The spectrum of idiopathic inflammatory myopathies in South Africa
ConclusionThe spectrum of IIM in our cohort of mainly African blacks is similar to other studies, with OM being the most frequent subtype.Key Points• As there is limited information on idiopathic inflammatory myopathies (IIM) in sub-Saharan Africa, this study reports the spectrum of IIM in a South African cohort of predominantly African blacks.• Our patients were younger at diagnosis, and overlap myositis was the most common phenotype.• Comparisons with other studies show similarities in the manifestations of IIM. (Source: Clinical Rheumatology)
Source: Clinical Rheumatology - March 24, 2020 Category: Rheumatology Source Type: research

Inpatient Burden and Resource Utilization of Polymyositis and Dermatomyositis: A 10-year Study of National Inpatient Sample
Publication date: Available online 14 March 2020Source: Joint Bone SpineAuthor(s): Patompong Ungprasert, Thapat Wannarong, Wisit Cheungpasitporn, Karn Wijarnpreecha, Charat Thongprayoon, Paul T Kroner (Source: Joint Bone Spine)
Source: Joint Bone Spine - March 15, 2020 Category: Orthopaedics Source Type: research

Reduced miR-146a Promotes REG3A Expression and Macrophage Migration in Polymyositis and Dermatomyositis
Conclusions: Reduced miR-146a expression in PM/DM leads to increased REG3A expression that increases inflammatory macrophage migration, which may be a possible underlying mechanism of DM/PM pathogenesis. (Source: Frontiers in Immunology)
Source: Frontiers in Immunology - February 21, 2020 Category: Allergy & Immunology Source Type: research

Clinical and histological features of immune-mediated necrotising myopathy: a multi-centre South Australian cohort study
The idiopathic inflammatory myopathies (IIMs) are a group of systemic autoimmune diseases characterised primarily by muscle inflammation but also potentially accompanied by a range of extra-muscular manifestations. Dermatomyositis (DM), polymyositis (PM) and inclusion body myositis (IBM) constitute well-characterised subtypes of IIM, with the entity of non-specific idiopathic inflammatory myopathy (NSIIM) being more recently described [1]. Whilst these IIM subtypes are distinguished on clinical, serological and histological grounds, they are unified by the presence of a typically prominent intramuscular lymphocytic infiltr...
Source: Neuromuscular Disorders - February 18, 2020 Category: Neurology Authors: Jessica Day, Sophia Otto, Kathy Cash, Vidya Limaye Source Type: research

Targeted therapies in systemic sclerosis, myositis, antiphospholipid syndrome, and Sjögren's syndrome
Publication date: Available online 15 February 2020Source: Best Practice & Research Clinical RheumatologyAuthor(s): Lucas L. van den Hoogen, Jacob M. van LaarAbstractTargeted therapies using biological disease-modifying antirheumatic drugs (bDMARDs) and small molecule synthetic drugs have revolutionized rheumatological practice. Initially developed for the treatment of immune arthritis (rheumatoid arthritis, psoriatic arthritis, and spondylarthritis), both bDMARDs and small molecule synthetic drugs are now increasingly entering the space of connective tissue disease (CTD) treatment. Recent clinical trial data in system...
Source: Best Practice and Research Clinical Rheumatology - February 15, 2020 Category: Rheumatology Source Type: research

Muscle involvement in myasthenia gravis: Expanding the clinical spectrum of myasthenia-myositis association from a large cohort of patients
In conclusion we redefined the clinical spectrum of muscle involvement in MG-IM association, which represent a continuum among 3 main clinical groups: distal, proximal and subclinical muscle involvement. Minimal muscle involvement and focal myositis could be underestimated among myasthenic patients and early aggressive immunotherapy could be required in focal group.Graphical abstract (Source: Autoimmunity Reviews)
Source: Autoimmunity Reviews - February 15, 2020 Category: Allergy & Immunology Source Type: research

