BACKGROUND:Systemic lupus erythematosus (SLE) is a multifactorial autoimmune disease characterized by chronic inflammation and dysregulated interferon (IFN) signaling. Many patients remain refractory to existing treatments, underscoring the need for novel therapeutic approaches achievable through drug repurposing. Fluvoxamine, an antidepressant with anti-inflammatory and immunomodulatory properties, has not been systematically studied in SLE. METHODS:Differentially expressed genes (|Z| ≥ 2) were analyzed using Gene Ontology and Kyoto Encyclopedia of Genes and Genomes (KEGG) pathway enrichment. Gene Set Enrichment Analysis (GSEA) was applied to assess pathway-level modulation of six hallmark SLE-related signatures. RESULTS:Fluvoxamine induced cell line-specific transcriptomic changes. Jurkat and THP-1 cells showed strong enrichment of TNF-α/NF-κB signaling and regulation of apoptosis-related genes, while HEK293 and A549 cells displayed modulation of cytokine and fibrotic pathways, including NOS3 upregulation. U2OS cells exhibited prominent apoptotic signatures. Although hallmark interferon-α/γ responses were modestly altered at the pathway level, canonical interferon-stimulated genes (e.g. IFI44L, ISG15, MX1) were not significantly downregulated in immune-derived lines. Overall, TNF-α/NF-κB activation and apoptosis emerged as the most consistently affected pathways across models. CONCLUSION:This integrative transcriptomic study supports fluvoxamine as a candidate immunomodulator with potential relevance for SLE, particularly through modulation of NF-κB and apoptotic pathways. These findings provide a mechanistic rationale for further investigation of sigma-1 receptor-targeting agents in autoimmune diseases.

Behçet’s disease (BD) is a complex, relapsing multisystem disorder characterized by autoinflammatory features. Because disease-specific diagnostic biomarkers are lacking and clinical presentation varies according to geographic background and organ involvement, diagnosis may be delayed, particularly in non-endemic regions and in patients with atypical manifestations. Therapeutic management is highly individualized and guided by disease severity and dominant organ involvement. Severe, refractory, or organ-threatening disease often requires aggressive immunosuppressive treatment, including targeted biologic therapy with monoclonal anti-tumor necrosis factor (anti TNF) alpha agents such as infliximab and adalimumab. Therapeutic drug monitoring (TDM), based on serum trough drug concentrations and anti-drug antibodies, is routinely used to optimize anti-TNF therapy in inflammatory bowel disease and is increasingly discussed in other immune-mediated inflammatory diseases treated with monoclonal antibodies. In Behçet’s disease, however, TDM is not yet standard practice, and Behçet-specific therapeutic trough targets for infliximab and adalimumab have not been validated. Nevertheless, integrating TDM with clinical assessment may support more individualized decision-making by helping distinguish insufficient drug exposure, immunogenicity, and pharmacodynamic failure. In this article, we discuss the rationale for TDM in anti-TNF-treated BD, summarize the current direct and indirect evidence, and propose a cautious, clinically guided approach. Behçet’s disease; infliximab; adalimumab; anti-TNF therapy; therapeutic drug monitoring; trough levels; anti-drug antibodies; treatment optimization

The SLE Risk Probability Index (SLERPI) was developed as diagnostic aid in early disease. Previous studies applied it as a classification tool in established SLE, even when analyzing its performance in early disease. We assessed the performance of SLERPI as a classification tool but also at initial assessment, in antinuclear antibody positive patients. In a cross-sectional setting, we enrolled 104 consecutive SLE patients and 104 ANA-positive controls with inflammatory rheumatic conditions. The performance of SLERPI and the EULAR/ACR-2019 criteria was analyzed cumulatively and at initial assessment. Different SLERPI positivity thresholds were evaluated. The cumulative SLERPI score was higher in the SLE group (14.5; IQR 12.0-18.6) compared to controls (7.0; IQR 5.0-8.3). It exhibited a sensitivity of 98.1%, slightly higher compared to EULAR/ACR-2019 (97.1%), and a low specificity (54.8%), lower compared to EULAR/ACR-2019 (56.7%). At initial assessment, SLERPI was higher among SLE patients (median 10.0; IQR 8.5-12.6) compared to controls (median 5.0, IQR 4.5-7.0). Its sensitivity was 83.7% and specificity 76.0%, both higher compared to the EULAR/ACR-2019 (80.8 and 75.0%). Areas under the curve (AUC) for both criteria were 0.898 initially. Cumulatively, the AUC of SLERPI was 0.961, and the AUC of EULAR/ACR-2019 0.956. SLERPI misclassified 20.2% (cumulatively) and 5.8% (initially) of controls as "definite SLE". Higher thresholds of SLERPI (8 initially, 8.5 cumulatively) were associated with the highest Youden index. SLERPI retained a high sensitivity for early recognition and classification purposes, even in ANA-positive patients, with higher cut-off values of its positivity performing somewhat better.

