Focal segmental glomerulosclerosis differential diagnosis
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mehrian Jafarizade, M.D [2]
Overview
Focal segmental glomerulosclerosis should be differentiate from other causes of glomerular disease such as nephritic syndrome, nephrotic syndrome, Fabry's disease, poststreptococcal glomerulonephritis, lupus nephritis, antiglomerular basement membrane disease (goodpasture's syndrome), Cryoglobulinemia, Henoch-Schönlein purpura, amyloidosis, pulmonary-renal syndromes (vasculitis), thin basement membrane disease, Alport's Syndrome, anti-GBM Disease, hypertensive nephrosclerosis, and subacute bacterial endocarditis. The various types of glomerular diseases may be differentiated from each other based on associations, presence of pitting edema, hemeturia, hypertension, hemoptysis, oliguria, peri-orbital edema, hyperlipidemia, type of antibodies, light and electron microscopic features.
Differential Diagnosis
Focal segmental glomerulosclerosis should be differentiate from other causes of glomerular disease. The various types of glomerular diseases may be differentiated from each other based on associations, presence of pitting edema, hemeturia, hypertension, hemoptysis, oliguria, peri-orbital edema, hyperlipidemia, type of antibodies, light and electron microscopic features. The following table differentiates between various types of glumerular diseases:
Glomerular diseases | Disease | History and Symtoms | Laboratory Findings | Pathology | ||||||||||||
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
History | Systemic symptoms | Hemeturia | Proteinuria | Hypertension | Pitting edema | Oliguria | Nephrotic features | Nephritic features | Hyperlipidemia and hypercholesterolemia | Auto-antibodies,
Complements |
Light microscope | Electron microscope | Immunoflourescence pattern | |||
Acute Nephritic Syndromes | Poststreptococcal Glomerulonephritis[1][2][3] |
|
+/- | + | +/- | +/- | +/- | +/- | +/- | +/- |
|
|
| |||
Renal disease due to Subacute Bacterial Endocarditis, or cardiac shunt (Atrioventricular)[4][5] |
|
+/- | + | +/- | +/- | +/- | +/- | +/- | +/- |
|
|
|
| |||
Lupus Nephritis[6] |
|
|
+/- | + | +/- | +/- | +/- | +/- | +/- | +/- |
|
|
|
| ||
Antiglomerular Basement Membrane Disease (Goodpasture's syndrome)[7][8] |
|
|
+ | + | + | + | + | + | - | - | Diffuse thickening of the glomerular basement membrane with absence of sub-epithelial and sub-endothelial deposits |
| ||||
IgA Nephropathy[9][10] |
|
|
+ | +/- | + | +/- | + | - | + | - |
|
|
|
| ||
Disease | History | Systemic symptoms | Hemeturia | Proteinuria | Hypertension | Pitting edema | Oliguria | Nephrotic features | Nephritic features | Hyperlipidemia and hypercholesterolemia | Auto-antibodies,
Complements |
Light microscope | Electron microscope | Immunoflourescence pattern | ||
ANCA Small-Vessel Vasculitis[11][12] | Granulomatosis with Polyangiitis (Wegener's)[13][14][15] |
|
|
+ | + | + | +/- | + | - | + | - |
|
|
| ||
Microscopic Polyangiitis[16] | +/- |
|
+ | + | + | + | + | + | - |
| ||||||
Churg-Strauss Syndrome[17] | +/- | + | + | + | + | + | + | - |
| |||||||
Membranoproliferative Glomerulonephritis[18][19] |
|
+ | + | + | +/- | + | + | - | - | - |
|
| ||||
Henoch-Schönlein purpura [20] |
|
|
+ | + | + | +/- | + | + | - | - | - |
|
|
| ||
Disease | History | Systemic symptoms | Hemeturia | Proteinuria | Hypertension | Pitting edema | Oliguria | Nephrotic features | Nephritic features | Hyperlipidemia and hypercholesterolemia | Auto-antibodies,
Complements |
Light microscope | Electron microscope | Immunoflourescence pattern | ||
Cryoglobulinemia[21] | Patients having cryoglobulinemia may have positive history of:
|
Pulmonary symptoms:
Cutaneous symptoms: Gastrointestinal symptoms:
General symptoms:
|
+/- | + | +/- | + | +/- | +/- | +/- | +/- | +/- |
|
| |||
Nephrotic Syndrome | Minimal Change Disease[22][23] |
|
- | + | - | + | +/- | + | - | + |
|
|
- | |||
Focal Segmental Glomerulosclerosis[24][25][26] |
|
- | + | - | + | +/- | + | - | + |
|
|
- | ||||
Membranous Glomerulonephritis[27][28] |
|
- | + | - | + | +/- | + | - | + | Immune complex deposition |
|
Immune complex GN, granular deposite | ||||
Diabetic Nephropathy[29][30][31][32][33][34][35][36][37][38] | For more information on diabetes click here. | - | + | - | + | +/- | + | - | + |
|
|
- | ||||
