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

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

scholarly journals P112 Muscles in myositis with normal MRI appearance have higher quantitative T2 compared to those in healthy controls

Rheumatology ◽  
2020 ◽  
Vol 59 (Supplement_2) ◽  
Author(s):  
Matthew Farrow ◽  
John Biglands ◽  
Andrew Grainger ◽  
Elizabeth Hensor ◽  
Philip O'Connor ◽  
...  
Keyword(s):  
Muscle Strength ◽  
Spin Echo ◽  
Muscle Pathology ◽  
Healthy Controls ◽  
Muscle Enzymes ◽  
Spin Echo Sequence ◽  
Healthy Control ◽  
And Gender ◽  
The Individual ◽  
T2 Mri

Abstract Background Myositis is an autoimmune disease which can cause a decrease in quality of life and increased mortality, presenting with muscle weakness, raised muscle enzymes and myalgia. Diagnosis is reliant on subjective clinical examinations, blood tests, conventional MRI and invasive muscle biopsies. Quantitative T2 MRI offers a non-invasive measurement of muscle oedema which could help improve the understanding of muscle pathology and potentially inform diagnosis. The aim of this study was to evaluate whether quantitative T2 MRI of muscles is sensitive enough to detect differences in myositis patients compared to healthy controls, and how it compares with current radiologist scoring methods. Methods 16 myositis patients were recruited (10/16 female, 10 polymyositis, 6 dermatomyositis, mean age 50 ± 26), median CK 1000IU/L ± 3100IU/L, and 16 age and gender matched healthy controls were recruited. MRI of the dominant thigh were performed. Imaging was performed using a fat-suppressed turbo-spin echo sequence. Quantitative T2 measurements were obtained from regions of interest (ROI) drawn manually within the individual muscles that make up the quadriceps and hamstrings with no distinction made between affected and unaffected muscles. A mono-exponential fit was used to obtain an estimate of the T2 from each ROI. Two radiologists blinded to the diagnosis, semi-quantitatively scored by consensus the muscles on a 4-point visual scale as either no oedema (0), mild oedema (1), moderate oedema (2) or severe oedema (3). Muscle strength was assessed using an isokinetic dynamometer. Results T2 values were higher in myositis patients compared to healthy controls [mean (SD) hamstring myositis 47.8ms (7.7ms), healthy 39.9ms (1.5ms), p &lt; 0.001; quadriceps myositis 53.8ms (12.1ms), healthy 42.1ms (2.1ms), p &lt; 0.001]. Quantitative T2 correlated with the radiologists’ oedema scores with rs=0.7 in the hamstrings (p &lt; 0.001) and rs=0.6 in the quadriceps (p &lt; 0.001), with an upward trend in T2 as radiologist scored visible oedema increased. Patients who had been classified as normal by the radiologists were compared with matched healthy controls (n = 8), T2 values for patients with ‘normal muscle’ were still higher than those for healthy controls: mean T2 in the hamstrings (myositis 42.2ms, healthy controls 38.7ms, p = 0.004); mean T2 in the quadriceps (myositis 43.9ms, healthy controls 40.1ms, p = 0.001). T2 was inversely correlated with muscle strength in all participants. Conclusion Quantitative T2 measurements can detect muscle differences between myositis patients and healthy control groups, which suggests that this measurement could be used as an objective method to monitor muscles. They are also sensitive to differences that may not be detected by radiologists. This suggests that subtle systemic changes in muscle in myositis patients, which go undetected in semi-quantitative visual scoring, can be detected using quantitative T2 measurements. This shows the potential for T2 measurements to be a diagnostic measure in the diagnosis and management of myositis. Disclosures M. Farrow None. J. Biglands None. A. Grainger None. E. Hensor None. P. O'Connor None. A. Ladas None. S. Tanner None. A. Aslam None. P. Emery None. A. Tan None.

scholarly journals 3420 Estradiol levels are elevated in older men with diffuse cutaneous SSc and are associated with decreased survival