Systemic autoimmune diseases can adversely affect the health of childbearing women and pregnancy outcomes. Additionally, some of theadministered medications are teratogenic, prompting international rheumatological societies to recommend effective contraception and preconception counselling. Given the limited structured education in this field, this study aimed to assess reproductive health awareness and perceptions of family planning among women with systemic autoimmune diseases. A survey-based study was conducted at the Division of Clinical Immunology and Rheumatology, University Hospital Centre Zagreb, Zagreb, Croatia. The sample included 165 women aged 18–50 years who completed the ReproKnow questionnaire assessing reproductive health knowledge, while sociodemographic and clinical data were obtained through interviews and medical records. The median ReproKnow score was 5/10 correct answers indicating moderate knowledge of the studied topic. Greater reproductive health awareness was observed among patients with higher education levels and those diagnosed with SLE or vasculitis. Although one-third of participants stated that their diagnosis affected family planning, only one-fifth reported consistent contraception use, even among those receiving teratogenic medications. Knowledge of contraception efficacy was low (25

Behçet’s disease (BD) is a systemic vasculitis of unknown origin affecting both arterial and venous vessels, resulting in diverse clinical features. Its manifestations vary due to ethnic, geographic, and individual differences. Although the highest prevalence of BD is reported along the ancient Silk Road route, nowadays, due to population migrations, the disease can be detected worldwide. Our study aimed to evaluate the demographic and clinical characteristics of patients with BD in a cohort from a non-endemic country and compare it with other cohorts from endemic and non-endemic countries worldwide. Our retrospective observational case-series study included the data from a single Rheumatology centre. We analyzed the data from 38 patients (17 men and 21 women) with a mean age at diagnosis of 29 years ± SD 8.87 and with a mean follow-up of 12.7 years. The most common manifestations were oral (97.4

Patients with immune-mediated inflammatory diseases (IMIDs) are at increased risk for vaccine-preventable infections (VPIs), yet vaccination uptake remains suboptimal in European countries. This is the first study that assessed vaccination rates, adverse events, and attitudes toward COVID-19, influenza, and S. pneumoniae vaccines among IMID patients in Croatia. We conducted a cross-sectional survey (May–October 2023) using an adapted and translated version of the COVID-Vaccination Attitude Scale (C-VAS) incorporating Health Belief Model (HBM) constructs. Adult IMID patients under rheumatology follow-up for ≥3 years at a tertiary centre were invited to participate. Data were verified against medical records. Descriptive statistics, Chi-square tests, and Mann–Whitney U tests compared vaccination status with sociodemographic variables and HBM constructs. 71 patients (median age 55 years; 73

