Sarcoidosis: Is It a Possible Trigger of Inclusion Body Myositis?

Sarcoidosis is a multisystem disorder of unknown etiology, characterized pathologically by the presence of nonnecrotizing granulomatous inflammation in affected organs. Although skeletal muscle is involved in 50–80 percent of individuals with sarcoidosis, symptomatic myopathy has been shown to be a rare manifestation of the disease. Inclusion body myositis (IBM) is a rare acquired idiopathic inflammatory myopathy with the insidious onset of asymmetric and distal muscle weakness that characteristically involves the quadriceps, tibialis anterior, and forearm flexors. Moreover, dysphagia can be the presenting complaint in one-third of patients. Herein, we are presenting a case of 67-year-old African American female who presented with one-month history of new onset progressive dyspnea on exertion. She was diagnosed with stage IV sarcoidosis based on chest CT scan findings and transbronchial lung biopsy revealing nonnecrotizing granulomatous inflammation. Over the next three months after her diagnosis, she presented to the hospital with progressive dysphagia associated with asymmetrical distal muscle weakness. A quadriceps muscle biopsy revealed features consistent with inclusion body myositis. We are reporting this case as it may support the hypothesis of sarcoidosis being a trigger that possibly promotes the development of inclusion body myositis, leading to a very poor prognosis.

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Physical therapy improves motion in a patient with inclusion body myositis - a case report

Vojnosanitetski pregled ◽

10.2298/vsp171110165s ◽

2020 ◽

Vol 77 (11) ◽

pp. 1216-1220

Author(s):

Jelena Stevanovic ◽

Maja Vulovic ◽

Danijela Pavicevic ◽

Mihailo Bezmarevic ◽

Andjelka Stojkovic ◽

...

Keyword(s):

Case Report ◽

Physical Therapy ◽

Inclusion Body ◽

Muscle Weakness ◽

Muscle Biopsy ◽

Inclusion Body Myositis ◽

Clinical Symptoms ◽

Inflammatory Myopathy ◽

Functional Abilities ◽

Progressive Muscle Weakness

Introduction. Inclusion body myositis (IBM) is a rare form of inflammatory myopathy with a slowly progressive course. It is manifested by early weakness and atrophy of skeletal muscles, especially forearm muscles and the quadriceps. At the very beginning of the disease, clinical symptoms are not pronounced, therefore it is difficult to diagnose. Case report. A forty-eight-year-old female patient visited her doctor due to the weakness of muscles in arms and legs. Five years prior to this, she was treated by a neurologist and a physiatrician on several occasions with different diagnoses for progressive muscle weakness. During the last hospitalization, IBM was diagnosed after the muscle biopsy findings. After the diagnosis, the patient underwent intensive physical therapy in order to preserve the ability to independently perform everyday activities and stability of walk. Conclusion. IBM is a rare clinical entity which often takes several years to be diagnosed. Progressive muscle weakness in elderly should point to possible IBM diagnosis, which is only confirmed by muscle biopsy. Physical therapy has a significant role in the treatment as it leads to improvement of functional abilities of the patients in their daily activities, thus reducing the disability degree.

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Coexistence of TDP-43 and C5b-9 staining of muscle in a patient with inclusion body myositis

BMJ Case Reports ◽

10.1136/bcr-2020-238312 ◽

2021 ◽

Vol 14 (2) ◽

pp. e238312

Author(s):

Christina Law ◽

Huili Li ◽

Sankar Bandyopadhyay

Keyword(s):

