IGAN
MCID: IGN003
MIFTS: 40

Iga Nephropathy 1 (IGAN)

Categories: Genetic diseases, Immune diseases, Nephrological diseases

Aliases & Classifications for Iga Nephropathy 1

MalaCards integrated aliases for Iga Nephropathy 1:

Name: Iga Nephropathy 1 57
Berger Disease 57 76 29 6
Igan 57 75
Iga Nephropathy, Susceptibility to, Type 1 40
Iga Nephropathy, Susceptibility to, 1 57
Iga Glomerulonephritis 73
Nephritis, Iga Type 57
Iga Nephropathy 75
Igan1 57

Characteristics:

OMIM:

57
Inheritance:
?autosomal dominant

Miscellaneous:
histologic features overlap with henoch-schonlein purpura (hspn)
incidence 5-50 per million (children) and 10-40 per million (adults)


HPO:

32
iga nephropathy 1:
Inheritance heterogeneous autosomal dominant inheritance


Classifications:



Summaries for Iga Nephropathy 1

OMIM : 57 End-stage renal disease (ESRD) is a major public health problem, affecting 1 in 1,000 individuals and with an annual death rate of 20% despite dialysis treatment. IgA nephropathy (IgAN) is the most common form of glomerulonephritis, a principal cause of ESRD worldwide, affecting up to 1.3% of the population. Kidneys of patients with IgA nephropathy show deposits of IgA-containing immune complexes with proliferation of the glomerular mesangium. Typical clinical features include onset before age 40 with hematuria and proteinuria, and episodes of gross hematuria following mucosal infections are common; 30% of patients develop progressive renal failure. Although not generally considered a hereditary disease, striking ethnic variation in prevalence (Julian et al., 1985; D'Amico, 1987) and familial clustering (Scolari et al., 1999), along with subclinical renal abnormalities among relatives of cases, suggest a genetic component (Gharavi et al., 2000). (161950)

MalaCards based summary : Iga Nephropathy 1, also known as berger disease, is related to iga nephropathy 3 and iga nephropathy 2. An important gene associated with Iga Nephropathy 1 is IGAN2 (IgA Nephropathy, Susceptibility To, 2). The drugs Prednisolone phosphate and Methylprednisolone have been mentioned in the context of this disorder. Affiliated tissues include kidney, skin and liver, and related phenotypes are hypertension and proteinuria

UniProtKB/Swiss-Prot : 75 IgA nephropathy: Most common primary glomerulonephritis, which is partly due to aberrant or incomplete galactosylation of IgA1 molecules.

Wikipedia : 76 IgA nephropathy (IgAN), also known as IgA nephritis, Berger''s disease (/b�?�?r�?�?eɪ/) (and... more...

Related Diseases for Iga Nephropathy 1

Diseases in the Iga Nephropathy 1 family:

Iga Nephropathy 2 Iga Nephropathy 3

Diseases related to Iga Nephropathy 1 via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 226)
# Related Disease Score Top Affiliating Genes
1 iga nephropathy 3 12.6
2 iga nephropathy 2 12.3
3 hematuria, benign familial 11.2
4 loin pain hematuria syndrome 11.2
5 kidney disease 10.9
6 purpura 10.6
7 henoch-schoenlein purpura 10.5
8 nephrotic syndrome 10.3
9 focal segmental glomerulosclerosis 1 10.3
10 tonsillitis 10.3
11 membranous nephropathy 10.3
12 malignant hypertension 10.3
13 lipoid nephrosis 10.3
14 hepatitis 10.2
15 crescentic glomerulonephritis 10.2
16 vasculitis 10.2
17 chronic kidney failure 10.2
18 focal segmental glomerulosclerosis 10.2
19 crohn's disease 10.2
20 polycystic kidney disease 10.2
21 membranoproliferative glomerulonephritis 10.2
22 hemolytic-uremic syndrome 10.2
23 uveitis 10.2
24 interstitial nephritis 10.1
25 scleritis 10.1
26 rapidly progressive glomerulonephritis 10.1
27 psoriasis 10.1
28 fabry disease 10.1
29 hepatitis b 10.1
30 acute poststreptococcal glomerulonephritis 10.1
31 autosomal dominant polycystic kidney disease 10.1
32 end stage renal failure 10.0
33 glomerulonephritis 10.0
34 proliferative glomerulonephritis 10.0
35 mesangial proliferative glomerulonephritis 10.0
36 macrophagic myofasciitis 10.0
37 autoimmune disease 10.0
38 systemic lupus erythematosus 10.0
39 celiac disease 1 10.0
40 hemolytic uremic syndrome, atypical 1 10.0
41 panbronchiolitis, diffuse 10.0
42 microvascular complications of diabetes 3 10.0
43 microvascular complications of diabetes 4 10.0
44 microvascular complications of diabetes 6 10.0
45 microvascular complications of diabetes 7 10.0
46 diabetes mellitus 10.0
47 lymphoma 10.0
48 spondyloarthropathy 10.0
49 graves' disease 10.0
50 lymphadenitis 10.0