Incidence of different cancer types in dermatomyositis, polymyositis and dermatopolymyositis: results of a registry analysis.
Abstract Studies confirming Dermatomyositis/polymyositis/dermatopolymyositis (DM/PM/DPM) as a paraneoplastic disease have found cancer incidence rates elevated at the time point of diagnosis and remaining elevated over time. Guidelines promote cancer screening in newly diagnosed myositis patients. Data on the incidence of cancer types in DM/PM patients are insufficient to guide targeted screening. PMID: 32060906 [PubMed - as supplied by publisher] (Source: The British Journal of Dermatology)
Source: The British Journal of Dermatology - February 14, 2020 Category: Dermatology Authors: Zidane M, Dressler C, Nast A, Egeberg A Tags: Br J Dermatol Source Type: research

Comparison of cytokine profiles between anti-ARS antibody-positive interstitial lung diseases and those with anti-MDA-5 antibodies
ConclusionsAnti-MDA-5-positive ADM-ILD had poorer survival than anti-ARS-ILD. IP-10 seems to be most deeply involved in the pathophysiology of anti-MDA-5-associated ADM-ILD.Key Points•To clarify differences in the inflammatory and immunological features of anti-MDA-5-positive ADM-ILD and anti-ARS-ILD, we performed an observational study to measure serum cytokine concentrations before treatment using a multiplex immunoassay system.•Multivariate logistic regression models revealed that IP-10 was associated with the most significant relative risk for ADM-ILD with anti-MDA-5 antibodies.•Levels of IP-10 were elev...
Source: Clinical Rheumatology - February 13, 2020 Category: Rheumatology Source Type: research

HTLV-1 Infection and Rheumatic Diseases
Some major research and clinical questions about human T-cell leukemia virus type 1 (HTLV-1) infection and rheumatic diseases remain: (1) Does HTLV-1 infection cause rheumatic diseases? (2) Do patients with rheumatic diseases display different responses to treatment with anti-rheumatic agents when they are HTLV-1 carriers? (3) Is adult T-cell leukemia/lymphoma (ATL) or HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP) more prevalent in HTLV-1 carriers with rheumatic diseases who are treated with anti-rheumatic agents? These questions are important because increasing numbers of patients with rheumatic dise...
Source: Frontiers in Microbiology - February 11, 2020 Category: Microbiology Source Type: research

Inflammatory muscle disease – An update
Publication date: Available online 8 February 2020Source: Best Practice & Research Clinical RheumatologyAuthor(s): Sara Baig, Julie J. PaikAbstractIdiopathic inflammatory myopathies (IIM) are a heterogeneous group of inflammatory myopathies whose common feature is immune-mediated muscle injury. There are distinct subgroups including dermatomyositis (DM), polymyositis (PM), inclusion body myositis, and immune-mediated necrotizing myopathy. Antisynthetase syndrome is also emerging as a distinct subgroup with its unique muscle histopathological characteristic of perifascicular necrosis. While the newly updated EULAR/ACR C...
Source: Best Practice and Research Clinical Rheumatology - February 9, 2020 Category: Rheumatology Source Type: research

Recovering autonomy is a key advantage of home-based immunoglobulin therapy in patients with myositis: A qualitative research study
Immunoglobulins are 2nd or 3rd-line treatments in dermatomyositis (DM) or polymyositis (PM) refractory to high-dose corticosteroids and immunosuppressants. Immunoglobulins (2 g/kg/mo) are usually administered intravenously (IVIg) once a month and the patients stay at hospital for a few days. Recently, subcutaneous injections (SCIg) were proposed 2 to 3 times per week, in some dysimmune diseases. SCIg are administered at home preferably by the patient or by a nurse. We investigated the needs and attitudes of DM and PM patients with experience of IVIg and SCIg. Seven patients (6 PM and 1 DM) from a single center participa...
Source: Medicine - February 1, 2020 Category: Internal Medicine Tags: Research Article: Quality Improvement Study Source Type: research

