Scleromyxedema-Associated Myopathy and Vacuolar Myopathy with Monoclonal Gammopathy and Stiffness (VAMMGAS) as manifestations of Monoclonal Gammopathy of Clinical Significance (MGCS): a disease spectrum between autophagy dysfunction and inflammation.
Tommaso Nicoletti, Olivier Benveniste, Maxime Battistella et al.
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Case in brief
This report discusses two patients with Scleromyxedema-Associated Myopathy (SAM) and one with Vacuolar Myopathy with Monoclonal Gammopathy and Stiffness (VAMMGAS), both associated with Monoclonal Gammopathy of Clinical Significance (MGCS). All patients exhibited progressive proximal muscle weakness and abnormal electromyography findings. Histopathological analysis revealed features of impaired autophagy and immune activation. Treatment with immunomodulatory therapies led to improvement in symptoms.
Diagnostic / clinical pearl
Clinicians should recognize that SAM and VAMMGAS may present with overlapping features, including muscle weakness and specific electromyographic changes, which can aid in diagnosis and management.
Why it matters
Understanding the relationship between SAM and VAMMGAS within the spectrum of MGCS can enhance diagnostic accuracy and inform treatment strategies for affected patients.