Graphical network of the top 20 diseases related to Iga Nephropathy 1:



Diseases related to Iga Nephropathy 1

Symptoms & Phenotypes for Iga Nephropathy 1

Symptoms via clinical synopsis from OMIM:

57
Cardiovascular Vascular:
hypertension

Laboratory Abnormalities:
proteinuria
hematuria, macroscopic and microscopic

Immunology:
serum circulating immune complexes contain galactose-deficient iga1

Genitourinary Kidneys:
proteinuria
hematuria, microscopic
hematuria, macroscopic (seen in children and young adults coincident with mucosal infection)
galactose-deficient iga1 deposits in the glomerular mesangium, with or without igm and igg
glomerular crescents (rare), seen on biopsy
more
Skeletal:
arthralgia (in some patients)


Clinical features from OMIM:

161950

Human phenotypes related to Iga Nephropathy 1:

32 (show all 8)
# Description HPO Frequency HPO Source Accession
1 hypertension 32 HP:0000822
2 proteinuria 32 HP:0000093
3 arthralgia 32 occasional (7.5%) HP:0002829
4 hematuria 32 HP:0000790
5 purpura 32 HP:0000979
6 stage 5 chronic kidney disease 32 HP:0003774
7 nephritis 32 HP:0000123
8 iga deposition in the glomerulus 32 HP:0000794

Drugs & Therapeutics for Iga Nephropathy 1

Drugs for Iga Nephropathy 1 (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 160)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Prednisolone phosphate Approved, Vet_approved Phase 4,Phase 2,Phase 3,Not Applicable 302-25-0
2
Methylprednisolone Approved, Vet_approved Phase 4,Phase 2,Phase 3,Not Applicable 83-43-2 6741
3
Prednisolone Approved, Vet_approved Phase 4,Phase 2,Phase 3,Not Applicable 50-24-8 5755
4
Methylprednisolone hemisuccinate Approved Phase 4,Phase 2,Phase 3,Not Applicable 2921-57-5
5
Enalapril Approved, Vet_approved Phase 4,Phase 2,Not Applicable 75847-73-3 5362032 40466924
6
Enalaprilat Approved Phase 4,Phase 2,Not Applicable 76420-72-9 6917719
7
rituximab Approved Phase 4 174722-31-7 10201696
8
Angiotensin II Approved, Investigational Phase 4,Phase 3,Not Applicable 11128-99-7, 68521-88-0, 4474-91-3 172198 65143
9
Losartan Approved Phase 4,Phase 3,Not Applicable 114798-26-4 3961
10
Mycophenolic acid Approved Phase 4,Phase 3,Not Applicable 24280-93-1 446541
11
Benzocaine Approved, Investigational Phase 4 94-09-7, 1994-09-7 2337
12 tannic acid Approved Phase 4
13
Prednisone Approved, Vet_approved Phase 4,Phase 3,Phase 2,Not Applicable 53-03-2 5865
14
Valsartan Approved, Investigational Phase 4 137862-53-4 60846
15
Bortezomib Approved, Investigational Phase 4 179324-69-7 387447 93860
16
Probucol Approved, Investigational Phase 4 23288-49-5 4912
17
Hydroxychloroquine Approved Phase 4,Phase 2 118-42-3 3652
18
Azathioprine Approved Phase 4,Phase 3 446-86-6 2265
19
Ramipril Approved Phase 4,Phase 3 87333-19-5 5362129
20
Cyclophosphamide Approved, Investigational Phase 4,Phase 3,Phase 2,Not Applicable 6055-19-2, 50-18-0 2907
21
Basiliximab Approved, Investigational Phase 4 152923-56-3, 179045-86-4
22
Calcitriol Approved, Nutraceutical Phase 4,Not Applicable 32222-06-3 5280453 134070
23
Serine Approved, Nutraceutical Phase 4 56-45-1 5951
24
Prednisolone hemisuccinate Experimental Phase 4,Phase 2,Phase 3,Not Applicable 2920-86-7
25
N,N-dimethylarginine Experimental Phase 4 30315-93-6 123831
26
Dexchlorpheniramine Experimental, Investigational Phase 4 25523-97-1 33036
27 Methylprednisolone acetate Phase 4,Phase 2,Phase 3,Not Applicable
28 Hormone Antagonists Phase 4,Phase 2,Phase 3,Not Applicable
29 Antihypertensive Agents Phase 4,Phase 3,Not Applicable
30 Immunoglobulin A Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable
31 Immunologic Factors Phase 4,Phase 2,Phase 3,Not Applicable
32 Antineoplastic Agents, Hormonal Phase 4,Phase 2,Phase 3,Not Applicable
33 Hormones Phase 4,Phase 2,Phase 3,Not Applicable
34 Antiemetics Phase 4,Phase 2,Phase 3,Not Applicable
35 Anti-Inflammatory Agents Phase 4,Phase 2,Phase 3,Not Applicable
36 HIV Protease Inhibitors Phase 4,Phase 3,Phase 2,Not Applicable
37 Peripheral Nervous System Agents Phase 4,Phase 2,Phase 3,Not Applicable
38 Neuroprotective Agents Phase 4,Phase 2,Phase 3,Not Applicable
39 Prednisolone acetate Phase 4,Phase 2,Phase 3,Not Applicable
40
protease inhibitors Phase 4,Phase 3,Phase 2,Not Applicable
41 Gastrointestinal Agents Phase 4,Phase 2,Phase 3,Not Applicable
42 Angiotensin-Converting Enzyme Inhibitors Phase 4,Phase 3,Phase 2,Not Applicable
43 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 4,Phase 2,Phase 3,Not Applicable
44 Antirheumatic Agents Phase 4,Phase 3,Phase 2,Not Applicable
45 glucocorticoids Phase 4,Phase 2,Phase 3,Not Applicable
46 Autonomic Agents Phase 4,Phase 2,Phase 3,Not Applicable
47 Protective Agents Phase 4,Phase 2,Phase 3,Not Applicable
48 Angiotensin II Type 1 Receptor Blockers Phase 4,Phase 3,Not Applicable
49 Anti-Arrhythmia Agents Phase 4,Phase 3,Not Applicable
50 Angiotensin Receptor Antagonists Phase 4,Phase 3,Not Applicable