Surfactant protein D is associated with 3-month mortality of anti-MDA5 antibody-interstitial lung disease.
Authors: Lyu W, Zhou Y, Zhuang Y, Liu Y, Cao M, Xin X, Wu H, Wang J, Meng F, Cai H, Dai J Abstract OBJECTIVES: To investigate the associations between serum levels of matrix metalloproteinase 7 (MMP7), surfactant protein D (SPD), interleukin 18 (IL-18) and chemokine ligand 18 (CCL18) with dermatomyositis and polymyositis-associated interstitial lung disease (DM/PM-ILD) and evaluate their prognostic values in the disease. METHODS: Seventy-eight patients with multiple disciplinary team diagnosis of DM/PM-ILD were enrolled and classified as anti-melanoma differentiation-associated protein 5 antibody (MDA5)-ILD, an...
Source: Clinical and Experimental Rheumatology - January 30, 2020 Category: Rheumatology Tags: Clin Exp Rheumatol Source Type: research

18F-FDG PET/CT in Graft Versus Host Disease–Associated Polymyositis
We present a case of a 38-year-old man with a known case of mixed-phenotype acute leukemia after hematopoietic stem cell transplant presenting with GVHD-associated polymyositis. 18F-FDG PET/CT done in this case not only helped in the detection of the muscle involvement but also helped in obtaining precise muscle sample for histopathological diagnosis using PET-guided biopsy. (Source: Clinical Nuclear Medicine)
Source: Clinical Nuclear Medicine - January 8, 2020 Category: Nuclear Medicine Tags: Interesting Images Source Type: research

18F-FDG PET/CT in a Patient With Thymoma-Associated Paraneoplastic Polymyositis
A 43-year-old woman presented with progressive muscle weakness and myalgia for 1 month. Laboratory tests revealed the elevation of myogenic enzymes. MRI of the thighs demonstrated a pattern of edema for inflammatory myopathy. Electromyography showed diffuse myopathic disorder, and muscle biopsy indicated polymyositis. 18F-FDG PET/CT was performed to investigate the potential paraneoplastic syndrome and revealed a soft tissue lesion with 18F-FDG avidity in the right anterior mediastinum, as well as diffusely increased activity throughout the body muscles. The patient's symptoms and biochemical abnormalities were gradually r...
Source: Clinical Nuclear Medicine - January 8, 2020 Category: Nuclear Medicine Tags: Interesting Images Source Type: research

Mortality of Chinese patients with polymyositis and dermatomyositis
ConclusionsMortality of PM/DM patients in China is substantial, especially in females, and those with ILD, malignancy or infection. Infection was the leading cause of death. Patients with older age at onset, infection, ILD, and malignancy need to be paid more attention.Key Points•This is the first comprehensive report about the mortality situation with a large population in PM/DM patients in China including SMR, YLL, and cause of death, Kaplan-Meier survival analysis and Cox regression analysis for mortality risk factors.•The specific SMRs for PM/DM patients with malignancy and interstitial lung disease were also...
Source: Clinical Rheumatology - January 4, 2020 Category: Rheumatology Source Type: research

Polymyositis has minimal effect on primary total knee or hip arthroplasty outcomes
AbstractTo assess whether polymyositis is associated with more complications and higher healthcare utilization after total knee or hip arthroplasty (TKA/THA). Using the 1998 –2014 U.S. National Inpatient Sample data, we performed multivariable-adjusted logistic regression analyses to assess the association of polymyositis with in-hospital complications (implant infection, revision, transfusion, mortality) and healthcare utilization (hospital charges, length of hospita l stay, discharge to non-home setting), controlling for important covariates and confounders. Sensitivity analyses additionally adjusted the main model...
Source: Clinical Rheumatology - January 2, 2020 Category: Rheumatology Source Type: research

Chronic-graft-versus-host-disease-related polymyositis: a 17-months-old child with a rare and late complication of haematopoietic stem cell transplantation.
Conclusions:This is the first case of cGVHD-related-polymyositis described in a pediatric patient which was successfully treated with rituximab. (Source: Mediterranean Journal of Hematology and Infectious Diseases)
Source: Mediterranean Journal of Hematology and Infectious Diseases - December 31, 2019 Category: Hematology Source Type: research

