MCID: FBR085
MIFTS: 26

Fibrillary Glomerulonephritis

Categories: Nephrological diseases, Rare diseases

Aliases & Classifications for Fibrillary Glomerulonephritis

MalaCards integrated aliases for Fibrillary Glomerulonephritis:

Name: Fibrillary Glomerulonephritis 52 71
Congo Red-Negative Amyloidosis-Like Glomerulopathy 52 58
Non-Amyloid Fibrillary Glomerulonephritis 52 58
Non-Amyloid Fibrillary Glomerulopathy 52 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 71 C0268749

Summaries for Fibrillary Glomerulonephritis

NIH Rare Diseases : 52 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. The drugs Tacrolimus and Immunologic Factors have been mentioned in the context of this disorder. Affiliated tissues include kidney, liver and lung.

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 80)
# Related Disease Score Top Affiliating Genes
1 immunotactoid or fibrillary glomerulopathy 12.5
2 immunotactoid glomerulopathy 12.3
3 glomerulopathy with fibronectin deposits 2 11.7
4 glomerulonephritis 11.0
5 nephrotic syndrome 10.7
6 end stage renal failure 10.5
7 glomerular disease 10.5
8 amyloidosis 10.5
9 autoimmune disease 10.4
10 kidney disease 10.4
11 crescentic glomerulonephritis 10.3
12 hepatitis c 10.3
13 rapidly progressive glomerulonephritis 10.3
14 primary glomerular disease 10.3
15 membranous nephropathy 10.3
16 membranoproliferative glomerulonephritis 10.2
17 proliferative glomerulonephritis 10.2
18 systemic lupus erythematosus 10.2
19 goodpasture syndrome 10.2
20 cryoglobulinemia 10.2
21 thrombotic microangiopathy 10.2
22 rheumatoid arthritis 10.1
23 myeloma, multiple 10.1
24 sjogren syndrome 10.1
25 graves' disease 10.1
26 focal segmental glomerulosclerosis 10.1
27 thrombocytopenia 10.1
28 purpura 10.1
29 mesangial proliferative glomerulonephritis 10.1
30 monoclonal gammopathy of uncertain significance 10.1
31 chronic kidney disease 10.1
32 arthritis 10.1
33 lupus erythematosus 10.1
34 thrombocytopenic purpura, autoimmune 9.9
35 anemia, autoimmune hemolytic 9.9
36 lymphoma, hodgkin, classic 9.9
37 immune deficiency disease 9.9
38 myelofibrosis 9.9
39 lymphoma, non-hodgkin, familial 9.9
40 hepatitis c virus 9.9
41 lipoprotein glomerulopathy 9.9
42 cholangiocarcinoma 9.9
43 deficiency anemia 9.9
44 lymphoplasmacytic lymphoma 9.9
45 lymphoma 9.9
46 cutaneous t cell lymphoma 9.9
47 charcot-marie-tooth disease 9.9
48 interstitial nephritis 9.9
49 tooth disease 9.9
50 primary biliary cirrhosis 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):

(show all 13)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Tacrolimus Approved, Investigational Phase 4 104987-11-3 445643 439492 6473866
2 Immunologic Factors Phase 4
3 Melanocyte-Stimulating Hormones Phase 4
4 Pharmaceutical Solutions Phase 4
5 Adrenocorticotropic Hormone Phase 4
6 Hormone Antagonists Phase 4
7 beta-endorphin Phase 4
8 Immunosuppressive Agents Phase 4
9 Hormones Phase 4
10 Calcineurin Inhibitors Phase 4
11
rituximab Approved Phase 2 174722-31-7 10201696
12 Antineoplastic Agents, Immunological Phase 2
13 Antirheumatic Agents Phase 2

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 A Multicenterer, Comparative Safety Andof ACTHar Gel Alone or in Cwith oe Urinary in Patients Positive Fibrillary Glomerulopathy Recruiting NCT04080076 Phase 4 Acthar 80 UNT/ML Injectable Solution;Tacrolimus capsule
2 A Single Center Pilot Trial of Rituximab in the Treatment of Fibrillary Glomerulonephritis Completed NCT02197767 Phase 2 rituximab
3 Different Histopathological Patterns in Patients With HCV Nephropathy Not yet recruiting NCT03476031

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, Liver, Lung, Bone Marrow, Bone, Myeloid, T Cells

Publications for Fibrillary Glomerulonephritis

Articles related to Fibrillary Glomerulonephritis:

