Beta-2 microglobulin related amyloidosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Amandeep Singh M.D.[2]

Synonyms and Keywords: Dialysis related amyloidosis, β2-microglobulin amyloidosis, β2 amyloidosis, Amyloidosis due to dialysis, Amyloidosis due to Beta 2 microglobulin

Overview

In 1975, Warren and Otieno were thee first to discover the association between β2-microglobulin related amyloidosis and Carpal tunnel syndrome. In 1985, Gejyo et al. identified β2-microglobulin as the protein precursor of the amyloid associated with chronic hemodialysis. There is no established system for the classification of beta2-microglobulin related amyloidosis. The exact pathogenesis of β2-microglobulin related amyloidosis is not fully understood.

Historical Perspective

  • In 1975, Warren and Otieno were thee first to discover the association between β2-microglobulin related amyloidosis and Carpal tunnel syndrome.[1]
  • In 1985, Gejyo et al. identified β2-microglobulin as the protein precursor of the amyloid associated with chronic hemodialysis.[2]
  • In 1993, advanced glycation end products were first implicated in the pathogenesis of β2-microglobulin related amyloidosis by Miyata et al.[3]

Classification

There is no established system for the classification of beta2-microglobulin related amyloidosis.

Pathophysiology

  • The exact pathogenesis of β2-microglobulin related amyloidosis is not fully understood.
  • Advanced glycation end products can be responsible for β2-microglobulin deposition.[3]

Causes

The exact cause of β2-microglobulin related amyloidosis has not been identified.

Differentiating β2-Microglobulin Related Amyloidosis from other Diseases

β2-microglobulin related amyloidosis must be differentiated from other diseases that cause joint pain and joint stiffness, such as bursitis and Carpal tunnel syndrome.

Epidemiology and Demographics

  • The exact incidence/prevalence of β2-microglobulin related amyloidosis can not be determined as the biopsy is the only way to find out the diagnosis and can not be done in every patient undergoing hemodialysis.[4]
  • In a prospective postmortem study, amyloid deposition in joints was found in 21% of patients receiving hemodialysis for < 2 years, in 50% at 4 to 7 years, in 90 percent at 7 to 13 years, and in 100 percent at > 13 years.[5]
  • The incidence of β2-microglobulin related amyloidosis increases with age especially in patients on chronic dialysis.
  • It can be seen in as much as 20% of patients after 2–4 years of dialysis and in 100% of patients after 13 years of hemodialysis.
  • There is no racial predilection to β2-microglobulin related amyloidosis.

Risk Factors

The most potent risk factor in the development of beta2-microglobulin related amyloidosis is chronic Hemodialysis. Other risk factors include initiation of dialysis in young age, hemodialysis treatment with low purity dialysate and use of dialysis membranes with low flux.[6]

Screening

There is insufficient evidence to recommend routine screening for beta2-microglobulin related amyloidosis

Natural History, Complications, and Prognosis

Diagnosis

Diagnostic Study of Choice

  • The diagnostic study of choice is biopsy which is considered the gold standard diagnostic technique.[12]
  • The diagnosis of β2-microglobulin related amyloidosis is made with the combination of clincal and radiological features where biopsy is not or can't be performed.

History and Symptoms

  • The majority of patients with beta2 microglobulin related amyloidosis initially are asymptomatic.
  • A positive history of chronic hemodialysis expressing Carpal tunnel syndrome is suggestive of beta2 microglobulin related amyloidosis. The most common symptoms of β2-microglobulin related amyloidosis include joint pain, tingling; enlargement of the tongue, dysphonia,Dysphagia and Dysgeusia(when gastrointestinal tract is involved)

Physical Examination

Common physical examination findings of beta2 microglobulin related amyloidosis include Tinel's sign.

Laboratory Findings

  • There are no diagnostic laboratory findings associated with beta2-microglobulin related amyloidosis.

Electrocardiogram

  • There are no ECG findings associated with beta2-microglobulin related amyloidosis.

X-ray

  • An x-ray may be helpful in the diagnosis of beta2-microglobulin related amyloidosis. Findings on an x-ray suggestive of beta2-microglobulin related amyloidosis include radiolucent lesions or bone cysts, frequently with thin sclerotic margins.[13]

Echocardiography

  • Echocardiography may be helpful in the diagnosis of [disease name]. Findings on an echocardiography suggestive of beta2-microglobulin related amyloidosis include findings of heart failure and Mitral regurgitation.
  • For more findings of echocardiography of cardiac amyloidosis due to beta2-microglobulin related amyloidosis, please click here.

CT scan

  • CT scan may be helpful in the diagnosis of beta2-microglobulin related amyloidosis. Findings on CT scan suggestive of beta2-microglobulin related amyloidosis include [finding 1], [finding 2], and [finding 3].[13]

MRI

  • MRI may be helpful in the diagnosis of beta2-microglobulin related amyloidosis Findings on MRI suggestive of beta2-microglobulin related amyloidosis include [finding 1], [finding 2], and [finding 3].

Other Imaging Findings

  • Scintigraphy may be helpful in the diagnosis of beta2-microglobulin related amyloidosis. Findings suggestive of beta2-microglobulin related amyloidosis include amyloid deposits in the carpal tunnel.[14]

Other Diagnostic Studies

  • Biopsy is helpful in the diagnosis of beta2-microglobulin related amyloidosis . Findings diagnostic of beta2-microglobulin related amyloidosis include apple-green birefringence under polarized light when prestained with Congo red.

