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BACKGROUND: Pulmonary renal syndrome (PRS) is not a single entity but is caused by varied etiologies, including anti-neutrophil cytoplasmic antibody (ANCA), associated small vessel vasculitis (such as Wegener’s granulomatosis, microscopic polyangiitis, and Churg-Strauss vasculitis), Goodpasture’s syndrome, systemic lupus erythematosus, Henoch-Schonlein purpura, cryoglobulinemia, and rare causes such as druginduced vasculitis and subacute endocarditis. CASE REPORT: We report a case of a 51-year-old man who was referred to our hospital with a 2-month history of breathing difficulties, mild hemoptysis, and deteriorated renal function with a serum creatinine of 269 μmol/L. Serological testing was negative for anti-neutrophil cytoplasmic antibody (ANCA) and anti-glomerular basement membrane (GBM) antibody, and also all cultures, including blood and urine, remained negative. In the renal biopsy, not linear deposition of IgG along the GBM and crescents at varying stages with extracapillary glomerulonephritis emphasizes the possibility of a double-seronegative pulmonary renal syndrome. Regarding therapeutic dilemmas, on the 3rd day of hospitalization, we initiated immunosuppression with cyclophosphamide and corticosteroids as well as plasma exchange (5 treatments). Under immunosuppressive therapy and plasma exchange, the patient’s status continually improved; there was no pulmonary bleeding, but the serum creatinine remained high and renal function remained in stage 4 chronic kidney disease. Four weeks later, he was hospitalized again, and we faced a new therapeutic dilemma because of the rapid relapse during immunosuppressive therapy and renal function deterioration that required hemodialysis treatment. Despite repeatedly negative results for anti-GBM and ANCA, initial immunosuppressive therapy with plasma exchange (9 treatments) was included again. The patient was discharged 30 days after admission in a stable general condition, with the maintenance immunosuppressive therapy with mycophenolate mofetil and hemodialysis 3 times/week. After 24 months, we have received information from the hemodialysis center that he is on regular hemodialysis and that he is in good condition. CONCLUSION: We think that in this atypical case, intensive plasma exchange and immunosuppressive therapy are crucial in the early stage and maintenance therapy is necessary for vasculitis in remission. This reported case has important clinical implications because pulmonary-renal syndrome with negative ANCAs and anti-GBM antibodies is extremely rare and no treatment recommendations have been established yet.

Background and Aims The association between serum uric acid (UA) and left ventricular hypertrophy (LVH) is controversial in chronic kidney disease, whereas in hemodialysis (HD) patients has not been studied until now. Thus, we evaluated the relationship of baseline and time-averaged UA with echocardiographic LVH over a 5-year period in HD patients. Method This longitudinal study was conducted on 225 prevalent HD patients over a 5-year period. Patients were stratified into 3 groups according to their baseline and time-averaged UA levels: lower group (UA450µmol/l). Echocardiography was performed on a non-dialysis day and the presence of LVH was defined based on the left ventricular mass index (LVMI) >131 and >100 g/m2, for men and women, respectively. The patients were followed during a 60 month period. Results During the 5-year follow-up, 81 patients died (36%), and the main causes of death were cardiovascular (CV) related (70%). Survival analysis show that patients with time-averaged UA450µmol/l, but this difference was not statistically significant in terms of baseline UA level. A negative correlation was observed between LVH and time-averaged UA (r=-0.26, p=0.001), but not with LVH and baseline UA. Patients in lower time-averaged UA group had significantly higher LVMI compared to patients in intermediate and higher group (153.10± 59.89, 131.62±40.99, 131.19±44.49 g/m2, p=0.029), but from the lowest to the highest baseline UA levels, LVH was not significantly different (146.99±59.20, 141.38±37.52, 126.85±42.48 g/m2, p=0.07). Unadjusted odd ratio of LVH risk in the lower time-averaged UA compared to the reference time-averaged UA group was 3.11 (95% CI=1.38-7.05; p=0.006); and after adjustment for age, gender, diabetes and CV disease, ORs for LVH persisted significant only in the lower time-averaged UA group (OR = 2.82, 95% CI = 1.16–6.88, P = 0.002). On the contrary, baseline UA did not affect unadjusted and adjusted LVH. Conclusion In HD patients the prolonged exposure to hypouricemia is associated with LVH. This paradoxical association can only be explained by the hypothesis that uremic milieu in HD patients changes the influence of uric acid. However, these results should be the subject of further research.