MCID: FBR085
MIFTS: 24

Fibrillary Glomerulonephritis

Categories: Nephrological diseases, Rare diseases

Aliases & Classifications for Fibrillary Glomerulonephritis

MalaCards integrated aliases for Fibrillary Glomerulonephritis:

Name: Fibrillary Glomerulonephritis 20 70
Congo Red-Negative Amyloidosis-Like Glomerulopathy 20 58
Non-Amyloid Fibrillary Glomerulonephritis 20 58
Non-Amyloid Fibrillary Glomerulopathy 20 58

Characteristics:

Orphanet epidemiological data:

58
non-amyloid fibrillary glomerulopathy
Inheritance: Not applicable; Age of onset: Adult;

Classifications:

Orphanet: 58  
Rare renal diseases


External Ids:

ICD10 via Orphanet 33 N03.6
Orphanet 58 ORPHA97566
UMLS 70 C0268749

Summaries for Fibrillary Glomerulonephritis

GARD : 20 Fibrillary glomerulonephritis is an uncommon cause of glomerular disease. A more rare disorder known as immunotactoid glomerulpathy is a very similar condition. Both disorders probably result from deposits derived from immunoglobulins but in most cases the cause is idiopathic (unknown). The diagnosis is made with a kidney biopsy and by electron microscopy. Fibrillary glomerulonephritis and immunotactoid glomerulopathy can be differentiated from each other by electron microscopy; the fibrils in fibrillary glomerulonephritis are smaller and randomly oriented as opposed to the larger and often organized fibrils of immunotactoid glomerulopathy. The signs and symptoms include blood (hematuria) and protein ( proteinuria ) in the urine, kidney insufficiency and high blood pressure. Both fibrillary glomerulonephritis and immunotactoid glomerulopathy have been associated with hepatitis C virus infection and with malignancy and autoimmune disease. Because of this, patients should be screened for these conditions. Treatment is generally determined by the severity of the kidney problems.

MalaCards based summary : Fibrillary Glomerulonephritis, also known as congo red-negative amyloidosis-like glomerulopathy, is related to immunotactoid or fibrillary glomerulopathy and immunotactoid glomerulopathy. An important gene associated with Fibrillary Glomerulonephritis is DNAJB9 (DnaJ Heat Shock Protein Family (Hsp40) Member B9). The drugs rituximab and Antineoplastic Agents, Immunological have been mentioned in the context of this disorder. Affiliated tissues include kidney, bone marrow and myeloid.

Related Diseases for Fibrillary Glomerulonephritis

Diseases related to Fibrillary Glomerulonephritis via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 77)
# Related Disease Score Top Affiliating Genes
1 immunotactoid or fibrillary glomerulopathy 11.8
2 immunotactoid glomerulopathy 11.6
3 glomerulopathy with fibronectin deposits 2 11.2
4 glomerulonephritis 11.0
5 proteinuria, chronic benign 10.7
6 nephrotic syndrome 10.6
7 glomerular disease 10.5
8 end stage renal disease 10.5
9 amyloidosis 10.5
10 crescentic glomerulonephritis 10.4
11 rapidly progressive glomerulonephritis 10.3
12 kidney disease 10.3
13 autoimmune disease 10.3
14 membranoproliferative glomerulonephritis 10.3
15 membranous nephropathy 10.2
16 hepatitis c 10.2
17 proliferative glomerulonephritis 10.2
18 systemic lupus erythematosus 10.1
19 goodpasture syndrome 10.1
20 cryoglobulinemia 10.1
21 mesangial proliferative glomerulonephritis 10.1
22 thrombotic microangiopathy 10.1
23 sjogren syndrome 10.0
24 graves' disease 10.0
25 purpura 10.0
26 monoclonal gammopathy of uncertain significance 10.0
27 lupus erythematosus 10.0
28 type 2 diabetes mellitus 9.9
29 rheumatoid arthritis 9.9
30 thrombocytopenic purpura, autoimmune 9.9
31 anemia, autoimmune hemolytic 9.9
32 lymphoma, hodgkin, classic 9.9
33 immune deficiency disease 9.9
34 myelofibrosis 9.9
35 myeloma, multiple 9.9
36 lymphoma, non-hodgkin, familial 9.9
37 lipoprotein glomerulopathy 9.9
38 cholangiocarcinoma 9.9
39 deficiency anemia 9.9
40 disease by infectious agent 9.9
41 lymphoplasmacytic lymphoma 9.9
42 cutaneous t cell lymphoma 9.9
43 charcot-marie-tooth disease 9.9
44 interstitial nephritis 9.9
45 tooth disease 9.9
46 primary biliary cholangitis 9.9
47 tolosa-hunt syndrome 9.9
48 focal segmental glomerulosclerosis 9.9
49 polyneuropathy 9.9
50 hypothyroidism 9.9

