Glomerular deposition disease

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Aarti Narayan, M.B.B.S [2], Aida Javanbakht, M.D., Mehrian Jafarizade, M.D [3]

Synonyms and keywords: light chain deposition disease, LCDD

Overview

Localized deposition of fibrils and proteins in glumerole is called glomerular deposition disease. Light chain deposition disease (LCDD) is one of the glomerular deposition disease. It's a rare blood cell disease which is characterized by deposition of fragments of infection-fighting immunoglobulins, called light chains, in the body. These light chain deposits damage organs and cause disease. The kidneys are almost always affected and this often leads to Chronic renal failure. About half of people with light chain deposition disease also have multiple myeloma. Unlike in AL Amyloidosis, in which light chains are laid down in characteristic amyloid deposits, in LCDD, light chains are deposited in non-amyloid granules. Light chains in LCDD are kappa light chains in granular shape.[1]

Classification

Glomerular deposition disease may be classified according to pathology findings into into 5 groups: [2][3]

  • LCDD
  • Amyloidosis
  • Fabry's disease
  • Fibrillary immuno-tactoid glumerulopathy
  • Collagenofibrotic glomerulopathy

Pathophysiology

Pathogenesis:

Deposits of abnormal or extra production of proteins, lipids and fibrills in the glomeruls cause glomerular deposition diseases.

-In LCDD:

light chains are small polypeptides produced by B lymphocytes.They are sub units of antibodies. Kappa and Lambda are two types of light chains. Excess production of Kappa chain and accumulation in the renal glumerulus cause LCDD. The exact mechanism of increase production of light chains and reason that renal attracts them is unknown. These chains can deposit in all parts of renal glumeruls and tubuls.

Accumulation of monoclonal light chains and matrix proteins cause increase quantity and activity of transforming growth factor-beta (TGF-beta). TGF-beta inhibits mesangial cell proliferation and increase matrix protein production

Besides glumerulus,light chains may accumulate in renal tubular and make tubular casts. These casts cause interstitial inflammation and renal failure.[5]

- In Amyloidosis:  

Amyloids (misfolding and aggregation of normally soluble proteins) deposit in the nephrones and cause renal failure.

- In Fabry's disease:

A deficiency of the enzyme alpha galactosidase A causes deposition of glycolipid in the nephrones and cause renal failure.

- In Fibrillary immuno-tactoid glomerulopathy:

Fibrills (larger than amyloids) deposit in subendothelial and mesangium of the nephrones and cause function impairment.

- In Collagenofibrotic glomerulopathy:

Type III collagen fibers deposit in the subendothelial and mesangium in the kidney.

Microscopic Pathology

On light microscopy:

-In LCDD:

- In Amyloidosis:

- In Fabry's disease:

- In Fibrillary immuno-tactoid glumerulopathy:

- In Collagenofibrotic glomerulopathy:

On electron microscopy :

-In LCDD:

- In Amyloidosis:

- In Fabry's disease:

- In Fibrillary immuno-tactoid glomerulopathy:

- In Collagenofibrotic glomerulopathy:

  • Deposition of irregular collagenous fibers in the mesangium and subendothelial space of the renal glumerol.[10]

Genetics

  • Fabry's disease is transmitted in X-linked recessive pattern.
  • Hereditory types of amyloidosis is transmitted in autosomal dominant pattern.
  • There exact genetic association for LCDD, Fibrillary immuno-tactoid glumerulopathy, and Collagenofibrotic glomerulopathy are unknown.

Causes

The specific etiology that cause extra or abnormal production of fibrills and chains in the glomerular depositional diseases is unknown.

Differentiating from Other Diseases

Glomerular deposition disease 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[11][12][13] +/- + +/- +/- +/- +/- +/- +/-
  • Immune complex GN
  • Granular deposit
Renal disease due to Subacute Bacterial Endocarditis, or cardiac shunt (Atrioventricular)[14][15] +/- + +/- +/- +/- +/- +/- +/-
  • Crescentic GN is the most common pathological features
  • Mesangial deposits,
  • Subendothelial deposits
  • Subepithelial "humps," in minority of cases
  • Pauci-immune GN
Lupus Nephritis[16]
  • History of SLE features
+/- + +/- +/- +/- +/- +/- +/-
  • Differs based on the disease classification
  • Differs based on the disease classification
  • Differs based on the disease classification, mostly immune complex GN
  • Granular deposit
Antiglomerular Basement Membrane Disease (Goodpasture's syndrome)[17][18]
  • Young adults
+ + + + + + - - Diffuse thickening of the glomerular basement membrane with absence of sub-epithelial and sub-endothelial deposits 
  • Immune complex GN
  • Linear deposit
IgA Nephropathy[19][20] + +/- + +/- + - + -
  • Immune complex deposition
  • Crescent formation
  • Immune complex GN, granular deposite
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[21][22] Granulomatosis with Polyangiitis (Wegener's)[23][24][25]
  • Middle age male
+ + + +/- + - + -
  •  Pauci-immune GN
Microscopic Polyangiitis[26] +/- + + + + + + -
  •  Pauci-immune GN
Churg-Strauss Syndrome[27] +/- + + + + + + -
  •  Pauci-immune GN
Membranoproliferative Glomerulonephritis[28][29] + + + +/- + + - - -
  • Immune complex GN
  • Granular deposite
Henoch-Schönlein purpura [30] + + + +/- + + - - -
  • Diffuse mesangial IgA deposits often associated with mesangial hypercellularity
  • Diffuse mesangial IgA deposits often associated with mesangial hypercellularity
  • Immune complex GN, granular deposite
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[31] Patients having cryoglobulinemia may have positive history of: Pulmonary symptoms:
  • Cough

