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Autoimmune diseases (ADs) and myelodysplastic syndrome (MDS) may be associated in approximately 10-20% of cases. Although this association has been well recognized, it is not always easily diagnosed. The exact physiopathological mechanism involved has yet to be determined but seems to be multifactorial. The therapeutic decision is not well codified and often represents a challenge. But overall, glucocorticosteroids have generally proven to be effective at the expense of a high incidence of dependence and relapse. This review aims to summarize and analyze all aspects of this association to provide an overview for practitioners and clinicians. A scientific search in databases (PubMed, Scopus, Web of Science, Google Scholar) was conducted using a combination of key words such as autoimmune diseases, myelodysplastic syndromes, and association. Articles from 1992 to 2022 were considered and relevant data were collected and summarized to provide a coherent detailed overview of the coexistence of ADs and MDS., Competing Interests: The authors declare no conflict of interest., (Copyright: © 2024 Narodowy Instytut Geriatrii, Reumatologii i Rehabilitacji w Warszawie.)

Hughes-Stovin syndrome is a rare disease characterized by thrombophlebitis associated with arterial or bronchial aneurysms. Even though it was described first in 1911, it is scarcely reported in the literature. Hughes-Stovin syndrome diagnosis is based on clinical manifestations as well as radiological findings. There are no validated criteria or specific laboratory findings to confirm the diagnosis. Computed tomography pulmonary angiography remains the gold standard for the diagnosis and follow-up of radiological findings, as they were recently described in a critical analysis of the largest cohort in the literature. The aim of this review is to draw attention to this rare but potentially fatal disease and to discuss its therapeutic options., Competing Interests: The authors declare no conflict of interest., (Copyright: © 2022 Narodowy Instytut Geriatrii, Reumatologii i Rehabilitacji w Warszawie.)

Objectives: To investigate the changes over time in extraocular and ocular manifestations of Behçet's disease (BD) in Tunisian patients., Material and Methods: Retrospective study of 246 patients divided into two groups: group 1 (147 patients examined from 1995 to 2005) and group 2 (99 patients examined from 2006 to 2017)., Results: Active or scarred genital ulcers observed by physician at presentation were significantly less frequent in group 2 (47.2% vs. 29.6%; p = 0.007), as were articular involvement (50.3% vs. 34.7%; p = 0.016) and erythema nodosum (18.4% vs. 8.1%; p = 0.024). One hundred-seven patients (43.5%) developed ocular manifestations during the 23-year study period. Intermediate uveitis was significantly more frequent in group 2 than in group 1 (11.7% vs. 28.4%; p = 0.003), and posterior uveitis less frequent in group 2 than in group 1 (34.2% vs. 19.7%; p = 0.016). Patients from group 2 were more likely to have macular edema (19.8% vs. 45.6%; p = 0.001). However, better visual prognosis, with a lower rate of legal blindness, was noted in group 2., Conclusions: Changes over time included a decrease in the rate of articular involvement and cutaneous involvement. There was an increase in the rate of intermediate uveitis and a decrease in the rate of posterior uveitis over time. Despite an increase in the rate of macular edema, there was an improvement in visual prognosis, with less legal blindness over time., Competing Interests: The authors declare no conflict of interest., (Copyright © 2020 Narodowy Instytut Geriatrii, Reumatologii i Rehabilitacji w Warszawie.)

Competing Interests: The authors declare no conflict of interest.

Background: Primary Sjögren's syndrome (pSS) is a chronic systemic autoimmune disease with an elevated risk of developing lymphoproliferative malignancies (LM). Whether pSS is a risk factor or not for non-lymphoma malignancies (NLM) has been scarcely evaluated in the literature. Age is per se a risk factor for malignancies: patients over 70 years old have 4 times higher risk for cancers than adults. Even if the mean age of pSS onset usually is in the 4 th and 5 th decade, its onset in patients aged over 65 years (Elderly Onset pSS - EOpSS) is not uncommon., Material and Methods: To evaluate pSS as a risk factor for NLM we performed a systematic electronic search on PubMed in the period 2006-2016 to identify all the publications on this topic. The studies were eligible for inclusion if they reported specific Standardized Incidence Ratio (SIR) with 95% CI. Studies that did not report sufficient published and/or original data were excluded., Results: Only 7 articles of 494 that we found in PubMed fulfilled the inclusion criterion. In the vast majority of these, SIR values were not statistically significant for NLM. The occurrence of NLM after LM was statistically significant in some studies and a NLM represented the most frequent cause of death. The possibility that NLM may represent a paraneoplastic syndrome seems much more frequent than LM, the risk of which increases with time after the diagnosis. Data regarding the neoplastic weight of EOpSS are mainly pointed out by case reports., Conclusions: Primary Sjögren's syndrome is not associated with an increased risk for NLM. However the possibility that NLM may appear after recovery from lymphoma should be carefully considered because it could be cause of the patient's death. Similarly the possibility that NLM may represent a paraneoplastic syndrome must be highlighted. The relationship between EOpSS and SIRs for NLM should be deepened with studies on ad hoc cohorts., Competing Interests: The authors declare no conflict of interest.