Background ANCA vasculititides are autoimmune diseases that produce systemic and renal damage and have important consequences on patients` health.
Objectives To investigate the epidemiology, clinical characteristics and response to therapy in renal pauciimmune vasculitis patients thet presented to “C. Davila” Nephrology Teaching Hospital in Bucharest.
Methods Prospective study in patients admitted in our centre from December 1998 to December 2012. Patients were monitored using Birmingham Vasculitis Activity Score (BVAS) and Vascular Damage Index. Statistical analysis was performed using the programs EpiInfo and SPSS.
Results 91 patients were investigated (59,3% female, 40,7% male), medium age 61,1+/-15,6 years. Time lag until diagnosis: 2 months (2-6). ANCA positivity: ANCA c(+): 20%, ANCA p (+): 47% and ANCA negative: 33%. At initial clinical presentation, renal involvement was present in 100% of patients, with a BVAS score of 18 (9-36), macroscopic hematuria: 40%, proteinuria: 1,46 +/- 1,6 g/g creatinine, medium serum creatinine 6 +/- 3,8 mg/dl. Patients presented systemic symptoms in 74,7% of cases, with a median BVAS score of 2,5+/-1,8. Pulmonary infiltrates were present in 44% of patients, and cutaneous manifestations in 12,2%.
ANCA c positivity was more frequent in males (p=0,01) and in younger patients (p=0,01). ANCA c vasculitis had the most precocious start (p=0,05), most frequent pulmonary involvement (p=0,002) and had highest BVAS score (p=0,0017). Also ANCA c vasculitis patients presented with more severe disease, lowest hemoglobin (p=0,02), lowest albumin (p=0,01) and highest number of hematic casts (p=0,0001) and highest demand of dialysis, either temporary (p=0,03) or definitive (p=0,01).
After induction therapy we obtained significant decrease of renal score (14,15 +/- 9,3), proteinuria (1,26+/- 0,28 g/g creatinin)and serum creatinine (3,2+/-2.2 mg/dl), that was maintained at 6 months post induction : renal score: 15,2+/- 10,7, proteinuria 1,2 +/-0,2 g/g creatinine and serum creatinine 2,7+/-1,9 mg/dl. Severe complications were sepsis followed by ESRD (19,8%) and death (6,6%).
Conclusions Pauciimmune vasculitis is a severe condition, diagnosis may be delayed because of nonspecific signs and symptoms at onset. ANCA c positive patients have a more active glomerular disease, a better response to therapy but a higher risk of death, probably due to more frequent pulmonary damage.
Disclosure of Interest None Declared
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