scholarly journals Clinical Characteristics and Management of Patients With Clinical Amyopathic Dermatomyositis: A Retrospective Study of 64 Patients at a Tertiary Dermatology Department

2021 ◽  
Vol 8 ◽  
Author(s):  
Keyun Tang ◽  
Hanlin Zhang ◽  
Hongzhong Jin
Keyword(s):  
Muscle Weakness ◽  
Inflammatory Markers ◽  
English Literature ◽  
Initial Visit ◽  
Amyopathic Dermatomyositis ◽  
Dermatology Department ◽  
Inflammatory Myositis ◽  
Idiopathic Inflammatory Myositis ◽  
Acr Criteria ◽  
Systemic Treatments

Background: Clinical amyopathic dermatomyositis (CADM) represents a subtype of 5–20% of patients with dermatomyositis (DM), which can be categorized into amyopathic dermatomyositis (ADM) and hypomyopathic dermatomyositis (HDM). The characteristics of patients with CADM are still limited in English literature.Objective: To investigate clinical features, cutaneous findings, diagnostic accuracy, and treatment regimen of CADM patients.Methods: Sixty-four patients diagnosed with CADM at Peking Union Medical College Hospital by dermatologists were retrospectively analyzed. Data were recorded in the electronic database at each offline clinical consultation and directly extracted from medical records. 2017 EULAR/ACR criteria for idiopathic inflammatory myositis (IIM) classification was used to identify and classify patients with CADM. Published studies were searched to extract relevant data of CADM patients.Results: This cohort included 38 ADM patients and 26 HDM patients. 2017 EULAR/ACR criteria classified 67.2% of patients with CADM into probable or definite DM. Antimalarials were given to a majority of CADM patients (72.6%, n = 45). However, 68.8% (31 out of 45) required at least one aggressive agent combined with hydroxychloroquine due to insufficient response or side effects. The median of systemic treatments in HDM was significantly higher than ADM (p = 0.007). The number of ADM patients using antimalarials as monotherapy was significantly higher than that of HDM patients (p = 0.031), while the number of HDM patients receiving steroids combined with immunosuppressants was significantly higher (p = 0.025). The median of Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI) score improvement was 11.5 and 10.5 for ADM and HDM after a median follow-up of 31.5 and 32.5 months, respectively. Six patients with normal muscle strength developed muscle weakness after a median of 10.5 months (IQR 9-13), and elevated inflammatory markers at initial visit might indicate their muscle weakness development.Conclusions: 32.8% of patients may be overlooked using the three skin variables of 2017 EULAR/ACR criteria. The response rate to single hydroxychloroquine in our cohort was 68.8%. Detailed treatment modalities were different among ADM and HDM. Long-term monitoring for the development of myositis in patients with CADM, especially those with elevated inflammatory markers at initial visit, may be warranted.

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

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

scholarly journals Paget’s Disease of the Breast in a Patient with Amyopathic Dermatomyositis

2012 ◽  
Vol 2012 ◽  
pp. 1-4 ◽  
Author(s):  
Sirin Yasar ◽  
Gunay Gurleyik ◽  
Yesim Sabuncuoglu ◽  
Ali Aktekin ◽  
Bulent Yasar ◽  
...  
Keyword(s):  
Rare Case ◽  
Paget’S Disease ◽  
Paget's Disease ◽  
Lymph Node Biopsy ◽  
Amyopathic Dermatomyositis ◽  
Dermatology Department ◽  
Node Biopsy ◽  
Areola Complex ◽  
Underlying Malignancy ◽  
Recurrent Malignancy

Amyopathic dermatomyositis (AD) can be a part of paraneoplastic syndrome of an underlying malignancy. Paget’s disease is a rare form of breast cancer. We present a very rare case of Paget’s disease associated with AD. Paget’s disease has been diagnosed in a patient with AD who is under surveillance of dermatology department. The patient has undergone central lumpectomy with removal of the nipple-areola complex and sentinel lymph node biopsy. Surgical margins after lumpectomy and sentinel node biopsy were negative. The whole breast irradiation was performed after surgery. The patient receives medical treatment for AD of which lesions regressed in 1 year during the follow-up period. This is a very rare case of Paget’s disease diagnosed in a patient with AD. Female patients with dermatomyositis have been absolutely recommended to undergo screening for breast and gynaecological malignancies. AD may be an early finding of primary or recurrent malignancy of the breast.

scholarly journals Changes in antibody titres in patients with anti-transcription intermediary factor 1-gamma antibody-positive idiopathic inflammatory myositis

Rheumatology ◽  
2018 ◽  
Vol 58 (1) ◽  
pp. 179-181 ◽  
Author(s):  
Yoshiyuki Abe ◽  
Taiki Ando ◽  
Masakazu Matsushita ◽  
Kurisu Tada ◽  
Ken Yamaji ◽  
...  
Keyword(s):  
Inflammatory Myositis ◽  
Idiopathic Inflammatory Myositis ◽  
Antibody Titres

scholarly journals P8 To study the demographic and clinical characteristics of in-patients with idiopathic inflammatory myositis

2008 ◽  
Vol 3 (3) ◽  
pp. S34
Author(s):  
J Thomas ◽  
NV Jayachandran ◽  
PKS Chandrasekhara ◽  
S Kanchinadham ◽  
JK Kadel ◽  
...  
Keyword(s):  
Clinical Characteristics ◽  
Inflammatory Myositis ◽  
Idiopathic Inflammatory Myositis

scholarly journals Juvenile dermatomyositis - A case report with review on oral manifestations and oral health considerations

