The Influence of Neuroleptanalgesia on the Serum Activity of Muscle Enzymes in Ponies

Abstract Purpose of review To identify clues to disease activity and discuss therapy options. Recent findings The diagnostic evaluation includes documenting symmetrical proximal muscle damage by exam and MRI, as well as elevated muscle enzymes—aldolase, creatine phosphokinase, LDH, and SGOT—which often normalize with a longer duration of untreated disease. Ultrasound identifies persistent, occult muscle inflammation. The myositis-specific antibodies (MSA) and myositis-associated antibodies (MAA) are associated with specific disease course variations. Anti-NXP-2 is found in younger children and is associated with calcinosis; anti-TIF-1γ+ juvenile dermatomyositis has a longer disease course. The diagnostic rash—involving the eyelids, hands, knees, face, and upper chest—is the most persistent symptom and is associated with microvascular compromise, reflected by loss of nailfold (periungual) end row capillaries. This loss is associated with decreased bioavailability of oral prednisone; the bioavailability of other orally administered medications should also be considered. At diagnosis, at least 3 days of intravenous methyl prednisolone may help control the HLA-restricted and type 1/2 interferon–driven inflammatory process. The requirement for avoidance of ultraviolet light exposure mandates vitamin D supplementation. Summary This often chronic illness targets the cardiovascular system; mortality has decreased from 30 to 1–2% with corticosteroids. New serological biomarkers indicate occult inflammation: ↑CXCL-10 predicts a longer disease course. Some biologic therapies appear promising.

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SAT0290 HIGH SERUM MYOSTATIN LEVELS SUGGEST ACCELERATED MUSCLE SENESCENCE IN ACTIVE IDIOPATHIC INFLAMMATORY MYOSITIS

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2020-eular.5641 ◽

2020 ◽

Vol 79 (Suppl 1) ◽

pp. 1089.2-1090

Author(s):

A. Anuja ◽

M. Singh ◽

M. K. Rai ◽

H. Singh ◽

V. Agarwal ◽

...

Keyword(s):

Young Adults ◽

Elevated Serum ◽

High Serum ◽

Inactive Disease ◽

Healthy Controls ◽

Muscle Enzymes ◽

Inflammatory Myositis ◽

Idiopathic Inflammatory Myositis ◽

Healthy Control ◽

Accelerated Senescence

Background:Inflammation is the forerunner to fibrosis and premature ageing in various systemic diseases. Hence it seems plausible that idiopathic inflammatory myopathies (IIM) may exhibit accelerated senescence too.Objectives:Hence we investigated the Myostatin: Follistatin system in the serum as a reflection of early senescence in myositis as compared with healthy and diseased controls.Methods:Patients with inflammatory myositis (ACR/EULAR criteria) presenting to the wards and outpatient clinic between December 2017 to August 2019 were recruited. Those with active infection, pregnancy, renal dysfunction or chronic kidney disease were excluded. Apart from patient and disease variables, activity and damage were assessed using standard IMACS score set measures. Patients in inception cohort were additionally followed up at 1 and 6 months. Myostatin and Follistatin were estimated in sera using ELISA (R&D systems, USA). Juvenile myositis and young adults (18-40 years) were subsequently analyzed separately. Non-parametric tests were used for paired and unpaired analysis. Results expressed as median.Results:95 myositis (8 Juvenile myositis, 26 DM, 10 PM, 29 Overlap, 2 NAM 1 CAM and 19 ASS) patients (23 Male and 72 Female) with median age 38 (24.5-46.0) years and disease duration 0.9 (2.3-5.1) years were included. Serum Myostatin was lower in IIM than in healthy control (HC) (153.5 vs. 243.6 p<0.0001, Fig 1A) but higher in IIM as compared with disease controls (153.5 vs 86.1 p=0.0174 Fig. 1B). Serum myostatin was comparable between juvenile and adult myositis and in the various subsets of adult myositis (Fig. 1 C and D). Myostatin levels were higher in active as compared with inactive myositis in young adults (211.7 vs. 158.9, p=0.0149, Figure 1E). Serum Myostatin correlated with height (r 0.3, p=0.003) and weight (r 0.2, p=0.047) but not MMT8 or muscle enzymes.Figure 1.Serum Myostatin levels in IIM as compared with healthy controls (A) and disease controls (B). Levels in juvenile myositis as compared with adult IIM (C) and in various subsets of IIM (D). Serum Myostatin levels in active and inactive disease (E).Although Follistatin was lower in IIM than HC (198.4 vs 243.6, p=<0.0001), the neither Follistatin nor Myostatin: Follistatin ratios differ between subsets, and in active versus inactive disease Figure 2 A-D). On follow-up, the serial Myostatin estimation paralleled change in disease activity.Figure 2.Serum Follistatin levels in IIM as compared with healthy controls (A) and disease controls (C). Levels in juvenile and adult IIM (D) and in various subsets of IIM (D).Conclusion:Elevated serum Myostatin levels in active myositis raise the possibility of accelerated senescence in the inflamed muscle tissues which need further investigation.Acknowledgments: :Partly funded by APLAR and IRA research grants awarded to LG.Disclosure of Interests:None declared

