PRIMARY AMYLOIDOSIS (AL) AS A CAUSE OF NEPHROTIC SYNDROME

Y. BatailleDepartment of nephrology and hypertensionUniversité de Liège, C.H.U.- Domaine universitaire du Sart Tilman - B.35, B 4000 LIEGE 1 – (Belgique)

C. BovyDepartment of nephrology and hypertensionUniversité de Liège, C.H.U.- Domaine universitaire du Sart Tilman - B.35, B 4000 LIEGE 1 – (Belgique)

P LancellottiDepartment of cardiologyUniversité de Liège, C.H.U.- Domaine universitaire du Sart Tilman - B.35, B 4000 LIEGE 1 – (Belgique)

V. MelchiorDepartment of pathologyUniversité de Liège, C.H.U.- Domaine universitaire du Sart Tilman - B.35, B 4000 LIEGE 1 – (Belgique)

K. DelbecqueDepartment of pathologyUniversité de Liège, C.H.U.- Domaine universitaire du Sart Tilman - B.35, B 4000 LIEGE 1 – (Belgique)

Y. BeguinDepartment of haematologyUniversité de Liège, C.H.U.- Domaine universitaire du Sart Tilman - B.35, B 4000 LIEGE 1 – (Belgique)

J-M KrzesinskiDepartment of nephrology and hypertensionUniversité de Liège, C.H.U.- Domaine universitaire du Sart Tilman - B.35, B 4000 LIEGE 1 – (Belgique)

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Abstract

AL amyloidosis is a rare systemic disease resulting from tissue accumulation of amyloid fibrils derived from monoclonal immunoglobulin light chains. It can disrupt the tissue architecture and consequently cause organ dysfunction. The prognosis is poor with a median survival of 13 months in untreated patients.

By illustrating the case of a patient whose AL amyloidosis was detected after presenting a nephrotic syndrome, the characteristics of the disease are reviewed as well as diagnostic criteria and current available therapeutics.

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PRIMARY AMYLOIDOSIS (AL) AS A CAUSE OF NEPHROTIC SYNDROME

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PRIMARY AMYLOIDOSIS (AL) AS A CAUSE OF NEPHROTIC SYNDROME

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