DISEASE PHENOTYPE IN ANCA ASSOCIATED VASCULITIS.
- Filipa D. Silva, Portugal
Abstract
Background and Aims
The optimal management of ANCA vasculitis patients consists of accurate identification of disease phenotypes, relapses and preventing complications such as infections and cancer. In this study, we pretend to profile the patients with ANCA vasculitis in terms of clinical features with an emphasis on kidney involvement and relapse.
Methods
Patients included in the study will be individuals ≥ 18 years or older, of both sexes, followed in an outpatient internal medicine and nephrology consultation for a period of up to ten years, with an established diagnosis of ANCA associated vasculitis. Statistical analysis was performed using SPSS software.
Results
Fifty-eight patients with ANCA-associated vasculitis were followed, fifty-four with renal involvement. The majority were male (55%), age mean of 61 years. 75.9% MPO positive. 88% had biopsy confirming vasculitis. Renal biopsies were classified into four classes: most patients (50%) had crescentic class, followed by mixed (33%), sclerotic (10.4%) and focal (6.3%). Creatinine at diagnosis was higher for the crescentic and sclerotic types. 17.2% evolved to ESRD and none was submitted to transplant. 20.7% had a relapse, with a mean time between diagnosis and relapse of 4.5 years. Females, GPA and ANCA MPO were the most affected. 5.2% of patients died. 51% of the patients had an infection in the course of the disease, being a major cause of death. 6 patients were diagnosed with cancer, mainly of the skin.
Conclusions
The prognosis of ANCA-associated vasculitis has been transformed in recent years. Once it was a set of acute and fatal conditions, but these disorders are currently considered to be chronic diseases.