Julien Dang1, Mukedaisi Abulizi2, Anissa Moktefi3, Khalil El Karoui1, Jean-François Deux4, Diane Bodez5, Fabien Le Bras6, Karim Belhadj6, Philippe Remy1, Pauline Issaurat5, Violaine Plante-Bordeneuve7, Valérie Molinier-Frenkel8, Pascale Fanen9, Soulef Guendouz5, Mounira Kharoubi5, Emmanuel Itti2, Thibaud Damy10, Vincent Audard11. 1. Assistance Publique des Hôpitaux de ParisService de Néphrologie et Transplantation, Créteil, 94000, France; Centre de Référence Maladie Rare Syndrome Néphrotique Idiopathique de l'Enfant et de l'Adulte, Créteil, 94000, France; Réseau Amylose Mondor, Groupe de Recherche Clinique sur les Amyloses Amyloid Research Institute, Centre de référence des amyloses cardiaques, Université Paris Est Créteil, France; Groupe Hospitalier Henri-Mondor/Albert Chenevier, Université Paris Est Créteil, France; and Equipe 21, Institut National de la Santé et de la Recherche Médicale (INSERM) U955, Institut Mondor de Recherche Biomédicale (IMRB), Université Paris Est Créteil, France. 2. Service de Médecine Nucléaire, Créteil, 94000, France; Réseau Amylose Mondor, Groupe de Recherche Clinique sur les Amyloses Amyloid Research Institute, Centre de référence des amyloses cardiaques, Université Paris Est Créteil, France. 3. Centre de Référence Maladie Rare Syndrome Néphrotique Idiopathique de l'Enfant et de l'Adulte, Créteil, 94000, France; Service de Pathologie, Créteil, 94000, France; Réseau Amylose Mondor, Groupe de Recherche Clinique sur les Amyloses Amyloid Research Institute, Centre de référence des amyloses cardiaques, Université Paris Est Créteil, France; Groupe Hospitalier Henri-Mondor/Albert Chenevier, Université Paris Est Créteil, France; and Equipe 21, Institut National de la Santé et de la Recherche Médicale (INSERM) U955, Institut Mondor de Recherche Biomédicale (IMRB), Université Paris Est Créteil, France. 4. Service d'Imagerie Médicale, Créteil, 94000, France; Réseau Amylose Mondor, Groupe de Recherche Clinique sur les Amyloses Amyloid Research Institute, Centre de référence des amyloses cardiaques, Université Paris Est Créteil, France. 5. Service de Cardiologie, Créteil, 94000, France; Réseau Amylose Mondor, Groupe de Recherche Clinique sur les Amyloses Amyloid Research Institute, Centre de référence des amyloses cardiaques, Université Paris Est Créteil, France. 6. Unité Hémopathies Lymphoïdes, Créteil, 94000, France; Réseau Amylose Mondor, Groupe de Recherche Clinique sur les Amyloses Amyloid Research Institute, Centre de référence des amyloses cardiaques, Université Paris Est Créteil, France. 7. Service de Neurologie, Créteil, 94000, France; Réseau Amylose Mondor, Groupe de Recherche Clinique sur les Amyloses Amyloid Research Institute, Centre de référence des amyloses cardiaques, Université Paris Est Créteil, France. 8. Département d'Hématologie-Immunologie biologique, Créteil, 94000, France; Réseau Amylose Mondor, Groupe de Recherche Clinique sur les Amyloses Amyloid Research Institute, Centre de référence des amyloses cardiaques, Université Paris Est Créteil, France. 9. Département de Biochimie, Biologie moléculaire, Pharmacologie et Génétique médicale, Créteil, 94000, France; Réseau Amylose Mondor, Groupe de Recherche Clinique sur les Amyloses Amyloid Research Institute, Centre de référence des amyloses cardiaques, Université Paris Est Créteil, France. 10. Service de Cardiologie, Créteil, 94000, France; Réseau Amylose Mondor, Groupe de Recherche Clinique sur les Amyloses Amyloid Research Institute, Centre de référence des amyloses cardiaques, Université Paris Est Créteil, France. Electronic address: thibaud.damy@aphp.fr. 11. Assistance Publique des Hôpitaux de ParisService de Néphrologie et Transplantation, Créteil, 94000, France; Centre de Référence Maladie Rare Syndrome Néphrotique Idiopathique de l'Enfant et de l'Adulte, Créteil, 94000, France; Réseau Amylose Mondor, Groupe de Recherche Clinique sur les Amyloses Amyloid Research Institute, Centre de référence des amyloses cardiaques, Université Paris Est Créteil, France; Groupe Hospitalier Henri-Mondor/Albert Chenevier, Université Paris Est Créteil, France; and Equipe 21, Institut National de la Santé et de la Recherche Médicale (INSERM) U955, Institut Mondor de Recherche Biomédicale (IMRB), Université Paris Est Créteil, France. Electronic address: vincent.audard@aphp.fr.
