No abstract

No abstract

Cardiac amyloidosis is an under-recognized and potentially fatal cause of heart failure and other cardiovascular manifestations. It is caused by deposition of misfolded precursor proteins as fibrillary amyloid deposits in cardiac tissues. The two primary subtypes of systemic amyloidosis causing cardiac involvement are immunoglobulin light chain (AL), a plasma cell dyscrasia, and transthyretin (ATTR), itself subdivided into a hereditary subtype caused by a gene mutation of the ATTR protein, and an age-related wild type, which occurs in the absence of a gene mutation. Clinical recognition requires a high index of suspicion, inclusive of the extracardiac manifestations of both subtypes. Diagnostic workup includes screening for serum and/or R ESUM E L'amylose cardiaque est une cause sous-reconnue et potentiellement mortelle d'insuffisance cardiaque et d'autres manifestations cardiovasculaires. Elle est caus ee par le d epôt, dans les tissus cardiaques, de prot eines pr ecurseurs mal repli ees prenant la forme de fibrilles amyloïdes. On distingue deux grands sous-types d'amylose syst emique entraînant une atteinte cardiaque : l'amylose à chaînes l egères d'immunoglobulines (AL), une forme de dyscrasie plasmocytaire; et l'amylose à transthyr etine (ATTR), dont il existe un sous-type h er editaire, caus e par une mutation du gène codant pour la TTR, et un sous-type à TTR sauvage, li e au vieillissement et se produisant en l'absence de mutation g enique. La reconnaissance clinique n ecessite

Objective: To describe successful therapeutic strategies in statin-induced anti-HMGCR myopathy. Methods: Retrospective data from a cohort of 55 patients with statin-induced anti-HMGCR myopathy, sequentially stratified by the presence of proximal weakness, early remission, and corticosteroid and IVIG use at treatment induction, were analyzed for optimal successful induction and maintenance of remission strategies. Results: A total of 14 patients achieved remission with a corticosteroid-free induction strategy (25%). In 41 patients treated with corticosteroids, only 4 patients (10%) failed an initial triple steroid/IVIG/steroid-sparing immunosuppressant (SSI) induction strategy. Delay in treatment initiation was independently associated with lower odds of successful maintenance with immunosuppressant monotherapy (OR 0.92, 95% CI 0.85 to 0.97, P = 0.015). While 22 patients (40%) presented with normal strength, only 9 had normal strength at initiation of treatment.Conclusion: While corticosteroid-free treatment of anti-HMGCR myopathy is now a safe option in selected cases, initial triple steroid/IVIG/SSI was very efficacious in induction. Delays in treatment initiation and, as a corollary, delays in achieving remission decrease the odds of achieving successful maintenance with an SSI alone. Avoiding such delays, most notably in patients with normal strength, may reset the natural history of anti-HMGCR myopathy from a refractory entity to a treatable disease.