2019 ◽  
Vol 3 (s1) ◽  
pp. 104-105
Author(s):  
DeAnna Baker Frost ◽  
Bethany Wolf ◽  
Christine Peoples ◽  
Jessica Fike ◽  
Katherine Silver ◽  
...  
Keyword(s):  
Cox Regression ◽  
Elevated Serum ◽  
High Serum ◽  
Organ Involvement ◽  
Healthy Controls ◽  
University Of Pittsburgh ◽  
Risk Of Death ◽  
Heart Involvement ◽  
Increased Risk ◽  
Impact On Survival

OBJECTIVES/SPECIFIC AIMS: Our objective was to examine serum E2 levels in dcSSc males in relation to disease characteristics (i.e autoanitbody profile and internal organ involvement) and its impact on survival. METHODS/STUDY POPULATION: We measured serum E2 levels in 83 dcSSc men >50 years old from the University of Pittsburgh Scleroderma Center and healthy controls of similar age. Using statistical modeling, we examined the associations between circulating E2 levels, internal organ involvement, autoantibody profiles, and survival. RESULTS/ANTICIPATED RESULTS: Male dcSSc patients had significantly higher serum E2 levels compared to healthy male controls and compared to dcSSc post-menopausal women of similar age. Male dcSSc patients with high serum E2 levels had significantly more heart involvement and worse survival. Using Cox regression modeling for risk of death, increasing serum E2 levels in anti-Scl-70 antibody positive dcSSc males were associated an increased risk of death. DISCUSSION/SIGNIFICANCE OF IMPACT: DcSSc male patients have higher levels of E2 compared to healthy controls and dcSSc postmenopausal women. Elevated serum E2 levels in dcSSc males >50 are associated with heart involvement and, if anti-Scl-70 antibody positive, worse survival. Our current study expands on our previous work, not only that that E2 exerts pro-fibrotic effects on skin, but also internal organ involvement, overall survival. These data suggest an important role of estrogen imbalance in SSc.

scholarly journals A study on demographic pattern of idiopathic inflammatory myopathies in a tertiary care hospital

2019 ◽  
Vol 6 (2) ◽  
pp. 236
Author(s):  
Srujith C. H. ◽  
Kavitha Mohanasundaram ◽  
Jagadeesan M. ◽  
Halleys Kumar E. ◽  
Kannan R. ◽  
...  
Keyword(s):  
Tertiary Care ◽  
Lactic Dehydrogenase ◽  
Idiopathic Inflammatory Myopathies ◽  
Care Hospital ◽  
Inflammatory Myopathies ◽  
Muscle Enzymes ◽  
Cross Sectional ◽  
Inflammatory Myositis ◽  
Idiopathic Inflammatory Myositis

Background: Idiopathic inflammatory myopathies (IIMs) are a group of chronic systemic autoimmune diseases characterized by proximal muscle weakness and elevated muscle enzymes. Aim and Objective was to analyze the demographic profile of patients with idiopathic inflammatory myopathies (IIM).Methods: This was a cross sectional observational study conducted over a period of two years (2016-2018). After obtaining institutional ethical committee clearance, informed consent from patients. 16 patients who fulfilled the criteria were included in the study. The demographic and the clinical data were analysed.Results: The mean age was 47.3±11.2 years. The study showed female predominance. ANA was positive in 11(68.7%) patients. Among the 16 patients, 5 (31.25%) had polymyositis and 11 (68.7%) had dermatomyositis. The median enzymes levels were creatinine kinase 1134 U/L, lactic dehydrogenase 477U/L, ALT (alanine aminotransferase) 154 IU/L, AST (aspartate aminotransferase) 236IU/L. Raynaud's phenomenon was seen in 37.5%. In our study, 31.25% had hypothyroidism and 6.25% had diabetic mellitus. On follow up 37.5% developed interstitial lung disease (ILD) and 18.75% were found to have malignancy.Conclusions: Steroids and immunomodulators are the mainstay of treatment in patients with idiopathic inflammatory myositis. All our patients improved with steroids. It is important to evaluate these patients during early stages and follow up to prevent complications.

