Inclusion body myositis: correlation of clinical outcomes with histopathology, electromyography and laboratory findings

Rheumatology ◽  
2021 ◽  
Author(s):  
Marcus V Pinto ◽  
Ruple S Laughlin ◽  
Christopher J Klein ◽  
Jay Mandrekar ◽  
Elie Naddaf
Keyword(s):  
Inclusion Body ◽  
Muscle Biopsy ◽  
Inclusion Body Myositis ◽  
Quadriceps Strength ◽  
Serum Markers ◽  
Clinical Severity ◽  
Clinical Feature ◽  
Motor Unit Potential ◽  
Laboratory Findings ◽  
Clinical Measures

Abstract Objective To determine whether histopathological, electromyographic and laboratory markers correlate with clinical measures in Inclusion Body Myositis (IBM) Methods We reviewed our electronic medical records to identify patients with IBM according to ENMC 2011 criteria, seen between 2015 and 2020. We only included patients who had a muscle biopsy and needle electromyography (EMG) performed on the same muscle (opposite or same side). We used a detailed grading system (0- normal to 4- severe) to score histopathological and EMG findings. Clinical severity was assessed by the modified Rankin scale (mRS), muscle strength sum score (SSS), quadriceps strength and severity of dysphagia on swallow evaluation. Serum markers of interest were creatine kinase level, and cN-1A antibodies. Results We included 50 IBM patients, with a median age of 69 years; 64% were males. Median disease duration at diagnosis was 51 months. On muscle biopsy, endomysial inflammation mainly correlated with dysphagia, and inversely correlated with mRS. Vacuoles and congophilic inclusions did not correlate with any of the clinical measures. On EMG, the shortness of motor unit potential (MUP) duration correlated with all clinical measures. Myotonic discharges, and not fibrillation potentials, correlated with the severity of inflammation. Serum markers did not have a statistically-significant correlation with any of the clinical measures. Conclusions Dysphagia was the main clinical feature of IBM correlating with endomysial inflammation. Otherwise, inclusion body myositis clinical measures had limited correlation with histopathological features in this study. The shortness of MUP duration correlated with all clinical measures.

scholarly journals A Rare Case of Sporadic Inclusion Body Myositis with Atypical Presentation

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.

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

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.

scholarly journals Validation of a score tool for measurement of histological severity in juvenile dermatomyositis and association with clinical severity of disease

2013 ◽  
Vol 74 (1) ◽  
pp. 204-210 ◽  
Author(s):  
Hemlata Varsani ◽  
Susan C Charman ◽  
Charles K Li ◽  
Suely K N Marie ◽  
Anthony A Amato ◽  
...  
Keyword(s):  
Disease Activity ◽  
Muscle Biopsy ◽  
Juvenile Dermatomyositis ◽  
Intraclass Correlation ◽  
Latin Square ◽  
Quadriceps Muscle ◽  
Clinical Severity ◽  
Intraobserver Agreement ◽  
Biopsy Tissue ◽  
Clinical Measures

ObjectivesTo study muscle biopsy tissue from patients with juvenile dermatomyositis (JDM) in order to test the reliability of a score tool designed to quantify the severity of histological abnormalities when applied to biceps humeri in addition to quadriceps femoris. Additionally, to evaluate whether elements of the tool correlate with clinical measures of disease severity.Methods55 patients with JDM with muscle biopsy tissue and clinical data available were included. Biopsy samples (33 quadriceps, 22 biceps) were prepared and stained using standardised protocols. A Latin square design was used by the International Juvenile Dermatomyositis Biopsy Consensus Group to score cases using our previously published score tool. Reliability was assessed by intraclass correlation coefficient (ICC) and scorer agreement (α) by assessing variation in scorers’ ratings. Scores from the most reliable tool items correlated with clinical measures of disease activity at the time of biopsy.ResultsInter- and intraobserver agreement was good or high for many tool items, including overall assessment of severity using a Visual Analogue Scale. The tool functioned equally well on biceps and quadriceps samples. A modified tool using the most reliable score items showed good correlation with measures of disease activity.ConclusionsThe JDM biopsy score tool has high inter- and intraobserver agreement and can be used on both biceps and quadriceps muscle tissue. Importantly, the modified tool correlates well with clinical measures of disease activity. We propose that standardised assessment of muscle biopsy tissue should be considered in diagnostic investigation and clinical trials in JDM.

Histopathological features of the idiopathic inflammatory myopathies

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.

scholarly journals Asymptomatic hyper-creatine-kinase-emia as sole manifestation of inclusion body myositis

2013 ◽  
Vol 5 (2) ◽  
pp. 11 ◽  
Author(s):  
Josef Finsterer ◽  
Claudia Stöllberger ◽  
Gabor G. Kovacs
Keyword(s):  
Creatine Kinase ◽  
Inclusion Body ◽  
Muscle Biopsy ◽  
Nerve Conduction ◽  
Inclusion Body Myositis ◽  
Nerve Conduction Studies ◽  
Differential Diagnoses ◽  
Sporadic Inclusion Body Myositis

Sporadic inclusion body myositis (sIBM) usually manifests with painless weakness of the hand, finger and hip flexors. Absence of symptoms or signs, but mild hyper-CK-emia as the sole manifestation of IBM, has not been reported. We report the case of a 73-year-old male who presented with asymptomatic recurrent hyper-CK-emia ranging from 200 to 1324 U/L (n<171 U/L), since 10 years. Clinical neurologic investigation, nerve conduction studies and EMG were non-informative. Muscle biopsy surprisingly revealed sIBM. sIBM may be asymptomatic and may manifest with hyper-CK-emia exclusively. So, it has to be included in the differential diagnoses of asymptomatic hyper-CK-emia.

