Search Results (5)

Background: Autoimmune diseases and other immune-mediated disorders are associated with an increased risk of malignancy, influenced by chronic inflammation, immune dysregulation, and treatment-related factors. Clarifying cancer risk patterns across specific conditions is essential to improve clinical vigilance and inform screening practices. Objective: The aim of this study was to synthesise current evidence on the association between autoimmune and immune-mediated diseases and cancer, with a focus on practical implications for clinicians. Methods: Recent cohort studies, meta-analyses, and expert consensus documents were analysed to describe cancer epidemiology, pathogenic mechanisms, high-risk phenotypes, and treatment considerations across major autoimmune diseases and other immune-mediated conditions. The review covers idiopathic inflammatory myopathies, Sjögren’s syndrome, systemic sclerosis, systemic lupus erythematosus, rheumatoid arthritis, antiphospholipid syndrome, ANCA-associated vasculitis, giant cell arteritis, polymyalgia rheumatica, sarcoidosis, mixed connective tissue disease, IgG4-related disease, VEXAS syndrome, and eosinophilic fasciitis. Special attention was given to identifying warning features for underlying malignancy and evaluating cancer screening strategies. Results: The magnitude and distribution of cancer risk vary across diseases. In some conditions such as dermatomyositis, systemic sclerosis or Sjögren’s syndrome, increased risk is well established, particularly for haematological and certain solid tumours. However, tumour patterns may differ across populations, and findings are not always consistent. Distinct clinical and serological features help stratify individual cancer risk and may guide the intensity of screening. The first years after disease onset often represent a window of higher vulnerability, during which intensified surveillance may be warranted in selected patients. Conclusions: Cancer risk in autoimmune diseases should be assessed on an individual basis. Awareness of disease-specific risk factors and clinical warning signs supports early recognition of malignancy and informs screening decisions in routine practice. Full article

Objectives: Fournier’s gangrene is an aggressive, rapidly progressing, and life-threatening necrotizing fasciitis of the perineal and genital regions. Various scoring systems have been developed for predicting survival and prognosis in Fournier’s gangrene. This retrospective study aimed to evaluate the effectiveness of the newly developed Fournier’s gangrene mortality index (FGMI) in predicting mortality associated with Fournier’s gangrene. Methods: The study included patients over the age of 18 years who were followed-up with a diagnosis of Fournier’s gangrene in the general surgery clinics of three different hospitals in Şanlıurfa province between 2014 and 2024. The patients included in this study were divided into two groups: deceased (n = 20) and surviving (n = 149). In FGMI, the parameters used were age, creatinine level, albumin level, lymphocyte percentage, and neutrophil-to-lymphocyte ratio. Based on the total score and risk assessment, <5 points were categorized as low-to-moderate mortality risk and ≥5 points as high mortality risk. Results: A total of 169 patients with a diagnosis of Fournier’s gangrene were included in the study; 87 were men (51.48%). The median age of all patients was 53 (40–63) years; 20 patients (11.8%) died. The Laboratory Risk Indicator for Necrotizing Fasciitis (LRINEC) score did not show a statistically significant difference between the deceased and surviving groups (p = 0.5). Compared to the survivors, the deceased had higher neutrophil counts, neutrophil percentages, neutrophil-to-lymphocyte ratios, platelet-to-lymphocyte ratios, and C-reactive protein-to-albumin ratios, whereas lymphocyte counts, lymphocyte percentages, eosinophil counts, eosinophil percentages, monocyte counts, and monocyte percentages were lower, and these differences were statistically significant. According to receiver operating characteristic (ROC) analysis, the ROC-area under the curve for predicting mortality based on an FGMI score of ≥5 was 0.88 (95% CI: 0.80–0.95) with a sensitivity of 90% and a specificity of 70% (p < 0.001). Univariate risk analysis was performed, and the odds ratio revealed that mortality risk in patients followed-up for Fournier’s gangrene with a FGMI score of ≥5 was 20 times higher (4.48–90.91) (p < 0.001). Conclusions: The results reveal that the FGMI score is a scoring system that can predict mortality at the initial clinical presentation of patients with Fournier’s gangrene. Another important finding of the present study is that the LRINEC score was not sufficiently effective in predicting mortality. Full article

Morphea is an autoimmune fibrotic skin disease. Eosinophilic fasciitis (EF) is considered to belong to the severe spectrum of morphea. We conducted a scoping review assessing the risk of secondary cancer among morphea/EF patients, paraneoplastic morphea/EF and morphea/EF developing secondary to cancer therapy. The search was conducted using MEDLINE, Embase, Cochrane databases for articles published from inception to September 2022 following the Preferred Reporting Items for Systematic reviews and Meta-Analyses for Scoping Reviews (PRISMA-ScR) guidelines with no language or date restrictions. Two hundred and one studies were included. Of these, 32 studies reported on secondary cancer in morphea/EF patients, 45 on paraneoplastic morphea/EF and 125 on cancer-treatment-induced morphea/EF. While the current evidence remains limited, data suggest an increased risk of secondary cutaneous and possibly pancreatic malignancy in morphea patients, particularly the generalized subtype. There were insufficient data for EF. On the other hand, paraneoplastic morphea was anecdotal, whereas several observational studies suggested that ~10% of EF cases may be paraneoplastic, primarily in the context of hematologic malignancies. Radiotherapy-induced morphea is rare, seen in ~0.2% of treated patients and is usually localized to the treatment site, except in patients with pre-existing autoimmunity. While chemotherapy-induced cases are reported, immunotherapy morphea/EF cases are emerging and are preferentially seen with PD-1 and not CTLA-4 inhibitors. This study is limited by the type of articles included (case reports, case series and observational studies), and hence, additional research on this important topic is needed. Full article

Immune checkpoint inhibitors (ICI) are the standard of care for various malignancies and have been associated with a wide spectrum of complications that are phenotypically akin to primary autoimmune diseases. While the literature on these toxicities is growing, there is a paucity of data regarding ICI-associated scleroderma which can carry significant morbidity and limit the ability to continue effective ICI therapy. Our review aimed to analyze the current literature on ICI-associated systemic scleroderma (ICI-SSc) and key scleroderma mimics. Cases of ICI-SSc had notable differences from primary SSc, such as fewer vascular features and less seropositivity (such as scleroderma-specific antibodies and antinuclear antibodies). We found that patients with a diagnosis of SSc prior to the start of ICI can also experience flares of pre-existing disease after ICI treatment used for their cancer. Regarding scleroderma mimics, several cases of ICI-eosinophilic fasciitis have also been described with variable clinical presentations and courses. We found no cases of scleroderma mimics: ICI-scleromyxedema or ICI-scleroedema. There is a critical need for multi-institutional efforts to collaborate on developing a patient database and conducting robust, prospective research on ICI-scleroderma. This will ultimately facilitate more effective clinical evaluations and management for ICI-scleroderma. Full article

Eosinophilic fasciitis (EF), defined as diffuse fasciitis with eosinophilia by Shulman in 1974, is a disease with unknown etiology and whose pathogenesis is still being researched. The diagnosis is based on the clinical aspects (skin induration with an “orange peel” appearance), the lab results (eosinophilia, increased inflammatory markers), the skin biopsy with the pathognomonic histopathological result, as well as the typical MRI changes. The treatment includes glucocorticoids and immunosuppressive drugs. Due to severe refractory cases, the treatment remains a challenge. EF is still a disease with potential for further research. Full article