Elevated Serum Levels of Soluble CD163 in Polymyositis and Dermatomyositis: Associated with Macrophage Infiltration in Muscle Tissue

2015 ◽  
Vol 42 (6) ◽  
pp. 979-987 ◽  
Author(s):  
Qing-Lin Peng ◽  
Yin-Li Zhang ◽  
Xiao-Ming Shu ◽  
Han-Bo Yang ◽  
Lu Zhang ◽  
...  
Keyword(s):  
Muscle Tissue ◽  
Laboratory Data ◽  
Elevated Serum ◽  
Clinical Manifestations ◽  
Serum Levels ◽  
High Serum ◽  
Macrophage Infiltration ◽  
Healthy Controls ◽  
Soluble Cd163 ◽  
Cell Counts

Objective.To investigate serum levels of soluble CD163 (sCD163) in patients with polymyositis (PM) and dermatomyositis (DM), and to correlate these to clinical manifestations and laboratory data.Methods.Serum levels of sCD163 were detected in 24 patients with PM, 84 patients with DM, and 46 healthy controls by using the ELISA method. Immunohistochemistry staining of macrophage infiltration in muscle tissue using anti-CD163 monoclonal antibody was conducted on muscle biopsy specimens from 13 patients with PM and 17 with DM.Results.Serum levels of sCD163 were significantly increased in patients compared with healthy controls (p < 0.001). Patients with interstitial lung disease (ILD) had statistically higher sCD163 levels than patients without ILD (p < 0.001). High serum sCD163 levels were associated with increased incidence of antinuclear antibody (p < 0.05), higher serum levels of immunoglobulin G (p < 0.01) and immunoglobulin A (p < 0.05), and increased erythrocyte sedimentation rates (p < 0.01). Serum sCD163 levels were inversely correlated with CD3+ T cell counts in peripheral blood of patients (r = −0.306, p < 0.01). Cross-sectional assessment and longitudinal study revealed a significant correlation between serum sCD163 levels and disease activity. Patients with high serum sCD163 levels showed a higher incidence of CD163+ macrophage infiltration in muscle tissue than patients with normal sCD163 levels (chi-square value = 10.804, p < 0.01).Conclusion.Serum levels of sCD163 were significantly elevated and correlated with disease severity in patients with PM/DM, suggesting serum sCD163 as a promising biomarker in the disease evaluation of PM/DM. Our finding of elevated serum sCD163 levels associated with muscle macrophage infiltration highlights the role activated macrophage plays in the pathogenesis of PM/DM.

scholarly journals AB0596 PREDICTORS, LONG TERM CLINICAL AND TREATMENT OUTCOMES IN SOUTH ASIAN PATIENTS WITH IDIOPATHIC INFLAMMATORY MYOSITIS: A SINGLE CENTER STUDY

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1594.2-1595
Author(s):  
A. Nair ◽  
R. Goel ◽  
P. Chebbi ◽  
A. Mathew ◽  
A. Ganapati ◽  
...  
Keyword(s):  
Pericardial Effusion ◽  
Median Duration ◽  
Muscle Power ◽  
Disease Free Survival ◽  
Muscle Enzymes ◽  
Inflammatory Myositis ◽  
Idiopathic Inflammatory Myositis ◽  
Disease Free

