g logo ipb green

Overview

This Guidelines  summary provides clinical guidance for primary care on the management of glomerular diseases in people of all ages. The full guideline has been separated into a series of summaries, as follows:

This summary covers the diagnosis of further glomerular diseases not already covered in parts 1 and 2. For recommendations and good practice points on general management of glomerular disease, see part 1. For recommendations and good practice points on diagnosis and management of IgAN, IgAV, membranous nephropathy, and NS in children, see part 2.

This summary consists of recommendations and practice points. Recommendations are presented in bold. Within each recommendation in this summary, the strength of the recommendation is indicated as level 1 (recommended) or 2 (suggested), and the quality of the supporting evidence is shown as A (high), B (moderate), C (low), or D (very low)—further information can be found on page 14 of the full guideline. 

For a complete set of recommendations and practice points, refer to the full guideline.

KDIGO=Kidney Disease: Improving Global Outcomes

View this summary online at guidelines.co.uk/456746.article

Minimal change disease in adults

Diagnosis

  • MCD in adults can be diagnosed only with a kidney biopsy.

Prognosis

  • Long-term kidney survival is excellent in patients with MCD who respond to glucocorticoids, but less certain for patients who do not respond. 

Treatment

  • KDIGO recommends high-dose oral glucocorticoids for initial treatment of MCD [1C]
  • For an algorithm on the initial treatment of MCD in adults, see Algorithm 1.

Algorithm 1: Algorithm for the initial treatment of minimal change disease in adults

alg 1 initial treatment of minimal change disease v2

The optimal glucocorticoid regimen is not well-defined; however, suggested doses are outlined in Figure 45 of the full guideline. The choice of medication should be based on physician and patient preference.

  • High-dose glucocorticoid treatment for MCD should be given for no longer than 16 weeks
  • Begin tapering of glucocorticoids 2 weeks after complete remission
  • Although daily oral glucocorticoids are used most often to treat MCD, the route and frequency of administration can be individualised to patient needs
  • For patients in whom glucocorticoids may be relatively contraindicated, consider initial therapy with cyclophosphamide, a calcineurin inhibitor (CNI), or mycophenolate mofetil (MMF).

Treatment of relapses

For more information on treatment of relapses, see Figures 46 and 47 in the full guideline.

  • Treat infrequent relapses with glucocorticoids
  • KDIGO recommends cyclophosphamide, rituximab, CNIs, or mycophenolic acid analogues (MPAA) for the treatment of frequently relapsing/steroid-dependent MCD, rather than prednisone alone or no treatment. [1C]

Focal segmental glomerulosclerosis in adults

Diagnosis

Differentiating between primary and secondary FSGS

  • Adults with FSGS who do not have NS should be evaluated for a secondary cause (see Figures 51 and 52 in the full guideline). 

Genetic testing  

  • Genetic testing may be beneficial for selected patients with FSGS who should be referred to specialised centres with such expertise (see Figure 53 in the full guideline).

Treatment

Management of FSGS of undetermined cause and secondary FSGS

  • Immunosuppression should not be used in adults with FSGS of undetermined cause, or in those with secondary FSGS.

Initial treatment of primary FSGS

  • KDIGO recommends that high-dose oral glucocorticoids are used as the first-line immunosuppressive treatment for primary FSGS [1D]
  • Suggested dosing schedule for glucocorticoids in the initial treatment of primary FSGS is outlined in Table 1
  • Initial high-dose glucocorticoids should be continued until complete remission is achieved, or as tolerated by patients up to a maximum of 16 weeks, whichever is earlier
  • Adults with primary FSGS who respond to glucocorticoid treatment should receive glucocorticoids for 6 months or more
  • In adults with relative contraindications or intolerance to glucocorticoids, alternative immunosuppression with CNIs should be considered as the initial therapy in patients with primary FSGS (Table 1).

