A Study of the Safety, Tolerability, Pharmacokinetics and Efficacy of Treatment With AP1189 in Patients With iMN and Severe Proteinuria

Inclusion Criteria:

• Written informed consent has been obtained prior to initiating any study-specificprocedures

• Male and female subjects, 18 to 85 years of age diagnosed with iMN within 6 monthsprior to inclusion

• Diagnosed as anti-PLA2-Receptor positive by local laboratory within 6 months priorto inclusion

• Severe proteinuria defined by a U-protein/creatinine ratio >3.0 g/g and/orU-albumin/creatinine ratio >2.0 g/g and a P-albumin below the lower normal limit

• eGFR > 30 ml/min/1.73m2

• Treated with ACE- inhibitors or angiotensin II receptor blocker for a minimum of 1months with a stable systemic arterial blood pressure OR treatment with ACEinhibitors and/or angiotensin receptor blocker was excluded or discontinued due tohypotension, intolerance or other side effect

Only Denmark and Norway:

• Females of child-bearing potential using reliable means of contraception or arepost-menopausal

• Females of childbearing potential with negative pregnancy test at screening andbaseline

Only Sweden:

• Post-menopausal women or women who are surgically sterilized.

Exclusion Criteria:

• Participation in any other study involving investigational drug(s) during the studyand within 4 weeks prior to study entry

• Clinicial findings that in the opinion of the investigator would suggestcondition(s) other than iMN as a major cause of severe proteinuria

• Major surgery within 8 weeks prior to screening or planned surgery within 1 monthfollowing randomization

• Blood pressure with systolic pressure above 160 mmHg and/or diastolic pressure above 100 mmHg despite antihypertensive treatment will in all cases be considered "uncontrolled"

• Treated with systemic corticosteroids, or other immune suppressive, or immunemodulating compounds within 4 weeks prior to screening and during the entiretreatment period and until the final visit

• Treated with rituximab within 12 months of screening

• Evidence of active malignant disease

• Uncontrolled disease states, such as asthma, psoriasis, or inflammatory boweldisease where flares are commonly treated with oral or parenteral corticosteroids

• Evidence of serious uncontrolled concomitant cardiovascular, nervous system,pulmonary, renal, hepatic, endocrine or gastrointestinal disease

• Pregnant women or nursing mothers

• History of alcohol, drug, or chemical abuse within the 6 months prior to screening

• Any condition that in the view of the investigator would suggest that the patient isunable to comply with study protocol and procedures

Only Sweden:

• Females of child-bearing potential.