Complement deficiency disorders, characterized by autoimmunity and recurrent bacterial infections, are diagnosed through complement pathway testing and genetic analysis, with management involving antibiotics, vaccination, and treating autoimmune manifestations. This case series outlines six patients with diverse presentations. First 3 patients had recurrent meningococcal infections: a 25-year-old male had meningococcal meningitis at the age of 15 and 22; a 30-year-old female had meningococcal sepsis at 10 and 19; and a 30-year-old female had meningococcal meningitis at 15 and sepsis at 22. In all three patients, terminal complement pathway deficiency was confirmed, where the first patient is homozygous for a C8B deficiency and heterozygous for C9. The second and third patients are also homozygous for C8B deficiency. Two other patients had a more complicated infectious diathesis: a 48-year-old male had pneumonia at 10, followed by sepsis at 19 and meningococcal meningitis at 47; a 53-year-old female had an episode of meningitis at the age of 1, followed by recurrent pneumonias since childhood and the development of emphysema and pulmonary arterial hypertension leading to lung transplantation. Functional analysis in the male patient showed a deficiency in all three complement pathways, but the terminal complex activity was elevated (genetic testing is pending), whereas in the female patient, a terminal complement pathway deficiency was confirmed with a homozygous variant in C2 gene. One patient had predominantly autoimmune manifestations: a 58-year-old female with systemic lupus erythematosus and thrombycytopenia. A severely reduced classical and lectin pathway was found, with low C4, C1 inhibitor, and factor B and I. Genetic testing is pending. The cases illustrate heterogeneity in complement deficiency presentations, leading to delayed diagnosis. Genetic testing remained incomplete in some patients. Early diagnosis and vigilant monitoring are crucial to reduce infection recurrence and autoimmune progression.

INTRODUCTION:The natural course of interstitial lung disease (ILD) in patients with systemic autoimmune rheumatic diseases (SARD) varies significantly and is linked to considerable morbidity and mortality. Therefore, effective screening is crucial for early detection of SARD-ILD. Biomarkers associated with mucin 1, Krebs von den Lungen-6 (KL-6) and carbohydrate antigen 15-3 (CA 15-3), are increased in various ILD. This study aimed to assess the diagnostic accuracy of the serum biomarker CA 15-3 as a potential screening tool for ILD in patients newly diagnosed with SARD. METHODS:Conducted as a single-center cross-sectional study, the research included newly diagnosed SARD patients consecutively examined for ILD according to the algorithm. All included patients underwent chest high-resolution CT scans (HRCT), and serum levels of CA 15-3, KL-6, and lactate dehydrogenase (LDH) were measured and correlated with other variables associated with possible ILD presence. RESULTS:Serum biomarker levels, specifically CA 15-3 and LDH, are significantly higher in ILD-positive patients (P<0.001 for both). An inverse relationship is observed between higher FVC values and lower CA 15-3 levels (Rho=-0.291, P=0.007). Similarly, higher DLCO values are associated with lower CA 15-3 levels (Rho=-0.317, P=0.003). Our findings revealed that elevated CA 15-3 levels are positively correlated with higher levels of KL-6 (Rho=0.268, P=0.01) and LDH (Rho=0.227, P=0.04). With a cut-off value of 24 U/mL, CA 15-3 showed the highest sensitivity and specificity (AUC=0.807, specificity=95.7%, sensitivity=71.1%). CA 15-3 emerged as the most significant predictor of a positive HRCT finding, accurately classifying 83% of cases. CONCLUSION:These results suggest that CA 15-3 shows promise as a valuable serum biomarker for screening SARD patients for ILD in routine clinical practice.