Table 1: Initial treatment of primary focal segmental glomerulosclerosis 

Treatment Dose and duration
Glucocorticoids Starting dose:
  • high-dose glucocorticoid therapy with prednisone at daily single doses of 1 mg/kg (maximum 80 mg) or alternate-day dose of 2 mg/kg (maximum 120 mg)
High-dose glucocorticoid treatment duration:
  • continue high-dose glucocorticoid therapy for at least 4 weeks and until complete remission is achieved, or a maximum of 16 weeks, whichever is earlier
  • patients who are likely to remit will show some degree of proteinuria reduction before 16 weeks of high-dose treatment
  • it may not be necessary to persist with glucocorticoid therapy until 16 weeks if the proteinuria is persistent and unremitting, especially in patients who are experiencing side effects
Glucocorticoid tapering:
  • if complete remission is achieved rapidly, continue high-dose glucocorticoid treatment for 2 weeks or after the disappearance of proteinuria, whichever is longer. Reduce prednisone by 5 mg every 1–2 weeks to complete a total duration of 6 months
  • if partial remission is achieved within 8–12 weeks of high-dose glucocorticoid treatment, continue until 16 weeks to ascertain whether further reduction of proteinuria and complete remission may occur. Thereafter, reduce the dose of prednisone by 5 mg every 1–2 weeks to complete a total duration of 6 months
  • if the patient proves to be steroid-resistant or develops significant toxicities, glucocorticoids should be rapidly tapered as tolerated and treatment with alternative immunosuppression like a CNI should be considered
Calcineurin inhibitors[A] Starting dose:
  • ciclosporine 3–5 mg/kg/day in two divided doses OR tacrolimus 0.05–0.1 mg/kg/day in two divided doses
  • target trough levels could be measured to minimise nephrotoxicity
  • ciclosporine target trough level: 100­–175 ng/ml (83–146 nmol/l)
  • tacrolimus target trough level: 5–10 ng/ml (6–12 nmol/l)
Treatment duration for determining CNI efficiency:
  • ciclosporine or tacrolimus should be continued at doses achieving target trough level for at least 4–6 months, before considering the patient to be resistant to CNI treatment
Total CNI treatment duration:
  • in patients with partial or complete remissions, ciclosporine or tacrolimus should be continued at doses achieving target trough level for at least 12 months to minimise relapses
  • the dose of ciclosporine or tacrolimus can be slowly tapered down over a course of 6–12 months as tolerated

[A] The CNI, while often used twice daily, may be dosed once a day, depending on individual formulations. Blood levels of CNIs do not provide information on intracellular levels. The target ranges for CNIs have been based on the transplant literature. The KDIGO Work Group acknowledges that targets for glomerular diseases are not known. Most clinicians check these levels to verify adherence and avoid CNI toxicity. At present, the most reasonable dosing of a CNI may be to titrate in the individual patient to obtain the desired effect on proteinuria, balancing dose escalation against serum creatinine and reducing the dose if serum creatinine increases but does not plateau or increases over 30% of baseline. If the serum creatinine level does not fall after dose reduction, the CNI should be discontinued

Abbreviations: CNI=calcineurin inhibitor; FSGS=focal segmental glomerulosclerosis; KDIGO=Kidney Disease: Improving Global Outcomes

Special situations

Steroid-resistant primary FSGS

  • For adults with steroid-resistant primary FSGS, KDIGO recommends that ciclosporine or tacrolimus is given for 6 months or longer rather than continuing with glucocorticoid monotherapy or not treating. [1C]

Dosing schedule for ciclosporine and tacrolimus

Treatment of steroid-resistant primary FSGS: for the suggested dosing schedule for ciclosporine and tacrolimus, see Figure 55 in the full guideline.

Duration of CNI treatment

  • Adults with steroid-resistant primary FSGS who respond to CNI treatment should receive CNIs for a minimum of 12 months to minimise the risk of relapses (see Figure 55 in the full guideline).

Patients resistant to or intolerant of CNIs

  • Adults who have steroid-resistant primary FSGS with resistance to or intolerance of CNIs should be referred to specialised centres for consideration of rebiopsy, alternative treatment, or enrolment in a clinical trial (see Figure 55 in the full guideline). 

