Paraneoplastic rheumatic syndromes

Fifteen percent of patients with malignancy are diagnosed endocrinal, hematological, rheumatic or neuromuscular paraneoplastic syndromes. They are more common in males than in females. Rheumatic paraneoplastic syndromes are quite rare. Differences in clinical features, course and outcomes of primary rheumatic disease and paraneoplastic rheumatic syndromes are reviewed. Attention is paid to the need to differentiate paraneoplastic syndromes and real rheumatic disease and the ways to do it. It is generally held that an extensive search for occult malignancy in most rheumatic syndromes is not recommended unless accompanied by specific findings of malignancy

Clinical characteristics and long-term outcomes of 35 patients with Wegener’s granulomatosis followed up at two rheumatology centers in Lithuania

Objective. The aim of this study was to investigate the survival of Lithuanian patients with Wegener’s granulomatosis, who were followed up at two tertiary rheumatology centers, and to find the factors possibly influencing the outcomes of this disease. Material and methods. Thirty-five patients were followed up prospectively from the onset of disease (the first patient was enrolled in 1994) at Vilnius University Hospital and the Center of Rheumatology of Kaunas University of Medicine (17 and 18 patients, respectively). All patients in both the centers were followed up on a routine basis, and their records contained necessary information about laboratory and biopsy data; the censoring date (end of follow-up) was stated in June 2006. Results. Among the patients, the most frequent organs involved were ear, nose, throat (ENT) (82.6%), lungs (74.3%), and kidney (renal involvement was defined by proteinuria/abnormal urine sediment) (45.7%). Renal insufficiency was present in 20.6% of all the patients. At the end of the study, 32.4% of patients had simultaneously all three organ systems involved, namely upper respiratory tract, pulmonary, and renal. ANCA positivity was found for 26 (74.3%) of all the patients. Overall mortality rate was 25.7% (9/35). The mean survival was 99.4 months (95% CI, 73.6; 125.3) limited to 149 months for the longest-surviving patient. Conclusions. Female gender and all three specific organ involvements being present at the same time and higher vasculitis damage index were associated with poor outcome. Overall mortality rate was 25.7% (9/35) during the 12-year follow-up, and it is similar to the data from other European countries