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Amyloidosis in Patients Undergoing Carpal Tunnel Release
Study Questions:
What is the prevalence and type of amyloid in patients who undergo carpal tunnel surgery?
Methods:
The authors performed a prospective, cross-sectional study of men ≥50 years and women ≥60 years undergoing carpal tunnel release surgery. Tenosynovial biopsy specimens were evaluated for amyloid with Congo red staining. Those patients with positive histology were further evaluated for the type of amyloidosis (with mass spectrometry) and for cardiac involvement (biomarkers, electrocardiography, echocardiography with strain, and technetium-99m pyrophosphate scintigraphy).
Results:
Of the 200 eligible patients, 98 were enrolled and biopsied. The mean age was 68 years and 51% were male. Ten patients (10.2%) were found to have amyloid in the tenosynovial biopsy with Congo red staining. Mass spectrometry identified seven patients with transthyretin (ATTR), one patient with presumed ATTR, and two patients with light chain (AL) amyloidosis. Of the 10 patients with amyloidosis, two were diagnosed with cardiac involvement (one AL, one ATTR). These patients were initiated on therapy with dexamethasone, cyclophosphamide, and bortezomib (AL patient) and diflunisal (ATTR patient). One patient had ATTR deposits in the tenosynovium and neuropathic symptoms, and was placed on diflunisal.
Conclusions:
In this prospective cross-sectional study of patients undergoing carpal tunnel release surgery, Congo red staining identified 10% of patients having amyloid deposits. Subsequent cardiac evaluation identified two patients with myocardial involvement, which led to disease-modifying targeted therapy.
Perspective:
Amyloid cardiomyopathy is an under-recognized etiology of heart failure. Amyloidosis is a systemic disorder, which often is misdiagnosed or recognized only late in its course. Early identification of this disease is paramount in order to provide patients with timely therapy, which varies depending on the type of amyloidosis (ATTR vs. AL). This study is the first to report prospectively the prevalence of amyloidosis in patients undergoing carpal tunnel release, and highlights the importance of recognizing idiopathic carpal tunnel syndrome as a possible predictor for amyloidosis and amyloid heart disease. Larger patient cohorts and long-term follow-up of patients with tenosynovial amyloidosis are needed to better characterize the natural history and frequency of cardiac involvement in this patient population. In addition, as tenosynovial biopsy is a relatively low-risk procedure, providers should consider this diagnostic test in patients with idiopathic carpal tunnel syndrome, as it may lead to early diagnosis of amyloidosis, possible recognition of cardiac involvement, and the opportunity to initiate early therapy.
Keywords: Amyloid, Amyloidosis, Biomarkers, Cardiomyopathies, Cardiotoxicity, Carpal Tunnel Syndrome, Cyclophosphamide, Dexamethasone, Diagnostic Imaging, Diflunisal, Diphosphates, Echocardiography, Electrocardiography, Heart Failure, Mass Spectrometry, Plaque, Amyloid, Prealbumin, Radionuclide Imaging, Secondary Prevention, Technetium, Technetium Tc 99m Pyrophosphate
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