Management of relapse

  • Adults with previous steroid-sensitive primary FSGS who experience a relapse can be treated using the same approach as that for adults with relapsing MCD (see Figure 47 in the full guideline).

Infection-related glomerulonephritis

Bacterial infection-related glomerulonephritis

Diagnosis

  • Kidney biopsy can be useful in suspected bacterial infection-related GN, particularly when culture evidence of infection is elusive or the diagnosis is in doubt, to assess prognosis, and/or for potential therapeutic reasons; in some cases, biopsy may be critical for arriving at the correct diagnosis, as comorbidities may contribute to confounding effects (see Figure 56 in the full guideline).

Prognosis and treatment

For more information on prognosis and suggested therapy of bacterial infection-related GN, see Figure 57 in the full guideline.

Viral infection-related GN

Hepatitis C virus infection-related GN

Hepatitis B virus infection-related GN

Diagnosis

  • Patients with proteinuric glomerular disease should undergo testing for hepatitis B virus (HBV) infection.

Prognosis

  • Adult patients with chronic HBV infection should be considered at risk for the development of kidney failure.

Treatment

  • KDIGO recommends that patients with replicative HBV infection (as denoted by HBV DNA levels greater than 2000 IU/ml) and GN receive treatment with nucleos(t)ide analogues as recommended for the general population by standard clinical practice guidelines for HBV infection [1C]
  • Pegylated interferon regimens should not be used to treat patients with replicative HBV infection and GN
  • Immunosuppressive agents, such as cyclophosphamide or rituximab, may accelerate HBV replication and should be avoided in patients with untreated replicative HBV infection and GN.

Special situations

  • Rituximab and cyclophosphamide should be avoided in patients with simultaneous HBV infection and anti-phospholipase A2 receptor (anti-PLA2R) antibody-mediated membranous nephropathy (MN) until a sustained virologic remission has been obtained by nucleos(t)ide analogue therapy
  • Plasma exchange may be tried in patients with accompanying cryoglobulinaemic vasculitis
  • Children with HBV infection and MN should be managed conservatively without immunosuppression due to a high likelihood of spontaneous remission of the kidney disease. 

HIV-related GN

Diagnosis

  • A kidney biopsy should be performed, when feasible, to evaluate the morphology of HIV-related kidney disease; a pathology-based description of HIV-related kidney disease should be used to help define and guide therapy.

Prognosis

  • The factors contributing to the long-term outcome of HIV infection associated with GN are numerous and include persistence of viral replication, response to antiviral treatment, genetic predisposition to glomerular injury (for example, APOL1 risk alleles), co-infection with other viruses, and development of immune complex disease or thrombotic microangiopathy; thus, the estimation of prognosis in individual patients can be very difficult.

Treatment

  • KDIGO recommends that antiretroviral therapy is initiated in all patients with HIV and chronic kidney disease, especially biopsy-proven HIV-associated nephropathy (HIVAN), regardless of CD4 count, adjusted to the degree of kidney function [1C]
  • A decision for the use of glucocorticoids as an adjunct therapy for HIVAN must be made on a case-by-case basis, as the risks and benefits long-term are uncertain.

Nephropathies due to infections with schistosomiasis, filariasis, and malaria

Schistosomal nephropathy

Diagnosis

  • Test for appropriate endemic co-infections (Salmonella, HBV, hepatitis C virus [HCV], HIV), as targeted treatment may alter the aggressiveness of an underlying GN or the sequelae of schistosomiasis
  • Obtain a kidney biopsy in patients suspected of having schistosomal GN in the presence of a viral co-infection (HCV, HBV, HIV).

Treatment

  • Treat patients with schistosomal infection and GN with an appropriate antiparasitic agent in sufficient dosage and duration to eradicate the organism; there are no indications for use of immunosuppressive agents in schistosomal nephropathy.

