Systemic sclerosis (SSc) is a rare chronic disease characterized by pathologic collagen deposits in the skin and internal organs. Although it is considered to be an autoimmune disease, ...immunosuppressants have a limited effect on severe SSc. Intravenous immunoglobulins (IVIG) have shown favorable effects in patients with SSc by suppressing the action of profibrotic cytokines, so they could have additional effect on standard treatment such as cyclophosphamide (CYC). This article presents the immunomodulatory effect of low-dose IVIG in addition to CYC in the treatment of severe SSc in this center during the last 9 years.
Methods
This retrospective observational study analyzed the medical documentation of nine patients with SSc treated with low-dose IVIG (0.4 g/kg and month) together with intravenous CYC (600 mg/m
2
and month). The therapeutic effect on lung and skin manifestations was assessed.
Results
Of the patients one had interstitial lung diseases (ILD), two had progressive skin diseases, and six had a combination of skin and lung involvement. The best results were achieved in skin changes, where complete healing of digital ulcers (DU) was recorded in every reported case. A decrease in the modified Rodnan skin score (mRSS) was noted in three patients and increased diffusion capacity of the lungs for carbon monoxide in another three patients.
Conclusion
The results of the study suggest that IVIG may be an additional treatment option together with CYC for patients for whom other therapies have failed, but further studies on the exact role of IVIG in the treatment of severe SSc are required.
Systemic sclerosis (SSc) is an autoimmune disease characterized by fibrosis of the skin and internal organs. Although its pathogenesis is not fully understood, connexins (Cxs) and pannexins (Panx) ...could be involved in the process of fibrosis. We analyzed the protein expression of Cx37, Cx40, Cx43, and Panx1 in the gastric mucosa of patients with SSc and healthy volunteers, using immunofluorescence staining. Protein levels of Cx37 were slightly increased, while the levels of Cx40 were significantly decreased in the lamina propria of the gastric mucosa of SSc patients compared to the controls. The changes were proportional to SSc severity, with the most prominent changes found in patients with severe diffuse cutaneous SSc. No differences in Cx43 or Panx1 levels were found between the analyzed groups of samples. The lack of changes in Cx43 expression, which has been previously associated with fibrosis, could be due to the weak expression of Cx43 in the gastric mucosa in general. Further studies on full-thickness gastric biopsies containing muscle layers and animal SSc models are needed to fully elucidate the role of Cxs and Panxs in SSc-associated fibrosis.
Sjögrenov sindrom (SS) je heterogena bolest koja se najčešće prezentira blagim kliničkim tijekom. Međutim, u manjeg dijela bolesnika poprima teški oblik bolesti s brojnim sistemskim manifestacijama i ...mogućim lošim ishodom. Sistemske manifestacije se pojavljuju u približno 25% bolesnika s primarnim Sjögrenovim sindromom (pSS). Klinička prezentacija sistemskih očitovanja SS-a vrlo je raznolika i može zahvatiti bilo koji organski sustav. Mogu nastati uslijed limfocitne infiltracije organa ili proliferacije limfocita B i odlaganja imunih kompleksa. Umor je najčešća sistemska manifestacija. Najznačajnije dermatološke manifestacije bolesti su palpabilna purpura, ulceracije, urtikarijalni i leukocitoklastični vaskulitis. Koštano-mišićne manifestacije u bolesnika s pSS-om pojavljuju se u širokom rasponu od artralgija do erozivnog artritisa. Zahvaćenost pluća može se očitovati kao fična intersticijska pneumonija s fibrozom i traheobronhalna bolest s povećanom reaktivnošću bronha, bronhiektazijama, bronhiolitisom ili ponavljajućim respiratornim infekcijama. Bubrežne promjene se uočavaju u 10–30% bolesnika sa SS-om. Kao posljedica limfocitne infiltracije razvija se tubularni intersticijski nefritis, renalna tubularna acidoza tipa I, nefrogeni dijabetes insipidus i ostali poremećaji tubularne funkcije. Znatno rjeđe upalni proces zahvaća glomerule dovodeći do glomerulonefritisa. Jetreni poremećaji se nalaze u oko 20% bolesnika sa SS-om, a najčešće se očituju upalnim promjenama intrahepatalnih žučnih vodova nalikujući primarnoj bilijarnoj cirozi. Zahvaćenost perifernoga živčanog sustava manifestira se senzomotornom aksonalnom polineuropatijom, senzornom ataksičnom i autonomnom neuropatijom, mononeuritisom multipleks, kranijalnim neuropatijama i radikulopatijama. Od promjena središnjega živčanog sustava opisuju se optička neuropatija, hemipareza, poremećaji pokreta, cerebelarni sindromi, tranzitorne ishemijske atake, rjeđe transverzalni mijelitis i progresivna mijelopatija. Simptomi vaskulitisa mogu varirati od mononeuritisa multipleksa do ishemije crijeva i disfunkcije zahvaćenih organa. Razvoj non-Hodgkinovog limfoma B-stanica predstavlja glavnu komplikaciju bolesti i pojavljuje se u 5–7% bolesnika sa Sjögrenovim sindromom.
