Rare Nephrology News
Spotlight On
IgA nephropathy
IgA nephropathy is a kidney disorder caused by deposits of the protein immunoglobulin A (IgA) inside the glomeruli
Prevalence
Age of Onset
ICD-10
N02.8
Inheritance
Autosomal dominant
Autosomal recessive
Mitochondrial/Multigenic
X-linked dominant
X-linked recessive
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
Interest over time
Google searches
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
| Title | Description | Phases | Status | Interventions | More Information |
|---|---|---|---|---|---|
| Open-Label Extension Study of BION-1301 in IgA Nephropathy | This 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 nephropathy | Phase 2 | Recruiting | Drug: BION-1301 | More Info |
| Study of Efficacy and Safety of LNP023 in Primary IgA Nephropathy Patients | The 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 3 | Recruiting | Drug: Placebo|Drug: LNP023 | More Info |
| A Study of Telitacicept for Injection (RC18) in Subjects With IgA Nephropathy | This 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 2 | Recruiting | Drug: Telitacicept 160mg|Drug: Telitacicept 240mg|Drug: Placebo | More Info |
| Atacicept in Subjects With IgA Nephropathy | The objective of the study is to evaluate the effect of atacicept compared to placebo on change in proteinuria in adult subjects with IGAN. | Phase 2 | Recruiting | Biological: Atacicept|Other: Placebo to match Atacicept | More Info |
| Atrasentan in Patients With IgA Nephropathy | The 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 3 | Recruiting | Drug: Atrasentan|Drug: Placebo | More Info |
| Efficacy and Safety of Nefecon in Patients With Primary IgA (Immunoglobulin A) Nephropathy | The 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 3 | Active, not recruiting | Drug: Nefecon|Drug: Placebo oral capsule | More Info |
| Study of the Safety and Efficacy of OMS721 in Patients With Immunoglobulin A (IgA) Nephropathy | The 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 3 | Recruiting | Biological: OMS721|Other: Vehicle (D5W or saline) | More Info |
| A Study of the Effect and Safety of Sparsentan in the Treatment of Patients With IgA Nephropathy | To 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 3 | Active, not recruiting | Drug: sparsentan|Drug: irbesartan | More 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 2 | Recruiting | Drug: BION-1301 Single Dose|Drug: Placebo Single Dose|Drug: BION-1301 Multiple Doses|Drug: Placebo Multiple Doses | More Info |
| Clinical Trial to Assess Efficacy and Safety of the Human Anti-CD38 Antibody Felzartamab (MOR202) in IgA Nephropathy | Randomized, placebo-controlled, multi-center, double-blind, proof of concept phase IIa trial and dose evaluation trial of felzartamab in IgAN | Phase 2 | Recruiting | Drug: Felzartamab|Other: Placebo | More Info |
Top Treatments in Research
| Agent | Class/Mechanism of Action | Development Status | Company | Clinical Studies | More Information |
|---|---|---|---|---|---|
| BION-1301 | An investigational humanized IgG4 monoclonal antibody that blocks APRIL binding to both the BCMA and TACI receptors, | Phase 2 | Chinook Therapeutics, Inc. | More Info | More Info |
| LNP023 (iptacopan) | Iptacopan (LNP023) is an oral, first-in-class, potent and selective small-molecule inhibitor of factor B (FB) | Phase 3 | Novartis | More Info | More Info |
| Telitacicept | Telitacicept (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 2 | Remegen | More Info | More Info |
| Atacicept | Atacicept (TACI-Ig) is a recombinant fusion protein of the extracellular domain of TACI and the human IgG1.Fc domain | Phase 2 | Vera Therapeutics, Inc. | More Info | More Info |
| Atrasentan | Atrasentan is a potent and selective, small molecule inhibitor of the endothelin A receptor | Phase 3 | Chinook Therapeutics, Inc. | More Info | More Info |
| OMS721 | Narsoplimab (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 3 | Omeros | More Info | More Info |
| sparsentan | Sparsentan 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) receptors | Phase 3 | Travere | More Info | More Info |
| Felzartamab | Felzartamab (MOR202) is a therapeutic human monoclonal antibody derived from MorphoSys' HuCAL antibody library and directed against CD38. | Phase 2 | Morphosys | More Info | More Info |
| VIS649 | VIS649 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 2 | Visterra | More Info | More Info |
| Ravulizumab | Ravulizuma, is a humanized monoclonal antibody complement inhibitor medication designed to bind to and prevent the activation of Complement component 5 | Phase 2 | Alexion | More Info | More Info |
| APL-2 | APL-2 is an (pegcetacoplan), investigational C3-targeted inhibitor | Phase 2 | Apellis | More Info | More Info |