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[edit | edit source]

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[edit | edit source]

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[edit | edit source]

### Pathogenesis:[edit | edit source]

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

* Accumulation of matrix proteins compress the glomerular capillaryies and cause renal failure.[4]

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[edit | edit source]

On light microscopy:

-In LCDD:

* No glomerular and vascular abnormality
* Some tubular dilation with flattened epithelium suggest acute tubular injury
* Negative Congo Red stain

\- In Amyloidosis:

* Typical green birefringence under polarized light after Congo red staining (appears in red under normal light)

\- In Fabry's disease:

* Hypertrophic podocytes of glomerul, foamy appearing vacuoles and mesangial widening. Vacuolated cells will be seen in PAS stain.[6]

\- In Fibrillary immuno-tactoid glumerulopathy:

* Capillary wall thickening, matrix expansion,interstitial infiltration and necrosis in renal tubules and negative Congo red stain.[7]

\- In Collagenofibrotic glomerulopathy:

* Diffuse increase in the mesangial matrix, capillary walls widening, and collagen fibers in the glomerular basement membrane.[8]

On electron microscopy :

-In LCDD:

* Ground-pepper–like deposits in the mesangial and the endothelial of the glomerular basement membrane. Also, in renal tubules if there is tubular involvement.

\- In Amyloidosis:

* Endothelium, glomerular basement membrane, and podocytes thickening.[9]

\- In Fabry's disease:

* Deposition of glicolipids in lysosomes of podocytes and glomerular parietal epithelial cells.

\- In Fibrillary immuno-tactoid glomerulopathy:

* Predominant fibrils in the subepithelial of glomerular capillary.

\- In Collagenofibrotic glomerulopathy:

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

## Genetics[edit | edit source]

* 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[edit | edit source]

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

## Differentiating from Other Diseases[edit | edit source]

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] |

* Streptococcal skin infections
* Streptococcal pharyngitis
* 2-3 weeks after infection

* Fever
* Fatigue
* Skin rash

| +/-  | \+  | +/-  | +/-  | +/-  | +/-  | +/-  | +/-  |

* Anti-dsDNA antibodies
* Anti-C1q antibodies
* Antineutrophil cytoplasmic antibodies (ANCA)

* Hypercellular and inflamed glomeruli

* Sub-epithelial immune complex deposits
* Obliteration of epithelial cell foot processes

* Immune complex GN
* Granular deposit

Renal disease due to Subacute Bacterial Endocarditis, or cardiac shunt (Atrioventricular)[14][15] |

* History of infective endocarditis mostly due to S. aureus
* Cardiac shunt

* Fever
* Fatigue
* Weight loss

| +/-  | \+  | +/-  | +/-  | +/-  | +/-  | +/-  | +/-  |

* ANCA (myeloperoxidase) positive in 1/3
* Activation of the alternative complement pathway (Decreased C3, C4)
* Positive RF
* Positive anti-GBM autoantibodies

* Crescentic GN is the most common pathological features

* Diffuse membranoproliferative glomerulonephritis features
* Focal proliferative GN
* Mesangial proliferative GN

* Mesangial deposits,
* Subendothelial deposits
* Subepithelial "humps," in minority of cases

* Pauci-immune GN

Lupus Nephritis[16] |

* History of SLE features

* Lupus criteria:
* Malar rash
* Arthritis
* Arthralgia
* Anemia
* Easy bruising

| +/-  | \+  | +/-  | +/-  | +/-  | +/-  | +/-  | +/-  |

* Anti-C1q antibodies
* Anti-dsDNA

* 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

* Dry cough
* Hemoptysis
* Malaise
* Fever and chills
* Arthralgia
* Fatigue
* Lethargy
* Pallor
* Anorexia
* Easy bruising

| \+  | \+  | \+  | \+  | \+  | \+  | \-  | \-  |

* ANCA (myeloperoxidase)
* Positive anti-GBM autoantibodies

* Hypercellular and inflamed glomeruli (Crescent formation)

| 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] |

* Young children
* History of mucosal infections (e.g. gastroenteritis) and upper respiratory tract infection
* 2-3 days after infection (synpharyngitic)