2018 ◽  
Vol 44 (1) ◽  
pp. 52-61
Author(s):  
Pritesh Ruparelia ◽  
Oshin Verma ◽  
Vrutti Shah ◽  
Krishna Shah
Keyword(s):  
Oral Health ◽  
Muscle Weakness ◽  
Juvenile Dermatomyositis ◽  
Oral Manifestations ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Inflammatory Myositis ◽  
Life Threatening

Juvenile Dermatomyositis is the most common inflammatory myositis in children, distinguished by proximal muscle weakness, a characteristic rash and Gottron’s papules. The oral lesions most commonly manifest as diffuse stomatitis and pharyngitis with halitosis. We report a case of an 8 year old male with proximal muscle weakness of all four limbs, rash, Gottron’s papules and oral manifestations. Oral health professionals must be aware of the extraoral and intraoral findings of this rare, but potentially life threatening autoimmune disease of childhood, for early diagnosis, treatment, prevention of long-term complications and to improve the prognosis and hence, the quality of life for the patient.

scholarly journals Identifying novel B-cell targets for chronic inflammatory autoimmune disease by screening of chemical probes in a patient-derived cell assay

2020 ◽  
Author(s):  
Yvonne Sundström ◽  
Ming-Mei Shang ◽  
Sudeepta Kumar Panda ◽  
Caroline Grönwall ◽  
Fredrik Wermeling ◽  
...  
Keyword(s):  
Autoimmune Disease ◽  
B Cell ◽  
Lupus Erythematosus ◽  
Chemical Probes ◽  
Cell Secretion ◽  
Protein Targets ◽  
Inflammatory Myositis ◽  
Idiopathic Inflammatory Myositis ◽  
Cell Functions ◽  
Future Drug

SUMMARYB-cell secretion of autoantibodies drives autoimmune diseases, including systemic lupus erythematosus and idiopathic inflammatory myositis. Few therapies are presently available for treatment of these patients, often resulting in unsatisfactory effects and helping only some of patients. We developed a screening assay for evaluation of novel targets suspending B-cell maturation into antibody secreting cells, which could contribute to future drug development. The assay was employed for testing 43 high quality chemical probes and compounds inhibiting under-explored protein targets, using primary cells from patients with autoimmune disease. Probes inhibiting bromodomain family proteins and histone methyl transferases demonstrated abrogation of B-cell functions to a degree comparable to a positive control, the JAK inhibitor tofacitinib. Inhibition of each target rendered a specific functional cell and potential disease modifying effect, indicating specific epigenetic protein targets as potential new intervention points for future drug discovery and development efforts.

Effect of Cotreatment with Corticosteroids in Connective Tissue Related Lung Disease (CTD-ILD) Patients on Mycophenolate Mofetil (MMF)

2021 ◽  
Author(s):  
Roberto Caricchio ◽  
Erin R Narewski ◽  
Ryan Townsend ◽  
Stephen Codella ◽  
Jin Sun Kim ◽  
...  
Keyword(s):  
Mycophenolate Mofetil ◽  
Connective Tissue ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Connective Tissue Disease ◽  
Pulmonary Function Tests ◽  
Usual Interstitial Pneumonia ◽  
University Hospital ◽  
Inflammatory Myositis ◽  
Idiopathic Inflammatory Myositis

Abstract Introduction: Connective Tissue Disease Related Interstitial Lung Disease (CTD-ILD) is often treated with immunosuppressant medications; common among these is Mycophenolate Mofetil (MMF). We hypothesized that co-treatment with corticosteroids would impact disease progression.Methods: We examined a consecutive cohort of CTD-ILD patients followed at Temple University Hospital in Philadelphia, PA since 2015 who had pulmonary function tests (PFTs) performed by American Thoracic Society (ATS)/European Respiratory Society (ERS) Criteria at least one year apart. All patients were treated for CTD-ILD with MMF used either as sole therapy or as combination therapy with prednisone. Univariate logistic analyses were performed revealing the odds ratio (OR) for improvement or worsening of several PFT values (including forced vital capacity (FVC), diffusion capacity of carbon monoxide (DLCO), and six-minute walk (6MW)) greater than the minimal clinically important difference (MCID) for each value.Results: We included 103 patients (74 women) with an average age of 60 ± 11 years, 49% of our cohort were current or former smokers, and mean BMI was 29 ± 7 kg/m2. Patients were observed on treatment for an average of 23 months. CTD distribution included 25% mixed connective tissue disease (MCTD), 24% systemic sclerosis (SSc), 17% rheumatoid arthritis (RA), 14% systemic lupus erythematosus (SLE), 10% other idiopathic inflammatory myositis (IIM) syndromes, 7% Antisynthetase Syndrome, 5% Sjӧgren’s syndrome. Non-specific interstitial pneumonia (NSIP) was the majority (45%) ILD pattern noted, Usual Interstitial Pneumonia (UIP) 35%, and other types were less prevalent (20%). The majority of patients received corticosteroids as co-treatment with MMF (75 patients (72%)) with a mean daily dose of 15 ± 16 mg of prednisone. Mean daily MMF dose was 1144 ± 675 mg. Glucocorticoid treatment was not associated with significant improvements in PFT values, including FVC, DLCO, and 6MW distance walked.Conclusion: In this small cohort, patients with CTD-ILD receiving MMF did not demonstrate improved lung function when receiving co-treatment with corticosteroids, but larger prospective studies are needed to better elucidate the effect of corticosteroids on this vulnerable group of patients.

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