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Immunotactoid glomerulopathy

MedGen UID:
1684714
•Concept ID:
C5202806
•
Disease or Syndrome
Synonyms: FGN; fibrillary glomerulonephritis; Glomerulonephritis with Organized Monoclonal Microtubular Immunoglobulin Deposits; Immunotactoid glomerulonephritis; Immunotactoid Glomerulopathy; immunotactoid glomerulopathy
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0019991
Orphanet: ORPHA97567

Definition

Immunotactoid glomerulopathy (ITG) is a very rare condition characterized by glomerular accumulation of microtubules in the mesangium and the glomerular basement membrane, that mainly presents with proteinuria, micro-hematuria, nephrotic syndrome, renal insufficiency and hematologic malignancy. ITG and non-amyloid fibrillary glomerulopathy (non-amyloid FGP, see this term) are often grouped together as pathogenetically related diseases. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVImmunotactoid glomerulopathy

Professional guidelines

PubMed

Nonamyloidotic fibrillary glomerulopathy, immunotactoid glomerulopathy, and the differential diagnosis of filamentous glomerulopathies.
Devaney K, Sabnis SG, Antonovych TT
Mod Pathol 1991 Jan;4(1):36-45. PMID: 2020659

Recent clinical studies

Etiology

Light Chain-Only Immunotactoid Glomerulopathy: A Case Report.
Bu L, Javaugue V, Chauvet S, Napier J, Dasari S, Theis JD, Vrana JA, McPhail ED, Nasr SH
Am J Kidney Dis 2023 May;81(5):611-615. Epub 2022 Oct 31 doi: 10.1053/j.ajkd.2022.08.025. PMID: 36328099
Dysproteinemias and Glomerular Disease.
Leung N, Drosou ME, Nasr SH
Clin J Am Soc Nephrol 2018 Jan 6;13(1):128-139. Epub 2017 Nov 7 doi: 10.2215/CJN.00560117. PMID: 29114004Free PMC Article
The Scope of Kidney Affection in Monoclonal Gammopathies at All Levels of Clinical Significance.
Mehtat Ünlü Ş, Özsan H, Sarıoğlu S
Turk J Haematol 2017 Dec 1;34(4):282-288. Epub 2017 Aug 23 doi: 10.4274/tjh.2017.0197. PMID: 28832010Free PMC Article
Clinical features, predictors of disease progression and results of renal transplantation in fibrillary/immunotactoid glomerulopathy.
Pronovost PH, Brady HR, Gunning ME, Espinoza O, Rennke HG
Nephrol Dial Transplant 1996 May;11(5):837-42. doi: 10.1093/oxfordjournals.ndt.a027409. PMID: 8671905
Immunotactoid glomerulopathy.
Korbet SM, Schwartz MM, Rosenberg BF, Sibley RK, Lewis EJ
Medicine (Baltimore) 1985 Jul;64(4):228-43. doi: 10.1097/00005792-198507000-00004. PMID: 4010500

Diagnosis

Glomerulopathies with Fibrillary Deposits.
Dzekova-Vidimliski P, Karanfilovski V, Nikolov IG, Rambabova-Bushljetik I, Ristovska V, Petrushevska G, Selim G
Pril (Makedon Akad Nauk Umet Odd Med Nauki) 2023 Jul 1;44(2):99-106. Epub 2023 Jul 15 doi: 10.2478/prilozi-2023-0030. PMID: 37453107
The Complexity and Heterogeneity of Monoclonal Immunoglobulin-Associated Renal Diseases.
Sethi S, Rajkumar SV, D'Agati VD
J Am Soc Nephrol 2018 Jul;29(7):1810-1823. Epub 2018 Apr 27 doi: 10.1681/ASN.2017121319. PMID: 29703839Free PMC Article
Dysproteinemias and Glomerular Disease.
Leung N, Drosou ME, Nasr SH
Clin J Am Soc Nephrol 2018 Jan 6;13(1):128-139. Epub 2017 Nov 7 doi: 10.2215/CJN.00560117. PMID: 29114004Free PMC Article
Immunotactoid glomerulopathy.
Schwartz MM, Korbet SM, Lewis EJ
J Am Soc Nephrol 2002 May;13(5):1390-7. doi: 10.1097/01.asn.0000013397.06964.19. PMID: 11961029
The fibrillary glomerulopathies.
Korbet SM, Schwartz MM, Lewis EJ
Am J Kidney Dis 1994 May;23(5):751-65. doi: 10.1016/s0272-6386(12)70290-1. PMID: 8172222