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Muscle biopsy essential diagnostic advice for pathologists

Surgical and Experimental Pathology ◽

10.1186/s42047-020-00085-w ◽

2021 ◽

Vol 4 (1) ◽

Author(s):

Ana Cotta ◽

Elmano Carvalho ◽

Antonio Lopes da-Cunha-Júnior ◽

Jaquelin Valicek ◽

Monica M. Navarro ◽

...

Keyword(s):

Liquid Nitrogen ◽

Muscle Biopsy ◽

Diagnostic Procedures ◽

Mitochondrial Respiratory Chain ◽

Muscle Disease ◽

Diagnostic Workup ◽

Main Body ◽

Mitochondrial Myopathies ◽

Muscle Enzymes ◽

Molecular Studies

Abstract Background Muscle biopsies are important diagnostic procedures in neuromuscular practice. Recent advances in genetic analysis have profoundly modified Myopathology diagnosis. Main body The main goals of this review are: (1) to describe muscle biopsy techniques for non specialists; (2) to provide practical information for the team involved in the diagnosis of muscle diseases; (3) to report fundamental rules for muscle biopsy site choice and adequacy; (4) to highlight the importance of liquid nitrogen in diagnostic workup. Routine techniques include: (1) histochemical stains and reactions; (2) immunohistochemistry and immunofluorescence; (3) electron microscopy; (4) mitochondrial respiratory chain enzymatic studies; and (5) molecular studies. The diagnosis of muscle disease is a challenge, as it should integrate data from different techniques. Conclusion Formalin-fixed paraffin embedded muscle samples alone almost always lead to inconclusive or unspecific results. Liquid nitrogen frozen muscle sections are imperative for neuromuscular diagnosis. Muscle biopsy interpretation is possible in the context of detailed clinical, neurophysiological, and serum muscle enzymes data. Muscle imaging studies are strongly recommended in the diagnostic workup. Muscle biopsy is useful for the differential diagnosis of immune mediated myopathies, muscular dystrophies, congenital myopathies, and mitochondrial myopathies. Muscle biopsy may confirm the pathogenicity of new gene variants, guide cost-effective molecular studies, and provide phenotypic diagnosis in doubtful cases. For some patients with mitochondrial myopathies, a definite molecular diagnosis may be achieved only if performed in DNA extracted from muscle tissue due to organ specific mutation load.

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Quantitative T2 mapping accelerated by GRAPPATINI for evaluation of muscles in patients with myositis

British Journal of Radiology ◽

10.1259/bjr.20190109 ◽

2019 ◽

Vol 92 (1102) ◽

pp. 20190109 ◽

Cited By ~ 3

Author(s):

Fengdan Wang ◽

Haiping Zhang ◽

Chanyuan Wu ◽

Qian Wang ◽

Bo Hou ◽

...