The aim of this paper was to investigate the prevalence of smoking using selected anthropometric variables in a sample of hospitalized coronary heart disease (CHD) patients in Croatia (N = 1,298). A ...total of 444 subjects (34.6%) were non-smokers, 548 (42.6%) were smokers and 293 (22.8%) were ex-smokers. Men, on average, smoked more cigarettes per day than women (22.62 vs. 19.84 cigarettes, p < 0.001) and they also had bigger index "pack-years" than women (36.96 vs. 33.91, p = 0.024). Men were more often smokers and ex-smokers than women (47.4% vs. 30.8% for smokers and 25.0% vs. 22.8% for ex-smokers, p < 0.001). In this study a high prevalence of smoking was found among CHD patients in Croatia. Unless it is decreased, it can be expected that CHD patients in Croatia will continue to experience adverse effects more often than other CHD patients in the rest of Europe.
Despite high prevalence of patients with gastric disease in systemic sclerosis (SSc), its pathogenesis is still poorly understood. We immunohistochemically analysed biopsies of gastric mucosa (GM) in ...5 controls and 15 patients with different forms of SSc: limited cutaneous (lc), diffuse cutaneous moderate (sys1) and severe (sys2). The number of positive cells was analysed by a Kruskall-Wallis test, P < 0.05 was considered statistically significant. Percentage of proliferating (Ki-67 positive) cells was highest in sys1 (3% in superficial and 4,6% in deeper parts of GM), which dropped to 1% in sys2. Percentage of α-smooth muscle actin (α-SMA) positive cells was 5% in controls, 9% in superficial GM, while in deeper GM rose from 7% to 19% in sys1 and sys2, thus indicating increased myofibroblast population. Caspase-3 positive apoptotic cells characterized 1,5-2% of controls, 8% of superficial and 6% of deeper GM cells in sys1. In sys2, apoptosis affected 50% of surface epithelial and gland cells and 30% of deeper glands, and correlated with increased fibrosis and decreased syndecan-1 expression. Our data demonstrate that sys1 is the most "active" proliferating form of SSc. Sys2 characterize collagen deposition, surface epithelium defects, extensive apoptosis and low proliferation, GM atrophy and loss of function.
Reumatska polimialgija (PMR) i temporalni arteritis (TA)
česte su upalne reumatske bolesti u osoba starijih od pedeset
godina. Usko su povezane i često se pojavljuju u istog
bolesnika. Uobičajeni ...simptomi su bol i nelagoda te zakočenost
mišića ramenog obruča, vrata i kukova, a praćeni su
povišenim vrijednostima upalnih reaktanata i anemijom
kronične bolesti. Uz navedeno u TA se javlja temporalna
glavobolja, smetnje vida i sluha te bol čeljusti. Dobar i brz
terapijski odgovor na primjenu glukokortikoida ključno je
obilježje ovih dvaju poremećaja. Kod nekih upalnih autoimunih
reumatskih bolesti zabilježena je veća incidencija
malignoma. Premda razlozi te veće pojavnosti nisu jasni,
smatra se da je u podlozi poremećena regulacija imunosnog
sustava. Više prikaza slučaja, serija slučaja i epidemioloških
studija sugerira poveći rizik od malignih bolesti
u bolesnika s PMR i TA, ali su rezultati epidemioloških
studija kontradiktorni. U svrhu istraživanja ove povezanosti
nedavno je provedena nekolicina prospektivnih istraživanja. Povišen rizik malignih oboljenja u bolesnika
s PMR/TA nedvojbeno je utvrđen u prvih 6 – 12 mjeseci
od dijagnoze, a poslije se taj rizik gubi i izjednačava s
rizikom opće populacije. Uočena je veća pojavnost karcinoma
urogenitalnog, limfatičkog, hematopoetskog i živčanog
sustava. Kako se do dijagnoze PMR i TA dolazi „per
exclusionem“, a klinička slika često nije specifična i može
nalikovati paraneoplastičkom sindromu, potrebna je vrlo
pažljiva klinička evaluacija i pridržavanje klasifikacijskih
kriterija. Nužno je razmotriti sve što diferencijalno dijagnostički
dolazi u obzir, uključujući malignitet, posebno
tijekom prve godine kliničkog praćenja. Unatoč tome u
oko 20 % bolesnika s PMR/TA dolazi do naknadne revizije
dijagnoze u neku od upalnih reumatskih ili malignih
bolesti.