Reclassification of Korean patients with polymyositis and dermatomyositis based on the Bohan and Peter criteria by the 2017 European League Against Rheumatism/American College of Rheumatology classification criteria for adult and juvenile idiopathic inflammatory myopathies.
Conclusions: The Bohan and Peter criteria were comparable to the 2017 EULAR/ ACR criteria for classifying PM and DM in Korean patients. Considering the convenience of the Bohan and Peter criteria in the real clinical settings, we suggest that the old criteria should be preferentially applied and then performing muscle biopsy should be considered in a patient suspected of PM without antihistidyl tRNA synthetase (anti-Jo-1). Moreover, we suggest that ADM could also clinically be classified by the old criteria. PMID: 31875667 [PubMed - as supplied by publisher] (Source: The Korean Journal of Internal Medicine)
Source: The Korean Journal of Internal Medicine - December 28, 2019 Category: Internal Medicine Authors: Yoo J, Ahn SS, Jung SM, Song JJ, Park YB, Lee SW Tags: Korean J Intern Med Source Type: research

Interstitial pneumonia with autoimmune features: A single center prospective follow-up study
ConclusionsIPAF seems to a distinct entity, with a low tendency to evolve in a definite CTD. Nevertheless, further studies are needed to better define the clinical evolution and the outcome of IPAF. (Source: Autoimmunity Reviews)
Source: Autoimmunity Reviews - December 13, 2019 Category: Allergy & Immunology Source Type: research

Differential and quantitative neuroimaging characteristics of inclusion body myositis
Publication date: Available online 12 December 2019Source: Journal of Clinical NeuroscienceAuthor(s): Minori Furuta, Natsumi Furuta, Kazuaki Nagashima, Yukio Fujita, Yoshito Tsushima, Yoshio IkedaAbstractIn clinical settings, it is often difficult to distinguish inclusion body myositis (IBM) from other neuromuscular diseases. In order to clarify clinically useful characteristics for making the differential diagnosis of IBM, we performed clinical, epidemiological, and neuroimaging analyses in patients with various types of neuromuscular disorders. We enrolled 333 patients with myopathy and 12 patients with amyotrophic later...
Source: Journal of Clinical Neuroscience - December 13, 2019 Category: Neuroscience Source Type: research

Human Trichinosis mimicking polymyositis
(Source: International Journal of Infectious Diseases)
Source: International Journal of Infectious Diseases - December 13, 2019 Category: Infectious Diseases Authors: Laura Nuzzolo-Shihadeh, Adri án Camacho-Ortiz, Daniel Villarreal-Salinas, Alvaro Barbosa-Quintana, Rita Pineda-Sic, Eduardo Perez-Alba Tags: Medical Imagery Source Type: research

Neutrophil extracellular traps activate lung fibroblast to induce polymyositis-related interstitial lung diseases via TLR9-miR-7-Smad2 pathway.
In this study, we found that NET accelerated the progression of ILD and promoted pulmonary fibrosis (PF) in vivo. miR-7 expression was down-regulated in lung tissue of PM group than control group, and NETs further decreased miR-7 expression. TLR9 and Smad2 were up-regulated in lung tissue of PM group than control group, and NETs further increased TLR9 and Smad2 expressions. In vitro experiments showed that PMA-treated NETs accelerated the proliferation of LF and their differentiation into myofibroblast (MF), whereas DNase I decreased the promotion effect of NETs. Neutrophil extracellular trap components myeloperoxidase (MP...
Source: J Cell Mol Med - December 10, 2019 Category: Molecular Biology Authors: Zhang S, Jia X, Zhang Q, Zhang L, Yang J, Hu C, Shi J, Jiang X, Lu J, Shen H Tags: J Cell Mol Med Source Type: research

Strength training and aerobic exercise training for muscle disease.
CONCLUSIONS: The evidence regarding strength training and aerobic exercise interventions remains uncertain. Evidence suggests that strength training alone may have little or no effect, and that aerobic exercise training alone may lead to a possible improvement in aerobic capacity, but only for participants with FSHD. For combined aerobic exercise and strength training, there may be slight increases in muscle strength and aerobic capacity for people with dermatomyositis and polymyositis, and a slight decrease in aerobic capacity and increase in muscle strength for people with juvenile dermatomyositis. More research with rob...
Source: Cochrane Database of Systematic Reviews - December 6, 2019 Category: General Medicine Authors: Voet NB, van der Kooi EL, van Engelen BG, Geurts AC Tags: Cochrane Database Syst Rev Source Type: research