* Low grade fever
* Flank pain

| \+  | +/-  | \+  | +/-  | \+  | \-  | \+  | \-  |

* Immune complex deposition

* Crescent formation

* Mesangial proliferation

* 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

* Constitutional symptoms
* Dyspnea
* Purpura
* Arthralgias
* Neurologic dysfunction
* Sinusitis
* Otitis media
* Epistaxis.

| \+  | \+  | \+  | +/-  | \+  | \-  | \+  | \-  |

* ANCA

* Necrotizing and crescentic glomerulonephritis

* Subendothelial edema

* Microthrombosis
* Degranulation of neutrophils

* Pauci-immune GN

Microscopic Polyangiitis[26] | +/-  |

* Constitutional symptoms
* Dyspnea
* Purpura
* Arthralgias
* Neurologic dysfunction

| \+  | \+  | \+  | \+  | \+  | \+  |  | \-  |

* P-ANCA

* Hypercellular and inflamed glomeruli (Crescent formation)

* Hypercellular and inflamed glomeruli (Crescent formation)

* Pauci-immune GN

Churg-Strauss Syndrome[27] | +/-  |

* Asthma
* Sinusitis
* Myalgia
* Arthralgia
* Purpura
* Arrythmias
* Peripheral neuropathy

| \+  | \+  | \+  | \+  | \+  | \+  |  | \-  |

* C-ANCA

* Hypercellular and inflamed glomeruli (Crescent formation)

* Hypercellular and inflamed glomeruli (Crescent formation)

* Pauci-immune GN

Membranoproliferative Glomerulonephritis[28][29] |

* Idiopathic
* Hepatitis B and C (Type 1)
* C3 nepritic factor (Type2)

* Hematuria
* Oliguria
* Periorbital edema
* Hypertension

| \+  | \+  | \+  | +/-  | \+  | \+  | \-  | \-  | \-  |

* Thick glomerular basement membrane (Tram-track appearance)

* Mesangial proliferation
* Leukocyte infiltration

* Immune complex GN
* Granular deposite

Henoch-Schönlein purpura [30] |

* Most common in young male, following upper respiratory infections

* Skin manifestations- Palpable purpura on buttocks
* Gastrointestinal manifestations-
* Abdominal pain
* Melena
* Bloody diarrhea
* Hematemesis
* Joints manifestations-
* Arthralgia

| \+  | \+  | \+  | +/-  | \+  | \+  | \-  | \-  | \-  |

* 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:

* Hepatitis C infection
* Hepatitis B infection
* Leg ulcers
* Recurrent thrombosis

| Pulmonary symptoms:

* Difficulty breathing

* Cough

Cutaneous symptoms:

* Purpura
* Skin ulcer

Gastrointestinal symptoms:

* Abdominal pain

General symptoms:

* Fever
* Arthralgia,
* Myalgia
* Fatigue

* Blurring/loss of vision
* Diplopia
* Confusion

| +/-  | \+  | +/-  | \+  | +/-  | +/-  | +/-  | +/-  | +/-  |

* Membranoproliferative glomerulonephritis

* Mesangial and subendothelial deposits

* Prominent IgM and C3

Nephrotic Syndrome  | Minimal Change Disease[32][33] |

* Young children
* Recent infection and immunization
* Atopy
* Hodgkin lymphoma
* Thrombosis (due to urinary loss of antithrombin-III)

| \-  | \+  | \-  | \+  | +/-  | \+  | \-  | \+  |  |

* Normal

* Fusion of podocytes

| \-
Focal Segmental Glomerulosclerosis[34][35][36] |

* Idiopathic
* HIV
* Heroine use
* Sickle cell disease
* Interferon
* Severe obesity
* Mixed cryoglobulinemia (Hepatitis C)

| \-  | \+  | \-  | \+  | +/-  | \+  | \-  | \+  |  |

* Focal (some glomeruli) and segmental (only part of glomerulus)

* Effacement of podocytes

| \-
Membranous Glomerulonephritis[37][38] |

* Idiopathic
* Hepatitis B and C
* Solid tumors
* Systemic lupus erythematosus
* Drugs (NSAIDS, pencilamine, gold, captopril)

| \-  | \+  | \-  | \+  | +/-  | \+  | \-  | \+  | Immune complex deposition  |

* Thick glomerular basement membrane

* Sub-epithelial immune complex depositis with 'spike and dome' appearance

| 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] |

* For the secondary causes of amloidosis:
* Tuberculosis
* Familial Mediterranean fever
* Rheumatoid arthritis
* Multiple myeloma

