Sistemske manifestacije Sjögrenovog sindroma

Sjögren’s syndrome (SS) is a heterogeneous disease which, in the majority of cases, includes a mild clinical course. However, in some patients it takes on a severe form with numerous systemic manifestations and results in an adverse outcome. Systemic manifestations occur in approximately 25% of patients with primary Sjögren’s syndrome (pSS). The clinical presentation of systemic manifestations of SS is very diverse and can involve any organ system. Systemic manifestations can occur due to lymphocytic infiltration of organs or proliferation of B lymphocytes and deposition of immune complexes. Fatigue is the most common systemic manifestation. The most significant cutaneous manifestations of the disease are palpable purpura, ulcerations, urticarial vasculitis and leukocytoclastic vasculitis. Musculoskeletal manifestations can range from arthralgias to erosive arthritis. Pulmonary involvement may include symptoms such as nonspecific interstitial pneumonia with fibrosis and tracheobronchial disease. Renal changes are observed in 10% to 30% of patients with SS. Tubulointerstitial nephritis, type 1 renal tubular acidosis and nephrogenic diabetes insipidus can develop as a consequence of lymphocytic infiltration. Less often, the inflammatory process affects the glomeruli which leads to glomerulonephritis. Liver diseases are found in approximately 20% of patients with SS and their symptoms usually include inflammation of intrahepatic bile ducts resembling primary biliary cirrhosis. The manifestations of peripheral nervous system involvement include sensorimotor axonal polyneuropathy, mononeuritis multiplex, neuropathies and radiculopathies. Optic neuropathy, hemiparesis, movement disorders, cerebellar syndromes, transient ischemic attacks, transverse myelitis (less commonly), and progressive myelopathy have been described as central nervous system changes. Symptoms of vasculitis can range from mononeuritis multiplex to intestinal ischemia and dysfunction of the affected organs. The development of non-Hodgkin’s B-cell lymphoma is a major complication of the disease which occurs in 5%–7% of patients with SS.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

VASCULITIDES – INTRODUCTION

Vaskulitis je rijetko upalno zbivanje stijenke krvne žile koje dovodi do potpune ili djelomične okluzije krvne žile te ishemije tkiva ili organa koje ta krvna žile opskrbljuje. Vaskulitisi su složena grupa bolesti uglavnom autoimunosnog podrijetla. Vaskulitični proces može biti primaran ili sekundaran i može zahvatiti jedan ili više organa. Upala zahvaća dio ili cijelu stijenku krvne žile s posljedičnim gubitkom vaskularnog integriteta. Vaskulitisi se klasificiraju na temelju upalnog oštećenja malih, srednjih ili velikih krvnih žila.Vasculitis is clinicopathologic process characterized by inflammation and damage of blood vessels, often resulting in complete or partial occlusion of the involved vessels and ischemic damage to the supplied organ or tissue. The vasculitides are a large group of heterogeneous diseases for which it has been assumed that pathogenesis is largely autoimmune. It may be a primary or secondary manifestation of a disease process and may affect single or multiple organs. Inflammation affects vessel’s walls partly or completely resulting with the loss of vascular integrity. Vasculitides has been classified by whether inflammation predominantly damage small, medium, or large vessels

SYSTEMIC SCLEROSIS AND MALIGNANT DISEASES

Bolesnici sa sistemskom sklerozom (SSc) imaju povećan rizik od malignoma u odnosu na opću populaciju. Najčešće je riječ o karcinomu pluća, hematološkim bolestima, malignomima probavnog sustava, dojke i nemelanomskim karcinomima kože. Trajanje bolesti, starija životna dob u trenutku postavljanja dijagnoze SSc-a, pušenje i konzumiranje alkohola znatno povećavaju rizik od razvoja malignih bolesti. SSc, skleroderma like sindrom i Raynaudov sindrom također se javljaju u sklopu paraneoplastičkog sindroma. Povezanost SSc-a i malignoma objašnjava se jedinstvenom genetskom osjetljivošću za razvoj malignoma i autoimunih bolesti te izloženošću zajedničkim rizičnim okolišnim čimbenicima ili autoprotutijelima.Patients with systemic sclerosis (SSc) are at increased risk of malignancies in comparison with the general population. The most common is lung cancer, followed by hematologic diseases, malignant tumors of the gastrointestinal tract, breast, and non-melanoma skin cancers. Duration of the disease, older age at the moment of diagnosis, smoking, and alcohol consumption significantly increase the risk of malignancy. SSc, scleroderma-like syndrome, and Raynaud phenomenon can develop within paraneoplastic syndrome. The association of SSc and malignancies could be explained by a unique genetic susceptibility for the development of malignancies or autoimmune diseases, or the exposure to certain environmental factors or autoantibodies

THE DIFFERENCES BETWEEN CLINICAL MANIFESTATIONS AND COMORBIDITIES BETWEEN WOMEN AND MEN WITH SLE TREATED IN UNIVERSITY HOSPITAL OF SPLIT FROM JANUARY 2007 TO DECEMBER 2017

Background: SLE is chronic multisystem autoimmune disease with numerous clinical manifestations. Many comorbidities have significant impact on clinical course of SLE. Th ey are partly mediated by the primary disease; some are partly caused by the treatment and some of the comorbidities are the result of genetic susceptibility, independently of the main disease Objectives: The aim of this study was to determine the differences between clinical manifestations and comorbidities in women and men with SLE treated in University Hospital Centre Split from January 2007 to December 2017. Methods: The study included 268 patients with SLE diagnosis from the beginning of 2007 to the end of 2017. The data were collected from outpatient clinics, stationers and daily hospital of the Department of Rheumatology and Clinical Immunology of the Clinic for Internal Diseases of University Hospital Centre Split. During the collection process, the data were included in the Microsoft Office program package, or in Microsoft Excel, a program designed to create a table of budgets. For statistical analysis, SPSS 25 was used. We used χ² test and multivariate logistic regression. Results: Among 268 SLE patients, there were 26 (10%) males and 242 (90%) females. The median age of the patients was 52 years (min-max: 22–88 years; Q1-Q3: 41-62.75 years). We explored the association of individual clinical manifestations and comorbidities with gender with the χ² test. A statistically significant association was obtained for Sjögren’s syndrome and associated neoplasms with female gender, and for antiphospholipid syndrome (APS) and vasculitis with male gender. According the median age we divided our respondents into three groups. In the oldest age group >70 years there were no males, so we excluded patients > 70 years. The χ² test showed statistically significant association between younger age and skin changes and lupus nephritis. In older patients, statistical significant relation was found for dyslipidemia, hypertension, osteoporosis, gastritis and hear involvement. In multivariate logistic regression with the age and gender as independent variables, significantly higher frequency of Sjogren’s syndrome (P = 0.04) and associated neoplasms (P = 0.004) were found in females, while vasculitides (P = 0.014) and APS (P = 0.003) were more frequent in males with SLE. Conclusions: Women with SLE are more frequently affected by Sjögren’s syndrome and associated neoplasms, while men with SLE suffer more frequently of vasculitis and APS. Lupus nephritis and skin changes usually occur in both sexes in younger patients. Dyslipidemia, hypertension, heart failure, osteoporosis and gastritis are more frequent in older patients than in younger patients with SLE