Disease | History | Systemic symptoms | Hemeturia | Proteinuria | Hypertension | Pitting edema | Oliguria | Nephrotic features | Nephritic features | Hyperlipidemia and hypercholesterolemia | Auto-antibodies,
Complements |
Light microscope | Electron microscope | Immunoflourescence pattern | ||
Glomerular Deposition Diseases | Light Chain Deposition Disease[39] |
|
- | - | + | - | + | +/- | + | - | + | - |
|
|
| |
Renal Amyloidosis[40][41][42][43] |
|
- | + | - | + | +/- | + | - | + | - |
|
|
| |||
Fibrillary-Immunotactoid Glomerulopathy[44] | - | +/- | + | +/- | +/- | +/- | + | +/- | +/- | - |
|
|
| |||
Fabry's Disease[45][46][47] |
|
|
- | + | - | + | +/- | + | - | + | - |
|
|
- | ||
Basement Membrane Syndrome | Alport's Syndrome[48][49][50][51][52][53] |
|
Auditary:
Occular problems:
|
- | + | - | + | +/- | + | - | + | - |
|
|
| |
Disease | History | Systemic symptoms | Hemeturia | Proteinuria | Hypertension | Pitting edema | Oliguria | Nephrotic features | Nephritic features | Hyperlipidemia and hypercholesterolemia | Auto-antibodies,
Complements |
Light microscope | Electron microscope | Immunoflourescence pattern | ||
Thin Basement Membrane Disease[54][55] |
|
- | - | + | -/+ | - | -/+ | - | -/+ | - | - | - | Diffuse thinning of the glomerular basement membranes (GBM) | - | ||
Nail-Patella Syndrome[56][57] |
|
|
+ | + | - | - | - | - | - | - | - |
|
|
| ||
Glomerular-Vascular Syndromes | Hypertensive Nephrosclerosis[58] | Chronic hypertension |
|
+/- | +/- | + | +/- | +/- | +/- | - | +/- | - | ||||
Cholesterol Emboli[59] |
|
|
+/- | +/- | + | +/- | +/- | +/- | - | +/- | - |
|
|
| ||
Disease | History | Systemic symptoms | Hemeturia | Proteinuria | Hypertension | Pitting edema | Oliguria | Nephrotic features | Nephritic features | Hyperlipidemia and hypercholesterolemia | Auto-antibodies,
Complements |
Light microscope | Electron microscope | Immunoflourescence pattern | ||
Sickle Cell Disease[60] |
|
|
+/- | +/- | +/- | - | - | - | - | - | - |
| ||||
Thrombotic Microangiopathies[61] | Click for more information on Thrombotic Microangiopathies. | + | +/- | + | +/- | +/- | +/- | - | - | - |
|
|
| |||
Antiphospholipid Antibody Syndrome [62][63][64] |
|
|
+ | +/- | + | +/- | +/- | +/- | - | - | - |
|
|
|
Some infectious diseases such as HIV, HBV, HCV, syphilis, leprosy, malaria, and schistosomiasis may cause glomerular diseases.
References
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- ↑ Alchi B, Jayne D (August 2010). "Membranoproliferative glomerulonephritis". Pediatr. Nephrol. 25 (8): 1409–18. doi:10.1007/s00467-009-1322-7. PMC 2887509. PMID 19908070.
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- ↑ Gephardt GN, Tubbs RR, Popowniak KL, McMahon JT (October 1986). "Focal and segmental glomerulosclerosis. Immunohistologic study of 20 renal biopsy specimens". Arch. Pathol. Lab. Med. 110 (10): 902–5. PMID 2429634.
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- ↑ Wasserstein AG (April 1997). "Membranous glomerulonephritis". J. Am. Soc. Nephrol. 8 (4): 664–74. PMID 10495797.
- ↑ Drummond K, Mauer M, International Diabetic Nephropathy Study Group (2002). "The early natural history of nephropathy in type 1 diabetes: II. Early renal structural changes in type 1 diabetes". Diabetes. 51 (5): 1580–7. PMID 11978659.
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- ↑ Alpers CE, Biava CG (1989). "Idiopathic lobular glomerulonephritis (nodular mesangial sclerosis): a distinct diagnostic entity". Clin Nephrol. 32 (2): 68–74. PMID 2766585.
- ↑ Toyoda M, Najafian B, Kim Y, Caramori ML, Mauer M (2007). "Podocyte detachment and reduced glomerular capillary endothelial fenestration in human type 1 diabetic nephropathy". Diabetes. 56 (8): 2155–60. doi:10.2337/db07-0019. PMID 17536064.
- ↑ Najafian B, Crosson JT, Kim Y, Mauer M (2006). "Glomerulotubular junction abnormalities are associated with proteinuria in type 1 diabetes". J Am Soc Nephrol. 17 (4 Suppl 2): S53–60. doi:10.1681/ASN.2005121342. PMID 16565248.
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- ↑ Najafian B, Alpers CE, Fogo AB (2011). "Pathology of human diabetic nephropathy". Contrib Nephrol. 170: 36–47. doi:10.1159/000324942. PMID 21659756.
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- ↑ Korbet SM, Schwartz MM, Lewis EJ (March 1991). "Immunotactoid glomerulopathy". Am. J. Kidney Dis. 17 (3): 247–57. PMID 1996564.
