249. ANCA-associated vasculitis: predictors of progression to end stage renal disease and mortality
Creators: name: Ventura1, Sofia
name: Dias2, Bruno
name: Fernandes2, João
name: Oliveira2, João
name: Sala2, Inês
Publisher: name: Zenodo
Year: 2022
Subjects: Vasculitis
ANCA
Abstracts
Giant Cell Arthritis
MPA
GPA
EGPA
Takayasu
IgA vasculitis

description: <strong>Background:</strong> Vasculitis is a process defined by inflammation of any blood vessel with necrosis in variable degrees. ANCA-associated vasculitis (AAV) are characterized by necrotizing inflammation of small blood vessels. Renal involvement is present in the majority of patients with AAV and can be severe, being associated with poor survival and increased risk for progression to end stage renal disease (ESRD). The goals of this study were to describe the predictors for mortality and progression to ESRD in our population, as well as to assess possible prognostic factors at presentation and during follow-up. <strong>Methods:</strong> We conducted an observational retrospective study including all the patients diagnosed with AAV with biopsy-proven renal involvement diagnosed in our centre in the last 20 years. We collected information on demographics, clinical presentation, laboratory parameters, treatment, and progression to ESRD or death. Laboratory parameters were assessed at presentation and at 3, 6, 12, 18 and 24 months of follow-up, and included: antibody type (peroxidase 3 [PR3] or myeloperoxidase [MPO]) and titters, serum creatinine, haemoglobin, C-reactive protein, and the presence of urine sample abnormalities (proteinuria, haematuria, leukocyturia). <strong>Results:</strong> We included 52 patients, with a median age of 65.1 years (57.9-76.6 years) at presentation and no differences concerning the gender (50% males). Constitutional symptoms were the most frequent clinical picture (82% of the patients) and 32% of the cases presented with upper airway involvement. The majority of patients were positive for MPO antibody (n=49, 83%). ESRD-free survival of our study population was of 76%, 64% and 61%, respectively at 12, 36 and 60 months of follow-up. We observed global mortality rates of 15%, 29% and 37%, at the same timings, irrespectively of treatment or progression to ESRD. The patients that did not progress to ESRD nor death were significantly younger at presentation than their counterparts – 60.0 years, versus 71.1 years (ESRD), versus 76.4 years (death); p=0,008. This group of patients also had more frequent airway involvement (55%, versus 15% [ESRD], versus 20% [death]; p=0,027) and presented with lower creatinine concentration (2.77mg/dL, versus 4.84mg/dL [ESRD], versus 3.37mg/dL [death]; p=0,001). No differences were observed concerning haemoglobin or C-reactive protein levels, nor the presence of haematuria or leukocyturia at presentation. After adjusting for possible confounders, the independent predictors of progression to ESRD were higher serum creatinine levels (HR 1.164, P=0.031) and ANCA titters &gt;1/640 (HR: 3.326, P=0.049). The independent risk factors for mortality were higher age at diagnosis (HR 1.12 per year, p=0,001) and the presence of MPO (versus PR3; HR 4.34, p=0,043). <strong>Conclusions:</strong> The high rate of progression of AAV to ESRD and the high mortality rate that we observed in our study are in accordance with what was previously described in the literature. Since the consequences of a missed or delayed diagnosis of this condition are potentially life threatening, we defend that it is of upmost importance to identify the risk factors for the progression of the disease, in order to better and more efficaciously treat our patients. Hence, our work highlights the importance of classic risk factors, including higher serum creatinine and older age, on the renal and global prognosis of these patients. <strong>Disclosures:</strong> None