Graphical network of the top 20 diseases related to Fibrillary Glomerulonephritis:



Diseases related to Fibrillary Glomerulonephritis

Symptoms & Phenotypes for Fibrillary Glomerulonephritis

Drugs & Therapeutics for Fibrillary Glomerulonephritis

Drugs for Fibrillary Glomerulonephritis (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):


# Name Status Phase Clinical Trials Cas Number PubChem Id
1
rituximab Approved Phase 2 174722-31-7 10201696
2 Antineoplastic Agents, Immunological Phase 2
3 Immunologic Factors Phase 2
4 Antirheumatic Agents Phase 2

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 A Single Center Pilot Trial of Rituximab in the Treatment of Fibrillary Glomerulonephritis Completed NCT02197767 Phase 2 rituximab

Search NIH Clinical Center for Fibrillary Glomerulonephritis

Genetic Tests for Fibrillary Glomerulonephritis

Anatomical Context for Fibrillary Glomerulonephritis

MalaCards organs/tissues related to Fibrillary Glomerulonephritis:

40
Kidney, Bone Marrow, Myeloid

Publications for Fibrillary Glomerulonephritis

Articles related to Fibrillary Glomerulonephritis:

(show top 50) (show all 208)
# Title Authors PMID Year
1
DNA J homolog subfamily B member 9 and other advances in fibrillary glomerulonephritis. 61
33767057 2021
2
Complete remission of DnaJ homolog subfamily B member 9-positive fibrillary glomerulonephritis following steroid monotherapy in an elderly Japanese woman. 61
33656648 2021
3
Immunofluorescence Staining for Immunoglobulin Heavy Chain/Light Chain on Kidney Biopsies is a Valuable Ancillary Technique For the Diagnosis of Monoclonal Gammopathy-Associated Kidney Diseases. 61
33774084 2021
4
Spectrum of Kidney Involvement in Patients with Myelodysplastic Syndromes. 61
33732989 2021
5
Immunoglobulin-Negative DNAJB9-Associated Fibrillary Glomerulonephritis: A Report of 9 Cases. 61
32711071 2021
6
Fibrillary glomerulonephritis or complement 3 glomerulopathy: a rare case of diffuse necrotising crescentic glomerulonephritis with C3-dominant glomerular deposition and positive DNAJB9. 61
33602773 2021
7
Treatment of fibrillary glomerulonephritis with rituximab: a 12-month pilot study. 61
32617582 2021
8
Genetic or pharmacologic Nrf2 activation increases proteinuria in chronic kidney disease in mice. 61
32818518 2021
9
The Evolving Role of Novel Biomarkers in Glomerular Disease: A Review. 61
33077315 2021
10
Rituximab in fibrillary glomerulonephritis: fumble or forward progress? 61
32780101 2021
11
Familial Fibrillary Glomerulonephritis in Living Related Kidney Transplantation. 61
33426405 2021
12
Fibrillary glomerulonephritis presenting as crescentic glomerulonephritis in a young female: a case study. 61
32657244 2020
13
Recurrence of DNAJB9-Positive Fibrillary Glomerulonephritis After Kidney Transplantation: A Case Series. 61
32414663 2020
14
Letter Regarding "Fibrillary Glomerulonephritis Is Associated With HLA-DR7 and HLA-B35 Antigens". 61
33102980 2020
15
Fibrilo-Tactoid Glomerulonephritis: A Possible Novel Morphological Variant. 61
33363217 2020
16
DNAJB9-positive monotypic fibrillary glomerulonephritis is not associated with monoclonal gammopathy in the vast majority of patients. 61
32622524 2020
17
Fibrillary Glomerulonephritis Is Associated With HLA-DR7 and HLA-B35 Antigens. 61
32775835 2020
18
Successful treatment of a unique case of congophilic fibrillary glomerulonephritis: A case report. 61
32664131 2020
19
Clinicopathological characteristics and outcome of patients with fibrillary glomerulonephritis: DNAJB9 is a valuable histologic marker. 61
32557205 2020
20
IgM monoclonal gammopathy with heavy-and-light-chain amyloidosis resembling fibrillary glomerulonephritis determined by tandem mass spectrometry: a case report. 61
32448180 2020
21
DNAJB9 Is Not Transcriptionally Upregulated in the Glomerulus in Fibrillary Glomerulonephritis. 61
32154459 2020
22
