RESUMO
Mixed connective tissue disease (MCTD) was initially described as a chronic immune-mediated disease with overlapping features of systemic lupus erythematosus, scleroderma, and polymyositis. We conducted a cross-sectional study to describe the clinical and immunological profile of patients with MCTD and to compare the four diagnostic criteria, namely, Sharp, Kasukawa, Alarcón-Segovia, and Khan criteria. A total of 291 patients who were admitted from June 2007 to June 2017 and fulfilled the inclusion criteria were included in the study. A clinical diagnosis of MCTD was made in 111 patients, of whom 103 (92.8%) were women. The mean age at presentation was 39.3 years (SD ± 11.6). The most common organ systems that were involved were musculoskeletal system (95.5%), skin and mucosa (78.4%), and the gastrointestinal and hepatobiliary systems (56%). The maximum sensitivity was for the Kasukawa criteria with a sensitivity of 77.5% (95% CI 68.4-84.6) and specificity of 92.2% (95% CI 87-95.5). The Kahn criteria and Alarcón-Segovia criteria had the maximum specificity; the Alarcón-Segovia criteria had a sensitivity of 69.4% (95% CI 59.8-77.6) and a specificity of 99.4% (95% CI 96.5-99.9), while the Kahn criteria had a sensitivity of 52.3% (95% CI 42.6-61.7) and a specificity of 99.4% (95% CI 96.5-99.9). The sensitivity and specificity of Sharp criteria were 57.7% (95% CI 47.9-66.87) and 90% (95% CI 84.4-93.8), respectively.
RESUMO
CONTEXT: Skull base osteomyelitis (SBO) is an uncommon disease with substantial morbidity and mortality. AIMS: The aim of this study is to characterize clinical features, outcomes, and complications of SBO. We also looked at differences in clinical profile in otogenic and non-otogenic SBO. MATERIALS AND METHODS: This is a single-center retrospective observational study. Patients aged more than 15 years of age with clinical and radiological diagnosis of SBO admitted in general medicine department in a teaching hospital in South India from March 2006 to February 2018 were recruited. RESULTS: A total of 41 patients with SBO were identified and included. Mean age was 56.9 ± 10.7 years. In all, 90% of patients (37/41) had diabetes mellitus and 29% (12/41) had recent head/neck surgery. Only 19% (8/41) needed ICU care, and mortality was 21% (9/41). Most common symptom was headache seen in 73% (30/41) of patients. Majority, 61% (25/41), had otogenic infections. Otogenic infections were associated with longer duration of diabetes mellitus (mean = 11.5 vs. 5 years, P = 0.01), higher creatinine levels (mean = 1.66 vs. 0.9 mg/dL, P = 0.014, odds ratio [OR] = 3.8), and higher incidence of cranial nerve palsy (92% vs. 56%; OR = 8.9) compared to non-otogenic SBO. Cranial nerve palsy (78%), meningitis (63%), and cerebral venous thrombosis (43%) were frequent complications of SBO in this study. The causative organisms for SBO in our cohort was bacterial in 60% (15/25) and fungal in 40% (10/25) of the patients. Surgical debridement for source control was done in 54% of patients (22/41) and was associated with survival at discharge (P = 0.001). CONCLUSIONS: Bacterial infections are the most common cause of SBO. Otogenic SBO is associated with longer duration of diabetes mellitus and higher incidence of cranial nerve palsy. Therapeutic surgical debridement plays an important role in treatment of SBO and is associated with improved survival.