(show top 50) (show all 185)
# Title Authors PMID Year
1
Fibrillary Glomerulonephritis: Clinicopathologic Features and Atypical Cases from a Multi-Institutional Cohort. 61
31685544 2019
2
New developments in the diagnosis of fibrillary glomerulonephritis. 61
31227146 2019
3
Heavy Chain Fibrillary Glomerulonephritis: A Case Report. 61
30955945 2019
4
Fibrillary Glomerulonephritis: An Update. 61
31317113 2019
5
Serum levels of DNAJB9 are elevated in fibrillary glomerulonephritis patients. 61
31010480 2019
6
Elevated serum concentrations of DNAJB9 in fibrillary glomerulonephritis: another step toward understanding a progressive disease. 61
31010477 2019
7
A Case of Essential Thrombocythemia and IgA Nephropathy with Literature Review of the Concurrence. 61
31565452 2019
8
Monoclonal Immunoglobulin Deposition Disease and Related Diseases. 61
30918151 2019
9
Treatment of fibrillary glomerulonephritis with use of repository corticotropin injections. 61
30524713 2018
10
Rituximab and Fibrillary Glomerulonephritis: Interest of B Cell Reconstitution Monitoring. 61
30423930 2018
11
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
12
Congophilic Fibrillary Glomerulonephritis: A Case Series. 61
29866458 2018
13
[Chronic lymphoid leukemia and renal complication: Report on 10 cases from Marseille over 16 years]. 61
29891261 2018
14
Fibrillary Glomerulonephritis with Crescentic and Necrotizing Glomerulonephritis and Concurrent Thrombotic Microangiopathy. 61
30320122 2018
15
Fibrillary glomerulonephritis in an HIV patient without concurrent hepatitis C infection: Case report and review of the literature‚Ä©. 61
29578397 2018
16
Fibrillary Glomerulonephritis in a Patient with Sjogren's Syndrome. 61
29922524 2018
17
DNAJB9 Is a Specific Immunohistochemical Marker for Fibrillary Glomerulonephritis. 61
29340314 2018
18
Proliferative lupus nephritis in the absence of overt systemic lupus erythematosus: A historical study of 12 adult patients. 61
29310419 2017
19
Fibrillary Glomerulonephritis in Primary Sjogren's Syndrome: A Rare Cause of Renal Failure. 61
29196420 2017
20
Histologic regression of fibrillary glomerulonephritis: the first report of biopsy-proven spontaneous resolution of disease. 61
29225801 2017
21
Fibrillary glomerulonephritis presenting as crescentic glomerulonephritis. 61
28356674 2017
22
Paraneoplastic fibrillary glomerulonephritis associated with intrahepatic cholangiocarcinoma: When diagnosis of a rare kidney disease leads to successful hepatic cancer treatment. 61
27542513 2017
23
Clinical Features and Outcomes of a Racially Diverse Population with Fibrillary Glomerulonephritis. 61
28161700 2017
24
A Case of Fibrillary Glomerulonephritis with Fibril Deposition in the Arteriolar Wall and a Family History of Renal Disease. 61
28503551 2017
25
Renal pathology in hematopoietic cell transplant recipients: a contemporary biopsy, nephrectomy, and autopsy series. 61
27015134 2016
26
IgG Subclass Staining in Routine Renal Biopsy Material. 61
26848798 2016
27
Treatment of fibrillary glomerulonephritis by corticosteroids and tripterygium glycoside tablets: A case report. 61
27022729 2016
28
Fibrillary glomerulonephritis associated with limited scleroderma: a case report. 61
26709524 2016
29
Acthar gel in the treatment of nephrotic syndrome: a multicenter retrospective case series. 61
27036111 2016
30
Spectrum of manifestations of monoclonal gammopathy-associated renal lesions. 61
26735145 2016
31
Rapidly progressive glomerulonephritis due to coexistent anti-glomerular basement membrane disease and fibrillary glomerulonephritis. 61
26798468 2016
32
A case of mistaken identity: fibrillary glomerulonephritis masquerading as crescentic anti-glomerular basement membrane disease. 61
26636330 2016
33
Simultaneous occurrence of fibrillary glomerulonephritis and renal lesions in nonmalignant monoclonal IgM gammopathy. 61
26892336 2016
34
[Glomerulopathies with organized monoclonal immunoglobulin deposits]. 61
26810049 2016
35
Fibrillary glomerulonephritis masquerading as rapidly progressive glomerulonephritis with pseudo-linear glomerular basement membrane staining. 61
26249548 2015
36
AJKD Atlas of Renal Pathology: Fibrillary Glomerulonephritis. 61
26408242 2015
37
A case of membranoproliferative glomerulonephritis associated with curved fibril deposition. 61
26370133 2015
38
Fibrillary glomerulonephritis: An apparent familial form? 61
26063488 2015
39
Fibrillary glomerulonephritis combined with chronic inflammatory demyelinating polyneuropathy. 61
26484033 2015
40
A case of fibrillary glomerulonephritis associated with thrombotic microangiopathy and anti-glomerular basement membrane antibody. 61
25873933 2015
41
End-Stage Kidney Disease Due to Fibrillary Glomerulonephritis and Immunotactoid Glomerulopathy - Outcomes in 66 Consecutive ANZDATA Registry Cases. 61
26418732 2015
42
Rituximab treatment for fibrillary glomerulonephritis. 61
24867652 2014
43
Long-term outcome of kidney transplantation in a patient with coexisting lipoprotein glomerulopathy and fibrillary glomerulonephritis. 61
25852917 2014
44
Hepatitis C virus associated glomerulopathies. 61
24976695 2014
45
Pathological patterns of mesangioproliferative glomerulonephritis seen at a tertiary care center. 61
28197459 2014
46
Long-term kidney disease outcomes in fibrillary glomerulonephritis: a case series of 27 patients. 61
23759297 2013
47
Fibrillary glomerulonephritis complicated by membranous nephropathy in a patient with tuberculosis. 61
22826141 2013
48
Rituximab-associated interstitial lung disease in fibrillary glomerulonephritis. 61
26064515 2013
49
Recurrence of fibrillary glomerulonephritis in a renal transplant recipient. 61
22826142 2013
50
[Role of tubulin in the pathogenesis of fibrillary glomerulonephritis]. 61
24006767 2013

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

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44 MESH via Orphanet
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48 NCI
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50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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