* Dyspnea
* Lethargy
* Weight loss

* Bleeding tendency

| \-  | \+  | \-  | \+  | +/-  | \+  | \-  | \+  | \-  |

* 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] |

* Malignancy
* Monoclonal gammopathy
* Autoimmune disease

| \-  | +/-  | \+  | +/-  | +/-  | +/-  | \+  | +/-  | +/-  | \-  |

* 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] |

* Family history suggestive of the disorder

* Anhidrosis or decreased sweating
* Fatigue
* Angiokeratomas
* Burning pain of the extremities
* Corneal opacities.
* Dysphagia
* Abdominal pain
* Steatorrhea
* Delayed puberty
* Raynaud's phenomenon
* Hearing loss
* Telangiectasis
* Ataxia

| \-  | \+  | \-  | \+  | +/-  | \+  | \-  | \+  | \-  |

* 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:

* Early Tinnitus
* Vertigo
* High-frequency progressive bilateral hearing loss

Occular problems:

* Refractory Error

* Arcus
* Glaucoma
* Band Keratopathy
* Lenticonus
* Spherophakia
* Cataracts

| \-  | \+  | \-  | \+  | +/-  | \+  | \-  | \+  | \-  |

* Early stage: unremarkable

* Late stages: glomerulosclerosis, interstitial fibrosis, and interstitial foam cells (due to prolonged proteinuria).

* Within glomerular basement membrane (GBM): longitudinal splitting of the lamina densa

* Absence of staining of the alpha-3, alpha-4, and alpha-5 (IV) chains in the glomerular basement membrane
* Minimal binding to a glomerulus in indirect immunofluorescence microscopy with anti-glomerular basement membrane antibodies

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

* Gross hematuria following upper respiratory tract infection

| \-  | \-  | \+  | -/+  | \-  | -/+  | \-  | -/+  | \-  | \-  | \-  | 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.

* Immunofluorescence studies are typically negative.

* Nonspecific IgM and C3 deposition may be seen in sclerotic glomeruli.

Glomerular-Vascular Syndromes | Hypertensive Nephrosclerosis[67] | Chronic hypertension |

* Hypertensive retinal changes.

* High jugular venous pressure

* Rales from pulmonary edema on auscultation
* .Signs of left ventricular hypertrophy

* Left ventricular heave
* Shifting of apex towards the left

* S3 and gallop rhythm
* Loud S2

* Ascites

* Paralysis from stroke secondary to hypertension

* Inferior limb edema

| +/-  | +/-  | \+  | +/-  | +/-  | +/-  | \-  | +/-  | \-  |

* Interstitial fibrosis and atrophy
* Medial thickening and intimal fibrosis of medium-sized and larger vessels
* Arteriolar thickening, and hyalinosis
* Chronic stages:
* Global sclerosis
* Focal segmental sclerosis

Cholesterol Emboli[68] |

* Atherosclerotic cardiovascular disease
* Anticoagulation therapy
* Cardiopulmonary resuscitation
* Hypertension
* Aortic aneurysm
* Hypercholesterolemia
* Smoking history
* Male sex
* Age over 55 years
* Vascular procedures
* Invasive angiography
* Aortic aneurysm rupture or surgery
* Vascular surgery

* 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

* Pain and/or bone pain
* Dactylitis
* Blurry vision
* Persistent and painful erection
* Numbness
* Tingling
* Motor skill loss
* Speech deficits
* Gait disturbance
* Leg ulceration
* Jaundice

| +/-  | +/-  | +/-  | \-  | \-  | \-  | \-  | \-  | \-  |

* 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.

* Thrombi stain positive for fibrinogen

Antiphospholipid Antibody Syndrome [71][72][73] |

* Thrombosis
* Miscarriage
* History of nephropathy
* History of hematologic abnormalities
* Nonthrombotic neurologic symptoms, such as migraine headaches
* Pulmonary hypertension

* Fatigue
* Fever
* Weight loss

* Venous thrombosis
* Arterial thrombosis
* Thrombocytopenia
* Recurrent fetal loss

| \+  | +/-  | \+  | +/-  | +/-  | +/-  | \-  | \-  | \-  |

* 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.

* Thrombi stain positive for fibrinogen

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

## Epidemiology and Demographics[edit | edit source]

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[edit | edit source]

There are no established risk factors for glomerular deposition disease.

