Search

Dermatomyositis (DM) is an idiopathic inflammatory myositis (IIM) characterized by skin manifestations and muscle involvement. Spontaneous intramuscular hemorrhage (SIH) is a fatal complication that is very rare in the course of DM, but not well known to rheumatologists. Our aim was to determine the frequency and possible risk factors of DM-related SIH. A retrospective analysis was conducted on a cohort of DM patients who were observed in the rheumatology department of the university hospital between 1998 and January 2024. The clinical, laboratory, radiological data of the patients and the treatments they received during the follow-up were analyzed. To determine possible risk factors for the development of SIH in the course of DM, our patients with DM were analyzed together with other rare SIH cases in the literature. The study included 42 of our DM patients. 32 of the patients (76.2%) were female. The median age of the patients was 53 (24-82) years, the median age of DM diagnosis of the patients was 47 (18-75) years, and the median duration of DM of the patients was 36 (2-276) months. 7.1% of patients had dysphagia, and 16.7% had intertitial lung disease (ILD). 5 (11.9%) patients were diagnosed with malignancy. The incidence rate of SIH development in our DM cohort was 0.238/100 patient years (95% CI 0.006-1.256). We tried to identify independent risk factors for SIH development by comparing our 41 DM patients without SIH with the data of patients with 23 DM-related SIH collected from the literature by adding our 1 patient (24 pts). Male sex (OR 4.97, 95% CI 1.66-14.92, p = 0.003), ILD presence (OR 9.71, 95% CI 2.99-31.47, p < 0.001), anti-MDA5 positivity (OR 16.0, 95% CI 1.60-159.3, p = 0.006), anti-Ro52 positivity (OR 11.6, 95% CI 2.93-46.34, p < 0.001), heparin use (OR 4.42, 95% CI 2.68-7.24, p < 0.001), intravenous immunoglobulin (IVIG) use (OR 11.7, 95% CI 2.26-60.54, p < 0.001), and steroid dose (OR 1.03, 95% CI 1.00-1.05, p = 0.005) were identified as risk factors for the development of SIH in the univariate analysis. The death rate due to hemorrhage was 50%. No single risk factor was found to be associated with death. As a result, SIH may occasionally arise in patients with DM. Rheumatologists should be aware that patients with dysphagia and/or ILD, who are on heparin, getting high doses of steroids, and test positive for anti-MDA5 and/or anti-Ro52 antibodies may develop SIH in the early stages of DM., (© 2024. The Author(s).)

Objective: Idiopathic inflammatory myopathies (IIM) are a group of connective tissue diseases that are characterized with proximal muscle inflammation and immune mediated muscle damage. Compared to the general population, both malignancy and mortality rates are found to be higher in IIM patients. The aim of our study was to define the predictive factors for mortality and to evaluate the types and frequencies of concomitant malignities in IIM patients. Methods: A retrospective evaluation was conducted in 53 patients who were diagnosed with IIM using 1975 Bohan-Peter criteria in university hospital rheumatology department between January 1998 and January 2020. The clinical and laboratory findings, malignancy status and prognostic factors for mortality were analyzed for the patients. Results: Among the 53 patients (32 women, 21 men), the mean age was 55.24±16.12 years, the mean age of myopathy diagnosis was 44.45±17.62 years. When the subgroups of myositis were analyzed, 27 patients (50.9%) had dermatomyositis and 23 patients (43.4%) had polymyositis. The most frequent clinical finding was fatigue with 77.2%. Steroid usage rate was 98.1% and azathioprine usage rate was 34% among patients. Malignancy frequency was 13% (n=7) (2 nasopharyngeal cancer, 2 colon cancer, 1 non-small cell lung cancer, 1 breast cancer and 1 pancreas cancer) and the age of patients with malignancy and myositis was significantly higher (p=0.03, p=0.02 respectively). When the mortality frequency was evaluated by different parameters, it was found that gender, disease duration and malignity preexisting myositis were statistically significant parameters. The most frequent mortality cause was malignancy. Cumulative survival rate in our cohort was 80.75% in 5 years and 72.68% in 10 years. Conclusion: As a result, the frequencies of mortality and malignancy in our patients with IIM were generally similar to those in the literature. The most important factors affecting life expectancy and mortality were advanced age at diagnosis and the presence of malignancy. [ABSTRACT FROM AUTHOR]