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Ramonatxo Arthur

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  • Randall-type monoclonal immunoglobulin deposition disease: description of cardiac involvement    - Ramonatxo Arthur  -  11 septembre 2020  - Thèse d'exercice

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    Background: Randall-type monoclonal immunoglobulin disease (MIDD) is a rare complication of a monoclonal plasma cell clone. MIDD differs from AL amyloidosis by the presence of Congo red negative non-organized immunoglobulin (Ig) deposits, most commonly light chains (LCDD) along basement membranes and sometimes heavy chains (HCDD) or light and heavy chains (LHCDD). As AL amyloidosis MIDD is a multi-systemic disease, and affect the heart. To date no study has focused on the clinical characteristics of heart disease in MIDD.

    Purpose: The aim of this study was to describe the cardiac characteristics (clinical, ECG, holter ECG, ultrasound and MRI) of patients with Randall's disease

    Methods: This multi-center, nation-wide retrospective study extracted from the database of the French reference center for AL amyloidosis and other Ig deposition diseases between 2011 to 2019. Histological evidence was obtained on biopsies, most often renal with typical linear non-organized Ig deposits along basement membranes. Patients whose ECG and cardiac ultrasounds were not available were excluded.

    Results: Among 22 patients included (mean age was 66 ± 10 years), 12 (55%) were male; 16 (73%) were LCDD, 1 (5%) HCDD and 5 (23%) LHCDD. At baseline, 13 (59%) had a history of hypertension, 1 (3%) had a history of atrial fibrillation and in terms of clinical characteristics, 4 (18%) patients had NYHA class 3 or 4, 11 (50%) had edematous syndrome and 5 (23%) signs of left heart failure. The mean NT-ProBNP level was 693 ± 15 829 ng/L and mean high sensitivity troponin was 0.03 ± 0.05 ng/mL. The most frequent ECG feature was Q waves of pseudo necrosis in 41% of patients and microvoltage in 18%. On the 24-hour Holter monitoring, alteration of sinus variability was found in 65% of patients; one patient had a high-grade conduction disorder and another had ventricular tachycardia. On echocardiography, mean LVEF was 64 ± 10% and only two patients had a LVEF < 50% but diastolic dysfunction was present in 95% of patients; the global longitudinal strain was 19 ± 3 % with impaired values in 36% of patients. Contrarily to AL amyloidosis, the interventricular septum shiny aspect was noted in only one patient and no patient had late gadolinium enhancement on cardiac MRI.

    After median follow-up of 44 [25; 73] months, 5 (23%) were hospitalized for episodes of heart failure, including one patient for cardiogenic shock; 4 (18%) patients presented with atrial fibrillation after the diagnosis of Randall's disease. Regarding mortality, 6 (27%) patients died with a median of death at 67 [65; 73] years old.

    Conclusions: To our knowledge, we present the first case series dedicated to the description of cardiac parameters in MIDD patients with cardiac involvement. Except for MRI appearance of cardiac infiltration and interventricular septum shiny aspect in echocardiography, these patients showed features close to that of AL amyloid heart disease.

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