## Screening[edit | edit source]

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

## Natural History, Complications, and Prognosis[edit | edit source]

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]

* Age
* underlying hematologic disease
* Light chain deposition in other organs
* Renal function
* other medical diseases like diabetic nephropathy

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[edit | edit source]

### Diagnostic Study of Choice[edit | edit source]

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

### History and Symptoms[edit | edit source]

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[edit | edit source]

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

### Laboratory Findings[edit | edit source]

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

* Proteinuria (30-50% of cases have nephrotic syndrome)
* Hematuria (usually microscopic)
* Abnormal liver enzyme ( in case of liver involvement)
* Presence of a monoclonal protein in serum or urine

### Electrocardiogram[edit | edit source]

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

### X-ray[edit | edit source]

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

### Echocardiography or Ultrasound[edit | edit source]

* 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]

* Low EF
* Diffuse hypokinesia
* Left ventricular concentric hypertrophy
* Low diastolic compliance

* Ultrasound:

There are no ultrasound findings associated with glomerular deposition disease.

### CT scan[edit | edit source]

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

### MRI[edit | edit source]

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

### Other Imaging Findings[edit | edit source]

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

### Other Diagnostic Studies[edit | edit source]

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

## Treatment[edit | edit source]

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).

* If patient has LCDD and Multiple Myeloma (MM), should receive treatment per MM guideline like Bortezomib1.3 mg/m^2 IV on days 1,4,8,11,22,25,29,32 of 42 day cycle for cycles 1-4.
* High-dose melphalan in conjunction with autologous stem cell transplantation

### Surgery[edit | edit source]

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[edit | edit source]