Treatment

Medical Therapy

  • The mainstay of treatment for beta2-microglobulin related amyloidosis is Kidney transplantation
  • Those who are not able to get a kidney transplant needs Hemodialysis with high flux membrane and dialysate[15]

Surgery

  • Surgical intervention is not recommended for the management of beta2-microglobulin related amyloidosis.
  • Arthroscopic surgery done in the cases with joint infiltration with B2 microglobulin where removal of synovium provides relief.[16]

Primary Prevention

  • There are no established measures for the primary prevention of beta2-microglobulin related amyloidosis.
  • Effective measures for the primary prevention of beta2-microglobulin related amyloidosis include kidney transplant as soon as available.

Secondary Prevention

  • Effective measures for the secondary prevention of beta2-microglobulin related amyloidosis include avoiding the use of low flux membrane, clearing dialysate regularly, performing DEXA scan.

References

  1. Warren DJ, Otieno LS (1975). "Carpal tunnel syndrome in patients on intermittent haemodialysis". Postgrad Med J. 51 (597): 450–2. doi:10.1136/pgmj.51.597.450. PMC 2496055. PMID 1187497.
  2. Gejyo F, Yamada T, Odani S, Nakagawa Y, Arakawa M, Kunitomo T; et al. (1985). "A new form of amyloid protein associated with chronic hemodialysis was identified as beta 2-microglobulin". Biochem Biophys Res Commun. 129 (3): 701–6. doi:10.1016/0006-291x(85)91948-5. PMID 3893430.
  3. 3.0 3.1 Miyata T, Oda O, Inagi R, Iida Y, Araki N, Yamada N; et al. (1993). "beta 2-Microglobulin modified with advanced glycation end products is a major component of hemodialysis-associated amyloidosis". J Clin Invest. 92 (3): 1243–52. doi:10.1172/JCI116696. PMC 288264. PMID 8376584.
  4. Kay, Jonathan (2009). "β2-microglobulin amyloidosis". Amyloid. 4 (3): 187–211. doi:10.3109/13506129709014384. ISSN 1350-6129.
  5. Jadoul M, Garbar C, Noël H, Sennesael J, Vanholder R, Bernaert P; et al. (1997). "Histological prevalence of beta 2-microglobulin amyloidosis in hemodialysis: a prospective post-mortem study". Kidney Int. 51 (6): 1928–32. doi:10.1038/ki.1997.262. PMID 9186884.
  6. Davison AM (1995). "beta 2-microglobulin and amyloidosis: who is at risk?". Nephrol Dial Transplant. 10 Suppl 10: 48–51. PMID 8825433.
  7. Kopeć J, Gadek A, Drozdz M, Miśkowiec K, Dutka J, Sydor A; et al. (2011). "Carpal tunnel syndrome in hemodialysis patients as a dialysis-related amyloidosis manifestation--incidence, risk factors and results of surgical treatment". Med Sci Monit. 17 (9): CR505–9. doi:10.12659/msm.881937. PMC 3560521. PMID 21873947.
  8. Ohashi K, Hara M, Kawai R, Ogura Y, Honda K, Nihei H; et al. (1992). "Cervical discs are most susceptible to beta 2-microglobulin amyloid deposition in the vertebral column". Kidney Int. 41 (6): 1646–52. doi:10.1038/ki.1992.237. PMID 1501421.
  9. Yamamoto S, Kazama JJ, Maruyama H, Nishi S, Narita I, Gejyo F (2008). "Patients undergoing dialysis therapy for 30 years or more survive with serious osteoarticular disorders". Clin Nephrol. 70 (6): 496–502. doi:10.5414/cnp70496. PMID 19049706.
  10. Matsumoto K, Kikuchi J, Kaneko Y, Yasuoka H, Suzuki K, Tokuyama H; et al. (2018). "Persistent fever and destructive arthritis caused by dialysis-related amyloidosis: A case report". Medicine (Baltimore). 97 (1): e9359. doi:10.1097/MD.0000000000009359. PMC 5943088. PMID 29505515.
  11. Campistol JM, Solé M, Muñoz-Gomez J, Lopez-Pedret J, Revert L (1990). "Systemic involvement of dialysis-amyloidosis". Am J Nephrol. 10 (5): 389–96. doi:10.1159/000168154. PMID 2080789.
  12. National Kidney Foundation (2003). "K/DOQI clinical practice guidelines for bone metabolism and disease in chronic kidney disease". Am J Kidney Dis. 42 (4 Suppl 3): S1–201. PMID 14520607.
  13. 13.0 13.1 Kiss E, Keusch G, Zanetti M, Jung T, Schwarz A, Schocke M; et al. (2005). "Dialysis-related amyloidosis revisited". AJR Am J Roentgenol. 185 (6): 1460–7. doi:10.2214/AJR.04.1309. PMID 16303998.
  14. Tan SY, Baillod R, Brown E, Farrington K, Soper C, Percy M; et al. (1999). "Clinical, radiological and serum amyloid P component scintigraphic features of beta2-microglobulin amyloidosis associated with continuous ambulatory peritoneal dialysis". Nephrol Dial Transplant. 14 (6): 1467–71. doi:10.1093/ndt/14.6.1467. PMID 10383009.
  15. National Kidney Foundation (2003). "K/DOQI clinical practice guidelines for bone metabolism and disease in chronic kidney disease". Am J Kidney Dis. 42 (4 Suppl 3): S1–201. PMID 14520607.
  16. Takenaka R, Fukatsu A, Matsuo S, Ishikawa K, Toriyama T, Kawahara H (1992). "Surgical treatment of hemodialysis-related shoulder arthropathy". Clin Nephrol. 38 (4): 224–30. PMID 1424310.



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