- ↑ Alroy J, Sabnis S, Kopp JB (June 2002). "Renal pathology in Fabry disease". J. Am. Soc. Nephrol. 13 Suppl 2: S134–8. PMID 12068025.
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|month=
ignored (help) - ↑ Branton MH, Schiffmann R, Sabnis SG; et al. (2002). "Natural history of Fabry renal disease: influence of alpha-galactosidase A activity and genetic mutations on clinical course". Medicine. 81 (2): 122–38. PMID 11889412. Unknown parameter
|month=
ignored (help) - ↑ McCarthy PA, Maino DM (2000). "Alport syndrome: a review". Clin Eye Vis Care. 12 (3–4): 139–150. PMID 11137428.
- ↑ Chugh KS, Sakhuja V, Agarwal A, Jha V, Joshi K, Datta BN; et al. (1993). "Hereditary nephritis (Alport's syndrome)--clinical profile and inheritance in 28 kindreds". Nephrol Dial Transplant. 8 (8): 690–5. PMID 8414153.
- ↑ Chugh KS, Sakhuja V, Agarwal A, Jha V, Joshi K, Datta BN; et al. (1993). "Hereditary nephritis (Alport's syndrome)--clinical profile and inheritance in 28 kindreds". Nephrol Dial Transplant. 8 (8): 690–5. PMID 8414153.
- ↑ McCarthy PA, Maino DM (2000). "Alport syndrome: a review". Clin Eye Vis Care. 12 (3–4): 139–150. PMID 11137428.
- ↑ Amari F, Segawa K, Ando F (1994). "Lens coloboma and Alport-like glomerulonephritis". Eur J Ophthalmol. 4 (3): 181–3. PMID 7819734.
- ↑ Govan JA (1983). "Ocular manifestations of Alport's syndrome: a hereditary disorder of basement membranes?". Br J Ophthalmol. 67 (8): 493–503. PMC 1040106. PMID 6871140.
- ↑ Savige J, Rana K, Tonna S, Buzza M, Dagher H, Wang YY (2003). "Thin basement membrane nephropathy". Kidney Int. 64 (4): 1169–78. doi:10.1046/j.1523-1755.2003.00234.x. PMID 12969134. Unknown parameter
|month=
ignored (help) - ↑ Hou P, Chen Y, Ding J, Li G, Zhang H (2007). "A novel mutation of COL4A3 presents a different contribution to Alport syndrome and thin basement membrane nephropathy". Am. J. Nephrol. 27 (5): 538–44. doi:10.1159/000107666. PMID 17726307.
- ↑ Najafian B, Smith K, Lusco MA, Alpers CE, Fogo AB (October 2017). "AJKD Atlas of Renal Pathology: Nail-Patella Syndrome-Associated Nephropathy". Am. J. Kidney Dis. 70 (4): e19–e20. doi:10.1053/j.ajkd.2017.08.001. PMID 28941488.
- ↑ Guidera KJ, Satterwhite Y, Ogden JA, Pugh L, Ganey T (1991). "Nail patella syndrome: a review of 44 orthopaedic patients". J Pediatr Orthop. 11 (6): 737–42. PMID 1960197.
- ↑ Hughson MD, Puelles VG, Hoy WE, Douglas-Denton RN, Mott SA, Bertram JF (July 2014). "Hypertension, glomerular hypertrophy and nephrosclerosis: the effect of race". Nephrol. Dial. Transplant. 29 (7): 1399–409. doi:10.1093/ndt/gft480. PMC 4071048. PMID 24327566.
- ↑ Lusco MA, Najafian B, Alpers CE, Fogo AB (April 2016). "AJKD Atlas of Renal Pathology: Cholesterol Emboli". Am. J. Kidney Dis. 67 (4): e23–4. doi:10.1053/j.ajkd.2016.02.034. PMID 27012950.
- ↑ Wesson DE (June 2002). "The initiation and progression of sickle cell nephropathy". Kidney Int. 61 (6): 2277–86. doi:10.1046/j.1523-1755.2002.00363.x. PMID 12028473.
- ↑ Lusco MA, Fogo AB, Najafian B, Alpers CE (December 2016). "AJKD Atlas of Renal Pathology: Thrombotic Microangiopathy". Am. J. Kidney Dis. 68 (6): e33–e34. doi:10.1053/j.ajkd.2016.10.006. PMID 27884283.
- ↑ Jayakody Arachchillage D, Greaves M (2014). "The chequered history of the antiphospholipid syndrome". Br J Haematol. 165 (5): 609–17. doi:10.1111/bjh.12848. PMID 24684307.
- ↑ Jayakody Arachchillage D, Greaves M (2014). "The chequered history of the antiphospholipid syndrome". Br J Haematol. 165 (5): 609–17. doi:10.1111/bjh.12848. PMID 24684307.
- ↑ Popa A, Voinea L, Pop M, Stana D, Dascalu AM, Alexandrescu C; et al. (2008). "[Primary antiphospholipid syndrome]". Oftalmologia. 52 (1): 13–7. PMID 18714484.