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

### Secondary Prevention[edit | edit source]

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

## References[edit | edit source]

1. ↑ 1.0 1.1 1.2 Pozzi C, D'Amico M, Fogazzi GB, Curioni S, Ferrario F, Pasquali S, Quattrocchio G, Rollino C, Segagni S, Locatelli F (December 2003). "Light chain deposition disease with renal involvement: clinical characteristics and prognostic factors". Am. J. Kidney Dis. 42 (6): 1154–63. PMID 14655186.
2. ↑ Alpers CE, Kowalewska J (January 2008). "Fibrillary glomerulonephritis and immunotactoid glomerulopathy". J. Am. Soc. Nephrol. 19 (1): 34–7. doi:10.1681/ASN.2007070757. PMID 18045849.
3. ↑ Nimmagadda S, Mukku K, Devaraju SR, Uppin MS (2017). "Unusual cause of glomerular deposition disease: Collagenofibrotic glomerulopathy". Indian J Nephrol. 27 (1): 62–65. doi:10.4103/0971-4065.179300. PMC 5255993. PMID 28182050.
4. ↑ Zhu L, Herrera GA, Murphy-Ullrich JE, Huang ZQ, Sanders PW (August 1995). "Pathogenesis of glomerulosclerosis in light chain deposition disease. Role for transforming growth factor-beta". Am. J. Pathol. 147 (2): 375–85. PMC 1869812. PMID 7639331.
5. ↑ Herrera GA (June 2000). "Renal manifestations of plasma cell dyscrasias: an appraisal from the patients' bedside to the research laboratory". Ann Diagn Pathol. 4 (3): 174–200. PMID 10919389.
6. ↑ 6.0 6.1 Alroy J, Sabnis S, Kopp JB (June 2002). "Renal pathology in Fabry disease". J. Am. Soc. Nephrol. 13 Suppl 2: S134–8. PMID 12068025.
7. ↑ Rosenstock JL, Markowitz GS, Valeri AM, Sacchi G, Appel GB, D'Agati VD (April 2003). "Fibrillary and immunotactoid glomerulonephritis: Distinct entities with different clinical and pathologic features". Kidney Int. 63 (4): 1450–61. doi:10.1046/j.1523-1755.2003.00853.x. PMID 12631361.
8. ↑ Gubler MC, Dommergues JP, Foulard M, Bensman A, Leroy JP, Broyer M, Habib R (August 1993). "Collagen type III glomerulopathy: a new type of hereditary nephropathy". Pediatr. Nephrol. 7 (4): 354–60. PMID 8398640.
9. ↑ SPIRO D (1959). "The structural basis of proteinuria in man; electron microscopic studies of renal biopsy specimens from patients with lipid nephrosis, amyloidosis, and subacute and chronic glomerulonephritis". Am. J. Pathol. 35 (1): 47–73. PMC 1934814. PMID 13617410.
10. ↑ Aoki T, Hayashi K, Morinaga T, Tomida H, Hishida M, Yamamoto S, Kajiwara N, Tamai H (May 2015). "Two brothers with collagenofibrotic glomerulopathy". CEN Case Rep. 4 (1): 85–89. doi:10.1007/s13730-014-0145-y. PMC 5413714. PMID 28509277.
11. ↑ GERMUTH FG (1953). "A comparative histologic and immunologic study in rabbits of induced hypersensitivity of the serum sickness type". J Exp Med. 97 (2): 257–82. PMC 2136196. PMID 13022878.CS1 maint: PMC format (link)
12. ↑ Germuth FG, Senterfit LB, Dreesman GR (1972). "Immune complex disease. V. The nature of the circulating complexes associated with glomerular alterations in the chronic BSA-rabbit system". Johns Hopkins Med J. 130 (6): 344–57. PMID 5031005.CS1 maint: Multiple names: authors list (link)
13. ↑ Radhakrishnan J, Cattran DC (2012). "The KDIGO practice guideline on glomerulonephritis: reading between the (guide)lines--application to the individual patient". Kidney Int. 82 (8): 840–56. doi:10.1038/ki.2012.280. PMID 22895519.
14. ↑ Neugarten J, Baldwin DS (August 1984). "Glomerulonephritis in bacterial endocarditis". Am. J. Med. 77 (2): 297–304. PMID 6380288.
15. ↑ Arze RS, Rashid H, Morley R, Ward MK, Kerr DN (January 1983). "Shunt nephritis: report of two cases and review of the literature". Clin. Nephrol. 19 (1): 48–53. PMID 6831779.
16. ↑ Weening JJ, D'Agati VD, Schwartz MM, Seshan SV, Alpers CE, Appel GB, Balow JE, Bruijn JA, Cook T, Ferrario F, Fogo AB, Ginzler EM, Hebert L, Hill G, Hill P, Jennette JC, Kong NC, Lesavre P, Lockshin M, Looi LM, Makino H, Moura LA, Nagata M (February 2004). "The classification of glomerulonephritis in systemic lupus erythematosus revisited". Kidney Int. 65 (2): 521–30. doi:10.1111/j.1523-1755.2004.00443.x. PMID 14717922.