Abstract
OBJECTIVE: To describe the prevalence of and risk factors for renal infarction (RI) in patients with cardiac amyloidosis. PATIENTS AND METHODS: We evaluated 87 patients with cardiac amyloidosis who underwent renal technetium-99m-labeled dimercaptosuccinic acid scintigraphy in the Amyloidosis Referral Center of Henri-Mondor Hospital from October 1, 2015, through February 28, 2018. RESULTS: Three groups of patients were identified according to the underlying amyloidosis disorder: AL amyloidosis in 24 patients, mutated-transthyretin amyloidosis in 24 patients, and wild-type transthyretin amyloidosis in 39 patients. Patients with wild-type transthyretin amyloidosis were older (P<.001), more likely to be men (P=.02), to have arrhythmic heart diseases (P<.001), and to be receiving anticoagulation treatment (P<.001). Patients with AL amyloidosis had significantly higher N-terminal pro-B-type natriuretic peptide levels (P=.02) and were more likely to have nephrotic syndrome (P<.001). Renal infarction was detected in 18 patients (20.7%), at similar frequencies in the various groups. Baseline urinary protein to creatinine ratio was the only parameter for which a significant difference (P=.03) was found between patients with and without RI diagnoses. The likelihood of RI diagnosis was 47.1% (8 of 17) in the presence of AKI and 14.5% (10 of 69) in its absence (P=.003). Overall, heart transplant-censored patient survival did not differ significantly between patients with and without RI (P=.64), but death- and heart transplant-censored renal survival was significantly lower in patients with RI (P<.001). CONCLUSION: Our study suggests that prevalence of RI in patients with cardiac amyloidosis is higher than previously thought, regardless of the underlying amyloidosis disorder. Acute kidney injury in a patient with cardiac amyloidosis should alert clinicians to the possibility of RI.
OBJECTIVE: To describe the prevalence of and risk factors for renal infarction (RI) in patients with cardiac amyloidosis. PATIENTS AND METHODS: We evaluated 87 patients with cardiac amyloidosis who underwent renal technetium-99m-labeled dimercaptosuccinic acid scintigraphy in the Amyloidosis Referral Center of Henri-Mondor Hospital from October 1, 2015, through February 28, 2018. RESULTS: Three groups of patients were identified according to the underlying amyloidosis disorder: AL amyloidosis in 24 patients, mutated-transthyretin amyloidosis in 24 patients, and wild-type transthyretin amyloidosis in 39 patients. Patients with wild-type transthyretin amyloidosis were older (P<.001), more likely to be men (P=.02), to have arrhythmic heart diseases (P<.001), and to be receiving anticoagulation treatment (P<.001). Patients with AL amyloidosis had significantly higher N-terminal pro-B-type natriuretic peptide levels (P=.02) and were more likely to have nephrotic syndrome (P<.001). Renal infarction was detected in 18 patients (20.7%), at similar frequencies in the various groups. Baseline urinary protein to creatinine ratio was the only parameter for which a significant difference (P=.03) was found between patients with and without RI diagnoses. The likelihood of RI diagnosis was 47.1% (8 of 17) in the presence of AKI and 14.5% (10 of 69) in its absence (P=.003). Overall, heart transplant-censored patient survival did not differ significantly between patients with and without RI (P=.64), but death- and heart transplant-censored renal survival was significantly lower in patients with RI (P<.001). CONCLUSION: Our study suggests that prevalence of RI in patients with cardiac amyloidosis is higher than previously thought, regardless of the underlying amyloidosis disorder. Acute kidney injury in a patient with cardiac amyloidosis should alert clinicians to the possibility of RI.