Therapy

Glomerulopathies with Fibrillary Deposits.
Dzekova-Vidimliski P, Karanfilovski V, Nikolov IG, Rambabova-Bushljetik I, Ristovska V, Petrushevska G, Selim G
Pril (Makedon Akad Nauk Umet Odd Med Nauki) 2023 Jul 1;44(2):99-106. Epub 2023 Jul 15 doi: 10.2478/prilozi-2023-0030. PMID: 37453107
Immunotactoid glomerulopathy - an enigmatic case in the setting of nodal marginal zone lymphoma and systemic sclerosis sine scleroderma.
Mohamed MW, Al-Hammadi M, Hussein AM, Alarab D, Albreak HA, Tungekar MF, Dandi B
BMC Nephrol 2022 Mar 15;23(1):103. doi: 10.1186/s12882-022-02730-w. PMID: 35291980Free PMC Article
Hepatitis C virus associated glomerulopathies.
Ozkok A, Yildiz A
World J Gastroenterol 2014 Jun 28;20(24):7544-54. doi: 10.3748/wjg.v20.i24.7544. PMID: 24976695Free PMC Article
Clinical features, predictors of disease progression and results of renal transplantation in fibrillary/immunotactoid glomerulopathy.
Pronovost PH, Brady HR, Gunning ME, Espinoza O, Rennke HG
Nephrol Dial Transplant 1996 May;11(5):837-42. doi: 10.1093/oxfordjournals.ndt.a027409. PMID: 8671905
Immunotactoid glomerulopathy.
Korbet SM, Schwartz MM, Rosenberg BF, Sibley RK, Lewis EJ
Medicine (Baltimore) 1985 Jul;64(4):228-43. doi: 10.1097/00005792-198507000-00004. PMID: 4010500

Prognosis

Immunotactoid glomerulopathy is a rare entity with monoclonal and polyclonal variants.
Nasr SH, Kudose SS, Said SM, Santoriello D, Fidler ME, Williamson SR, Damgard SE, Sethi S, Leung N, D'Agati VD, Markowitz GS
Kidney Int 2021 Feb;99(2):410-420. Epub 2020 Aug 18 doi: 10.1016/j.kint.2020.07.037. PMID: 32818517
The Complexity and Heterogeneity of Monoclonal Immunoglobulin-Associated Renal Diseases.
Sethi S, Rajkumar SV, D'Agati VD
J Am Soc Nephrol 2018 Jul;29(7):1810-1823. Epub 2018 Apr 27 doi: 10.1681/ASN.2017121319. PMID: 29703839Free PMC Article
Dysproteinemias and Glomerular Disease.
Leung N, Drosou ME, Nasr SH
Clin J Am Soc Nephrol 2018 Jan 6;13(1):128-139. Epub 2017 Nov 7 doi: 10.2215/CJN.00560117. PMID: 29114004Free PMC Article
The fibrillary glomerulopathies.
Korbet SM, Schwartz MM, Lewis EJ
Am J Kidney Dis 1994 May;23(5):751-65. doi: 10.1016/s0272-6386(12)70290-1. PMID: 8172222
Immunotactoid glomerulopathy.
Korbet SM, Schwartz MM, Lewis EJ
Am J Kidney Dis 1991 Mar;17(3):247-57. doi: 10.1016/s0272-6386(12)80469-0. PMID: 1996564

Clinical prediction guides

Results of a nation-wide cohort study suggest favorable long-term outcomes of clone-targeted chemotherapy in immunotactoid glomerulopathy.
Javaugue V, Dufour-Nourigat L, Desport E, Sibille A, Moulin B, Bataille P, Bindi P, Garrouste C, Mariat C, Karlin L, Nouvier M, Goujon JM, Gnemmi V, Fermand JP, Touchard G, Bridoux F
Kidney Int 2021 Feb;99(2):421-430. Epub 2020 Jul 30 doi: 10.1016/j.kint.2020.06.039. PMID: 32739419
Fibrillary glomerulonephritis in siblings.
Chan TM, Chan KW
Am J Kidney Dis 1998 May;31(5):E4. doi: 10.1016/s0272-6386(98)70066-6. PMID: 10074581
Clinical features, predictors of disease progression and results of renal transplantation in fibrillary/immunotactoid glomerulopathy.
Pronovost PH, Brady HR, Gunning ME, Espinoza O, Rennke HG
Nephrol Dial Transplant 1996 May;11(5):837-42. doi: 10.1093/oxfordjournals.ndt.a027409. PMID: 8671905
The racial prevalence of glomerular lesions in nephrotic adults.
Korbet SM, Genchi RM, Borok RZ, Schwartz MM
Am J Kidney Dis 1996 May;27(5):647-51. doi: 10.1016/s0272-6386(96)90098-0. PMID: 8629623
Immunotactoid glomerulopathy and cutaneous vasculitis.
Orfila C, Meeus F, Bernadet P, Lepert JC, Suc JM
Am J Nephrol 1991;11(1):67-72. doi: 10.1159/000168276. PMID: 1904680

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