Background:Idiopathic inflammatory myositis (IIM) are a heterogeneous group of immune-mediated disorders with varied presentations and multiple organ involvement. Data on long term outcome among South Asian patients with IIM is sparse.Objectives:To study the long term clinical outcome, treatment responses and factors predicting outcome among adult patients with IIMMethods:Patients diagnosed as ‘Idiopathic Inflammatory Myositis’ under the department of Clinical Immunology and Rheumatology at CMC, Vellore, India were screened retrospectively. Patients aged 18 years and above, satisfying Bohan and Peter criteria, having follow up of one year or more with atleast two outpatient or inpatient visits between January 2010 and April 2019 were included in this study. Those patients with connective tissue disease associated myositis were not included. Details on muscle weakness, extramuscular involvement, muscle enzymes and treatment administered were recorded at baseline, 3, 6, 12, 18, 24 months and yearly thereafter. After assessing their cumulative response, categorization of patients into complete and partial responders was done. Complete responders were defined as patients with persistent muscle power of more than 4/5 and/or MMT 8 more than 76/80, complete resolution of skin, articular and lung involvement (if any) as well as muscle enzymes less than twice the upper limit of normal without any documented flares during the entire follow up period. Patients not satisfying the said criterias were grouped as Partial responders. Disease free survival duration was also analyzed.Results:Out of 310 patients of IIM identified, 187 (60.3%) patients satisfied the inclusion criteria. Women were 2.2 times more than men and mean age at symptom onset was 35.7±12.6 years. Dermatomyositis was the predominant myositis subtype seen. All patients were put on steroids with the mean dose being 45.9 ± 18.6 mg/day. At baseline, the key immunosuppressants used were methotrexate in 44.9% and mycophenolate in 37.6% patients. The median follow up duration was 48 (25-80) months. An associated malignancy was diagnosed in 3.2% after a median duration of 24.5 months. Five patients expired after a median duration of 80 months from diagnosis. Normal muscle power was attained in 76.1% patients and 88.6% were vocational by the last follow up visit. Steroids were discontinued in 56.7% patients after a median duration of 24 months (p=0.0002). Discontinuation of the immunosuppressant was feasible in 10.2% patients after a median duration of 44 months. Assessment of the cumulative responses revealed a relapsing and remitting course in 45.9%. Outcome predictors in univariate analysis were Jo-1 status, presence of arthritis, interstitial lung disease and pericardial effusion at baseline. On multivariate analysis, absence of pericardial effusion (p=0.011) and interstitial lung disease (p=0.067) at baseline were found to be predictors of complete response. Disease free survival probability estimated at 5 years and 10 years was 91.6% and 72.4% respectively. Estimating the probability gender wise, males achieved disease free status earlier than females.Conclusion:A favorable clinical and functional outcome was seen in a significant proportion of these patients with IIM on long term follow up. Pericardial effusion and ILD were identified as predictors of poor clinical outcome.References:[1]Taborda AL, Azevedo P, Isenberg DA. Retrospective analysis of the outcome of patients with idiopathic inflammatory myopathy: a long-term follow-up study. Clin Exp Rheumatol. 2014 Apr; 32(2):188–93.Acknowledgments:NilDisclosure of Interests:None declared

High serum myostatin level suggests accelerated muscle senescence in active idiopathic inflammatory myositis

2021 ◽  
Vol 16 (3) ◽  
pp. 284
Author(s):  
Latika Gupta ◽  
AnamikaKumari Anuja ◽  
Danveer Bhadu ◽  
R Naveen ◽  
MantabyaKumar Singh ◽  
...  
Keyword(s):  
High Serum ◽  
Inflammatory Myositis ◽  
Idiopathic Inflammatory Myositis

scholarly journals Circulating Total and Active Metalloproteinase-9 and Tissue Inhibitor of Metalloproteinases-1 in Patients with Systemic Lupus Erythomatosus

2006 ◽  
Vol 2006 ◽  
pp. 1-7 ◽  
Author(s):  
Ewa Robak ◽  
Agnieszka Wierzbowska ◽  
Magdalena Chmiela ◽  
Liliana Kulczycka ◽  
Anna Sysa-Jędrejowska ◽  
...  
Keyword(s):  
Serum Levels ◽  
Tissue Inhibitor Of Metalloproteinase ◽  
Inactive Disease ◽  
Enzyme Linked Immunosorbent Assay ◽  
Control Group ◽  
Healthy Controls ◽  
Tissue Inhibitor ◽  
Positive Correlation ◽  
Healthy Control ◽  
Metalloproteinase 9