Cutaneous symptoms:

Gastrointestinal symptoms:

  • Abdominal pain

General symptoms:

+/- + +/- + +/- +/- +/- +/- +/-
  • Prominent IgM and C3
Nephrotic Syndrome Minimal Change Disease[32][33] - + - + +/- + - +
  • Normal
-
Focal Segmental Glomerulosclerosis[34][35][36] - + - + +/- + - + -
Membranous Glomerulonephritis[37][38] - + - + +/- + - + Immune complex deposition Immune complex GN, granular deposite
Diabetic Nephropathy[39][40][41][42][43][44][45][46][47][48] For more information on diabetes click here. - + - + +/- + - +
  • Diffuse mesangial matrix expansion (nodular glomerulosclerosis)
  • Increased mesangial hypercellularity
  • Prominent glomerular basement membranes
  • Thick basement membrane without any deposit
  • Nodular glomerulosclerosis
-
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[49]
  • Occurs in the setting of high tumor burden
- - + - + +/- + - + -
  • Light-chain deposits
  • Granular deposits on electron microscopy
  • Detection of light chain deposits using anti–light chain antibody
Renal Amyloidosis[50][51][52][53] - + - + +/- + - + -
  • Diffuse glomerular deposition of amorphous hyaline material (nodular pattern), in mesangium (weakly staining with periodic acid-Schiff (PAS)
  • Nodular deposit
  • AA amyloidosis type: negative for immunoglobulins and complement
  • AL amyloidosis type: Positive for lambda or kappa light chains
Fibrillary-Immunotactoid Glomerulopathy[54] - +/- + +/- +/- +/- + +/- +/- -
  • Diffuse sclerosing glomerulonephritis
  • Diffuse proliferative glomerulonephritis
  • Membranoproliferative glomerulonephritis
  • Mesangioproliferative/sclerosing disease
  • Membranous glomerulonephritis
  • Large fibrillar deposits in the mesangium randomly
  • Glomerular capillary walls different from amloidosis
  • No staining with Congo red or thioflavine-T or with antibodies to a specific type
  • Positive for immunoglobulin G (IgG), C3
  • Kappa and lambda (ie, polyclonal) light chains
Fabry's Disease[6][55][56] - + - + +/- + - + -
  • Vacuolization of visceral glomerular epithelial cells (podocytes) and distal tubular epithelial cells
  • Glycolipid accumulation
  • Myeloid or zebra bodies: Gb3 deposition within enlarged secondary lysosomes as lamellated membrane structures
  • Inclusions, composed of concentric layers (onion skin appearance)
-
Basement Membrane Syndrome Alport's Syndrome[57][58][59][60][61][62]
  • Positive family history
Auditary:

Occular problems:

  • Refractory Error
- + - + +/- + - + -
  • Early stage: unremarkable
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[63][64]
  • Positive family history
- - + -/+ - -/+ - -/+ - - - Diffuse thinning of the glomerular basement membranes (GBM) -
Nail-Patella Syndrome[65][66]
  • Positive family history
  • Poorly developed fingernails, toe nails, and patellae (kneecaps).
  • Elbow deformities
  • Abnormally shaped pelvis bone (hip bone)
  • Knee may be small, deformed or absent
+ + - - - - - - -
  • Mostly unremarkable changes
  • Secondary FSGS
  • Late stages:
    • Global glomerulosclerosis,
    • Tubulointerstitial fibrosis
  • Glomerular basement membranes (GBMs): Focal or diffuse irregular thickening with electron-lucent areas (moth-eaten appearance) containing type III collagen bundles.
  • Similar collagen fibrils can be seen in mesangial matrix.
  • Podocytes: Segmental effacement of foot processes.
  • Nonspecific IgM and C3 deposition may be seen in sclerotic glomeruli.
 Glomerular-Vascular Syndromes  Hypertensive Nephrosclerosis[67] Chronic hypertension +/- +/- + +/- +/- +/- - +/- -
  • Interstitial fibrosis and atrophy
  • Medial thickening and intimal fibrosis of medium-sized and larger vessels
  • Arteriolar thickening, and hyalinosis
  • Chronic stages:
Cholesterol Emboli[68]
  • Depends on the organ involved
+/- +/- + +/- +/- +/- - +/- -
  • Atheroemboli are seen in interlobular and arcuate arteries, as lance-shaped clefts, due to dissolution of cholesterol crystals
  • Acute lesions:
    • Atheroemboli are surrounded by red blood cells, fibrin, and leukocytes, with multinucleated giant cell reactions
  • Chronic lesions:
    • Cholesterol clefts are surrounded by intimal fibrosis
    • Vessel recanalization of chronic lesions can occur.
  • Global and segmental sclerosis of glomeruli may be present.
  • Extensive foot process effacement can be seen
  • Not specific changes
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[69]
  • Positive family history
+/- +/- +/- - - - - - -
  • Glomerular hypertrophy
  • Hemosiderin deposits
  • Focal areas of hemorrhage or necrosis
  • Chronic stage: interstitial inflammation, edema, fibrosis, tubular atrophy, and papillary infarcts
  • Glomerular enlargement and focal segmental glomerulosclerosis (FSGS)
Thrombotic Microangiopathies[70] Click for more information on Thrombotic Microangiopathies. + +/- + +/- +/- +/- - - -
  • Acute stage:
    • Inravasculr fibrin thrombi
  • Chronic stage:
    • Endocapillary hypercellularity.
    • Intimal proliferation of arterioles
  • Swollen glomerular endothelial cells with loss of fenestrations
  • Chronic stage: interposed cells with new GBM matrix material deposition.
Antiphospholipid Antibody Syndrome [71][72][73]
  • Fatigue
  • Fever
  • Weight loss
+ +/- + +/- +/- +/- - - -
  • Swollen glomerular endothelial cells with loss of fenestrations
  • Chronic stage: interposed cells with new GBM matrix material deposition.


