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==Epidemiology and Demographics==
==Epidemiology and Demographics==
The incidence of LCDD is unknown. Most of the patients are men with the mean age of  58 years  
The incidence of LCDD is unknown. Most of the patients are men with the mean age of  58 years <ref name="pmid14655186">{{cite journal |vauthors=Pozzi C, D'Amico M, Fogazzi GB, Curioni S, Ferrario F, Pasquali S, Quattrocchio G, Rollino C, Segagni S, Locatelli F |title=Light chain deposition disease with renal involvement: clinical characteristics and prognostic factors |journal=Am. J. Kidney Dis. |volume=42 |issue=6 |pages=1154–63 |date=December 2003 |pmid=14655186 |doi= |url=}}</ref>.


[[Renal|Renal involvement]] is the most common cause of [[mortality]] and [[morbidity]] in these patients. [[Survival rate|Survival]] varies between months to 10 years <ref name="pmid11423577">{{cite journal |vauthors=Lin J, Markowitz GS, Valeri AM, Kambham N, Sherman WH, Appel GB, D'Agati VD |title=Renal monoclonal immunoglobulin deposition disease: the disease spectrum |journal=J. Am. Soc. Nephrol. |volume=12 |issue=7 |pages=1482–92 |date=July 2001 |pmid=11423577 |doi= |url=}}</ref>.
[[Renal|Renal involvement]] is the most common cause of [[mortality]] and [[morbidity]] in these patients. [[Survival rate|Survival]] varies between months to 10 years <ref name="pmid11423577">{{cite journal |vauthors=Lin J, Markowitz GS, Valeri AM, Kambham N, Sherman WH, Appel GB, D'Agati VD |title=Renal monoclonal immunoglobulin deposition disease: the disease spectrum |journal=J. Am. Soc. Nephrol. |volume=12 |issue=7 |pages=1482–92 |date=July 2001 |pmid=11423577 |doi= |url=}}</ref>.

Revision as of 03:13, 8 June 2018


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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

Synonyms and keywords: light chain deposition disease

Overview

Light chain deposition disease (LCDD) is 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 diseases are classified in 5 types of diseases:

  1. Amyloidosis
  2. LCDD
  3. Fabry's disease
  4. Fibrillary immuno-tactoid glumerulopathy: The diagnosis is based on pathology finding. Infiltration of fibrills make glomerular structures in the kidney. Fibrills are larger than amyloid fibrils. Patients usually asymtomatic but, sometimes have proteinuria and hematuria. Prognosis is poor and ESRD will happen in half of the patients [2].
  5. Collagenofibrotic glomerulopathy: A rare disease and the diagnosis is based on pathology finding. Type III collagen fibers deposit in the subendothelial and mesangium in the kidney. Negetive with Congo red and thioflavin stains. There is no specific threatment for it[3] .

Pathophysiology

Pathogenesis:

The exact pathogenesis of LCDD is not fully understood. Accumulation of monoclonal light chains and matrix proteins → ↑ quantity and activity of transforming growth factor-beta (TGF-beta).

Accumulation of light chains→ tubular casts→  interstitial inflammationrenal failure [5].

Microscopic Pathology

On light microscopy in LCDD:

On electron microscopy in LCDD:

Genetics

There exact genetic association for LCDD is unknown.

Causes

The specific etiology is unknown.

Differentiating from Other Diseases

Epidemiology and Demographics

The incidence of LCDD is unknown. Most of the patients are men with the mean age of 58 years [1].

Renal involvement is the most common cause of mortality and morbidity in these patients. Survival varies between months to 10 years [6].

Risk Factors

There are no established risk factors for LCDD.

Screening

There is insufficient evidence to recommend routine screening for LCDD.

Natural History, Complications, and Prognosis

Prognostic factors at presentation [1]:

The median time to progression to chronic renal failure 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

History and Symptoms

All organs can be effected by LCDD. Most of the time kidney is involved [8]. Usually patients are asymptomatic in early stages. Symptoms are nonspecific like fatigue and weight loss. Rapidly progressive glomerulonephritis or acute tubulointerstitial nephritis cause renal failure in these patients. Other than the kidneys, liver and heart are the most commonly involved organs. Deposition of light chains in the liver may lead to hepatomegaly, portal hypertension and liver failure. The heart is affected in up to 80% of patients with LCDD, and may cause arrhythmias restrictive cardiomyopathy, cardiomegaly, and congestive heart failure [9].

Physical Examination

Laboratory Findings

Electrocardiogram

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

X-ray

There are no pathognomonic and specific x-ray findings associated with LCDD.

Echocardiography or Ultrasound

  • Echocardiography:

In case of heart involvement: ↓ EF, diffuse hypokinesia, left ventricular concentric hypertrophy, ↓ diastolic compliance [11]

  • Ultrasound:

No finding findings associated with renal LCDD.

CT scan

  • In severe cases the size of the organ will ↑.
  • No pathognomonic and specific finding associated with LCDD.

MRI

  • In severe cases the size of the organ will ↑.
  • No pathognomonic and specific finding associated with LCDD.

Other Imaging Findings

There are no other imaging findings associated with LCDD.

Other Diagnostic Studies

There are no other diagnostic studies associated with LCDD.

Treatment

Medical therapy:

70% of cases without therapy will have ESRD. There is no standard treatment for LCDD. Medical therapy options are:

Surgery

In case of ESRDKidney transplant

Primary Prevention

There are no established measures for the primary prevention of LCDD.

Secondary Prevention

There are no established measures for the secondary prevention of LCDD.

References

  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. 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.
  7. Yadav P, Leung N, Sanders PW, Cockwell P (April 2015). "The use of immunoglobulin light chain assays in the diagnosis of paraprotein-related kidney disease". Kidney Int. 87 (4): 692–7. doi:10.1038/ki.2014.333. PMC 4863638. PMID 25296094.
  8. Ronco PM, Alyanakian MA, Mougenot B, Aucouturier P (July 2001). "Light chain deposition disease: a model of glomerulosclerosis defined at the molecular level". J. Am. Soc. Nephrol. 12 (7): 1558–65. PMID 11423587.
  9. 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.
  10. 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.
  11. 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.


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