Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2014 Aug;32(3):595-628, vii.
doi: 10.1016/j.ncl.2014.04.007.

Idiopathic inflammatory myopathies

Mazen M Dimachkie  1 , Richard J Barohn  2 , Anthony A Amato  3
Affiliations
Review

Idiopathic inflammatory myopathies

Mazen M Dimachkie et al. Neurol Clin. 2014 Aug.

Abstract

The idiopathic inflammatory myopathies (IIM) consist of rare heterogeneous autoimmune disorders that present with marked proximal and symmetric muscle weakness, except for distal and asymmetric weakness in inclusion body myositis. Despite many similarities, the IIM are fairly heterogeneous from the histopathologic and pathogenetic standpoints, and also show some clinical and treatment-response differences. The field has witnessed significant advances in our understanding of the pathophysiology and treatment of these rare disorders. This review focuses on dermatomyositis, polymyositis, and necrotizing myopathy, and examines current and promising therapies.

Keywords: Clinical presentation; Dermatomyositis; Diagnosis; Inclusion body myositis; Necrotizing myopathy; Pathology; Polymyositis; Treatment.

PubMed Disclaimer

Figures

Figure 1
Figure 1
Figure 1a. Heliotrope rash and periorbital edema and erythema in DM Figure 1b. Violaceous erythematous scaly papular rash in DM - Gottron’s papules, with cutaneous ulceration and perinungal hyperemia. Figure 1c. Periungal hyperemia in DM
Figure 1
Figure 1
Figure 1a. Heliotrope rash and periorbital edema and erythema in DM Figure 1b. Violaceous erythematous scaly papular rash in DM - Gottron’s papules, with cutaneous ulceration and perinungal hyperemia. Figure 1c. Periungal hyperemia in DM
Figure 1
Figure 1
Figure 1a. Heliotrope rash and periorbital edema and erythema in DM Figure 1b. Violaceous erythematous scaly papular rash in DM - Gottron’s papules, with cutaneous ulceration and perinungal hyperemia. Figure 1c. Periungal hyperemia in DM
Figure 2
Figure 2
Figure 2a. DM: Perifascular atrophy - Hematoxylin and eosin Figure 2b. DM: Perimysial inflammation - Hematoxylin and eosin Figure 2c. PM: Central invasion of non-necrotic fibers by inflammatory infiltrates - Hematoxylin and eosin Figure 2d. NM: Multiple necrotic fibers undergoing phagocytosis - Hematoxylin and eosin longitudinal section
Figure 2
Figure 2
Figure 2a. DM: Perifascular atrophy - Hematoxylin and eosin Figure 2b. DM: Perimysial inflammation - Hematoxylin and eosin Figure 2c. PM: Central invasion of non-necrotic fibers by inflammatory infiltrates - Hematoxylin and eosin Figure 2d. NM: Multiple necrotic fibers undergoing phagocytosis - Hematoxylin and eosin longitudinal section
Figure 2
Figure 2
Figure 2a. DM: Perifascular atrophy - Hematoxylin and eosin Figure 2b. DM: Perimysial inflammation - Hematoxylin and eosin Figure 2c. PM: Central invasion of non-necrotic fibers by inflammatory infiltrates - Hematoxylin and eosin Figure 2d. NM: Multiple necrotic fibers undergoing phagocytosis - Hematoxylin and eosin longitudinal section
Figure 2
Figure 2
Figure 2a. DM: Perifascular atrophy - Hematoxylin and eosin Figure 2b. DM: Perimysial inflammation - Hematoxylin and eosin Figure 2c. PM: Central invasion of non-necrotic fibers by inflammatory infiltrates - Hematoxylin and eosin Figure 2d. NM: Multiple necrotic fibers undergoing phagocytosis - Hematoxylin and eosin longitudinal section
See this image and copyright information in PMC

References

    1. van der Meulen MFG, Bronner IM, Hoogendijk JE, et al. Polymyositis: an overdiagnosed entity. Neurol. 2003;61:316–321. - PubMed
    1. Chahin N, Engel AG. Correlation of muscle biopsy, clinical course, and outcome in PM and sporadic IBM. Neurol. 2008;5;70(6):418–424. - PubMed
    1. Tan JA, Roberts-Thomson PJ, Blumbergs P, et al. Incidence and prevalence of idiopathic inflammatory myopathies in South Australia: a 30-year epidemiologic study of histology-proven cases. Int J Rheum Dis. 2013 Jun;16(3):331–8. - PubMed
    1. Kuo CF, See LC, Yu KH, et al. Incidence, cancer risk and mortality of dermatomyositis and polymyositis in Taiwan: a nationwide population study. Br J Dermatol. 2011;165(6):1273–9. - PubMed
    1. Regardt M, Welin Henriksson E, Alexanderson H, et al. Patients with polymyositis or dermatomyositis have reduced grip force and health-related quality of life in comparison with reference values: an observational study. Rheumatology (Oxford) 2011;50(3):578–85. - PubMed

Publication types