Background Data from several large prospective studies have demonstrated that secukinumab is an efficient and safe treatment choice for axial spondyloarthritis (axSpA) [1]. Objectives The aim of our study was to evaluate data from a real-life cohort of patients with axSpA treated with secukinumab. Methods We retrospectively analysed data from our institutional registry of axSpA patients (radiographic and non-radiographic) treated with secukinumab. We analysed the features of patients who continued secukinumab at their last visit and those who discontinued the drug over the follow-up period. We used the Kaplan-Meier curve to estimate the cumulative retention rate of secukinumab. Results A total of 57 patients were enrolled in our study. Among them, 26 (45,6%) were women, and 31 (54,4%) were men. The median age was 50 (22-71). There were 26 (45,6%) patients who were bDMARD naïve and 31 (54,4%) patients that had used at least one bDMARD before secukinumab. Among the naïve group, 4 (14,4%) patients discontinued secukinumab, whereas among the group with former bDMARD usage, 12 (38,7%) people discontinued treatment. The ineffectiveness of the drug was the most common reason for discontinuation in both groups. Regarding adverse effects, two people reported respiratory infections. We did not record significant drug safety complications in our cohort. The duration of treatment was 6,5 years. The survival rate of the drug was 91% after six months, 83,4% after 12 months, 75% after 24 months, and 66,6% after 48 months. Table 1 shows detailed clinical aspects of the cohort. Conclusion Our data confirmed a good survival rate of secukinumab in patients with axial spondyloarthritis. However, results showed that fewer biologically naïve patients discontinued the secukinumab treatment than those who received the drug after the failure of another biologic. Reference [1]Baeten D, Sieper J, Braun J, et al. MEASURE 1 Study Group; MEASURE 2 Study Group. Secukinumab, an Interleukin-17A Inhibitor, in Ankylosing Spondylitis. N Engl J Med. 2015 Dec 24;373(26):2534-48. doi: 10.1056/NEJMoa1505066. PMID: 26699169. Acknowledgements: NIL. Disclosure of Interests Ljiljana Smiljanic Tomicevic Speakers bureau: Novartis, Ely Lilly, Pliva, Pfizer, Abbvie, Ivan Padjen Speakers bureau: Novartis, Ely Lilly, Pliva, Pfizer, Abbvie, Matea Martinić: None declared, Miroslav Mayer Speakers bureau: Novartis, Ely Lilly, Pliva, Pfizer, Abbvie, Branimir Anić Speakers bureau: Novartis, Ely Lilly, Pliva, Pfizer, Abbvie.Table 1Clinical characteristics of the cohortNaive bDMARDn=24≥1 bDMARDn= 33Patients continuing the secukinumabn=41Patients discontinuing the secukinumabn=16Totaln=57n(%)n(%)n(%)n(%)n(%)Male13 (54,2)13 (39,4)22 (53,7)4 (17,9)31 (54,4%)Female11 (45,8)20 (60,6)19 (46,3)10 (43,5)26 (45,6%)HLAB27 positive13 (54,2)19 (57,6)25 (61)8 (50)32 (56,1)Median (range)Median (range)Median (range)Median (range)Median (range)Age (year)48 (32-66)52 (22-71)50 (22-71)50,5 (30-62)50 (22-71)Diagnosis duration (year)8 (2-17)12 (4-41)9 (2-21)12 (2-41)9 (2-41)Initial BASDAI7,15 (3,10-9,4)7,3 (1,9-9)7,1 (1,4-9,4)7,65 (4,2-8,9)7,25 (1,4-9,4)Δ BASDAI (3 months)1,10,42,80,852,3Initial BASFI6,3 (3,4-8,9)6,5 (0,6-9,6)6,5 (3,4-9,4)6,2 (0,6-8,6)6,4 (0,6 -9,4)Initial CRP5,5 (0,8-44,4)3,6 (0,3-115)4,4 (0,3-44,4)3,45 (1,4-115)4 (0,3-115)Δ CRP (3 months)1,60,41,450,50,6Secukinumab therapy duration (days)883,5(79-1973)1127(112-2359)1413(79-2359)293,5(112-1408)1050(79-2359)

Bicipitoradial bursitis (BRB) is the inflammation of the bicipitoradial bursa, a bursa located in the cubital fossa between the biceps tendon and radial tuberosity. It is a relatively uncommon condition mostly attributed to repetitive use; however, it can be associated with other conditions, including rheumatoid arthritis (RA). We present a 52-year-old woman who presented with novel joint pain with swelling lasting 2 months with elevated rheumatoid factor (RF) 246.2 IU/mL and anti-cyclic citrullinated peptide (CCP) antibodies >1200 IU/mL, highly suggestive of RA. It should be noted the patient works as a butcher, with earlier conditions, including carpal tunnel syndrome (CTS) which was operated on. The patient also had numbness of the right hand with electromyoneurography (EMNG) suggestive of CTS relapse and noticed swelling of the cubital fossae. As a part of the workup, a right elbow ultrasound was done showing a mass, which was confirmed by magnetic resonance imaging as BRB. Given the clinical picture, elevated RF and anti-CCP antibodies, and BRB, it was concluded that the patient likely has early RA, and BRB as a result of both repetitive use in combination with the early RA onset which possibly exacerbated an earlier, stable condition. BRB can be large enough to compress local neural structures causing symptoms, which may be an explanation for the EMNG result of CTS relapse, possibly caused by BRB instead as CTS relapse is relatively uncommon. Although a rare condition usually attributed to repetitive use, BRB can also be associated with other conditions including RA as in our case.