Interventional clinical trials:

(show top 50) (show all 105)
# Name Status NCT ID Phase Drugs
1 Rituximab in Recurrent IgA Nephropathy Unknown status NCT02571842 Phase 4 Intravenous Rituximab;ACEI/ARB and corticosteroids
2 Efficacy and Safety of Abelmoschus Manihot for IgA Nephropathy Unknown status NCT02231125 Phase 4 Losartan;Abelmoschus manihot
3 Efficacy and Safety of a Combination of Mycophenolate Mofetil and Corticosteroid in Advanced IgA Nephropathy Unknown status NCT02981212 Phase 4 Mycophenolate Mofetil;ACE inhibitor;Corticosteroid;ARB
4 Calcitriol in the Treatment of Immunoglobulin A Nephropathy Unknown status NCT00862693 Phase 4 Calcitriol
5 A Prospective Study of the Kidney Protective Effect of Aliskiren in Hypertensive Patients With IgA Nephropathy Unknown status NCT01184599 Phase 4 aliskiren
6 Inhibition of the Renin Angiotensin System Plus Corticosteroids for the Treatment of Proteinuria in IGA Nephropathy Completed NCT00367562 Phase 4 ENALAPRIL VALSARTAN METHYLPREDNISONE
7 Mycophenolate Mofetil for IgA Nephropathy Completed NCT00863252 Phase 4 mycophenolate mofetil;angiotensin blockade
8 Pilot Study of Velcade® in IgA Nephropathy Completed NCT01103778 Phase 4 Bortezomib (Velcade®)
9 A Study of the Antioxidant Probucol Combined With Valsartan in Patients With IgA Nephropathy Completed NCT00426348 Phase 4 Valsartan;Probucol;Placebo
10 Hydroxychloroquine Sulfate for Reduction of Proteinuria in Patients With IgA Nephropathy: a Self- Controlled Study Completed NCT02351752 Phase 4 Hydroxychloroquine Sulfate
11 "Steroids and Azathioprine Versus Steroids Alone in IgAN" Completed NCT00755859 Phase 4 steroids plus azathioprine;steroids
12 Rituximab in Progressive Immunoglobulin A (IgA) Nephropathy Completed NCT00498368 Phase 4 Intravenous Rituximab;ACE/ARB
13 Calcitriol in the Treatment of Immunoglobulin A (IgA) Nephropathy Completed NCT00319761 Phase 4 Calcitriol
14 Aliskiren for Proteinuric IgAN Despite Angiotensin Blockade Completed NCT00922311 Phase 4 Aliskiren
15 Inhibitors of Angiotensin II in Proteinuric Mesangioproliferative Glomerulonephritis Completed NCT01115426 Phase 4 Ramipril or losartan
16 Treatment of Resistant Nephrotic Syndrome With ACTH Gel (ACTHAR) Completed NCT01129284 Phase 4 ACTHAR gel
17 Hydroxychloroquine Sulfate Alleviates Persistent Proteinuria in IgA Nephropathy Recruiting NCT02765594 Phase 4 Hydroxychloroquine Sulfate;Valsartan
18 Treatment of Prednisone Plus Cyclophosphamide in Patients With Advanced-stage IgA Nephropathy Active, not recruiting NCT01758120 Phase 4 prednisone plus cyclophosphamide;Prednisone alone
19 Extended Follow-up of Treatment of Prednisone Plus Cyclophosphamide in Patients With Advanced-stage IgA Nephropathy Active, not recruiting NCT03218852 Phase 4 prednisone and cyclophosphamide;prednisone alone
20 Prevention in Recipients With Primary IgA Nephropathy of Recurrence After Kidney Transplantation: ATG-F Versus Basiliximab as Induction Immunosuppressive Treatment Active, not recruiting NCT02523768 Phase 4 ATG-F;Simulect
21 Effect of Immunosuppression in IgA Nephropathy Not yet recruiting NCT03468972 Phase 4 Immunosuppressive treatment
22 Aldosterone Breakthrough During Diovan, Tekturna, and Combination Therapy in Patients With Proteinuric Kidney Disease Terminated NCT01129557 Phase 4 Aliskiren;Valsartan
23 Acthar on Proteinuria in IgA Nephropathy Patients Withdrawn NCT02382523 Phase 4 Acthar 80 unit injection
24 Validity and Security of Reh-acteoside Therapy for Patients of IgA Nephropathy Unknown status NCT02662283 Phase 2, Phase 3 Prednisolone;Reh-acteoside
25 Treatment of IgAN With Multi-glycoside of Tripterygium Wilfordii HOOK. f. Unknown status NCT02187900 Phase 3 Multi-glycoside of Tripterygium Wilfordii HOOK. f. (TWH);Mycophenolate mofetil (MMF)
26 BRIGHT-SC: Blisibimod Response in IgA Nephropathy Following At-Home Treatment by Subcutaneous Administration Completed NCT02062684 Phase 2, Phase 3 Blisibimod;Placebo