Special situations

  • Monitor patients with hepatic fibrosis from schistosomiasis for the development of kidney disease
  • Evaluate patients with a history of schistosomiasis and an elevated serum creatinine (SCr) and/or haematuria for bladder cancer and/or urinary obstruction.

Filariasis and glomerular disease

Treatment

  • Treat patients with filarial infection and GN with an appropriate antiparasitic agent in sufficient dosage and duration to eradicate the organism.

Malarial nephropathy

Treatment

  • Treat patients with malarial infection and GN with an appropriate antiparasitic agent in sufficient dosage and duration to eradicate the organism from blood and hepatosplenic sites; there are no indications for use of immunosuppressive agents in malarial nephropathy.

Immunoglobulin- and complement-mediated glomerular diseases with a membranoproliferative glomerulonephritis pattern of injury

Diagnosis

  • Evaluate patients with immune complex-mediated GN (ICGN) for underlying disease (see Figure 68 in the full guideline)
  • Evaluate patients with GN and monoclonal immunoglobulin deposits for a haematological malignancy
  • If no underlying aetiology is found for ICGN after extensive workup, evaluate for both complement dysregulation and drivers of complement dysregulation (see Figure 70 in the full guideline)
  • Rule out infection-related GN or post-infectious GN prior to assigning the diagnosis of C3 glomerulopathy (C3G)
  • Evaluate for the presence of a monoclonal protein in patients who present for the first time with a C3G diagnosis at 50 years of age or older (see Figure 69 in the full guideline). 

Treatment

Immune complex-related glomerulonephritis

  • When the cause of ICGN is determined, the initial approach to treatment should focus on the underlying pathologic process
  • Indolent ICGN, whether idiopathic or linked to a primary disease process, is best managed with supportive care and carefully considered use of immunosuppression
  • For patients with idiopathic ICGN and proteinuria under 3.5 g/day, the absence of the nephrotic syndrome, and a normal estimated glomerular filtration rate (eGFR), KDIGO suggests supportive therapy with renin–angiotensin system inhibition alone
  • For patients with idiopathic ICGN, a nephrotic syndrome, and normal or near-normal SCr, try a limited treatment course of glucocorticoids[A]
  • For patients with idiopathic ICGN, abnormal kidney function (but without crescentic involvements), active urine sediment, with or without nephrotic-range proteinuria, add glucocorticoids and immunosuppressive therapy to supportive care[A]
  • For patients presenting with a rapidly progressive crescentic idiopathic ICGN, treat with high-dose glucocorticoids and cyclophosphamide
  • For most patients with idiopathic ICGN presenting with an eGFR less than 30 ml/min per 1.73 m2, treat with supportive care alone
  • Patients who fail to respond to the treatment approaches discussed in the fourth and fifth practice points in this section should be considered for a clinical trial where available.

C3 glomerulopathy

  • In the absence of a monoclonal gammopathy, C3G in patients with moderate-to-severe disease should be treated initially with MMF plus glucocorticoids, and if this fails, eculizumab should be considered; patients who fail to respond to the treatment approaches discussed in Practice Point 8.2.2.1 of the full guideline should be considered for a clinical trial where available.

ANCA-associated vasculitis

Diagnosis

  • In the case of a clinical presentation compatible with small-vessel vasculitis in combination with positive myeloperoxidase- or proteinase 3-ANCA serology, waiting for a kidney biopsy to be performed or reported should not delay starting immunosuppressive therapy, especially in patients who are rapidly deteriorating (see Figure 71 in the full guideline)
  • Patients with AAV should be treated at centres with experience in AAV management.

Prognosis

Relapses

  • The persistence of ANCA positivity, an increase in ANCA levels, and a change in ANCA from negative to positive are only modestly predictive of future disease relapse and should not be used to guide treatment decisions. 