Tofacitinib for the Treatment of Refractory Polymyositis
No abstract available (Source: JCR: Journal of Clinical Rheumatology)
Source: JCR: Journal of Clinical Rheumatology - November 26, 2019 Category: Rheumatology Tags: Letters to the Editor Source Type: research

PET Scan: Nuclear Medicine Imaging in Myositis
AbstractPurpose of ReviewPositron emission tomography (PET) combined with computed tomography (CT) has proven useful as a cancer screening technique in patients with inflammatory myopathy, mainly dermatomyositis. In this review, we focus on advances in this direction and other potential applications of PET/CT in patients with inflammatory myopathy.Recent FindingsCancer screening by PET/CT seems suitable and cost-effective in patients with myositis. It has also shown value as a hybrid technique for diagnosing myositis versus controls and could be of interest for differentiating between polymyositis and sporadic inclusion bo...
Source: Current Rheumatology Reports - November 21, 2019 Category: Rheumatology Source Type: research

Disease patterns in connective tissue disease-associated interstitial lung disease: a population based study
Conclusions: A minority of patients with CTD had a diagnosis of ILD, but the presence of ILD was associated with significant excess mortality comparable to ILD without CTD. (Source: European Respiratory Journal)
Source: European Respiratory Journal - November 21, 2019 Category: Respiratory Medicine Authors: Hyldgaard, C., Bendstrup, E., Pedersen, A. B., Pedersen, L., Ellingsen, T. Tags: Rare ILD/DPLD Source Type: research

Clinical characteristics of anti-Jo-1-positive interstitial lung disease
Anti-Jo-1-syndrome is the most common form of antisynthetase syndrome, which often presents with interstitial lung disease (ILD) and polymyositis/dermatomyositis(PM/DM). Little is understood how the presence of PM/DM affects the clinical presentation of ILD patients with anti-Jo-1-antibody (Jo-1-ILD).We aim to characterize Jo-1-ILD by comparing patients with PM/DM (PM/DM-ILD) and without PM/DM (non-PM/DM-ILD).In this retrospective review, 49 Jo-1-ILD patients diagnosed at our institution from January 2005 to June 2018 were included. Clinical, radiologic, and histologic features of 20 PM/DM-ILD and 29 non-PM/DM-ILD patients...
Source: European Respiratory Journal - November 21, 2019 Category: Respiratory Medicine Authors: Chang, S., Shin, M. H., Shin, J. H., Park, M. S. Tags: ILD/DPLD of known origin Source Type: research

Drop Head Syndrome as a Rare Complication in Mixed Connective Tissue Disease: A Case Report.
Authors: Akagi M, Umeda M, Hashisako M, Hara K, Tsuji S, Endo Y, Takatani A, Shimizu T, Fukui S, Koga T, Kawashiri SY, Iwamoto N, Igawa T, Ichinose K, Tamai M, Nakamura H, Origuchi T, Niino D, Kawakami A Abstract A 54-year-old woman developed drop head syndrome (DHS), Raynaud's phenomenon and CK elevation. She did not meet the international classification criteria of dermatomyositis/polymyositis, as we observed no muscle weakness, grasping pain or electromyography abnormality in her limbs, and anti-ARS antibody was negative. Cervical magnetic resonance imaging and a muscle biopsy of the trapezius muscle revealed my...
Source: Internal Medicine - November 20, 2019 Category: Internal Medicine Tags: Intern Med Source Type: research