Some infectious diseases such as HIV, HBV, HCV, syphilis, leprosy, malaria, and schistosomiasis may cause glomerular diseases.

Epidemiology and Demographics

The incidence of glomerular deposition disease depends on the type of the disease. Collagenofibrotic glomerulopathy and Fibrillary immuno-tactoid glumerulopathy are rare. LCDD is unknown. Most of the patients are men with the mean age of 58 years [1]. The incidence of amyloidosis is 1.2 per 100,000 individuals per year [74]. The incidence of Fabry's disease is 1 in 50,000 males [75].

Renal involvement is the most common cause of mortality and morbidity in these patients. Survival varies between months to 10 years in patients with LCDD [76].

Risk Factors

There are no established risk factors for glomerular deposition disease.

Screening

There is insufficient evidence to recommend routine screening for glomerular deposition disease.

Natural History, Complications, and Prognosis

If left untreated, most of the patients will suffer from ESRD in the future. Common complication of glomerular deposition diseases is renal failure. Prognostic factors of glomerular deposition diseases include:[1]

Prognosis is generally not good. The median time to progression to chronic renal failure in LCDD is 2.7 years. After 5 years, about 37% of patients with LCDD are alive and do not have end stage renal disease.

Diagnosis

Diagnostic Study of Choice

  • Biopsy is the gold standard test for the diagnosis of glomerular deposition disease.[77]

History and Symptoms

Usually patients are asymptomatic in early stages. Symptoms are nonspecific like fatigue and weight loss. In case of edema patients may come with edema. Patients may come with abdominal pain because of deposition of light chains in the liver may lead to hepatomegaly, portal hypertension and liver failure. Patients may come with palpitation because the heart is affected in up to 80% of patients with LCDD, and may cause arrhythmias restrictive cardiomyopathy, cardiomegaly, and congestive heart failure.[78]

Physical Examination

  • Physical exam is normal most of the time.
  • Sometimes there is an organomegaly which depends on involvement of the organ like hepatomegaly

Laboratory Findings

Laboratory findings consistent with the diagnosis of glomerular deposition disease include:[79][80][81]

Electrocardiogram

Arrhythmia like atrial fibrillation ( in case of heart involvement).

X-ray

There are no x-ray findings associated with glomerular deposition disease.

Echocardiography or Ultrasound

  • Echocardiography:

Echocardiography may be helpful in the diagnosis of glomerular deposition disease. Findings on echocardiography suggestive of glomerular deposition disease in case of heart involvement include:[82]

  • Ultrasound:

There are no ultrasound findings associated with glomerular deposition disease.

CT scan

  • There are no CT scan findings associated with glomerular deposition disease.
  • In severe cases the size of the organ will increase.

MRI

  • There are no MRI findings associated with glomerular deposition disease.
  • In severe cases the size of the organ will increase.

Other Imaging Findings

There are no other imaging findings associated with Glomerular deposition diseases.

Other Diagnostic Studies

There are no other diagnostic studies associated with Glomerular deposition diseases.

Treatment

Medical therapy:

70% of cases untreated patients will become to ESRD. There is no standard treatment for Glomerular deposition diseases. Medical therapy options for LCDD include:[83][84][85]

  • Symptomatic treatment for renal dysfunction like ACE inhibitors like Lisinopril 2.5 to 5 mg orally once a day ( dose depends on the level of the kidney damage).

Surgery

Surgery is not the first-line treatment option for patients with glomerular deposition disease. Surgery is usually reserved for patients with either:[86][87]

  • LCDD patients who have detectable light chains in urine or serum
  • ESRD

Primary Prevention

There are no established measures for the primary prevention of Glomerular deposition diseases.

Secondary Prevention

There are no established measures for the secondary prevention of Glomerular deposition diseases.

References

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