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Myalgia with the Presence of Pathologic EMG Correlates with Perimysial Inflammatory Infiltrates Kirsten Johannssen, Nicholas Schwab, Carsten P

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Myalgia with the Presence of Pathologic EMG Correlates with Perimysial Inflammatory Infiltrates Kirsten Johannssen, Nicholas Schwab, Carsten P ARTICLE OPEN ACCESS Myalgia with the presence of pathologic EMG correlates with perimysial inflammatory infiltrates Kirsten Johannssen, MD, Nicholas Schwab, PhD, Carsten P. Wessig, MD, Karlheinz Reiners, MD, Correspondence Heinz Wiendl, MD, and Claudia Sommer, MD Dr. Sommer [email protected] Neurol Neuroimmunol Neuroinflamm 2019;6:e549. doi:10.1212/NXI.0000000000000549 Abstract Objective We aimed to define normal numbers of inflammatory cells in muscle biopsies and to identify the predictive value of isolated muscle pain and increased creatine kinase regarding the diagnosis of myositis. Methods We analyzed muscle biopsies of 71 patients using immunostains for CD3+, CD4+, CD8+, CD68+, major histocompatibility complex class I, perforin, and myeloid-related protein (MRP) 8. Patients were categorized as follows—group 1: myalgia without further clinical or laboratory abnormalities (n = 24); group 2: asymptomatic elevation of creatine kinase (hyperCKemia, n = 26); group 3: myalgia and pathologic EMG findings (n = 9); and group 4: otherwise healthy controls who had malignant hyperthermia susceptibility testing (n = 12). Results In the normal muscle biopsy specimens from group 4, mean endomysial macrophage (CD68+) density was 21.7 ± 5.6/mm2, and perimysial density was 13.0 ± 5.6/mm2. Numbers of T-lymphocytes (CD3+) were 5 ± 3.5 endomysially and 2.2 ± 3.9/mm2 perimysially. This was not different from groups 1 and 2. Only group 3 patients had increased mean numbers of perimysial macrophages (24.1 ± 6.3/mm2; p = 0.0005), CD3+ (7.6 ± 4.9/mm2; p = 0.0056), and CD8+ T-lymphocytes (5.4 ± 3.1/mm2; p = 0.0008) and displayed the activation marker MRP8 in all cases. Although inflammatory cells were increased in the perimysium in group 3, histology did not fulfill the criteria for dermatomyositis, polymyositis, or inclusion body myositis. Conclusions Normal muscle contains a considerable number of macrophages and T-lymphocytes. Muscle biopsy is likely to detect inflammatory changes in patients with myalgia or hyperCKemia only if pathologic EMG findings are present. From the Department of Neurology (K.J., N.S., C.P.W., K.R., H.W., C.S.), University of Wurzburg;¨ Department of Neurology (N.S., H.W.), University of Munster,¨ Munster,¨ Germany; and Department of Neurology (K.J.), University of Erlangen, Germany. Funding information and disclosures are provided at the end of the article. Full disclosure form information provided by the authors is available with the full text of this article at Neurology.org/NN. The Article Processing Charge was funded by the University Hospital Wurzburg.¨ This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivatives License 4.0 (CC BY-NC-ND), which permits downloading and sharing the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal. Copyright © 2019 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology. 1 Glossary ATPase = adenosine triphosphatase; H&E = hematoxylin and eosin; hyperCKemia = elevation of serum creatine kinase; MH = malignant hyperthermia; MHC = major histocompatibility complex; MRP = myoloid-related protein. Muscle pain is a common complaint in clinical practice. laboratory abnormalities other than hyperCKemia that were Sometimes, an underlying myopathy or myositis is suspected, possibly related to the complaints. We used biopsies from and patients are referred to a specialized center for neuro- patients with definite polymyositis (n = 4), dermatomyositis (n muscular diagnostic workup. Similarly, patients with isolated = 5), and inclusion body myositis (n = 4) as positive controls for elevation of serum creatine kinase (hyperCKemia) may be histology. Diagnoses were based on published standard criteria.2 referred for diagnostic workup of myopathy or for malignant For each patient, the following clinical and laboratory data were hyperthermia (MH) susceptibility testing.1 If clinical exami- considered: age, clinical symptoms, routine serum chemistry nation and EMG findings are normal, making a diagnosis in including CK levels, C-reactive protein, erythrocyte sedimenta- these patients can be difficult, and muscle biopsy may be tion rate, whole-blood and differential cell counts, serum elec- performed. After exclusion of metabolic or mitochondrial trolytes, renal and liver function tests, thyroid function tests, myopathy or muscular dystrophy, immunohistochemistry may autoantibodies (all: antinuclear antibodies, anti-neutrophil identify inflammatory cells and thus possibly detect myositis. cytoplasmic antibodies, rheumatoid