A case of overlap syndrome (scleroderma and polymyositis) associated with the development of sudden chest pain due to myocardial damage.
We report a rare case of overlap syndrome (SSc and polymyositis) who presented with sudden chest pain secondary to myocardial fibrosis. Although the etiology of chest symptoms in collagen disease was difficult to identify, cardiac magnetic resonance imaging (MRI) revealed not myocarditis but myocardial fibrosis in our case. Synthetic judgement of serum brain natriuretic peptide/ troponin T levels and cardiac MRI is useful in the search for the cause of chest symptoms even in patients with collagen diseases. PMID: 31723102 [PubMed - in process] (Source: Drug Discoveries and Therapeutics)
Source: Drug Discoveries and Therapeutics - November 16, 2019 Category: Drugs & Pharmacology Tags: Drug Discov Ther Source Type: research

Colonic Perforation as Initial Presentation of Amyloid Disease: Case Report and Literature Review
We present a case of primary amyloidosis of the colon in a patient affected by polymyositis who underwent Hartmann ’s procedure after a spontaneous colonic perforation. After 2 months of well-being, the patient underwent two consecutive surgical procedures for stenosis of the ostomy orifice.Areas CoveredA review of the literature has been performed, gathering case reports highlighting the distribution of this disease by age, gender, location, and treatment when available.Expert CommentaryGastrointestinal amyloid disease is a rare condition, and it could be considered among the rare causes of intestinal perforati...
Source: Digestive Diseases and Sciences - November 14, 2019 Category: Gastroenterology Source Type: research

M406 what came first the immunodeficiency or immunosuppression
64 year old male with polymyositis previously treated with rituximab 5 years prior presented with hypogammaglobulinemia. Polymyositis was poorly controlled and the patient needed clearance prior to restarting rituximab. The patient had no significant infection history. Screening laboratories included a low IgG of 610, low IgM of 31 and protection to 4/14 post vaccination pneumococcal titers. (Source: Annals of Allergy, Asthma and Immunology)
Source: Annals of Allergy, Asthma and Immunology - November 1, 2019 Category: Allergy & Immunology Authors: M. Karasov, J. Carlson Source Type: research

Genes, Vol. 10, Pages 864: Meta-Analysis of Polymyositis and Dermatomyositis Microarray Data Reveals Novel Genetic Biomarkers
In this study, three gene expression datasets (PM: 7, DM: 50, Control: 13) available in public databases were used to conduct meta-analysis. We then conducted expression quantitative trait loci analysis to detect the variant sites that may contribute to the pathogenesis of PM and DM. Six-hundred differentially expressed genes were identified in the meta-analysis (false discovery rate (FDR) < 0.01), among which 317 genes were up-regulated and 283 were down-regulated in the disease group compared with those in the healthy control group. The up-regulated genes were significantly enriched in interferon-signaling pat...
Source: Genes - October 30, 2019 Category: Genetics & Stem Cells Authors: Jaeseung Song Daeun Kim Juyeon Hong Go Woon Kim Junghyun Jung Sejin Park Hee Jung Park Jong Wha J. Joo Wonhee Jang Tags: Article Source Type: research

Texture analysis of sonographic muscle images can distinguish myopathic conditions.
Authors: Nodera H, Sogawa K, Takamatsu N, Hashiguchi S, Saito M, Mori A, Osaki Y, Izumi Y, Kaji R Abstract Given the recent technological advent of muscle ultrasound (US), classification of various myopathic conditions could be possible, especially by mathematical analysis of muscular fine structure called texture analysis. We prospectively enrolled patients with three neuromuscular conditions and their lower leg US images were quantitatively analyzed by texture analysis and machine learning methodology in the following subjects :  Inclusion body myositis (IBM) [N=11] ; myotonic dystrophy type 1 (DM1) [N=19...
Source: Journal of Medical Investigation - October 29, 2019 Category: General Medicine Tags: J Med Invest Source Type: research