Immunopathogenesis of Inflammatory Myopathies

2019 ◽  
pp. 177-192
Author(s):  
Marinos C. Dalakas
Keyword(s):  
Inclusion Body ◽  
Clinical Features ◽  
Muscle Tissue ◽  
Muscle Biopsy ◽  
Inclusion Body Myositis ◽  
Treatment Options ◽  
Inflammatory Myopathies ◽  
Antisynthetase Syndrome ◽  
Autoimmune Myositis ◽  
Overlap Myositis

This chapter looks at inflammatory myopathies (IM), which constitute a heterogeneous group of acquired myopathies that have in common the presence of inflammation in the muscle tissue. The chapter looks at specific clinical features such as dermatomyositis, polymyositis, necrotizing autoimmune myositis, antisynthetase syndrome-overlap myositis, and inclusion body myositis. It then considers diagnosis, which can be made through muscle biopsy and the detection of autoantibodies. Finally, it looks at treatment options.

scholarly journals Stance control orthosis trial in patients with inclusion body myositis

2011 ◽  
Vol 35 (1) ◽  
pp. 39-44 ◽  
Author(s):  
Kathie Bernhardt ◽  
Terry Oh ◽  
Kenton Kaufman
Keyword(s):  
Inclusion Body ◽  
Inclusion Body Myositis ◽  
Inflammatory Myopathy ◽  
Knee Extensor ◽  
Positive Outcome ◽  
Quadriceps Strength ◽  
Quadriceps Weakness ◽  
Functional Deficits ◽  
Stance Control ◽  
Kinematic Measures

Background: Inclusion Body Myositis (IBM) is an inflammatory myopathy that commonly affects quadriceps strength, resulting in knee buckling and falls. Therefore, patients with IBM should be ideal candidates for stance control orthoses (SCOs).Objectives: Evaluate the effectiveness of SCO use in patients with IBM who have functional deficits due to quadriceps weakness.Study Design: Cohort study.Methods: Nine subjects with IBM were provided a stance control orthosis and followed for six months of home use. All patients had objective testing of their strength and gait and completed a questionnaire at baseline and six months. Gait analysis was performed both with and without the orthosis.Results: Velocity and cadence were lower and step width was higher when using the orthosis. Kinematic measures were largely unchanged with orthosis use. Subjects with less knee extensor weakness had a better outcome than weaker participants. Those who spent more time wearing the orthosis also had a more positive outcome. The participants felt that the SCO was helpful in safeguarding against falls and providing stability.Conclusions: SCO use will benefit patients with IBM, but care should be taken to choose the SCO option that best suits their individual clinical presentation.Clinical relevanceThis is the first study to examine SCO use in the IBM population. Patients with IBM have quadriceps weakness and will potentially benefit from SCO use. This study suggests that successful SCO management of patients with IBM depends on severity of weakness.

scholarly journals Overlapping features of polymyositis and inclusion body myositis in HIV-infected patients

Neurology ◽  
2017 ◽  
Vol 88 (15) ◽  
pp. 1454-1460 ◽  
Author(s):  
Thomas E. Lloyd ◽  
Iago Pinal-Fernandez ◽  
E. Harlan Michelle ◽  
Lisa Christopher-Stine ◽  
Katherine Pak ◽  
...  
Keyword(s):  
Hiv Infection ◽  
Inclusion Body ◽  
Muscle Biopsy ◽  
Inclusion Body Myositis ◽  
Age At Onset ◽  
Case Series ◽  
Knee Extensors ◽  
Hiv Positive ◽  
Rimmed Vacuoles ◽  
The Mean

Objective:To characterize patients with myositis with HIV infection.Methods:All HIV-positive patients with myositis seen at the Johns Hopkins Myositis Center from 2003 to 2013 were included in this case series. Muscle biopsy features, weakness pattern, serum creatine kinase (CK) level, and anti–nucleotidase 1A (NT5C1A) status of HIV-positive patients with myositis were assessed.Results:Eleven of 1,562 (0.7%) patients with myositis were HIV-positive. Myositis was the presenting feature of HIV infection in 3 patients. Eight of 11 patients had weakness onset at age 45 years or less. The mean time from the onset of weakness to the diagnosis of myositis was 3.6 years (SD 3.2 years). The mean of the highest measured CK levels was 2,796 IU/L (SD 1,592 IU/L). On muscle biopsy, 9 of 10 (90%) had endomysial inflammation, 7 of 10 (70%) had rimmed vacuoles, and none had perifascicular atrophy. Seven of 11 (64%) patients were anti-NT5C1A-positive. Upon presentation, all had proximal and distal weakness. Five of 6 (83%) patients followed 1 year or longer on immunosuppressive therapy had improved proximal muscle strength. However, each eventually developed weakness primarily affecting wrist flexors, finger flexors, knee extensors, or ankle dorsiflexors.Conclusions:HIV-positive patients with myositis may present with some characteristic polymyositis features including young age at onset, very high CK levels, or proximal weakness that improves with treatment. However, all HIV-positive patients with myositis eventually develop features most consistent with inclusion body myositis, including finger and wrist flexor weakness, rimmed vacuoles on biopsy, or anti-NT5C1A autoantibodies.

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