U ovom radu prikazali smo bolesnicu s gubitkom vida u sklopu temporalnog arteritisa potvrđenog biopsijom temporalne arterije. Klinički se bolest prezentirala oteklinom parotidnih žlijezda, amaurosis ...fugax i klaudikacijama čeljusti, a potom i glavoboljom. Nakon uvođenja glukokortikoidne terapije simptomi bolesti su regredirali, no zaostao
je gubitak vida na jedno oko. Ovaj slučaj nas upozorava da je važno razmotriti temporalni arteritis kao diferencijalnu dijagnozu u bolesnika starijih od 50 godina s amaurosis fugax i ostalim atipičnim simptomima koji se mogu razviti prije pojave glavobolje. Dobra suradnja između neurologa, otorinolaringologa, infektologa, oftalmologa i reumatologa
ključna je za rano postavljanje dijagnoze i početak odgovarajućeg liječenja koji smanjuje rizik od trajnog gubitka vida.
Uvod. Reumatska polimialgija (lat. polymialghia reumatica – PMR) je kronična upalna reumatska bolest koja se javlja uglavnom kod bolesnika starijih od 50 godina. Najčešće se prezentira bolovima i ...ukočenošću u području ramenog i zdjeličnog obruča te vrata. U pojedinih bolesnika dolazi do razvoja arteritisa divovskih stanica (engl. Giant Cell Arteritis – GCA). Cilj ovoga istraživanja jest prikazati kliničke karakteristike bolesnika kojima je dijagnosticirana PMR u razdoblju od početka 2015. godine do srpnja 2020. godine na Zavodu za reumatologiju i kliničku imunologiju ničkoga bolničkog centra (KBC -a) Split. Materijali i metode. U istraživanje su uključeni bolesnici kojima je dijagnosticirana PMR prema EULAR /ACR klasifikacijskim kriterijima iz 2012. godine. Podatci su prikupljeni pregledom dostupne medicinske dokumentacije. U analizi rezultata korištene su metode deskriptivne statistike. Ukupno je dijagnosticirano 49 bolesnika s PMR-om. Prosječna dob je 77 godina, a prevladavaju osobe ženskog spola (67,35%). Rezultati.
PMR se najčešće manifestirala bolovima u zglobovima (u 91,84% bolesnika) te febrilitetom (u 28,57% bolesnika). U 6 bolesnika (12,24%) je naknadno dijagnosticiran GCA. Najčešći pridruženi komorbiditeti bili su arterijska hipertenzija (73,47%) i šećerna bolest tipa 2 (38,78%). U troje bolesnika (6,12%) maligna bolest je dijagnosticirana u periodu od jednu godinu prije i nakon postavljanja dijagnoze PMR-a. Liječenje glukokortikoidima (GC) provedeno je u svih bolesnika, a u njih 45 (91,84%) postignuta je remisija bolesti. Zaključak. Zaključno, PMR je bolest koja se javlja u starijoj životnoj dobi i uspješno se liječi GC. Najčešća klinička prezentacija u naših bolesnika bila je bol u zglobovima, dok je najčešći komorbiditet bila arterijska hipertenzija. Nije potvrđeno da je PMR paraneoplastična bolest, što se poklapa s podatcima dostupnim iz literature.
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