Search

Objective: Compared to the general population, malignancy is more common in patients with systemic sclerosis (SSc) and is one of the leading causes of mortality. The objective of this study was to assess the frequency and types of malignancies, and its association with SSc features. Methods: Medical records of 141 patients who were followed up in the Department of Rheumatology at Kocaeli University Medical Faculty Hospital and who met the 2013 American College of Rheumatology/European League Against Rheumatism SSC classification criteria were retrospectively analyzed. Clinical, demographic, laboratory and radiological information of the patients were obtained from the patient files. Results: Of the seven cases with malignancy, two had non-small cell lung cancer, two had breast cancer, one had larynx cancer, one had endometrium cancer, and one had chondrosarcoma. Apart from malignancy, a female patient was diagnosed with monoclonal gammopathy of undetermined significance. Four of the patients diagnosed with malignancy were evaluated as limited, and three with diffuse skin involvement. Modified Rodnan skin score was significantly higher in SSc patients with malignancy than in the patients without malignancy (p=0.045). No significant difference was found in terms of disease subtype, gender, age, auto-antibody and disease duration. Conclusion: Since the risk of malignancy is increased in patients with SSc, patients should be followed carefully. Malignancy can be diagnosed before the onset of SSc, concurrently or during the follow-up. SSc can be seen as a paraneoplastic syndrome, especially in patients with positive anti-RNAP-III antibody, and these patients should be monitored in terms of malignancy. [ABSTRACT FROM AUTHOR]

Objective: Digital ulcers related to systemic sclerosis (SS) are associated with disability and mortality in the progression of the disease. In our study, we investigated the duration of treatment and drug-related side effects on patients, who were started bosentan for the treatment of digital ulcers. Methods: SS patients with digital ulcers, who had a follow-up period in outpatient and inpatient clinics of Rheumatology Department, were included in our study. Duration of treatment and drug-related side effects were retrieved from the patient’s files. For the patients who did not come to the clinic in the last 6 months, visits were made over the phone. Results: A total of 30 patients were included in the study. The mean age of SS diagnosis was 37.5±14.7 years and 25 of these patients were female. The distribution of disease patterns was limited cutaneous for 25 patients and diffuse cutaneous for 5 patients. The median length of bosentan treatment was 31.0±23.9 months. Five patients required the discontinuation of bosentan due to adverse events including toxic hepatitis (2 patients) and headache (3 patients). Also, 3 patients discontinued the treatment on their own, despite the absence of adverse effects or toxicity. Conclusion: Bosentan, used for the treatment of digital ulcers in SS patients, has been shown to have an acceptable side-effect profile. [ABSTRACT FROM AUTHOR]