Inclusion Body ◽

Transcriptional Repression ◽

Inclusion Body Myositis ◽

Inflammatory Myopathy ◽

Membrane Attack Complex ◽

Muscle Fibres ◽

Rimmed Vacuoles ◽

Histocompatibility Complex ◽

Immune Mediated ◽

Sporadic Inclusion Body Myositis

While sporadic inclusion body myositis (sIBM) is the most commonly acquired inflammatory myopathy above 50 years of age, its refractory response to conventional immunosuppressive treatments raises questions about its perplexing pathogenesis. Muscle biopsy typically reveals major histocompatibility complex I antigens and CD8+ T cell endomysial infiltrates invading non-necrotic muscle fibres early in the disease course with rimmed vacuoles, protein aggregates and amyloid inclusions later in the disease. Transactive response DNA-binding protein-43 (TDP-43), a protein implicated in transcriptional repression in neurodegenerative diseases, is also found in sIBM. C5b-9 membrane attack complex, an effector protein involved in the complement cascade of the immune response, is commonly found in dermatomyositis, but has rarely been reported in IBM. We describe a novel case of IBM with simultaneous C5b-9 and TDP-43 staining on quadriceps biopsy, raising the question of a possibility of concurrent immune-mediated inflammatory and myodegenerative pathogenesis.

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1001. Experimental Targeted Gene Therapy for Quadriceps Muscle Weakness for Sporadic Inclusion Body Myositis (sIBM) with Implications for Other Neuromuscular Disorders

Molecular Therapy ◽

10.1016/s1525-0016(16)40404-1 ◽

2008 ◽

Vol 16 ◽

pp. S375-S376

Keyword(s):

Gene Therapy ◽

Inclusion Body ◽

Muscle Weakness ◽

Inclusion Body Myositis ◽

Neuromuscular Disorders ◽

Quadriceps Muscle ◽

Sporadic Inclusion Body Myositis ◽

Targeted Gene Therapy

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A Rare Case of Sporadic Inclusion Body Myositis with Atypical Presentation

Journal of Health and Allied Sciences NU ◽

10.1055/s-0041-1739230 ◽

2021 ◽

Author(s):

Manokaran Chinnusamy ◽

Sathiyanarayanan Janakiraman ◽

Ramesh Bala Arivazhagan

Keyword(s):

Inclusion Body ◽

Muscle Biopsy ◽

Inclusion Body Myositis ◽

Rare Case ◽

Immunosuppressive Agents ◽

Inflammatory Myopathy ◽

Atypical Presentation ◽

Sporadic Inclusion Body Myositis

AbstractSporadic inclusion body myositis (IBM) is the most common acquired inflammatory myopathy that occurs after the age of 50 years. IBM typically involves wrist and finger flexors and quadriceps, but all sporadic IBM may not have the classic presentation of distal arm and proximal leg involvement. Treating physicians must be aware of this atypical presentation to avoid the misdiagnosis of IBM, leading to treatment with immunosuppressive agents. The aim of this study is to increase the awareness among physicians about the atypical presentation of IBM and to emphasize the importance of muscle biopsy in such cases. Here we report a case of 52 years old male diagnosed with sporadic IBM by muscle biopsy presented with atypical presentation.

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Betekintés az idiopathiás inflammatorikus myopathiában szenvedő betegek gyógytornájába

Orvosi Hetilap ◽

10.1556/650.2016.30549 ◽

2016 ◽

Vol 157 (39) ◽

pp. 1557-1562

Author(s):

Andrea Váncsa

Keyword(s):

Randomized Controlled Trials ◽

Inclusion Body ◽

Aerobic Capacity ◽

Inclusion Body Myositis ◽

Inflammatory Myopathy ◽

Idiopathic Inflammatory Myopathy ◽

Muscle Performance ◽

Controlled Trials ◽

Randomized Controlled ◽

Active Disease

Using current recommended treatment, a majority of patients with idiopathic inflammatory myopathy develop muscle impairment and poor health. Beneficial effects of exercise have been reported on muscle performance, aerobic capacity and health in chronic polymyositis and dermatomyositis, as well as in active disease and inclusion body myositis to some extent. Importantly, randomized controlled trials indicate that improved health and decreased clinical disease activity could be mediated through increased aerobic capacity. Recently, reports seeking pathomechanisms of the underlying effects of exercise on skeletal muscle indicate increased aerobic capacity (i.e. increased mitochondrial capacity and capillary density, reduced lactate levels), activation of genes of aerobic phenotype and muscle growth programs and down regulation of genes related to inflammation. Exercise contributes to both systemic and within-muscle adaptations demonstrating that it is fundamental for improving muscle performance and health in patients with idiopathic inflammatory myopathy. There is a need for randomized controlled trials to study the effects of exercise in patients with active disease and inclusion body myositis. Orv. Hetil., 2016, 157(39), 1557–1562.

