Clinical manifestations and diagnosis of inclusion body myositis
- Marc L Miller, MD
Marc L Miller, MD
- Clinical Assistant Professor of Medicine
- Tufts University School of Medicine
- Thomas E Lloyd, MD, PhD
Thomas E Lloyd, MD, PhD
- Codirector, Johns Hopkins Myositis Center
- Associate Professor of Neurology, Neuroscience, and Genetics
- Johns Hopkins University School of Medicine
- Section Editors
- Ira N Targoff, MD
Ira N Targoff, MD
- Section Editor — Muscle Disease
- Professor of Medicine, Section of Rheumatology
- University of Oklahoma Health Sciences Center
- Jeremy M Shefner, MD, PhD
Jeremy M Shefner, MD, PhD
- Section Editor — Neuromuscular Disease
- Professor and Chair of Neurology, Barrow Neurological Institute
- Professor of Neurology, University of Arizona, Phoenix
- Clinical Professor of Neurology, Creighton University
Sporadic inclusion body myositis (IBM) is classified along with polymyositis, dermatomyositis, and autoimmune necrotizing myopathy as one of the idiopathic inflammatory myopathies. However, despite some similarities, the clinicopathologic manifestations of IBM are clearly distinct from the other disorders (table 1). (See "Clinical manifestations of dermatomyositis and polymyositis in adults".)
The clinical manifestations and diagnosis of IBM will be reviewed here. The treatment and prognosis are discussed separately. (See "Management of inclusion body myositis".)
Inclusion body myositis (IBM) is a rare sporadic disorder with a prevalence that is estimated at five to nine cases per million adults [1-3]; however, some estimates of prevalence have been as high as 70 per million population . There is a paucity of data on the annual incidence; the reported range in two studies is from 1 to 7.9 per million [4,5]. Due to the high incidence of weakness complaints in older adults and the insidious onset of weakness in IBM, this disease is frequently misdiagnosed . Indeed, it is the most common acquired idiopathic inflammatory myopathy in individuals over the age of 50 .
The disease affects men more often than women . The mean age at onset of symptoms is approximately 60 years, with a range from the fourth to the ninth decade.
Symptoms and signs — Patients with inclusion body myositis (IBM) present with the insidious onset of weakness. The average duration of symptoms before diagnosis is about five years [3,7]. The most common initial presentation is slowly progressive proximal leg weakness, with difficulty getting up out of a chair or frequent falls. In some patients, the initial complaint may be with weakness of grip strength, such as difficulty opening jars. Rarely, patients may present with isolated dysphagia .
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- CLINICAL MANIFESTATIONS
- Symptoms and signs
- Laboratory abnormalities
- Physical examination
- Laboratory testing
- Magnetic resonance imaging
- Muscle biopsy
- PROPOSED DIAGNOSTIC CRITERIA
- DIFFERENTIAL DIAGNOSIS
- INFORMATION FOR PATIENTS
- SUMMARY AND RECOMMENDATIONS