We investigated the serum concentration of total metalloproteinase-9 (tMPP-9), active MMP-9 (aMMP-9), and tissue inhibitor of metalloproteinase-1 (TIMP-1) in a group of 41 patients with SLE and 20 healthy controls. Serum levels of tMMP-9 and TIMP-1 were assessed by an enzyme-linked immunosorbent assay (ELISA) and aMMP-9 by fluorometric assay. The tMMP-9 level was lower in SLE patients (mean 262 ng/mL) than in healthy volunteers (mean 325 ng/mL) (P=.048). Similarly, aMMP-9 level was lower in SLE patients (mean 121 ng/mL) than in control group (mean 169 ng/mL) (P=.0355) and lower in active SLE (mean 54 ng/mL) than in inactive disease (mean 99 ng/mL) (P=.033). TIMP-1 level was also lower in SLE patients (mean 181 ng/mL) than in control group (mean 233 ng/mL) (P=.004). In SLE patients, a positive correlation was found between tMMP-9 and aMMP-9 (ρ=0.568;P=.001). We also found a positive correlation of tMMP-9 and TIMP-1 with VEGF concentrations (ρ=0.450,P=.005andρ=0.387;P=.018, resp). tMMP-9, aMMP-9, and TIMP-1 serum levels are lower in SLE patients than in healthy control group.

scholarly journals Raised TSH is associated with endothelial dysfunction in Metabolic Syndrome: A case control study

2017 ◽  
Vol 55 (4) ◽  
pp. 212-221 ◽  
Author(s):  
Ashok Kumar Ahirwar ◽  
Archana Singh ◽  
Anju Jain ◽  
Surajeet Kumar Patra ◽  
Binita Goswami ◽  
...  
Keyword(s):  
Metabolic Syndrome ◽  
Endothelial Dysfunction ◽  
Subclinical Hypothyroidism ◽  
Serum Insulin ◽  
Binary Logistic Regression ◽  
Binary Logistic Regression Analysis ◽  
Healthy Controls ◽  
Healthy Control ◽  
Study Population ◽  
Serum Tsh

AbstractIntroduction. Endothelial dysfunction has been considered as one of the important factors in pathogenesis of Metabolic Syndrome (Met S). Subclinical hypothyroidism (SCH) has also been reported to be associated with Met S. The aim of our study is to evaluate the association of raised TSH with mediators of endothelial dysfunction in Met S with Subclinical hypothyroidism as compared to healthy controls.Methods. Study population consisted of 100 subjects, out of which 50 were cases of Met S and 50 were healthy controls. Met S group were further divided into two, based on the presence & absence of SCH. Serum insulin, T3, T4, TSH were measured by chemiluminescence based immunoassay (CLIA). Serum nitric oxide (NO) levels were measured by Modified Griess’s method and serum endothelin-1 (ET-1) levels were measured by ELISA.Results. Out of 50 cases of Met S, SCH was diagnosed in 22. The mean serum TSH levels were significantly higher in Met S cases as compared to healthy controls (5.7 ± 1.2 μIU/mL vs. 2.3 ± 1.6 μIU/mL, P <0.0001). Mean serum NO levels were significantly lower in Met S cases as compared to healthy control (15.4 ± 10 μM vs. 21 ± 10 μM, p = 0.009). Mean serum ET-1 levels were significantly higher in Met S cases as compared to healthy controls (2.68 ± 1.7 fmol/mL vs. 2.1 ± 0.84 fmol/mL, p = 0.011). On Pearson’s correlation analysis, TSH showed positive correlation with ET-1 (r = 0.341, p = 0.001) and negative correlation with NO (r = −0.331, p = 0.001). Binary logistic regression analysis showed that TSH, NO and ET-1 has significant odd’s ratio for predicting Met S.Conclusion. Met S cases were screened for thyroid abnormalities and found to have 44% of SCH along with co-existing endothelial dysfunction. Raised TSH in SCH could cause endothelial dysfunction which may lead to Met S and associated co-morbidities. Present study gives new insight in linking endothelial dysfunction and raised TSH in Met S. Therefore, Met S cases should be screened for SCH and treated appropriately to attenuate endothelial dysfunction and associated comorbidities in Met S.