17. ↑ Bolton WK (November 1996). "Goodpasture's syndrome". Kidney Int. 50 (5): 1753–66. PMID 8914046.
18. ↑ Mathew TH, Hobbs JB, Kalowski S, Sutherland PW, Kincaid-Smith P (February 1975). "Goodpasture's syndrome: normal renal diagnostic findings". Ann. Intern. Med. 82 (2): 215–8. PMID 1090223.
19. ↑ Suzuki H, Kiryluk K, Novak J, Moldoveanu Z, Herr AB, Renfrow MB, Wyatt RJ, Scolari F, Mestecky J, Gharavi AG, Julian BA (October 2011). "The pathophysiology of IgA nephropathy". J. Am. Soc. Nephrol. 22 (10): 1795–803. doi:10.1681/ASN.2011050464. PMC 3892742. PMID 21949093.
20. ↑ Wyatt RJ, Julian BA (June 2013). "IgA nephropathy". N. Engl. J. Med. 368 (25): 2402–14. doi:10.1056/NEJMra1206793. PMID 23782179.
21. ↑ Higgins RM, Goldsmith DJ, Connolly J, Scoble JE, Hendry BM, Ackrill P, Venning MC (January 1996). "Vasculitis and rapidly progressive glomerulonephritis in the elderly". Postgrad Med J. 72 (843): 41–4. PMC 2398323. PMID 8746284.
22. ↑ Jennette JC (March 2003). "Rapidly progressive crescentic glomerulonephritis". Kidney Int. 63 (3): 1164–77. doi:10.1046/j.1523-1755.2003.00843.x. PMID 12631105.
23. ↑ Renaudineau Y, Le Meur Y (October 2008). "Renal involvement in Wegener's granulomatosis". Clin Rev Allergy Immunol. 35 (1–2): 22–9. doi:10.1007/s12016-007-8066-6. PMID 18172777.
24. ↑ Weiss MA, Crissman JD (October 1984). "Renal biopsy findings in Wegener's granulomatosis: segmental necrotizing glomerulonephritis with glomerular thrombosis". Hum. Pathol. 15 (10): 943–56. PMID 6384024.
25. ↑ Pagnoux C (March 2008). "[Wegener's granulomatosis and microscopic polyangiitis]". Rev Prat (in French). 58 (5): 522–32. PMID 18524109.CS1 maint: Unrecognized language (link)
26. ↑ Chung SA, Seo P (August 2010). "Microscopic polyangiitis". Rheum. Dis. Clin. North Am. 36 (3): 545–58. doi:10.1016/j.rdc.2010.04.003. PMC 2917831. PMID 20688249.
27. ↑ Sinico RA, Di Toma L, Maggiore U, Tosoni C, Bottero P, Sabadini E, Giammarresi G, Tumiati B, Gregorini G, Pesci A, Monti S, Balestrieri G, Garini G, Vecchio F, Buzio C (May 2006). "Renal involvement in Churg-Strauss syndrome". Am. J. Kidney Dis. 47 (5): 770–9. doi:10.1053/j.ajkd.2006.01.026. PMID 16632015.
28. ↑ 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.
29. ↑ Davis AE, Schneeberger EE, Grupe WE, McCluskey RT (May 1978). "Membranoproliferative glomerulonephritis (MPGN type I) and dense deposit disease (DDD) in children". Clin. Nephrol. 9 (5): 184–93. PMID 657595.
30. ↑ Jennette JC, Falk RJ (July 1994). "The pathology of vasculitis involving the kidney". Am. J. Kidney Dis. 24 (1): 130–41. PMID 8023818.
31. ↑ Fogo AB, Lusco MA, Najafian B, Alpers CE (February 2016). "AJKD Atlas of Renal Pathology: Cryoglobulinemic Glomerulonephritis". Am. J. Kidney Dis. 67 (2): e5–7. doi:10.1053/j.ajkd.2015.12.007. PMID 26802335.
32. ↑ Saha TC, Singh H (November 2006). "Minimal change disease: a review". South. Med. J. 99 (11): 1264–70. doi:10.1097/01.smj.0000243183.87381.c2. PMID 17195422.
33. ↑ Saleem MA, Kobayashi Y (2016). "Cell biology and genetics of minimal change disease". F1000Res. 5. doi:10.12688/f1000research.7300.1. PMC 4821284. PMID 27092244.
34. ↑ Rosenberg AZ, Kopp JB (March 2017). "Focal Segmental Glomerulosclerosis". Clin J Am Soc Nephrol. 12 (3): 502–517. doi:10.2215/CJN.05960616. PMC 5338705. PMID 28242845.
35. ↑ Jefferson JA, Shankland SJ (September 2014). "The pathogenesis of focal segmental glomerulosclerosis". Adv Chronic Kidney Dis. 21 (5): 408–16. doi:10.1053/j.ackd.2014.05.009. PMC 4149756. PMID 25168829.
36. ↑ 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.
37. ↑ Lai WL, Yeh TH, Chen PM, Chan CK, Chiang WC, Chen YM, Wu KD, Tsai TJ (February 2015). "Membranous nephropathy: a review on the pathogenesis, diagnosis, and treatment". J. Formos. Med. Assoc. 114 (2): 102–11. doi:10.1016/j.jfma.2014.11.002. PMID 25558821.
38. ↑ Wasserstein AG (April 1997). "Membranous glomerulonephritis". J. Am. Soc. Nephrol. 8 (4): 664–74. PMID 10495797.