Fibrillary Glomerulopathy with a High Level of Myeloperoxidase-ANCA: A Case Report. 61
32274231 2020
23
Multiple morphological phenotypes of monoclonal immunoglobulin disease on renal biopsy: Significance of treatment. 61
32318322 2020
24
Fibrillary Glomerulonephritis: Clinicopathologic Features and Atypical Cases from a Multi-Institutional Cohort. 61
31685544 2019
25
New developments in the diagnosis of fibrillary glomerulonephritis. 61
31227146 2019
26
Heavy Chain Fibrillary Glomerulonephritis: A Case Report. 61
30955945 2019
27
Fibrillary Glomerulonephritis: An Update. 61
31317113 2019
28
Elevated serum concentrations of DNAJB9 in fibrillary glomerulonephritis: another step toward understanding a progressive disease. 61
31010477 2019
29
Serum levels of DNAJB9 are elevated in fibrillary glomerulonephritis patients. 61
31010480 2019
30
A Case of Essential Thrombocythemia and IgA Nephropathy with Literature Review of the Concurrence. 61
31565452 2019
31
Monoclonal Immunoglobulin Deposition Disease and Related Diseases. 61
30918151 2019
32
Treatment of fibrillary glomerulonephritis with use of repository corticotropin injections. 61
30524713 2018
33
Rituximab and Fibrillary Glomerulonephritis: Interest of B Cell Reconstitution Monitoring. 61
30423930 2018
34
Fibrillary glomerulonephritis in a human immunodeficiency virus-positive, hepatitis C-negative Indian patient: Expanding the profile of renal involvement in human immunodeficiency virus infection. 61
30303165 2018
35
Congophilic Fibrillary Glomerulonephritis: A Case Series. 61
29866458 2018
36
[Chronic lymphoid leukemia and renal complication: Report on 10 cases from Marseille over 16 years]. 61
29891261 2018
37
Fibrillary Glomerulonephritis with Crescentic and Necrotizing Glomerulonephritis and Concurrent Thrombotic Microangiopathy. 61
30320122 2018
38
Fibrillary glomerulonephritis in an HIV patient without concurrent hepatitis C infection: Case report and review of the literature‚Ä©. 61
29578397 2018
39
Fibrillary Glomerulonephritis in a Patient with Sjogren's Syndrome. 61
29922524 2018
40
DNAJB9 Is a Specific Immunohistochemical Marker for Fibrillary Glomerulonephritis. 61
29340314 2018
41
Histologic regression of fibrillary glomerulonephritis: the first report of biopsy-proven spontaneous resolution of disease. 61
29225801 2017
42
Proliferative lupus nephritis in the absence of overt systemic lupus erythematosus: A historical study of 12 adult patients. 61
29310419 2017
43
Fibrillary Glomerulonephritis in Primary Sjogren's Syndrome: A Rare Cause of Renal Failure. 61
29196420 2017
44
Fibrillary glomerulonephritis presenting as crescentic glomerulonephritis. 61
28356674 2017
45
Paraneoplastic fibrillary glomerulonephritis associated with intrahepatic cholangiocarcinoma: When diagnosis of a rare kidney disease leads to successful hepatic cancer treatment. 61
27542513 2017
46
Clinical Features and Outcomes of a Racially Diverse Population with Fibrillary Glomerulonephritis. 61
28161700 2017
47
A Case of Fibrillary Glomerulonephritis with Fibril Deposition in the Arteriolar Wall and a Family History of Renal Disease. 61
28503551 2017
48
Renal pathology in hematopoietic cell transplant recipients: a contemporary biopsy, nephrectomy, and autopsy series. 61
27015134 2016
49
IgG Subclass Staining in Routine Renal Biopsy Material. 61
26848798 2016
50
Treatment of fibrillary glomerulonephritis by corticosteroids and tripterygium glycoside tablets: A case report. 61
27022729 2016

Variations for Fibrillary Glomerulonephritis

Expression for Fibrillary Glomerulonephritis

Search GEO for disease gene expression data for Fibrillary Glomerulonephritis.

Pathways for Fibrillary Glomerulonephritis

GO Terms for Fibrillary Glomerulonephritis

Sources for Fibrillary Glomerulonephritis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Apr-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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