27 Efficacy and Safety of Omega-3 Fatty Acids(Omacor®) for the Treatment of Immunoglobulin A Nephropathy Completed NCT00549692 Phase 3 Omega-3 fatty acid ethylester90
28 Treatment of Early Immunoglobulin A Nephropathy by Angiotensin Converting Enzyme Inhibitor - A Randomized Controlled Trial Completed NCT01225445 Phase 3 Ramipril
29 Supportive Versus Immunosuppressive Therapy for the Treatment Of Progressive IgA Nephropathy Completed NCT00554502 Phase 3 supportive therapy with: ACE-inhibitor / ARB / Statin;supportive and immunosuppressive therapy
30 Aliskiren for Immunoglobulin A (IgA) Nephropathy Completed NCT00870493 Phase 3 Aliskiren;Placebo
31 Steroids and Azathioprine in Advanced IgAN Completed NCT01392833 Phase 3 methylprednisolone;azathioprine;prednisone
32 Efficacy and Safety of Losartan in Children With Ig A Nephropathy Completed NCT02232776 Phase 3 Losartan
33 Treatment of Immunoglobulin A (IgA) Nephropathy by Angiotensin-Converting Enzyme (ACE) Inhibitor Completed NCT00437463 Phase 3 Ramipril
34 Treatment of IgA Nephropathy According to Renal Lesions Recruiting NCT03188887 Phase 3 corticotherapy;Renin Angiotensin system (RAS) blockade
35 A Study of the Effect and Safety of Sparsentan in the Treatment of Patients With IgA Nephropathy Recruiting NCT03762850 Phase 3 sparsentan;irbesartan
36 Treatment Effects of Chinese Medicine (Yi-Qi-Qing-Jie Herbal Compound) Combined With Immunosuppression Therapies in Patients of IgA Nephropathy With High-risk of ESRD Recruiting NCT03418779 Phase 2, Phase 3 The Yi-Qi-Qing-Jie Herbal Compound;Immunosuppressive Agents
37 Efficacy and Safety of Nefecon in Patients With Primary IgA (Immunoglobulin A) Nephropathy Recruiting NCT03643965 Phase 3 Nefecon;Placebo oral capsule
38 Study of the Safety and Efficacy of OMS721 in Patients With Immunoglobulin A (IgA) Nephropathy Recruiting NCT03608033 Phase 3
39 Mycophenolate Mofetil (MMF) in Patients With IgA Nephropathy (IgAN) Active, not recruiting NCT00657059 Phase 3 irbesartan;methylprednisolone (MP) or prednisone (pred);mycophenolate mofetil (MMF)
40 Pilot Study of ACTH in the Treatment of IgA Nephropathy at High Risk of Progression Active, not recruiting NCT02282930 Phase 3 ACTH (Acthar) Gel
41 Trial of Plasma Exchange for Severe Crescentic IgA Nephropathy Enrolling by invitation NCT02647255 Phase 2, Phase 3 Methylprednisolone pulse
42 Beginning of Effective and Safe Treatment in Immunoglobulin A-1 Nephropathy-1 Not yet recruiting NCT03357653 Phase 3 Losartan group;Placebo group
43 Mycophenolate Mofetil (MMF) in Patients With IgA Nephropathy Terminated NCT00318474 Phase 3 Mycophenolate Mofetil (MMF);MMF Placebo;ACEi;FOS
44 Paricalcitol for the Treatment of Immunoglobulin A Nephropathy Withdrawn NCT00599963 Phase 3 paricalcitol
45 BRILLIANT-SC: A Study of the Efficacy and Safety of Blisibimod Administration in Subjects With IgA Nephropathy Withdrawn NCT02052219 Phase 3 Blisibimod;Placebo
46 A Controlled Study of Steroids Therapy for Patients of IgA Nephropathy With Active Pathological Changes. Unknown status NCT02160132 Phase 2 Methylprednisolone(intravenously in the 1st-2nd-3rd month );Methylprednisolone(intravenously in the 1st-3rd-5th month)
47 The Effect of Nefecon® in Patients With Primary IgA Nephropathy at Risk of Developing End-stage Renal Disease Completed NCT01738035 Phase 2 NEFECON
48 Open-Label Study to Evaluate Safety and Efficacy of CCX168 in Subjects With Immunoglobulin A Nephropathy on Stable RAAS Blockade Completed NCT02384317 Phase 2 CCX168
49 Sirolimus Therapy for Poor Prognosis Immunoglobulin A Nephropathy Completed NCT00396721 Phase 2 ACE inhibitor + statin;Sirolimus (study drug)+ACE inhibitor + statin
50 Oral Treatment With PL-56 in Patients With IgA Nephropathy - an Explorative Study Completed NCT00767221 Phase 2 Budesonide