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Muscle fiber dysfunction contributes to clinical muscle weakness in inclusion body myositis

Neuromuscular Disorders ◽

10.1016/j.nmd.2017.06.222 ◽

2017 ◽

Vol 27 ◽

pp. S154

Author(s):

S. Lassche ◽

A. Rietveld ◽

A. Heerschap ◽

J. Van Hees ◽

M. Hopman ◽

...

Keyword(s):

Inclusion Body ◽

Muscle Weakness ◽

Muscle Fiber ◽

Inclusion Body Myositis

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Inclusion body myositis in twins

Neurology ◽

10.1212/wnl.51.2.598 ◽

1998 ◽

Vol 51 (2) ◽

pp. 598-600 ◽

Cited By ~ 29

Author(s):

Anthony A. Amato ◽

Robert T. Shebert

Keyword(s):

Genetic Susceptibility ◽

Inclusion Body ◽

Muscle Weakness ◽

Inclusion Body Myositis ◽

Late Onset ◽

Inclusion Body Myopathy ◽

Different Ages ◽

Sporadic Inclusion Body Myositis ◽

Volar Forearm ◽

Progressive Weakness

Sporadic inclusion body myositis (s-IBM) is characterized by late onset of slowly progressive weakness that involves the quadriceps and volar forearm muscles early in the course of the disease. There are hereditary forms of inclusion body myopathy (h-IBM) that histologically resemble s-IBM. The lack of inflammation on biopsy and the different ages at onset and patterns of muscle weakness distinguish s-IBM from h-IBM. We report twin brothers with the typical clinical and histologic features of s-IBM. The occurrence of s-IBM in these twins suggests the possibility of a genetic susceptibility to developing s-IBM.

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Distal muscle involvement in granulomatous myositis can mimic inclusion body myositis

Journal of Neurology Neurosurgery & Psychiatry ◽

10.1136/jnnp.2009.190751 ◽

2010 ◽

Vol 82 (6) ◽

pp. 674-677 ◽

Cited By ~ 18

Author(s):

S. Larue ◽

T. Maisonobe ◽

O. Benveniste ◽

C. Chapelon-Abric ◽

O. Lidove ◽

...

Keyword(s):

Inclusion Body ◽

Inclusion Body Myositis ◽

Muscle Involvement ◽

Distal Muscle ◽

Granulomatous Myositis

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Histopathological features of the idiopathic inflammatory myopathies

10.1093/med/9780198754121.003.0013 ◽

2018 ◽

Author(s):

Marianne de Visser and Eleonora M.A. Aronica

Keyword(s):

Inclusion Body ◽

Muscle Biopsy ◽

Inclusion Body Myositis ◽

Inflammatory Myopathy ◽

Adult Patients ◽

Idiopathic Inflammatory Myopathies ◽

Inflammatory Myopathies ◽

Histopathological Features ◽

Muscle Biopsies

In adult patients with presumed idipathic inflammatory myopathy (IIM) without a characteristic and diagnostic dermatomyositis rash, muscle biopsy is mandatory to confirm the IIM diagnosis and to exclude a myopathy which would not respond to glucocorticoids or other immunosuppressants, including inclusion body myositis. This chapter discusses when, where, and how to undertake muscle biopsies, when to repeat them, how to interpret their results, and how these relate to IIM subtypes and disease processes.

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