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IgA nephropathy

IgA nephropathy is a kidney disorder caused by deposits of the protein immunoglobulin A (IgA) inside the glomeruli

Prevalence

Unknown

US Estimated

Unknown

Europe Estimated

Age of Onset

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ICD-10

N02.8

Inheritance

Autosomal dominant

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Autosomal recessive

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Mitochondrial/Multigenic

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X-linked dominant

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X-linked recessive

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Rare View

IgA nephropathy is a chronic glomerulonephritis characterized by the deposition of IgA immune complexes in glomeruli. The classic presentation for the non-aggressive form (in 40–50% of the cases) is episodic hematuria, which usually starts within a day or two of a non-specific upper respiratory tract infection. n 1968, Jean Berger (1930–2011), a pioneering French nephrologist, was the first to describe IgA deposition in this form of glomerulonephritis and therefore it is sometimes called Berger’s disease.

5 Facts you should know

FACT

1

Primary IgA nephropathy is characterized by deposition of the IgA antibody in the glomerulus

FACT

2

IgA nephropathy can occur at any age, even in childhood

FACT

3

It affects men 2-6 times more frequently than women

FACT

4

Asians and whites are more likely to be affected

FACT

5

In non-aggressive IgA nephropathy there is traditionally a slow progression to chronic kidney failure in 20–40% of cases

IgA nephropathy is also known as...

IgA nephropathy

Berger's disease, synpharyngitic glomerulonephritis, IGAN; Glomerulonephritis, IGA

What’s your Rare IQ?

What is the most common presenting symptom of IgA nephropathy?

Common signs & symptoms

Arthralgia

Joint pain

Autosomal dominant inheritance

Hematuria

Blood in urine

Hypertension

IgA deposition in the glomerulus

Nephritis

Kidney inflammation

Proteinuria

High urine protein levels

Purpura

Red or purple spots on the skin

Top Clinical Trials

TitleDescriptionPhasesStatusInterventionsMore Information
Open-Label Extension Study of BION-1301 in IgA NephropathyThis is an open-label extension (OLE) study to evaluate the safety, pharmacokinetics (PK), pharmacodynamics (PD), and clinical activity of BION-1301 in adults with IgA nephropathyPhase 2RecruitingDrug: BION-1301More Info
Study of Efficacy and Safety of LNP023 in Primary IgA Nephropathy PatientsThe study is designed as a multicenter, randomized, double-blind, placebo controlled study to demonstrate the superiority of LNP023 at a dose of 200 mg b.i.d. compared to placebo on top of maximally tolerated ACEi or ARB on reduction of proteinuria and slowing renal disease progression in primary IgA Nephropathy patients.Phase 3RecruitingDrug: Placebo|Drug: LNP023More Info
A Study of Telitacicept for Injection (RC18) in Subjects With IgA NephropathyThis is a Phase 2, multi-center, randomized, double-blind, placebo-controlled clinical study to evaluate the safety and efficacy of Telitacicept for Injection (RC18) in the treatment of IgA nephropathy.Phase 2RecruitingDrug: Telitacicept 160mg|Drug: Telitacicept 240mg|Drug: PlaceboMore Info
Atacicept in Subjects With IgA NephropathyThe objective of the study is to evaluate the effect of atacicept compared to placebo on change in proteinuria in adult subjects with IGAN.Phase 2RecruitingBiological: Atacicept|Other: Placebo to match AtaciceptMore Info
Atrasentan in Patients With IgA NephropathyThe ALIGN Study is a phase 3, double-blind, placebo-controlled study to compare the efficacy and safety of atrasentan to placebo in patients with IgA nephropathy (IgAN) at risk of progressive loss of renal function.Phase 3RecruitingDrug: Atrasentan|Drug: PlaceboMore Info
Efficacy and Safety of Nefecon in Patients With Primary IgA (Immunoglobulin A) NephropathyThe overall aim of the study is to evaluate the efficacy, safety, and tolerability of Nefecon 16 mg per day in the treatment of patients with primary IgAN (Immunoglobulin A nephropathy) at risk of progressing to end-stage renal disease (ESRD), despite maximum tolerated treatment with renin-angiotensin system (RAS) blockade using angiotensin converting enzyme inhibitors (ACEIs) or angiotensin II type I receptor blockers (ARBs).
Phase 3Active, not recruitingDrug: Nefecon|Drug: Placebo oral capsuleMore Info
Study of the Safety and Efficacy of OMS721 in Patients With Immunoglobulin A (IgA) NephropathyThe purpose of this study is to evaluate the safety and efficacy of OMS721 in patients with IgA nephropathy. The study will assess proteinuria by 24-hour urine protein excretion (UPE) in g/day at 36 weeks from beginning of treatment.
Phase 3RecruitingBiological: OMS721|Other: Vehicle (D5W or saline)More Info
A Study of the Effect and Safety of Sparsentan in the Treatment of Patients With IgA NephropathyTo determine the long-term (approximately 2 years) nephroprotective potential of treatment with sparsentan as compared to an angiotensin receptor blocker in patients with immunoglobulin A nephropathy (IgAN).
Phase 3Active, not recruitingDrug: sparsentan|Drug: irbesartanMore Info
Safety and Tolerability of BION-1301 in Healthy Volunteers and Adults With IgA Nephropathy (IgAN)Multicenter study designed to evaluate the safety, tolerability, pharmacokinetics (PK), and pharmacodynamics (PD) of BION-1301 in healthy volunteers and adults with IgA Nephropathy (IgAN).Phase 1|Phase 2RecruitingDrug: BION-1301 Single Dose|Drug: Placebo Single Dose|Drug: BION-1301 Multiple Doses|Drug: Placebo Multiple DosesMore Info
Clinical Trial to Assess Efficacy and Safety of the Human Anti-CD38 Antibody Felzartamab (MOR202) in IgA NephropathyRandomized, placebo-controlled, multi-center, double-blind, proof of concept phase IIa trial and dose evaluation trial of felzartamab in IgANPhase 2RecruitingDrug: Felzartamab|Other: PlaceboMore Info

