THE OCCURRENCE OF PARANEOPLASTIC SYNDROMES IN PATIENTS WITH POLYMYALGIA RHEUMATICA TREATED AT THE UNIVERSITY HOSPITAL CENTER OSIJEK

Background: Polymyalgia rheumatica (PMR) is an inflammatory rheumatic disease that occurs in an elderly person, usually over fifty years of age. Disease is characterized by pain, discomfort, and tenderness of shoulder, throat and hip muscles, elevated erythrocyte sedimentation values, and a fast and effective therapeutic response to the applied glucocorticoid therapy. Clinical image of PMR may resemble the presentation of many malignant diseases, given that it is of great importance to do extensive diagnostic treatment of the patient. Objectives: Th e aim of this study was to investigate the occurrence of paraneoplastic syndromes in patients with PMR, treated at the Department of Rheumatology, Clinical Immunology and Allergology of the University Hospital Center of Osijek (UHCO). Methods: Th e study included PMR patients treated at the UHCO in the period from 1/2013. to 10/2018. A study was conducted using data from the General Practice Research Database of the UHCO. Results: In 46 patients with PMR the occurrence of paraneoplastic syndrome was 8.7% (N=4) with a 95% confidence interval of 2.42%–20.79%. Th e median age of the detection of the paraneoplastic syndrome was 73 (65–85) years, and the mean time of detection of the syndrome since the diagnosis of PMR was 1± 1 years. In total number of diagnosed, there is an equal number of male and female patients (N=2, p>0.999). Among males, the occurrence of paraneoplastic syndrome was 15.38%, and among women 6.02% (p = 0.585). The mean age of discovery of male paraneoplastic syndrome was 75±14.14, and in women 64±7.07 godina (p=0.699). Th ere was no statistically significant difference in the age of PMR patients (76.17±6.93) compared to those with paraneoplastic syndrome (71.5±9.11), p=0.213. Conclusions: According to the results of our research the time to diagnose paraneoplastic syndrome is approximately one year after the diagnosis of PMR. Therefore, more extensive diagnostic processing and disease control during the first year from the diagnosis of the PMR will reduce the risk of non-recognition of malignant disease disguised as a clinical image of PMR. In addition, the occurrence of paraneoplastic syndromes was 8.7% in the population of PMR patients included in this fi ve-year study. References: 1. Muller, Sara, et al. Is cancer associated with polymyalgia rheumatica? A cohort study in the General Practice Research Database. Ann Rheum Dis. 2014;73(10): 1769–73. 2. Muller, S., et al. Th e real evidence for polymyalgia rheumatica as a paraneoplastic syndrome. Reumatismo. 2018; 70(1): 23–34. 3. Ji, Jianguang, et al. Cancer risk in patients hospitalized with polymyalgia rheumatica and giant cell arteritis: a follow-up study in Sweden. Rheumatology.2010; 49(6): 1158–63. 4. Myklebust, Geirmund, et al. No increased frequency of malignant neoplasms in polymyalgia rheumatica and temporal arteritis. A prospective longitudinal study of 398 cases and matched population controls. J Rheum.2002; 29(10): 2143–7. 5. Mayer, Miroslav, and Branimir Anić. Paraneoplastički sindromi u reumatologiji. Reumatizam.2015; 62(Suppl. 1): 0–0

Systemic sclerosis-associated interstitial lung disease in the EUSTAR database: analysis by region

Objectives: The prevalence and characteristics of systemic sclerosis-associated interstitial lung disease (SSc-ILD) vary between geographical regions worldwide. The objectives of this study were to explore the differences in terms of prevalence, phenotype, treatment, and prognosis in patients with SSc-ILD from predetermined geographical regions in the EUSTAR database. Methods: Patients were clustered into seven geographical regions. Clinical characteristics and survival of patients with SSc-ILD were compared among these pre-determined regions. Results: For baseline analyses, 9260 SSc patients were included, with 6732 for survival analyses. The prevalence of SSc-ILD in the overall population was 50.2%, ranging from 44.0% in "Western Europe & Nordic countries" to 67.5% in "Eastern European, Russia & Baltic countries". In all regions, anti-topoisomerase antibodies were associated with SSc-ILD. Management also significantly differed; mycophenolate mofetil was prescribed at baseline in 31.6% of patients with SSc-ILD in "America (North & South)" and 31.7% in "Middle East" but only 4.3% in "Asia & Oceania" (P < 0.0001). Patients from "America (North & South)" and "Middle East" had the highest survival rate at the end of follow-up (85.8% and 85.2%, respectively). Conclusion: Our study highlights key differences among regions in terms of clinical presentation and prognosis of SSc-ILD. This work also demonstrates that the management of SSc-ILD is highly variable among the different regions considered, suggesting that efforts are still needed for the standardisation of medical practice in the treatment of this disease. Keywords: Interstitial lung disease; autoantibodies; lung fibrosis; scleroderma; systemic sclerosis