Background: Rheumatoid arthritis (RA) is a chronic systemic autoimmune and inflammatory disease. Conventional synthetic and biologic disease-modifying antirheumatic drugs (DMARDs), Janus kinase inhibitors, and rituximab are used to treat the disease. There are no recommendations or guidelines for the treatment of patients with both inflammatory arthritis and end-stage renal disease (ESRD), despite the safety and efficacy of the mentioned drugs. The anti-interleukin-6 receptor antibody tocilizumab (TCZ) has not been used as a long-term therapy for hemodialysis (HD) patients with RA, except in a few case reports. Case Description: We present the case of a 41-year-old patient with RA and ESRD on maintenance HD due to type 1 diabetes-related complications. Due to high RA disease activity, the patient was not a suitable candidate for a kidney transplant. Because TCZ is used to treat both RA and kidney transplant rejection, therapy with a full dose of TCZ was administered. The patient has achieved sustained clinical remission (for the past four years) with no adverse events reported. Conclusions: Herein, we present the safe and effective use of TCZ in an RA patient on HD who is also a candidate for kidney transplant. Consequently, TCZ could be the treatment of choice for RA patients with ESRD who have not achieved disease control (low activity or remission) with conventional synthetic DMARDs. Clinical studies are required to evaluate the efficacy and safety of biologic DMARDs and Janus kinase inhibitors in patients with both inflammatory arthritis and ESRD.

Giant cell arteritis (GCA) is the most common of the vasculitides, with the incidence varying from 21.57 in Scandinavia to 7.26 per 100,000 in the rest of Europe. GCA follows a polygenic inheritance pattern and rare familiar clustering.[1-6] Thomsen et al.[7] reported a study in which only 1% of observed individuals diagnosed with GCA had a first-degree relative with GCA. Moreover, calculated familiar concordant risk for GCA was 2.14, and it was only significant for individuals having a female relative affected. A similar study showed the concordant familiar risk for ankylosing spondylitis, systemic lupus erythematosus, Sjögren syndrome, systemic sclerosis, polymyositis/dermatomyositis, and rheumatoid arthritis to be 18.42, 14.04, 8.63, 4.50, 4.03, and 3.03, respectively.[9] Difference in familiar risk for concordant disease, presented as SIR (standardized incidence ratio), is shown in Table 1 using parents and siblings as probands, using both if applicable.[9] Herein, we report on the cases of two sisters, who had GCA diagnosed within less than a year, and their brother. Case 1- A 67-year-old female with an unremarkable medical history and positive family history of pancreatic cancer was admitted to our department following one month of fever without any infection signs. The patient complained about unilateral blurry vison, malaise, and weight-loss. Laboratory results showed elevated C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) without leukocytosis and normocytic anemia. The patient continued to spike fever despite being administered wide-spectrum antibiotics. Detailed work-up and computed tomography were performed and showed inflammatory changes in subclavian and axillary arteries. An inflammatory signal was later confirmed using color Doppler ultrasonography in both subclavian arteries and the right axillary artery, while temporal arteries showed no signs of inflammation. Case 2- A 63-year-old female with a positive family history of pancreatic cancer was hospitalized after several months of low-grade fever, fatigue, weight loss, and intermittent upper abdominal pain spreading to the back. The patient complained of left temporal headache. Initial laboratory workup also revealed elevated CRP and ESR without leukocytosis, discreate normocytic anemia, and no other pathological results. Color Doppler ultrasonography was done, and it showed signs of inflammation in both temporal arteries (halo sign). Case 3- A 58-year-old male with an unremarkable medical history, recently reported blurred vision, and progressive poor eyesight in the right eye presented to our clinic. The patient is currently under ophthalmologic workup, and the differential diagnosis includes ischemic optic neuropathy. Patient 1 is the older sister of Patient 2, and Patient 3 is their brother (Figure 1). Patients 1 and 2 were diagnosed with GCA, started on glucocorticoids, and are currently in remission on low-dose oral methylprednisolone. Patient 3 is still under examination, and his clinical symptoms might include elements of GCA. In conclusion, the majority of the studies about familiar risk of GCA are done in the North-European area, in which population the incidence of GCA is higher in general. Further studies of familiar risk are required in populations with lower background incidence of disease. GCA should be considered in elderly patients presenting with unexplained inflammatory condition, particularly if a first-degree relative with concordant disease is present.

