A Study With Imlifidase in Anti-GBM Disease

Inclusion Criteria:

1. Anti-GBM antibodies constituting an indication for PLEX as judged by theInvestigator

2. Haematuria on dipstick and/or urinary sediment

3. eGFR(MDRD) <20 mL/min/1.73 m^2

4. Patients aged ≥18 years

5. Willing and able to give written Informed Consent and to comply with therequirements of the study protocol

Exclusion Criteria:

1. Diagnosis of anti-GBM disease more than 14 days prior to randomisation

2. Anuria during the last 24-hour

3. Any constituent of SoC given more than 10 days prior to randomisation

4. IVIg within 4 weeks before randomisation

5. History or presence of any medical condition or disease which, in the opinion of theinvestigator, may place the patient at unacceptable risk, or jeopardise the purposeof the study

6. Patients previously randomised in the study

7. Unsuitable to participate in the trial for any other reason in the opinion of theinvestigator

8. Pregnancy or breast feeding

9. Contraception:

10. Men who are not vasectomised or abstinent or with a partner (of child-bearingpotential) not willing to use one of the highly effective contraceptives listedbelow from screening to 6 months following discontinuation of CYC

11. Men who are not willing to refrain from donating sperm from screening to 6months following discontinuation of CYC

12. Men who are not willing to use a condom during any form of sexual intercourse,regardless of a partner being of child-bearing potential from screening to 6months following discontinuation of CYC

13. Women of child-bearing potential not willing or not able to use at least onehighly effective contraceptive method from screening to 12 months followingdiscontinuation of CYC. In the context of this trial, a highly effective method is defined as those whichresult in low failure rate (i.e. less than 1% per year) when used consistently andcorrectly such as:

• combined (oestrogen and progestogen containing) hormonal contraceptionassociated with inhibition of ovulation (oral/intravaginal/transdermal)

• progestogen-only hormonal contraception associated with inhibition of ovulation (oral/injectable/implantable)

• intrauterine device (IUD)

• intrauterine hormone-releasing system (IUS)

• bilateral tubal occlusion

• vasectomised partner

• true abstinence: When this is in line with the preferred and usual lifestyle ofthe patient. [Periodic abstinence (such as calendar, ovulation, symptothermal,post-ovulation methods) and withdrawal are not acceptable methods ofcontraception]

10. Previous imlifidase treatment or known hypersensitivity to any of the excipients