39. ↑ 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.CS1 maint: Multiple names: authors list (link)
40. ↑ Hørlyck A, Gundersen HJ, Osterby R (1986). "The cortical distribution pattern of diabetic glomerulopathy". Diabetologia. 29 (3): 146–50. PMID 3699305.CS1 maint: Multiple names: authors list (link)
41. ↑ Alpers CE, Hudkins KL (2011). "Mouse models of diabetic nephropathy". Curr Opin Nephrol Hypertens. 20 (3): 278–84. doi:10.1097/MNH.0b013e3283451901. PMC 3658822. PMID 21422926.CS1 maint: PMC format (link)
42. ↑ Kimmelstiel P, Wilson C (1936). "Intercapillary Lesions in the Glomeruli of the Kidney". Am J Pathol. 12 (1): 83–98.7. PMC 1911022. PMID 19970254.CS1 maint: PMC format (link)
43. ↑ Alpers CE, Biava CG (1989). "Idiopathic lobular glomerulonephritis (nodular mesangial sclerosis): a distinct diagnostic entity". Clin Nephrol. 32 (2): 68–74. PMID 2766585.
44. ↑ 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.CS1 maint: Multiple names: authors list (link)
45. ↑ 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.CS1 maint: Multiple names: authors list (link)
46. ↑ Najafian B, Kim Y, Crosson JT, Mauer M (2003). "Atubular glomeruli and glomerulotubular junction abnormalities in diabetic nephropathy". J Am Soc Nephrol. 14 (4): 908–17. PMID 12660325.CS1 maint: Multiple names: authors list (link)
47. ↑ Najafian B, Alpers CE, Fogo AB (2011). "Pathology of human diabetic nephropathy". Contrib Nephrol. 170: 36–47. doi:10.1159/000324942. PMID 21659756.CS1 maint: Multiple names: authors list (link)
48. ↑ Najafian B, Alpers CE, Fogo AB (2011). "Pathology of human diabetic nephropathy". Contrib Nephrol. 170: 36–47. doi:10.1159/000324942. PMID 21659756.CS1 maint: Multiple names: authors list (link)
49. ↑ Hutchison CA, Cockwell P, Stringer S, Bradwell A, Cook M, Gertz MA, Dispenzieri A, Winters JL, Kumar S, Rajkumar SV, Kyle RA, Leung N (June 2011). "Early reduction of serum-free light chains associates with renal recovery in myeloma kidney". J. Am. Soc. Nephrol. 22 (6): 1129–36. doi:10.1681/ASN.2010080857. PMC 3103732. PMID 21511832.
50. ↑ Baker KR, Rice L (2012). "The amyloidoses: clinical features, diagnosis and treatment". Methodist Debakey Cardiovasc J. 8 (3): 3–7. PMC 3487569. PMID 23227278.
51. ↑ Gillmore JD, Hawkins PN (October 2013). "Pathophysiology and treatment of systemic amyloidosis". Nat Rev Nephrol. 9 (10): 574–86. doi:10.1038/nrneph.2013.171. PMID 23979488.
52. ↑ Jerzykowska S, Cymerys M, Gil LA, Balcerzak A, Pupek-Musialik D, Komarnicki MA (2014). "Primary systemic amyloidosis as a real diagnostic challenge - case study". Cent Eur J Immunol. 39 (1): 61–6. doi:10.5114/ceji.2014.42126. PMC 4439975. PMID 26155101.
53. ↑ Pepys MB (2006). "Amyloidosis". Annu. Rev. Med. 57: 223–41. doi:10.1146/annurev.med.57.121304.131243. PMID 16409147.
54. ↑ Korbet SM, Schwartz MM, Lewis EJ (March 1991). "Immunotactoid glomerulopathy". Am. J. Kidney Dis. 17 (3): 247–57. PMID 1996564.
55. ↑ Meikle PJ, Hopwood JJ, Clague AE, Carey WF (1999). "Prevalence of lysosomal storage disorders". JAMA : the Journal of the American Medical Association. 281 (3): 249–54. PMID 9918480. Unknown parameter `|month=` ignored (help)CS1 maint: Multiple names: authors list (link)
56. ↑ 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)CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)
57. ↑ McCarthy PA, Maino DM (2000). "Alport syndrome: a review". Clin Eye Vis Care. 12 (3–4): 139–150. PMID 11137428.
58. ↑ 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.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)
59. ↑ 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.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)
60. ↑ McCarthy PA, Maino DM (2000). "Alport syndrome: a review". Clin Eye Vis Care. 12 (3–4): 139–150. PMID 11137428.
61. ↑ Amari F, Segawa K, Ando F (1994). "Lens coloboma and Alport-like glomerulonephritis". Eur J Ophthalmol. 4 (3): 181–3. PMID 7819734.CS1 maint: Multiple names: authors list (link)
62. ↑ 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.CS1 maint: PMC format (link)