scholarly journals Immunoglobulin G4-related masses surrounding coronary arteries: a case report

2021 ◽  
Vol 5 (3) ◽  
Author(s):  
Takuya Nakamura ◽  
Yutaka Goryo ◽  
Takuya Isojima ◽  
Hiroyuki Kawata
Keyword(s):  
Coronary Artery ◽  
Coronary Angiography ◽  
Cardiovascular System ◽  
Coronary Arteries ◽  
Elevated Serum ◽  
High Serum ◽  
Immunoglobulin G4 ◽  
Coronary Arteritis ◽  
Serum Igg4 ◽  
Mass Formation

Abstract Background Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is an immune-mediated fibroinflammatory condition with high serum IgG4 levels affecting various organs, such as the pancreas, lacrimal and salivary glands, thyroid, kidney, and lung. Typical cardiovascular manifestations of IgG4-RD include periaortitis, coronary arteritis, and pericarditis. However, reports of IgG4-RD associated with coronary arteritis are rare. Here, we report a case of IgG4-related masses surrounding the coronary arteries. Case summary A 59-year-old man was referred to our hospital because of mediastinal masses detected by computed tomography (CT). Coronary CT angiography revealed masses surrounding the right coronary artery and the left anterior descending coronary artery. An elevated serum level of IgG4 and histological findings led to the diagnosis of IgG4-related coronary arteritis with mass formation. Coronary angiography showed numerous feeding arteries to the masses, which were demonstrated as multiple microchannels in the intravascular ultrasound (IVUS) images. Discussion IgG4-RD involving the cardiovascular system has been reported. However, coronary artery disease associated with IgG4-RD is very rare, and the mechanism of mass formation in IgG4-related coronary arteritis is unclear. In our case, within the cardiovascular system, IgG4-RD was limited to the coronary arteries, suggesting that the affected coronary arteries may provide the necessary blood supply to the mass, thus, aiding its growth. These findings were supported by the images from coronary angiography and IVUS.

SAT0323 MYOSITIS-SPECIFIC AND MYOSITIS-ASSOCIATED AUTOANTIBODIES IN A LARGE INDIAN COHORT OF INFLAMMATORY MYOSITIS REVEAL NOVEL CLINICO-PHENOTYPIC PATTERNS

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1107.1-1107
Author(s):  
L. Gupta ◽  
P. Gaur ◽  
V. Agarwal ◽  
R. Aggarwal ◽  
R. Misra
Keyword(s):  
Disease Duration ◽  
Unique Feature ◽  
Diagnostic Value ◽  
Immunofluorescence Assay ◽  
Major Influence ◽  
Antibody Prevalence ◽  
Research Support ◽  
Inflammatory Myositis ◽  
Idiopathic Inflammatory Myositis ◽  
Physician Diagnosis

