Primarni vaskulitis središnjega živčanog sustava - dijagnostički izazov

Primary angiitis of the central nervous system (PACNS) is a rare and severe disease confined to the central nervous system, i.e., the brain and spinal cord. The etiology, pathogenesis and immune mechanism of PACNS have not yet been completely elucidated. The diagnosis is challenging; it is based upon constellation of clinical picture, cerebrospinal fluid analysis, imaging methods or tissue biopsy as the gold standard. In differential diagnosis of PACNS, it is necessary to rule out infectious, malignant or systemic inflammatory diseases, as well as reversible cerebral vasoconstriction syndrome. Immunosuppressants are cornerstone therapy for PACNS, although evidence-based strategies for the management are lacking so far. PACNS is an entity with considerable morbidity and mortality. Awareness of this rare and heterogeneous disease is crucial for establishing early diagnosis and treatment initiation.Primarni vaskulitis središnjega živčanog sustava (PVSŽS) je rijetka i teška bolest ograničena na središnji živčani sustav, tj. mozak i leđnu moždinu. Etiologija, patogeneza i imuni mehanizam PVSŽS-a još nisu u potpunosti razjašnjeni. Dijagnoza je zahtjevna i postavlja se na temelju kliničke slike, nalaza lumbalne punkcije, slikovnih metoda ili biopsije tkiva kao zlatnog standarda. U diferencijalnoj dijagnozi PVSŽS-a potrebno je isključiti infektivne, maligne ili sistemske upalne bolesti, kao i reverzibilni vazokonstrikcijski sindrom. Imunosupresivi su temelj terapije, iako zasad nema jasnih smjernica i preporuka za liječenje ove bolesti. PVSŽS je entitet sa značajnim pobolom i smrtnošću. Svijest o ovoj rijetkoj bolesti složene kliničke prezentacije ključna je za postavljanje rane dijagnoze i početak liječenja

Rana primjena ciklofosfamida u liječenju novonastale progresivne kožne sistemske skleroze – prikaz BOLESNIKA i pregled literature

Systemic sclerosis (SSc) is a chronic autoimmune disease characterized by fibrosis of the skin and internal organs. Treatment of SSc remains a challenge, as it depends on disease extent, pattern and organ involvement. The use of cyclophosphamide (CYC) is mainly recommended for treatment of SSc interstitial lung disease, however its efficacy on skin involvement in SSc has been demonstrated by several studies. We present a case of a patient with new-onset progressive cutaneous diffuse SSc, who was successfully treated with a three-month course of CYC. Significant improvement of skin fibrosis was recorded, as well as a favourable disease outcome in the five-year follow up period. A brief literature review is also presented, demonstrating that CYC can be an effective and safe first-line treatment option in similar cases.Sistemska skleroza (SSc) je kronična autoimunosna bolest koju karakterizira fibroza kože i unutarnjih organa. Liječenje SSc-a izazov je za liječnika jer izbor terapije ovisi o proširenosti i obrascu bolesti, kao i zahvaćenosti unutarnjih organa. Upotreba ciklofosfamida (CYC) većinom je preporučena za liječenje intersticijske plućne bolesti u SSc-u. Međutim, njegova učinkovitost u liječenju kožnih manifestacija SSc-a dokazana je u više istraživanja. Prikazujemo slučaj bolesnice s novonastalom progresivnom, difuznom kožnom SSc, koja je uspješno liječena upotrebom CYC-a tijekom tri mjeseca. Zabilježeno je značajno poboljšanje kožne fibroze, kao i povoljan ishod bolesti tijekom idućih pet godina praćenja. Prikazan je i kraći pregled literature koji govori u prilog tomu da CYC može biti učinkovita i sigurna prva linija liječenja u sličnim slučajevima