Top Treatments in Research

AgentClass/Mechanism of ActionDevelopment StatusCompanyClinical StudiesMore Information
BION-1301An investigational humanized IgG4 monoclonal antibody that blocks APRIL binding to both the BCMA and TACI receptors,Phase 2Chinook Therapeutics, Inc.More InfoMore Info
LNP023 (iptacopan)Iptacopan (LNP023) is an oral, first-in-class, potent and selective small-molecule inhibitor of factor B (FB)Phase 3NovartisMore InfoMore Info
TelitaciceptTelitacicept (RC18) is a first-in-class TACI-Fc fusion protein for injection which targets two important cell-signaling molecules, B-cell lymphocyte stimulator (BLyS) and A proliferation inducing ligand (APRIL).Phase 2RemegenMore InfoMore Info
AtaciceptAtacicept (TACI-Ig) is a recombinant fusion protein of the extracellular domain of TACI and the human IgG1.Fc domainPhase 2Vera Therapeutics, Inc.More InfoMore Info
AtrasentanAtrasentan is a potent and selective, small molecule inhibitor of the endothelin A receptorPhase 3Chinook Therapeutics, Inc.More InfoMore Info
OMS721Narsoplimab (OMS721) Narsoplimab is a human monoclonal antibody targeting mannan-binding lectin-associated serine protease-2 (MASP-2), the effector enzyme of the lectin pathway of the complement system. Phase 3OmerosMore InfoMore Info
sparsentanSparsentan is a first-in-class, orally active, single molecule that functions as a high affinity dual-acting antagonist of both endothelin type A (ETA) and angiotensin II subtype 1 (AT1) receptorsPhase 3TravereMore InfoMore Info
FelzartamabFelzartamab (MOR202) is a therapeutic human monoclonal antibody derived from MorphoSys' HuCAL antibody library and directed against CD38.Phase 2MorphosysMore InfoMore Info
VIS649VIS649 is a humanized immunoglobulin G (IgG2) monoclonal antibody that binds to and blocks the biological actions of the cytokine A PRoliferation Inducing Ligand (APRIL)Phase 2VisterraMore InfoMore Info
RavulizumabRavulizuma, is a humanized monoclonal antibody complement inhibitor medication designed to bind to and prevent the activation of Complement component 5Phase 2AlexionMore InfoMore Info
APL-2APL-2 is an (pegcetacoplan), investigational C3-targeted inhibitorPhase 2ApellisMore InfoMore Info