factor, Anti-Sj¨ogren’s- Although there are precise clinical and histologic criteria for the syndrome-related antigen A, Anti-Sj¨ogren’s-syndrome-related diagnosis of polymyositis, dermatomyositis, and inclusion body antigen B, and Jo-1; from 2000: signal recognition particle, Mi- myositis,2 sometimes, inflammatory infiltrates are found in 2 alpha and beta, PL-7, PL-12, and Ro-52, immunology labo- muscle biopsies, which do not fulfill either of these criteria. ratory of Universit¨atsklinikum W¨urzburg and Euroimmun, Some of these may be diagnosed as “interstitial myositis,” in L¨ubeck, Germany), and EMG recording. None of the patients particular, in the context of collagenoses.3,4 So far, normal limits was treated with immunosuppressive drugs including cortico- for the presence of lymphocytes and macrophages in muscle steroids or immunomodulators at the time of biopsy. None of serving to establish inflammation in a muscle biopsy still need to the patients’ medical history showed relevant comorbidities at be defined. Our study therefore aimed at (1) determining the the time of presentation, particularly no evidence of rheuma- numbers of T cells and macrophages in normal muscle and (2) tologic, infectious, metabolic, or endocrine disorders. evaluating the frequency of myositis in patients with isolated muscle pain or hyperCKemia. In particular, we used markers for EMG T-cell and macrophage subtypes, with the hypothesis that Concentric needle EMG of deltoid, biceps brachii, anterior subtype profiles might be different between groups. tibial, and rectus femoris muscles was performed. EMG was classified as “myopathic” if amplitudes of motor unit potentials were abnormally low, duration was shorter than laboratory Methods normal values, and recruitment of motor units was increased. Standard protocol approvals and Histology and immunohistochemistry patient consents Muscle biopsy specimens were obtained for diagnostic purposes Patients were recruited retrospectively from our histology with informed consent. Open muscle biopsies were taken from database. All patients who had a muscle biopsy for diagnostic a muscle selected following clinical or EMG criteria under local reasons between 1996 and 2006 were eligible (n = 1,065). anesthesia. To avoid EMG-associated artifacts in biopsy speci- Tissue sampling and analysis was approved by the Ethics mens, the contralateral limb was selected for biopsy. In MH Committee of the W¨urzburgMedical Faculty. Written in- testing, the vastus lateralis muscle was biopsied according to 6 formed consent was obtained from all participants. standard protocols for the in vitro contracture testing. Routine histology and histochemistry were performed, including the Patients following stains on 10-μm cryosections: hematoxylin and eosin Inclusion criteria for the study were as follows: (1) myalgia (H&E), Elastica van Gieson, modified Gomori trichrome, without further clinical or laboratory abnormalities, (2) asymp- succinate dehydrogenase, nicotinamide-adenine dinucleotide, tomatic hyperCKemia, (3) myalgia with and without hyper- adenosine triphosphatase (ATPase), periodic acid–Schiff,oil CKemia and pathologic findings in EMG, and (4) asymptomatic red, acid phosphatase, cytochrome oxidase, and myophos- (negative testing for MH susceptibility to the University De- phorylase activity, as well as dystrophin, sarcoglycan, caveolin-3, partment of Anesthesiology).5 Exclusion criteria were the pres- and dysferlin immunohistochemistry. For immunohistochem- ence of pareses, any other diagnosis of myopathy by clinical and istry, flash-frozen muscle biopsy specimens were cut into 10-μm histologic examination (including histochemistry and immuno- cryostat sections. Unspecific binding in acetone-fixed, air-dried histochemistry for metabolic and hereditary disorders), positive sections was blocked for 30 minutes in 10% normal bovine genetic testing for myotonic dystrophy type 2, a history of statin serum, and sections were then incubated with primary anti- use, recent immunization into an affected muscle, and any bodies (supplemental table 1, links.lww.com/NXI/A101) or 2 Neurology: Neuroimmunology & Neuroinflammation | Volume 6, Number 3 | May 2019 Neurology.org/NN corresponding nonimmune immunoglobulin G (IgG) isotype the slides. Endo- and perimysial immunoreactivity was controls, diluted in 1% normal bovine serum at 4°C overnight. evaluated across 5 visual fields in a magnification of ×200 in Thereafter, a biotinylated secondary antibody (anti-mouse IgG each biopsy specimen, including 1 visual field that contained [H + L], Vector Laboratories, Burlingame, CA) and avidin- a larger blood vessel. The total number of immunoreactive biotin–horseradish peroxidase complex (DAKO, Hamburg, cells was counted in a prespecified area of interest Germany) were applied before incubating and staining with (1.25 mm2) and expressed as
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