In these AAV patients, not only AH but also ILD was frequently observed. AH was associated with the prognosis, but ILD was associated with the long-term prognosis of AAV.
Abbreviations ACEI Angiotensin converting enzyme inhibitor AKI Acute kidney injury ARB Angiotensin receptor blocker BMI Body mass index BP Blood pressure CCB Calcium channel blocker CKD Chronic kidney disease CVD Cardiovascular disease eGFR Estimated glomerular filtration rate ESKD End-stage kidney disease MRB Mineralocorticoid receptor blocker QOL Quality of life RAS Renin angiotensin system Levels of evidence A High: We are confident that the true effect lies close to that of the estimate of the effect. B Moderate: The true effect is likely to be close to the estimate of the effect, but there is a possibility that it is substantially different. C Low: The true effect may be substantially different from the estimate of the effect. D Very low: The estimate of the effect is very uncertain and might often be far from the true effect. None Grade of recommendation 1 "We recommend" 2 "We suggest" None Chapter 1. Diagnosis and definition of chronic kidney disease CQ1-1: How can we diagnose CKD? Statement: CKD is defined as the presence of either of the conditions listed below lasting for more than 3 months. (Level: None, Grade: 1) (1) Findings suggesting kidney damage, i.e., abnormal findings in blood or urinary tests, imaging studies or pathological evaluations. In particular, evidence of proteinuria ≥ 0.15 g/gCr (albuminuria ≥ 30 mg/gCr) is important. (2) GFR < 60 mL/min/1.73 m 2 In clinical practice, eGFR is calculated by the following GFR equation adjusted for the Japanese: eGFR (mL/min/1.73 m 2) = 194 x Cr-1.094 x Age-0.287 (x 0.739 if female) Note: We recommend that serum creatinine (Cr) value (mg/dL) should be evaluated by the enzymatic assay method and rounded off to 2 decimal places. The Japanese GFR equation is applicable to adults aged 18 years or older. CQ 1-2: How can we evaluate the severity of CKD? Statement: We recommend that CKD severity should be evaluated by cause, GFR category, and degree of proteinuria/ Japanese Society of Nephrology published Evidence-based Clinical Practice Guidelines for CKD 2018 (in Japanese) in the Journal of Japanese Society of Nephrology (in press). This is the English digest version of the above guidelines.
Objective: To compare disease severity classification systems for 6-month outcome prediction in patients with antineutrophil cytoplasmic antibody-associated vasculitis (AAV).
Methods:Patients with newly diagnosed AAV from 53 tertiary institutions were enrolled. Six-month remission, overall survival, and end-stage renal disease (ESRD)-free survival were evaluated.Results: According to the European Vasculitis Study Group (EUVAS)-defined disease severity, the 321 enrolled patients were classified as follows: 14, localized; 71, early systemic; 170, generalized; and 66, severe disease. According to the rapidly progressive glomerulonephritis (RPGN) clinical grading system, the patients were divided as follows: 60, grade I; 178, grade II; 66, grade III; and 12, grade IV. According to the Five-Factor Score (FFS) 2009, 103, 109, and 109 patients had <1, 2, and ≥3 points, respectively. No significant difference in remission rates was found in any severity classification. The overall and ESRD-free survival rates significantly differed between grades I/II, III, and IV, regardless of renal involvement. Severe disease was a good predictor of 6-month overall and ESRD-free survival. The FFS 2009 was useful to predict 6-month ESRD-free survival but not overall survival.
Conclusions:The RPGN grading system was more useful to predict 6-month overall and ESRD-free survival than the EUVAS-defined severity or FFS 2009.
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