Treatment

Induction

  • KDIGO recommends that glucocorticoids in combination with cyclophosphamide or rituximab are used as initial treatment of new-onset AAV [1B]
  • A recommended treatment algorithm for AAV with kidney involvement is given in Algorithm 2
  • In patients presenting with markedly reduced or rapidly declining glomerular filtration rate (GFR; SCr over 4 mg/dl [over 354 mcmol/l]), there are limited data to support rituximab and glucocorticoids—cyclophosphamide and glucocorticoids are preferred for induction therapy; the combination of rituximab and cyclophosphamide can also be considered in this setting
  • Considerations for choosing between rituximab and cyclophosphamide for induction therapy are given in Figure 77 in the full guideline
  • Considerations for choosing the route of administration of cyclophosphamide are given in Figure 78 in the full guideline
  • Discontinue immunosuppressive therapy after 3 months in patients who remain on dialysis and who do not have any extrarenal manifestations of disease
  • Recommendations for oral glucocorticoid tapering are given in Table 2
  • Recommendations for immunosuppressive dosing are given in Table 3
  • Consider plasma exchange for patients with an SCr greater than 5.7 mg/dl (500 mcmol/l) requiring dialysis or with rapidly increasing SCr, and in patients with diffuse alveolar haemorrhage who have hypoxaemia
  • Add plasma exchange for patients with an overlap syndrome of ANCA vasculitis and anti-GBM antibody GN.

alg 2 recommneded treatment regimen for AAV v3

Table 2: Prednisolone tapering regimen for ANCA-associated vasculitis

Week‘Reduced corticosteroid dose’ in PEXIVAS trial
  <50 kg 50–75 kg >75 kg
1 50 60 75
2 25 30 40
3–4 20 25 30
5–6 15 20 25
7–8 12.5 15 20
9–10 10 12.5 15
11–­12 7.5 10 12.5
13–14 6 7.5 10
15–16 5 5 7.5
17–18 5 5 7.5
19–20 5 5 5
21–22 5 5 5
23–52 5 5 5
>52 Investigators’ local practice

Table 3: Immunosuppressive drug dosing for ANCA-associated vasculitis

Oral cyclophosphamideIV cyclophosphamideRituximabRituximab and IV cyclophosphamideMMF

2 mg/kg/day for 3 months, continue for ongoing activity to a maximum of 6 months

15 mg/kg at weeks 0, 2, 4, 7, 10, 13 (16, 19, 21, 24 if required)

375 mg/m2/week x4 weeks OR
1 g at weeks 0 and 2

Rituximab 375 mg/m2/week x4 weeks, with IV cyclophosphamide 15 mg/kg at weeks 0 and 2 OR
Rituximab 1 g at 0 and 2 weeks with cyclophosphamide 500 mg/2 weeks x6

2000 mg/day (divided doses), may be increased to 3000 mg/day for poor treatment response

Reduction for age:

  • 60 years: 1.5 mg/kg/day
  • 70 years: 1.0 mg/kg/day

Reduce by 0.5 mg/kg/day for GFR <30 ml/min/1.73 m2

Reduction for age:

  • 60 years: 12.5 mg/kg

  • 70 years: 10 mg/kg

Reduce by 2.5 mg/kg/day for GFR <30 ml/min/1.73 m2

 

 

 

Abbreviations: GFR=glomerular filtration rate; IV=intravenous; MMF= mycophenolate motefil

Maintenance therapy

  • KDIGO recommends maintenance therapy with either rituximab or azathioprine and low-dose glucocorticoids after induction of remission [1C]
  • Following cyclophosphamide induction, either azathioprine plus low-dose glucocorticoids or rituximab without glucocorticoids should be used to prevent relapse
  • Following rituximab induction, maintenance immunosuppressive therapy should be given to most patients
  • The optimal duration of azathioprine plus low-dose glucocorticoids is not known, but should be between 18 months and 4 years after induction of remission
  • The optimal duration of rituximab maintenance is not known, but studies to date have evaluated a duration of 18 months after remission; there is no role for the routine use of an oral glucocorticoid or oral immunosuppressive with rituximab maintenance
  • When considering withdrawal of maintenance therapy, the risk of relapse should be considered, and patients should be informed of the need for prompt attention if symptoms recur (see Figure 82 in the full guideline)
  • Consider methotrexate for maintenance therapy in patients, after induction with methotrexate or for those who are intolerant of azathioprine and MMF, but not if GFR is less than 60 ml/min per 1.73 m2
  • Considerations for choosing rituximab or azathioprine for maintenance therapy are presented in Figure 83 in the full guideline
  • Recommendations for dosing and duration of maintenance therapy are given in Figure 84 in the full guideline.

