Monoclonal gammopathy of undetermined significance with associated necrotizing myopathy: a case report and review of the literature

Background: Monoclonal gammopathies encompass many types of plasma cell proliferative disorders ranging from benign to malignant. Monoclonal gammopathies that meet diagnostic criteria for monoclonal gammopathies of undetermined significance (MGUS) but with clinical manifestations are now being refer...

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Main Authors: Anna Bode, Oscar Borja Montes, Karen SantaCruz, Abu Baker Sheikh
Format: Article
Language:English
Published: SMC MEDIA SRL 2025-04-01
Series:European Journal of Case Reports in Internal Medicine
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Online Access:https://www.ejcrim.com/index.php/EJCRIM/article/view/5229
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author Anna Bode
Oscar Borja Montes
Karen SantaCruz
Abu Baker Sheikh
author_facet Anna Bode
Oscar Borja Montes
Karen SantaCruz
Abu Baker Sheikh
author_sort Anna Bode
collection DOAJ
description Background: Monoclonal gammopathies encompass many types of plasma cell proliferative disorders ranging from benign to malignant. Monoclonal gammopathies that meet diagnostic criteria for monoclonal gammopathies of undetermined significance (MGUS) but with clinical manifestations are now being referred to as monoclonal gammopathies of clinical significance (MGCS). MGUS associated myopathies are a rare form of MGCS. Case description: We present a case of MGUS associated necrotizing myositis. The patient had been previously diagnosed with MGUS and myositis around the same time, but prior testing had not revealed the etiology of the myopathy. Repeat biopsy and work-up revealed a necrotizing myopathy. Discussion: MGUS associated myopathies are rare, with the most common being sporadic late-onset nemaline myopathy (SLONM-MGUS) and amyloid light chain (AL) amyloidosis-associated myopathy. MGUS associated necrotizing myopathy is even rarer. Because this condition is so uncommon, there are no standardized guidelines on how to treat this condition. Some case reports or studies suggest treating the myositis with standard of care for the myositis type without considering the monoclonal gammopathy while other studies have suggested that treating the monoclonal gammopathy would be beneficial in treating the associated clinical syndrome. Conclusion: Our case report of MGUS associated necrotizing myopathy encourages internists to broaden their differential diagnosis of myopathy, increasing awareness of a condition that is still not well understood.
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spelling doaj-art-e267ed6aa5ed478f962d6aa83376e1712025-08-20T02:13:56ZengSMC MEDIA SRLEuropean Journal of Case Reports in Internal Medicine2284-25942025-04-0110.12890/2025_0052294764Monoclonal gammopathy of undetermined significance with associated necrotizing myopathy: a case report and review of the literatureAnna Bode0Oscar Borja Montes1Karen SantaCruz2Abu Baker Sheikh3Department of Internal Medicine, University of New Mexico, Albuquerque, USADepartment of Internal Medicine, University of New Mexico, Albuquerque, USADepartment of Pathology, University of New Mexico, Albuquerque, USADepartment of Internal Medicine, University of New Mexico, Albuquerque, USABackground: Monoclonal gammopathies encompass many types of plasma cell proliferative disorders ranging from benign to malignant. Monoclonal gammopathies that meet diagnostic criteria for monoclonal gammopathies of undetermined significance (MGUS) but with clinical manifestations are now being referred to as monoclonal gammopathies of clinical significance (MGCS). MGUS associated myopathies are a rare form of MGCS. Case description: We present a case of MGUS associated necrotizing myositis. The patient had been previously diagnosed with MGUS and myositis around the same time, but prior testing had not revealed the etiology of the myopathy. Repeat biopsy and work-up revealed a necrotizing myopathy. Discussion: MGUS associated myopathies are rare, with the most common being sporadic late-onset nemaline myopathy (SLONM-MGUS) and amyloid light chain (AL) amyloidosis-associated myopathy. MGUS associated necrotizing myopathy is even rarer. Because this condition is so uncommon, there are no standardized guidelines on how to treat this condition. Some case reports or studies suggest treating the myositis with standard of care for the myositis type without considering the monoclonal gammopathy while other studies have suggested that treating the monoclonal gammopathy would be beneficial in treating the associated clinical syndrome. Conclusion: Our case report of MGUS associated necrotizing myopathy encourages internists to broaden their differential diagnosis of myopathy, increasing awareness of a condition that is still not well understood.https://www.ejcrim.com/index.php/EJCRIM/article/view/5229monoclonal gammopathymyopathy
spellingShingle Anna Bode
Oscar Borja Montes
Karen SantaCruz
Abu Baker Sheikh
Monoclonal gammopathy of undetermined significance with associated necrotizing myopathy: a case report and review of the literature
European Journal of Case Reports in Internal Medicine
monoclonal gammopathy
myopathy
title Monoclonal gammopathy of undetermined significance with associated necrotizing myopathy: a case report and review of the literature
title_full Monoclonal gammopathy of undetermined significance with associated necrotizing myopathy: a case report and review of the literature
title_fullStr Monoclonal gammopathy of undetermined significance with associated necrotizing myopathy: a case report and review of the literature
title_full_unstemmed Monoclonal gammopathy of undetermined significance with associated necrotizing myopathy: a case report and review of the literature
title_short Monoclonal gammopathy of undetermined significance with associated necrotizing myopathy: a case report and review of the literature
title_sort monoclonal gammopathy of undetermined significance with associated necrotizing myopathy a case report and review of the literature
topic monoclonal gammopathy
myopathy
url https://www.ejcrim.com/index.php/EJCRIM/article/view/5229
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