Background:Idiopathic Inflammatory Myositis (IIM) are heterogenous, with distinct autoantibodies reflecting upon possible clinical evolution and outcomes. Ethnicity has major influence on both antibody prevalence patterns as well as phenotypic behaviours linked to them.Objectives:Thus we sought prevalence and co-existence of myositis specific autoantibodies (MSAs) and myositis associated autoantibodies (MAAs) and associated clinical characteristics in a large cohort of patients with IIM.Methods:Adult patients with a physician diagnosis of IIM as per ACR/EULAR classification criteria were investigated for the presence of MSAs/MAAs by Line immunoassay (G4, Euro-Immune, Lubeck, Germany). Anti-Nuclear Antibody (ANA) was tested by Immunofluorescence assay (IFA), and patterns in various antibody subsets explored. Prevalence and associations of different antibodies were assessed in disease subsets and clinical phenotypes.Results:MSA and MAAs were tested in 250 IIM patients (F:M 3.8:1) of median age 37 (25-47) and disease duration 6 (3-17) years. Dermatomyositis (DM) was seen in most patients 83 (33.2%) followed by overlap myositis (OM), juvenile DM, Anti-synthetase syndrome (ASS), polymyositis (PM), and cancer associated myositis (CAM). MSAs/MAAs were found in 148 (59.2%) of patients, of which 95 (64.2%) had an MSA and 53 (35.8%) had MAAs (Fig, 1A). 93 (62.8%) of autoantibody positive patients were positive for a single antibody, and only 2 (0.8%) of total had more than one MSA (Table 1).Table 1.Multiple antibodies positive upon testing for MSA and MAA by the LIANote: ** PL-7 co-exists with Ku + Pm/Scl, **PL-12 co-exists with Pm/Scl + Ro52, **SRP co-exists with Pm/Scl + Ro52The most frequently detected MSA was anti-Jo-1 (8%), with a further 9 specificities each found in 0.5–7.0% of patients. Amongst the autoantibody positive patients, 21% (n=53) had isolated MAA positivity, anti-Ro52 (33, 62.3%) being the most common, followed by anti-Pm/Scl (11, 20.8%) and anti-Ku (9, 17.0%) (Fig. 1B).Figure 1.A. MSA and MAA in Indian cohort of myositis B. ANA patterns in myositis C. MSA in ANA negative IIMOn ANA, 76.0% (172 of 226) were positive, with speckled being the most common pattern (37%,Fig. 1C). Of those ANA negative (n=54), 61% had either MSA or MAA (Fig 1D). 18 (54.6%) had autoantibodies associated with cytoplasmic patterns suggesting that cytoplasmic ANA may be underreported.Clinical presentation akin to DM was seen with all MSA except anti-SRP. PM group was heterogenous, and included ASS, OM and necrotizing phenotype (Fig. 2A). On occasion, anti-SRP, anti-Mi-2 and anti-MDA5 presented with clinical phenotype of ASS. (Fig 2A,C). Patients with ARS or anti-SAE were often clinically amyopathic (Figure 2B,C)Figure 2.A. Phenotypic associated with various antibody subsets B,C,and D. MSA/MAA in muscle weakness, rash and ILD phenotype. E. Unique feature of eye-lid edema in some patients with MDA-5 positive myositisARS were associated with mechanic’s hand (p<0.0001,OR 7.6), ILD (p<0.0001,OR 4.4), and arthritis (p=.002,OR 2.6) though there was no difference between Jo-1 and non-Jo-1 ASS. Anti-MDA-5 associated with fever (p=0.003,OR 12) and weight loss (p=0.008,OR 10.2) and unique phenotype of eye-lid edema in some adults (Figure 2E) and arthritis in children (p=0.01, OR 11.5). Anti-TIF-1ɣ associated with alopecia (p=0.007,OR 5.9) and malignancy (p= <0.0001,OR 34) in adults but not children.Conclusion:Myositis autoantibodies are seen in two-thirds IIM and identify distinct clinical subsets as well as unique phenotypes. MSA/MAA are positive in two-thirds of those negative on ANA, adding diagnostic value. MSAs are nearly always mutually exclusive and thus useful as biomarkers for diagnosis.Acknowledgments:MSA testing supported by grants from APLAR and Association of Physicians of India.Disclosure of Interests:Latika Gupta: None declared, Priyanka Gaur: None declared, Vikas Agarwal: None declared, Rohit Aggarwal Grant/research support from: Pfizer, Genentech, BMS, Mallinckrodt, Consultant of: Pfizer, Genentech, BMS, Mallinckrodt, Bristol Myers-Squibb, octapharma, CSL Behring, AstraZeneca, Corbus, Kezar, Abbvie, Ramnath Misra: None declared

Sign in / Sign up

Export Citation Format

Share Document