Relapsing disease

  • Patients with relapsing disease (life- or organ-threatening) should be reinduced (recommendation 9.3.1.1 of the full guideline), preferably with rituximab.

Special situations

For information on refractory disease and transplantation, refer to the full guideline. 

Lupus nephritis

Diagnosis

  • For an algorithm on the approach to the diagnosis of kidney involvement in systemic lupus erythematosus (SLE), see Figure 85 in the full guideline. 

Treatment

General management of patients with lupus nephritis

  • KDIGO recommends that patients with SLE, including those with LN, are treated with hydroxychloroquine or an equivalent antimalarial unless contraindicated [1C]
  • Adjunctive therapies to manage LN and attenuate complications of the disease or its treatments should be considered for all patients, as outlined in Figure 87 in the full guideline. 

Class I or II LN

  • For an algorithm on the approach to immunosuppressive treatment for patients with Class I or Class II LN, see Figure 88 in the full guideline. 

Class III or IV LN

Initial therapy of active Class III or IV LN

  • KDIGO recommends that patients with active Class III or IV LN, with or without a membranous component, are treated initially with glucocorticoids plus either low-dose intravenous cyclophosphamide or MPAA [1B]
  • A regimen of reduced-dose glucocorticoids following a short course of methylprednisolone pulses may be considered during the initial treatment of active LN when both the kidney and extrarenal disease manifestations show satisfactory improvement (for example glucocorticoid regimens for LN, see Figure 90 in the full guideline)
  • Intravenous cyclophosphamide should be used as the initial therapy for active Class III and Class IV LN in patients who may have difficulty adhering to an oral regimen
  • An MPAA-based regimen is the preferred initial therapy of proliferative LN for patients at high risk of infertility, patients who have a moderate-to-high prior cyclophosphamide exposure, and patients of Asian, Hispanic, or African ancestry
  • Initial therapy with a triple immunosuppressive regimen that includes a CNI (tacrolimus or ciclosporine) with reduced-dose MPAA and glucocorticoids is reserved for patients who cannot tolerate standard-dose MPAA or are unsuited for or will not use cyclophosphamide-based regimens
  • In patients with baseline eGFR of at least 45 ml/min per 1.73 m2, voclosporin can be added to MPAA and glucocorticoids as initial therapy for 1 year
  • There is an emerging role for B-lymphocyte targeting biologics in the treatment of LN—belimumab can be added to standard therapy in the treatment of active LN; rituximab may be considered for patients with persistent disease activity or repeated flares
  • Other therapies, such as azathioprine or leflunomide combined with glucocorticoids, may be considered in lieu of the recommended initial drugs for proliferative LN in situations of patient intolerance, lack of availability, and/or excessive cost of standard drugs, but these alternatives may be associated with inferior efficacy, including increased rate of disease flares and/or increased incidence of drug toxicities. 

Maintenance therapy for Class III and IV LN

  • KDIGO recommends that after completion of initial therapy, patients should be placed on MPAA for maintenance [1B]
  • Azathioprine is an alternative to MPAA after completion of initial therapy in patients who do not tolerate MPAA, who do not have access to MPAA, or who are considering pregnancy
  • Glucocorticoids should be tapered to the lowest possible dose during maintenance, except when glucocorticoids are required for extrarenal lupus manifestations; discontinuation of glucocorticoids can be considered after patients have maintained a complete clinical renal response for 12 months or longer
  • The dose of MMF in the early maintenance phase is approximately 750–1000 mg twice daily, and for MPA, approximately 540–720 mg twice daily
  • If MPAA and azathioprine cannot be used for maintenance, CNIs or mizoribine should be considered
  • The total duration of initial immunosuppression plus combination maintenance immunosuppression for proliferative LN should not be less than 36 months.