Search NIH Clinical Center for Iga Nephropathy 1

Genetic Tests for Iga Nephropathy 1

Genetic tests related to Iga Nephropathy 1:

# Genetic test Affiliating Genes
1 Berger Disease 29

Anatomical Context for Iga Nephropathy 1

MalaCards organs/tissues related to Iga Nephropathy 1:

41
Kidney, Skin, Liver, Heart, T Cells, Lung, Thyroid

Publications for Iga Nephropathy 1

Articles related to Iga Nephropathy 1:

# Title Authors Year
1
Enhanced expression of the CD71 mesangial IgA1 receptor in Berger disease and Henoch-SchAPnlein nephritis: association between CD71 expression and IgA deposits. ( 12538733 )
2003
2
Onset of Berger disease after Staphylococcus aureus infection: septic arthritis after anterior cruciate ligament reconstruction. ( 12671604 )
2003
3
Berger disease: thirty years later. ( 10378387 )
1999
4
Berger disease: Henoch-SchAPnlein syndrome without the rash. ( 2981307 )
1985
5
Berger disease/Henoch-Schönlein syndrome. ( 4045620 )
1985

Variations for Iga Nephropathy 1

ClinVar genetic disease variations for Iga Nephropathy 1:

6
# Gene Variation Type Significance SNP ID Assembly Location
1 PIGR NM_002644.3(PIGR): c.1739C> T (p.Ala580Val) single nucleotide variant Uncertain significance rs291102 GRCh37 Chromosome 1, 207106478: 207106478
2 PIGR NM_002644.3(PIGR): c.1739C> T (p.Ala580Val) single nucleotide variant Uncertain significance rs291102 GRCh38 Chromosome 1, 206933133: 206933133

Expression for Iga Nephropathy 1

Search GEO for disease gene expression data for Iga Nephropathy 1.

Pathways for Iga Nephropathy 1

GO Terms for Iga Nephropathy 1

Sources for Iga Nephropathy 1

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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