63. ↑ 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)CS1 maint: Multiple names: authors list (link)
64. ↑ 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.CS1 maint: Multiple names: authors list (link)
65. ↑ 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.
66. ↑ 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.
67. ↑ 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.
68. ↑ 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.
69. ↑ 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.
70. ↑ 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.
71. ↑ 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.
72. ↑ 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.
73. ↑ 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.CS1 maint: Explicit use of et al. (link) CS1 maint: Multiple names: authors list (link)
74. ↑ Khan MF, Falk RH (November 2001). "Amyloidosis". Postgrad Med J. 77 (913): 686–93. PMC 1742163. PMID 11677276.
75. ↑ Spada M, Pagliardini S, Yasuda M, Tukel T, Thiagarajan G, Sakuraba H, Ponzone A, Desnick RJ (July 2006). "High incidence of later-onset fabry disease revealed by newborn screening". Am. J. Hum. Genet. 79 (1): 31–40. doi:10.1086/504601. PMC 1474133. PMID 16773563.
76. ↑ Lin J, Markowitz GS, Valeri AM, Kambham N, Sherman WH, Appel GB, D'Agati VD (July 2001). "Renal monoclonal immunoglobulin deposition disease: the disease spectrum". J. Am. Soc. Nephrol. 12 (7): 1482–92. PMID 11423577.
77. ↑ Joh K (September 2007). "Pathology of glomerular deposition diseases". Pathol. Int. 57 (9): 551–65. doi:10.1111/j.1440-1827.2007.02139.x. PMID 17685926.
78. ↑ Koopman P, Van Dorpe J, Maes B, Dujardin K (December 2009). "Light chain deposition disease as a rare cause of restrictive cardiomyopathy". Acta Cardiol. 64 (6): 821–4. doi:10.2143/AC.64.6.2044752. PMID 20128164.
79. ↑ Buxbaum JN, Chuba JV, Hellman GC, Solomon A, Gallo GR (March 1990). "Monoclonal immunoglobulin deposition disease: light chain and light and heavy chain deposition diseases and their relation to light chain amyloidosis. Clinical features, immunopathology, and molecular analysis". Ann. Intern. Med. 112 (6): 455–64. PMID 2106817.
80. ↑ Katzmann JA, Clark RJ, Abraham RS, Bryant S, Lymp JF, Bradwell AR, Kyle RA (September 2002). "Serum reference intervals and diagnostic ranges for free kappa and free lambda immunoglobulin light chains: relative sensitivity for detection of monoclonal light chains". Clin. Chem. 48 (9): 1437–44. PMID 12194920.
81. ↑ Ozsan GH, Dispenzieri A (January 2011). "Serum free light chain analysis in multiple myeloma and plasma cell dyscrasias". Expert Rev Clin Immunol. 7 (1): 65–73. doi:10.1586/eci.10.80. PMID 21162651.
82. ↑ Mohan M, Gokden M, Gokden N, Schinke C (March 2016). "A Case of Cardiac Light Chain Deposition Disease in a Patient with Solitary Plasmacytoma". Am J Case Rep. 17: 173–6. PMC 4801155. PMID 26988342.
83. ↑ Gertz MA (February 2012). "Managing light chain deposition disease". Leuk. Lymphoma. 53 (2): 183–4. doi:10.3109/10428194.2011.622423. PMID 21913808.
84. ↑ Rajkumar SV (January 2011). "Multiple myeloma: 2011 update on diagnosis, risk-stratification, and management". Am. J. Hematol. 86 (1): 57–65. doi:10.1002/ajh.21913. PMID 21181954.
85. ↑ Gharwan H, Truica CI (June 2012). "Bortezomib-based chemotherapy for light chain deposition disease presenting as acute renal failure". Med. Oncol. 29 (2): 1197–201. doi:10.1007/s12032-011-9938-4. PMID 21479696.
86. ↑ Leung N, Lager DJ, Gertz MA, Wilson K, Kanakiriya S, Fervenza FC (January 2004). "Long-term outcome of renal transplantation in light-chain deposition disease". Am. J. Kidney Dis. 43 (1): 147–53. PMID 14712438.
87. ↑ Short AK, O'Donoghue DJ, Riad HN, Short CD, Roberts IS (2001). "Recurrence of light chain nephropathy in a renal allograft. A case report and review of the literature". Am. J. Nephrol. 21 (3): 237–40. doi:10.1159/000046254. PMID 11423695.

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