Class V LN

  • A suggested approach to the management of patients with pure Class V LN is described in Figure 94 in the full guideline.

Assessing treatment response in LN

  • Definitions of response to therapy in LN are provided in Figure 95 of the full guideline.

Management of unsatisfactory response to treatment

  • An algorithmic approach to patients whose response to therapy is deemed unsatisfactory is provided in Figure 96 of the full guideline.

Treatment of LN relapse

  • After a complete or partial remission has been achieved, LN relapse should be treated with the same initial therapy used to achieve the original response, or an alternative recommended first-line therapy.

Special situations

LN and thrombotic microangiopathy

  • Patients with LN and thrombotic microangiopathy (TMA) should be managed according to the underlying aetiology of TMA, as shown in Figure 97 in the full guideline.

Pregnancy in patients with LN

  • Patients with active LN should be counselled to avoid pregnancy while the disease is active or when treatment with potentially teratogenic drugs is ongoing, and for 6 months or more after LN becomes inactive
  • To reduce the risk of pregnancy complications, hydroxychloroquine should be continued during pregnancy, and low-dose aspirin should be started before 16 weeks of gestation
  • Only glucocorticoids, hydroxychloroquine, azathioprine, and CNIs are considered safe immunosuppressive treatments during pregnancy.

Treatment of LN in children

  • Treat paediatric patients with LN using immunosuppression regimens similar to those used in adults, but consider issues relevant to this population, such as dose adjustment, growth, fertility, and psychosocial factors, when devising the therapy plan.

Management of patients with LN and kidney failure

  • Patients with LN who develop kidney failure may be treated with haemodialysis, peritoneal dialysis, or kidney transplantation; kidney transplantation is preferred to long-term dialysis.

Antiglomerular basement membrane antibody glomerulonephritis

Diagnosis

  • Diagnosis of anti-GBM disease should be made without delay in all patients with suspected RPGN (see Figure 98 in the full guideline).

Treatment

  • KDIGO recommends initiating immunosuppression with cyclophosphamide and glucocorticoids plus plasmapheresis in all patients with anti-GBM GN except those who are treated with dialysis at presentation, have 100% crescents or greater than 50% global glomerulosclerosis in an adequate biopsy sample, and do not have pulmonary haemorrhage [1C]
  • Treatment for anti-GBM disease should start without delay if this diagnosis is suspected, even before the diagnosis is confirmed
  • Plasma exchange should be performed until anti-GBM titres are no longer detectable
  • Cyclophosphamide should be administered for 2–3 months and glucocorticoids for about 6 months (see Figure 99 in the full guideline)
  • No maintenance therapy of anti-GBM disease is necessary
  • Patients with GN who are anti-GBM- and ANCA-positive should be treated with maintenance therapy as for patients with AAV
  • In refractory anti-GBM disease, rituximab may be tried
  • Kidney transplantation in patients with kidney failure due to anti-GBM disease should be postponed until anti-GBM antibodies remain undetectable for 6 months or longer.

Footnote

[A] Patients who fail to respond to these treatment approaches discussed in Practice Points 8.2.1.4 and 8.2.1.5 of the full guideline should be considered for a clinical trial where available.

 

Full guideline:

Kidney Disease Improving Global Outcomes. KDIGO 2021 Clinical practice guideline for the management of glomerular diseases. Kidney Int 2021; 100 (4S): S1–S276. Available at: kdigo.org/wp-content/uploads/2017/02/KDIGO-Glomerular-Diseases-Guideline-2021-English.pdf  

Published date: October 2021.

Credit:

Lead image: Davizro Photography/stock.adobe.com

KDIGO general management of glomerular diseases guideline