SJÖGREN’S SYNDROME AND LYMPHOPROLIFERATIVE DISEASES

Sjögrenov sindrom (SS) kronična je autoimunosna bolest kod koje limfocitna infiltracija dovodi do oštećenja egzokrinih žlijezda, ponajprije suznih i pljuvačnih, što uzrokuje suhoću očiju i usta. Bolesnici sa SS-om imaju povećan rizik za razvoj limfoproliferativnih bolesti. Ne-Hodgkinov limfom (NHL) najteža je komplikacija i uzročnik je veće smrtnosti u bolesnika sa SS-om. Razvoj iz benigne limfocitne infiltacije do malignog NHL-a dugotrajan je i složen proces. Kronična upala i stimulacija B-limfocita, citokinska aktivacija i onkogeni mehanizmi uz prisutne prediktivne rizične čimbenike mogu dovesti do nastanka limfoma. Najučestaliji tip NHL-a su limfomi marginalne zone vezani za sluznice – MALT (engl. mucosa associated lymphoid tissue), a u većini slučajeva zahvaćene su žlijezde slinovnice.Sjögren’s syndrome (SS) is a chronic systemic autoimmune disease characterized by a lymphocytic infiltration and progressive destruction of exocrine glands, in particular the lacrimal and salivary glands, leading to ocular and oral dryness. Patients with SS have a higer risk for development of lymphoproliferative disorders. Non-Hodgkin’s lymphoma (NHL) is the most serious complication and carries a high risk of mortality in patients with SS. The evolution from benign lymphocytic infiltration to malignant NHL is a multi-step process. The chronic inflammation and excessive stimulation of B cells, cytokine activation, and oncogenic events with the presence of predictive risks factors may lead to the development of lymphoma. The most frequent type of NHL is marginal zone lymphoma of the mucosa-associated lymphoid tissue (MALT), affecting the salivary glands in the majority of cases

Pathogenesis of polymyositis and dermatomyositis

Idiopatske upalne miopatije, koje uobičajeno nazivamo miozitisi, heterogena su skupina bolesti od koji su polimiozitis i dermatomiozitis najpoznatije. Ova heterogena skupina kroničnih poremećaja dijeli zajedničko kliničko obilježje slabost proksimalnih mišića i, u tipičnim slučajevima, histološki nalaz nakupina upalnih stanica u mišićnom tkivu. Četiri su glavna tipa idiopatskih upalnih miopatija: dermatomiozitis, polimiozitis, miozitis inkluzijskih tjelešaca i imunosno-posredovane nekrotizirajuće miopatije (autoimune nekrotizirajuće miopatije). Kliničke i histopatološke različitosti među njima upućuju na različite patogenetske procese.The idiopathic inflammatory myopathies, collectively called myositis, are a heterogeneous group of diseases of which polymyositis and dermatomyositis are the best known. These heterogeneous group of chronic disordes sharing the clinical symptom of muscle weakness and, in typical cases, inflammatory cell infiltrates in muscle tissue. There are four major types of idiopathic inflammatory myopathies: dermatomyositis, polymyositis, inclusion body myositis and immune-mediated necrotizing myopathies (autoimmune necrotizing myopathies). Clinical and histopatological distinctions between these conditions suggest that different pathogenic processes underline each of the inflammatory myopathies

Very Early Diagnosis of Systemic Sclerosis in Clinical Practice – Case Report and Review of the Literature

Systemic sclerosis (SSc) is a systemic autoimmune disease charac- terised by generalized microangiopathy and fibrosis of skin and internal organs. The 2013 American College of Rheumatology (ACR) / European League Against Rheumatism (EULAR) criteria have contributed considerably to classifying pa- tients with SSc in earlier stages, but they still lack sensitivity for a very early stage of the disease. Criteria for a very early diagnosis of SSc (VEDOSS) have been pro- posed by EULAR Scleroderma Trial and Research group (EUSTAR) which include three red flags: Raynaud’s phenomenon, puffy fingers and antinuclear antibody positivity, plus SSc specific antibodies positivity and/or abnormal nailfold capil- laroscopy. We report a case of a 54-year-old female patient with 6-week history of puffy fingers, Raynaud phenomenon and positive antinuclear antibodies. Further workup revealed early pathologic capillary pattern by nailfold capillaroscopy and positive anticentromere antibodies. Screening for internal organ involve- ment detected no heart, lung, or upper gastrointestinal tract involvement. The patient was started on pentoxifylline with further follow-up. The aim of the implementation of VEDOSS criteria is to diagnose SSc at the earli- est possible stage, so that subclinical internal organ involvement could be de- tected and appropriate treatment started at a potentially reversible stage

Very Early Diagnosis of Systemic Sclerosis in Clinical Practice – Case Report and Review of the Literature

Systemic sclerosis (SSc) is a systemic autoimmune disease charac- terised by generalized microangiopathy and fibrosis of skin and internal organs. The 2013 American College of Rheumatology (ACR) / European League Against Rheumatism (EULAR) criteria have contributed considerably to classifying pa- tients with SSc in earlier stages, but they still lack sensitivity for a very early stage of the disease. Criteria for a very early diagnosis of SSc (VEDOSS) have been pro- posed by EULAR Scleroderma Trial and Research group (EUSTAR) which include three red flags: Raynaud’s phenomenon, puffy fingers and antinuclear antibody positivity, plus SSc specific antibodies positivity and/or abnormal nailfold capil- laroscopy. We report a case of a 54-year-old female patient with 6-week history of puffy fingers, Raynaud phenomenon and positive antinuclear antibodies. Further workup revealed early pathologic capillary pattern by nailfold capillaroscopy and positive anticentromere antibodies. Screening for internal organ involve- ment detected no heart, lung, or upper gastrointestinal tract involvement. The patient was started on pentoxifylline with further follow-up. The aim of the implementation of VEDOSS criteria is to diagnose SSc at the earli- est possible stage, so that subclinical internal organ involvement could be de- tected and appropriate treatment started at a potentially reversible stage