Primary Sjögren’s syndrome (pSS) is a systemic autoimmune disease that is manifested by the sensation of dry eyes and dry mouth. The higher incidence of non-Hodgkin lymphoma (NHL) among pSS has already been extensively researched. However, there are uncertanties whether the mortality risk in pSS patients and in pSS patients with NHL is increased. The purpose of this study was to describe the prevalence of NHL among pSS patients and to calculate their mortality risk. We retrospectively analysed data on 1367 patients treated in our rheumatology department under the ICD-10 code M35.0. The study finally recruited 155 patients who met the 2016 ACR/EULAR criteria for the diagnosis of pSS. Descriptive statistics was used in data analysis. We applied the indirect standardization by age to compare the incidence rate of NHL in our cohort to general population. Additionally, we compared the mortality in our study to the general population by calculating the standardized mortality ratio (SMR). The overall incidence rate of NHL was 440 per 100,000 patient-years. The SIR compared to the general population was 30.13 (95

sjögren's syndrome (ss) is a chronic, systemic autoimmune disease of unknown aetiology that manifests with various clinical manifestations.it is characterized by dense lymphocytic infiltration of exocrine glands which leads to functional impairment.involvement of the salivary and lacrimal glands, and the consequent dryness of the eyes and mouth, are among the most common clinical manifestations of the disease.ss may occur as a primary disease or secondary to another organ-specific autoimmune disease or overlap with other rheumatic conditions.The disease is named after the swedish ophthalmologist Henrik sjögren.The diagnosis of ss is made based on clinical and laboratory indicators and objective findings of involvement of the salivary glands and lacrimal glands. in clinical practice, classification criteria often help in making a diagnosis, although they were initially developed and validated to standardize a cohort of patients for inclusion in the clinical trials and studies.over the years, more than ten different classification criteria for ss have been proposed.in 1993, the first multicentric preliminary european classification criteria for ss were proposed.These criteria were subsequently revised by the american-european consensus group (aecg) in 2002. in 2012, the new modified classification criteria of the american college of rheumatology (acr) for ss were published, which did not provide any significant diagnostic changes.The 2016 criteria of the acr and the european alliance of associations for rheumatology (eUlar) are currently used for the classification of the disease.a significant shift with the new criteria is that symptoms of dry eyes or mouth are not necessary for classification, and it is sufficient to have only one of the systemic manifestations of the disease.

Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease that most commonly affects the young, working, female population. Musculoskeletal manifestations are one of the most prevalent and presenting features in SLE. Arthralgia, myalgia, non-erosive arthritis, myositis but also tenosynovitis and enthesitis are present in more than 90% of SLE patients. Although not considered very severe SLE manifestations, they significantly affect the patient’s quality of life and daily functioning. Clinical assessment of joints, tendons, entheses, and muscles is still the gold diagnostic standard. There are many radiological imaging methods, i.e., classic radiograms, ultrasound, bone scintigraphy, and magnetic resonance imaging that provide morphological information regarding damage and activity of musculoskeletal diseases in SLE and other rheumatic diseases. Musculoskeletal ultrasound stands out as an accessible and affordable method. Recognizing musculoskeletal manifestations may help establish an early diagnosis of SLE and assess disease activity, thus leading to early initiation of treatment and preventing chronic and irreversible changes with a beneficial effect on the quality of life.

