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Presentation and Outcome of Patients with Systemic Amyloidosis Undergoing Dialysis

Guillaume Bollée^*, Bruno Guery^*, Dominique Joly^*, Renaud Snanoudj, Benjamin Terrier^*, Mahmoud Allouache, Lucile Mercadal, Marie-Noëlle Peraldi, Béatrice Viron^||, Christine Fumeron^¶, Caroline Elie^**, and Fadi Fakhouri^*

^* Department of Nephrology, AP-HP, Hôpital Necker, Université Paris V, Paris, France; Department of Nephrology, AP-HP, Hôpital Kremlin Bicêtre, Université Paris XI, Kremlin Bicêtre, France; Department of Nephrology, AP-HP, Hôpital Pitié Salpêtrière, Université Paris VI, Paris, France; Department of Nephrology, AP-HP, Hôpital Saint-Louis, Université Paris VII, Paris, France; ^|| AURA Peupliers, Paris, France; ^¶ AURA Centre Henri Küntziger, Paris, France; ^** Department of Biostatistics, AP-HP, Hôpital Necker, Université Paris V, Paris, France

Correspondence: Dr. Fadi Fakhouri, Department of Nephrology, AP-HP, Hôpital Necker, Université Paris Descartes, Paris, France. Phone: 0033144495458; Fax: 0033144495450; E-mail: fadi.fakhouri{at}nck.aphp.fr

Background and objectives: Light chain (AL) and secondary (AA) amyloidosis usually present as a systemic disease frequently involving the kidney and leading to ESRD. Data regarding patients with AA or AL amyloidosis undergoing dialysis remain scarce.

Design, setting, participants, & measurements: We retrospectively studied patients with AA or AL amyloidosis who started dialysis in five French centers between January 1, 1995 and December 31, 2005.

Results: We identified 19 patients with AL and 20 patients with AA amyloidosis undergoing dialysis. Patients with AL amyloidosis had shorter time from diagnosis to dialysis (25.2 versus 69.3 mo, P < 0.05) and more extrarenal amyloidosis, especially cardiac (63.2 versus 5%, P < 0.0001). Mean duration of follow-up was 37.4 and 31.8 mo for patients with AL and AA amyloidosis, respectively. Fifteen patients (78.9%) with AL and three patients (15%) with AA amyloidosis died on dialysis. Median survival was shorter in patients with AL (26 mo) than AA amyloidosis [not definable (ND)] (P < 0.02). Sepsis and cardiac deaths were the main causes of mortality. Prognosis factors for death at 1 yr were AL type (P < 0.01), cardiac amyloidosis [odds ratio (OR) = 18, P < 0.01], heart failure (OR = 8, P < 0.04), and shorter time from diagnosis to dialysis (6.1 versus 56 mo, P < 0.03). Multivariate analysis indicated that AL type (P = 0.02), but not cardiac amyloidosis was independently associated with global mortality.

Conclusions: Survival of patients with amyloidosis undergoing dialysis, especially AL type, is probably better than previously reported. However, mortality is higher in AL than AA type, especially in the setting of cardiac involvement.