Keyword(s):

T2 Mapping ◽

Quantitative Information ◽

Thigh Muscle ◽

Mapping Technique ◽

Acquisition Time ◽

T2 Relaxation ◽

Muscle Enzymes ◽

Scanning Time ◽

Conventional Mri ◽

Thigh Muscles

Objective: Dermatomyositis (DM) and polymyositis (PM) make up the largest group of potentially treatable myopathies and require early diagnosis. This study investigates whether the edema of thigh muscles in DM/PM can be quantitatively assessed by a novel accelerated T2 mapping technique—GRAPPATINI. Methods: Three conventional MR sequences and GRAPPATINI accelerated T2 mapping of bilateral thighs from 20 patients (7 DM and 13 PM) and 10 healthy volunteers were prospectively carried out on a 3 T MR scanner. Afterwards, T2 values of 477 thigh muscles from the patients and the healthy controls were manually measured. In addition, the correlations between T2 values and serum muscle enzymes in patients were also analyzed. Results: The new GRAPPATINI technique made quantitative T2 mapping of bilateral thighs feasible with a scanning time of only 2 min 18 s. Moreover, GRAPPATINI-generated T2 values of muscles from patients were markedly higher than those from healthy subjects (p < 0.001). GRAPPATINI accelerated T2 mapping appeared a more sensitive technique in that some DM/PM muscles appearing normal per conventional MRI had increased T2 relaxation time. Furthermore, GRAPPATINI-generated T2 values of DM/PM thigh muscles positively correlated with serum enzyme levels (p < 0.001), which reflected the severity of myopathy. Conclusion: GRAPPATINI can significantly shorten acquisition time of T2 mapping and may potentially be applied clinically in DM and PM. Advances in knowledge: GRAPPATINI acceleration makes T2 mapping feasible in clinical practice in providing quantitative information regarding thigh muscle inflammation in DM and PM.

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FRI0248 PROGNOSTIC FACTORS FOR STEROID-FREE REMISSION IN PATIENTS WITH IDIOPATHIC INFLAMMATORY MYOPATHIES: IMPORTANCE OF ANTHROPOMETRIC MEASUREMENTS

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2020-eular.3010 ◽

2020 ◽

Vol 79 (Suppl 1) ◽

pp. 708.1-708

Author(s):

J. S. Lee ◽

S. H. Nam ◽

S. J. Choi ◽

W. J. Seo ◽

S. Hong ◽

...

Keyword(s):

Prognostic Factors ◽

Cox Regression ◽

Idiopathic Inflammatory Myopathies ◽

Inflammatory Myopathies ◽

Immunosuppressant Therapy ◽

Muscle Enzymes ◽

Cox Regression Analysis ◽

Factors Associated ◽

Early Use

Background:Several studies have been conducted on factors associated with mortality in idiopathic inflammatory myopathies (IIM), but few studies have assessed prognostic factors for steroid-free remission in IIM.Objectives:We investigated the various clinical factors, including body measurements, that affect IIM treatment outcomes.Methods:Patients who were newly diagnosed with IIM between 2000 and 2018 were included. Steroid-free remission was defined as at least three months of normalisation of muscle enzymes and no detectable clinical disease activity. The factors associated with steroid-free remission were evaluated by a Cox regression analysis.Results:Of the 106 IIM patients, 35 displayed steroid-free remission during follow-up periods. In the multivariable Cox regression analyses, immunosuppressants’ early use within one month after diagnosis [hazard ratio (HR) 6.21, 95% confidence interval (CI) 2.61–14.74, p < 0.001] and sex-specific height quartiles (second and third quartiles versus first quartile, HR 3.65, 95% CI 1.40–9.51, p = 0.008 and HR 2.88, 95% CI 1.13–7.32, p = 0.027, respectively) were positively associated with steroid-free remission. Polymyositis versus dermatomyositis (HR 0.21, 95% CI 0.09–0.53, p = 0.001), presence of dysphagia (HR 0.15, CI 0.05–0.50, p = 0.002) and highest versus lowest quartile of waist circumference (WC) (HR 0.24, 95% CI 0.07–0.85, p = 0.027) were negatively associated with steroid-free remission.Conclusion:The early initiation of immunosuppressant therapy, type of myositis and presence of dysphagia are strong predictors of steroid-free remission in IIM; moreover, height and WC measurements at baseline may provide additional important prognostic value.Disclosure of Interests:None declared

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