Objective: Prevalence of hypertension seems to be higher in patients with systemic lupus erythematosus (SLE) than in the general population, but there is scarce data on the epidemiology of hypertension in lupus nephritis (LN). Furthermore, to the best of our knowledge, there are no studies examining the trajectories of blood pressure (BP), treatment patterns and control of hypertension in LN. Design and method: We have conducted a retrospective cohort study to evaluate the prevalence, treatment and control of hypertension in patients with biopsy-proven LN. We have collected data on demographics, clinical and laboratory parameters, histopathology and office BP measurement at the time of biopsy and after long-term follow-up. BP measurement and definition of hypertension were according to 2018 ESC/ESH guidelines. Results: A total of 36 patients with biopsy-proven LN were followed up for 4.5 ± 2.9 years (81% women, mean age at biopsy 38 ± 14). Mean duration of SLE prior to biopsy was 4.3 years (min-max 0 to 27 years). Both systolic and diastolic BP decreased from the time of biopsy to last follow-up (137/85 mmHg vs. 125/79 mmHg, p < 0.001 for systolic BP and p = 0.075 for diastolic BP). Prevalence of hypertension at the time of biopsy was 58% and increased to 72% at the time of last follow-up (p = 0.22). Mean number of drugs per patient did not change (2.0 vs. 1.8, p > 0.05). Only 48% and 58% of patients with hypertension had achieved BP control (p = 0.67) and a total of 1 and 2 patients had resistant hypertension at the time of biopsy and at last follow-up, respectively. When examining treatment, 67% and 77% patients with hypertension had an ACEI/ARB, while 48% and 38% had calcium channel blocker, 43% and 35% had diuretics and 24% and 31% had beta blockers at the time of biopsy and follow-up, respectively. Conclusions: LN is associated with high cardiovascular risk and mortality as well as a high prevalence and inadequate control of hypertension. Achieving BP control is crucial and should be an important therapeutic goal in these patients.

Deskriptori SISTEMSKI ERITEMSKI LUPUS – dijagnoza, farmakoterapija, komplikacije; LONGITUDINALNI EKSTENZIVNI TRANZVERZALNI MIJELITIS – dijagnoza, etiologija, farmakoterapija; NEUROPSIHIJATRIJSKI SISTEMSKI ERITEMSKI LUPUS – dijagnoza, etiologija, farmakoterapija; IMUNOSUPRESIVI – terapijska uporaba; TETRAPLEGIJA – etiologija; RESPIRACIJSKA INSUFICIJENCIJA – etiologija; ANTIFOSFOLIPIDNA PROTUTIJELA SAŽETAK. Longitudinalni ekstenzivni transverzalni mijelitis (LETM) je iznimno rijetka, životno ugrožavajuća komplikacija sistemskog eritemskog lupusa (SLE). Riječ je o upalnoj leziji leđne moždine koja zahvaća najmanje tri susjedna vertebralna segmenta. Klinički se prezentira paraparezom ili tetraparezom, senzornim deficitom i vegetativnom disfunkcijom, a u teškim slučajevima uzrokuje respiratornu insuficijenciju. Prikazana je bolesnica u dobi od 28 godina koja se posljednjih 11 godina prati zbog SLE-a, a kod koje se bolest komplicirala razvojem LETM-a sa zahvaćanjem cervikalne i torakalne medule. LETM je nastupio nakon razdoblja stabilne remisije bolesti, a bio je praćen kliničkom, a prethodno i serološkom aktivacijom SLE-a. U sklopu LETM-a bolesnica je razvila tetraparezu i visoku hipesteziju, a zbog pareze respiratorne muskulature i posljedične respiratorne insuficijencije bila je potrebna mehanička ventilacija. Budući da obradom nije dokazana intratekalna infekcija, provedena je kombinirana imunosupresivna terapija kojom je postignut postupni i djelomični neurološki oporavak i poboljšanje neuroradiološkog nalaza. LETM je rijetka, ali ozbiljna komplikacija SLE-a, koja često ostavlja teške neurološke sekvele ili pak dovodi do letalnog ishoda. Uz prikaz bolesnice u pregledu literature navedena su dosadašnja saznanja o patogenezi, dijagnozi i terapijskom pristupu LETM-u u kontekstu neuropsihijatrijskog lupusa.