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Monoclonal gammopathy with renal significance (MGRS) - Monoclonal gammopathy with clinical significance (MGCS)

Monoklonale Gammopathie mit klinischer Signifikanz (MGCS)

Light chain amyloidosis is an entity from the group of rare but serious paraprotein-associated systemic diseases. In these diseases, uncontrolled cell proliferation is not the main cause, but the organ damage is caused by the irregularly formed paraprotein.

The classification of these diseases is currently based on the mechanism that causes the tissue and organ damage:

Organ damage due to:

A) organized and B) disorganized deposits: In most cases, organ damage is caused by different forms of deposits in the tissues (e.g. in the form of Light Chain Deposition Disease (LCDD), Heavy Chain Deposition Disease (HCDD), Crystall Storing Histiocytosis). Depending on the form of the deposits, a distinction is made between organized and non-organized deposits.

C) Organ damage due to autoantibody activity: Organ damage can also be caused by autoantibody activity, e.g. antibodies against the glomerular basement membrane of the kidney, membranous glomerulonephritis, acquired Von Willebrand syndrome, acquired C1 esterase inhibitor deficiency, cold agglutinin disease, cryoglobulin disease.

D) Organ damage due to pathological activation of the complement system: C3 nephropathy, atypical hemolytic uremic syndrome

E) Organ damage due to excessive cytokine production: VEGF in POEMS

F) Organ damage due to paraprotein but currently still unexplained mechanisms: systemic capillary leak syndrome, aquired cutis laxa, scleromyxedema, Schnitzler syndrome, sporadic late onset nemaline myopathy

A high level of awareness, special training and intensive interdisciplinary cooperation between many departments are required to diagnose these rare but very serious systemic diseases. These interdisciplinary collaborations were established in the course of the Amyloidosis Center and have also proved particularly successful in the diagnosis and treatment of monoclonal gammopathies (MGCS) with clinical significance.

Control Center 6i, Level 6
T: +43 (0)1 40400-44640

Secretariat: Renate Schoder,

Medical management:

Univ. Lektorin Hermine Agis Maria Krauth