KDIGO 2021 Clinical Practice Guideline for the Management of Glomerular Diseases

Abstract

S28 Introduction S30 Summary of recommendation statements and practice points S88 Chapter 1: General principles for the management of glomerular disease S115 Chapter 2: Immunoglobulin A nephropathy (IgAN)/immunoglobulin A vasculitis (IgAV) S128 Chapter 3: Membranous nephropathy S140 Chapter 4: Nephrotic syndrome in children S153 Chapter 5: Minimal change disease (MCD) in adults S161 Chapter 6: Focal segmental glomerulosclerosis (FSGS) in adults S172 Chapter 7: Infection-related glomerulonephritis S187 Chapter 8: Immunoglobulin-and complement-mediated glomerular diseases with a membranoproliferative glomerulonephritis (MPGN) pattern of injury S193 Chapter 9: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis S207 Chapter 10: Lupus nephritis S231 Chapter 11: Anti-glomerular basement membrane (Anti-GBM) antibody glomerulonephritis S235 Methods for guideline development S243 Biographic and disclosure information S254 Acknowledgments S256 ReferencesThis guideline is published as a supplement supported by KDIGO. The development and publication of this guideline are strictly funded by KDIGO, and neither KDIGO nor its guideline Work Group members sought or received monies or fees from corporate or commercial entities in connection with this work. The opinions or views expressed in this professional education supplement are those of the authors and do not necessarily reflect the opinions or recommendations of the International Society of Nephrology or Elsevier. Dosages, indications, and methods of use for products that are referred to in the supplement by the authors may reflect their clinical experience or may be derived from the professional literature or other clinical sources. Because of the differences between in vitro and in vivo systems and between laboratory animal models and clinical data in humans, in vitro and animal data may not necessarily correlate with clinical results.

Kidney biopsy-based epidemiologic analysis shows growing biopsy rate among the elderly

“…As a result, for an acceptable sample, at least 10 glomeruli are required 79 . This notion is supported by the recent guideline of the Kidney Disease: Improving Global Outcomes (KDIGO) organization on the management of glomerular diseases 80 . In case it is not yielded during the sampling, chances of missing the afflicted part of the kidney increase.…”

“…This rate varies between 0.3 and 8.5% in the international reports 9 , 22 , 36 , 47 , 48 , 57 , 61 , 70 . A biopsy is still the “gold standard” for the diagnostic evaluation of glomerular diseases and the biopsy should be performed when the value of the information obtained from the biopsy exceeds the risk entailed 80 . Most of the renal biopsy indications (e.g.…”

“…This correlation draws attention to the threshold for the execution of a renal biopsy: countries that perform a renal biopsy with more subtle clinical or laboratory abnormalities have a higher biopsy rate and increased number of negative histological results, while countries with low negative sample results and biopsy rate may interpret the indications rather strictly. In addition, local resources are also likely to determine the prevailing practice on performing kidney biopsies 80 .…”

How Times Have Changed! A Cornucopia of Antigens for Membranous Nephropathy

“…This has allowed providers in some situations to avoid the need to perform a kidney biopsy in patients who have with anti-PLA2R antibodies and rather to monitor them according to anti-PLA2R titers (204,205). This diagnostic strategy is now supported in the 2021 KDIGO Clinical Practice Guideline for the Management of Glomerular Diseases in not mandating a kidney biopsy to confirm PLA2R-associated MN in a seropositive patient (206). This is supported by our experience in which we see a lower frequency of PLA2R-positive kidney biopsies in our recent cohort, compared to historical studies suggesting that clinicians are already deciding not to biopsy patients diagnosed by the noninvasive serological test (33).…”

Hydroxychloroquine administration exacerbates acute kidney injury complicated by lupus nephritis

“…Over the past decade, growing clinical evidence has demonstrated that antimalarial drugs, mainly hydroxychloroquine (HCQ), show outstanding effectiveness and safety in the treatment of LN [ 4 ]. Several authoritative guidelines, such as KDIGO [ 5 , 6 ], and EULAR/ACR [ 7 ], recommend HCQ, in addition to corticosteroids and immunosuppressants, as a basic treatment for LN. Mechanistically, HCQ accumulates in lysosomes, elevating their pH values and resulting in impaired lysosomal proteolysis, which suppresses autoantigen presentation, inhibits endosomal Toll-like receptor signaling, and decreases proinflammatory cytokine production, to suppress immune cell-mediated inflammatory responses [ 8 , 9 ].…”