Fibrillary glomerular subepithelial deposits

MedGen UID:: 1782768
•Concept ID:: C5539712
•: Finding

HPO:	HP:0033600

Definition

A type of fibrillar glomerular subepithelial deposit characterized by extracellular aggregates of randomly arranged, straight, non-branching fibrils that are thicker than amyloid fibrils (average diameter about 20 nm) and are often admixed with smudgy, electron-dense material. This feature is associated with a prominent glomerular basement membrane (GBM) reflecting an diffuse and relatively uniform increase in thickness (subjective estimate). [from HPO]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVFibrillary glomerular subepithelial deposits

Abnormal nephron morphology
- Abnormal renal corpuscle morphology
  - Abnormal renal glomerulus morphology
    - Glomerular deposits
      - Glomerular subepithelial deposits
        Fibrillar glomerular subepithelial deposits
        Fibrillary glomerular subepithelial deposits

Recent clinical studies

Etiology

Fingerprint and other organised deposits in lupus nephritis.

Hvala A, Kobenter T, Ferluga D
Wien Klin Wochenschr 2000 Aug 25;112(15-16):711-5. PMID: 11020962

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Diagnosis

IgA-dominant glomerulonephritis with DNAJB9-negative fibrillar polytypic immunoglobulin deposits in the subepithelium.

Muto R, Maeda K, Fukui S, Saito S, Kato N, Kosugi T, Shimizu A, Maruyama S
CEN Case Rep 2023 Aug;12(3):323-328. Epub 2022 Dec 28 doi: 10.1007/s13730-022-00759-2. PMID: 36576710 Free PMC Article

A case of juvenile-onset fibrillary glomerulonephritis diagnosed by mass spectrometry and immunohistochemistry of DNAJB9.

Hattori K, Shimizu R, Tanaka S, Terashima T, Hiramatsu M, Shimomura T, Ito T, Morinaga T, Terasaki M, Shimizu A, Morozumi K, Tamai H
CEN Case Rep 2022 Nov;11(4):412-416. Epub 2022 Feb 23 doi: 10.1007/s13730-022-00693-3. PMID: 35199316 Free PMC Article

Fibrillary glomerulonephritis in an HIV patient without concurrent hepatitis C infection: Case report and review of the literature .

Zhang L, Carson JM, Lucia MS
Clin Nephrol 2018 May;89(5):381-386. doi: 10.5414/CN109215. PMID: 29578397

A case of fibrillary glomerulonephritis with linear immunoglobulin G staining of the glomerular capillary walls.

Sethi S, Adeyi OA, Rennke HG
Arch Pathol Lab Med 2001 Apr;125(4):534-6. doi: 10.5858/2001-125-0534-ACOFGW. PMID: 11260631

Nephrotic syndrome associated with fibrillary deposits in the glomeruli.

Yumura W, Nitta K, Horita S, Ozu H, Honda K, Nihei H
Intern Med 1995 Jan;34(1):46-50. doi: 10.2169/internalmedicine.34.46. PMID: 7718980

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