Sistemska skleroza kod starijeg bolesnika – prikaz bolesnika i pregled literature

Systemic sclerosis most commonly occurs in middle-aged patients. Younger or older people are rarely affected. It is very rare in people over the age of 80. In older patients, different clinical and laboratory characteristics of the disease, as well as a different therapeutic response are expected, in comparison to younger patients. Therefore, we present the case of an 84-year-old patient with systemic sclerosis, as well as a literature review in relation to this topic.Sistemska skleroza se najčešće javlja u bolesnika srednje životne dobi. Rjeđe obolijevaju mlađe ili starije osobe, a vrlo rijetko se javlja u osoba starijih od 80 godina. U starijih bolesnika možemo očekivati drugačija klinička i laboratorijska obilježja bolesti kao i terapijski odgovor u odnosu na mlađe bolesnike. Stoga, u ovom radu prikazujemo 84-godišnjeg bolesnika sa sistemskom sklerozom, uz pregled literature o ovoj temi

Simulation Model of Management of Shipping Company

Sustav dinamičkoga simulacijskog modeliranja jedan je od najprimjerenijih i najuspješnijih znanstvenih načina modeliranja dinamike složenih, nelinearnih, prirodnih, tehničkih i organizacijskih sustava. Takvi modeli u biti su kontinuirani modeli jer se realiteti predstavljaju skupom nelinearnih diferencijalnih jednadžba, tj. jednadžba stanja. Međutim, oni su istodobno i diskretni jer im se osnovni vremenski korak računanja, tj. diskretizacije (uzorkovanja) "DT" određuje u potpunom skladu s teoremom o uzorkovanju (Sampling Theorem) Shannona, Nyquista i Koteljnikova. Ovakav odabir osnovnoga vremenskog koraka DT omogućuje da se kompjutorski modeliraju kontinuirani simulacijski modeli na digitalnom računalu, što je veoma primjereno za edukaciju studenata pomorstva, strojarstva i elektrotehnike, te za teoretsko i praktično trenažiranje inženjera brodskih procesa jer im se primjenom sustavnoga dinamičkog pristupa omogućuje da stječu nova znanja i vještine o složenoj dinamici ponašanja pomorskih sustava i procesa. Brodarsko poduzeće u sustavu dinamičkoga modela čini s okolinom jednu cjelinu pa se ne može promatrati zasebno bez međusobnog utjecaja. Ono je u ovom modelu podijeljeno na nekoliko zasebnih jedinica a to su: komercijala, novac na žiro-računu, brodski kapaciteti, dugovanje, potraživanje, kreditni sektor, roba za prijevoz brodom. U ovom radu izrađeni su dinamički simulacijski modeli podsustava novca na žiro-računu i podsustava brodskih kapaciteta, uz simuliranu dinamiku ponašanja cijele brodarske organizacije.Systematic dynamic simulation modelling is one of the most adequate and successful scientific ways of modelling dynamics of complex non-linear natural, technical and organizational systems. Systematic dynamic models are in fact continual models because the subject has been shown by a set of non-linear differential equations i.e. equations of state. However they are at the same time discrete because the basic time unit of calculation i.e. discretization DC has been determined completely in accordance with Sampling Theorem by Shannon, Nyquista and Koteljnikov. Such a selection of basic time unit (DC) enables a computer modelling of continual simulation models on digital computer which is very adequate for education of the students of marine studies, marine engineering and marine electrical engineering, as well as theoretic and practical training of marine engineers because it enables them, by the application of dynamic process, acquisition of new knowledge and skills about complex dynamics of the behaviour of marine systems and processes. Shipping company is a constitutional part of a dynamic model and its surrounding and it cannot be studied independently without interrelations. Shipping company according to this model has been divided into several separated units, Commercial Department, deposits on the account, shipping capacities, debts, demands, loans, goods for transportation. This paper has analysed system of dynamic model of subsystem of the money on the account, and subsystems of shipping capacity. The dynamics of the behaviour of all shipping company has been simulated