Key result
A case of primary systemic amyloidosis with severe cardiac involvement was diagnosed only at autopsy, highlighting the diagnostic challenges of the disease.
Case Report (n=1)
Highlights the diagnostic challenge and poor prognosis of cardiac involvement in primary systemic amyloidosis, which may only be diagnosed post-mortem.
Reminds clinicians of missed cardiac amyloidosis diagnoses; leaves open need for improved antemortem detection strategies.
Cardiovascular involvement of amyloidosis is present in 90% of cases, which is frequently associated with the primary form of the disease (AL amyloidosis). Clinical manifestations are represented by heart failure due to restrictive myocardiopathy and electrical impulse conduction abnormalities, which are clinically remarkable in up to 50% of the cases. The prognosis of patients with systemic amyloidosis is directly associated with the presence of cardiac involvement, such that survival does not usually exceed 4 months after the onset of heart failure signs and symptoms. The authors report a case of primary systemic amyloidosis, diagnosed only at autopsy, with severe cardiac involvement.
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Morais et al. (2014) conducted a case report in Primary systemic amyloidosis (n=1). Primary systemic amyloidosis was evaluated. A case of primary systemic amyloidosis with severe cardiac involvement was diagnosed only at autopsy, highlighting the diagnostic challenges of the disease.
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