Absolute reduction of regulatory T cells and regulatory effect of short-term and low-dose IL-2 in polymyositis or dermatomyositis.
Abstract AIM: The study aimed to investigate the changes in peripherallymphocyte and CD4+T subsets and to observe the regulatory effect of low-dose interleukin-2 (ld-IL2) on these cells in polymyositis or dermatomyositis (PM/DM). METHODS: Lymphocyte subsets (CD3+T, CD4+T, CD8+T, B and natural killer (NK) cells), CD4+T subsets (Th1, Th2, Th17 and regulatory T (Treg) cells) and multiple cytokines of 71 patients after admission and treatment were measured by flow cytometry, as well as these indicators in 30 healthy controls (HCs). In DM, 35 cases were administrated with ld-IL2 combined with conventional therapy,...
Source: International Immunopharmacology - October 24, 2019 Category: Allergy & Immunology Authors: Feng M, Guo H, Zhang C, Wang Y, Liang Z, Zhao X, Qin Y, Wu Y, Liu G, Gao C, Luo J Tags: Int Immunopharmacol Source Type: research

Retrospective Analysis of Cancer-Associated Myositis Patients over the Past 3 Decades in a Hungarian Myositis Cohort.
kó K Abstract Association between cancer and myositis has been extensively reported and malignancy is a potentially life-threating complication in myositis. In this retrospective study authors give an overview of Hungarian cancer-associated myositis (CAM) patients treated at a single centre managing 450 myositis patients. All patients were diagnosed according to Bohan and Peter. Statistical analysis of disease onset, age, sex, muscle, skin and extramuscular symptoms, muscle enzymes, presence of antibodies, treatment and prognosis was performed. 43 patients could be considered as having CAM. 83.72% had cance...
Source: Pathology Oncology Research - October 23, 2019 Category: Pathology Authors: András C, Bodoki L, Nagy-Vincze M, Griger Z, Csiki E, Dankó K Tags: Pathol Oncol Res Source Type: research

Human muscle pathology is associated with altered phosphoprotein profile of mitochondrial proteins in the skeletal muscle.
Abstract Analysis of human muscle diseases highlights the role of mitochondrial dysfunction in the skeletal muscle. Our previous work revealed that diverse upstream events correlated with altered mitochondrial proteome in human muscle biopsies. However, several proteins showed relatively unchanged expression suggesting that post-translational modifications, mainly protein phosphorylation could influence their activity and regulate mitochondrial processes. We conducted mitochondrial phosphoprotein profiling, by proteomics approach, of healthy human skeletal muscle (n = 10) and three muscle diseases (n = 10 ...
Source: Journal of Proteomics - October 23, 2019 Category: Biochemistry Authors: Sunitha B, Kumar M, Gowthami N, Unni S, Gayathri N, Prasad TSK, Nalini A, Polavarapu K, Vengalil S, Preethish-Kumar V, Padmanabhan B, Bharath MMS Tags: J Proteomics Source Type: research

Muscle aches and pains: do I have leukemia?
We describe a 65-year-old man who presented with 'aches and pains' localized to the lower extremities, and was diagnosed with acute myeloid leukemia (AML). We hypothesize that this case represents an atypical presentation of AML with an immune-mediated necrotizing-like myopathy as a possible paraneoplastic manifestation of the disease, which improved after initiating chemotherapy. Our patient received a full course of 7 + 3 chemotherapy with cytarabine and daunorubicin. Proximal leg weakness and pain improved markedly following this treatment, establishing a temporal relationship between the possible paraneop...
Source: International Journal of Hematology - October 14, 2019 Category: Hematology Authors: Deschner M, Phua C, Saini L, Xenocostas A, Deotare U Tags: Int J Hematol Source Type: research

Staining of antinuclear antibodies and antibodies against removable nuclear antigens in connective tissue diseases.
-Huarcaya L Abstract INTRODUCTION AND OBJECTIVES: Connective tissue diseases are inflammatory, autoimmune diseases and threaten quality of life. To determine the relationship between staining patterns of antinuclear antibodies and antibodies against extractable nuclear antigens in patients with connective tissue disease. MATERIALS AND METHODS: Observational, basic, analytical and transversal study. Study conducted in the Immunology Service of the Arzobispo Loayza National Hospital between January 2017 and June 2017. We analyzed 291 samples of patients with CTD and for the detection of anti-nuclear antibody st...
Source: Allergologia et Immunopathologia - October 14, 2019 Category: Allergy & Immunology Authors: OlivaMenacho JE, Arroyo-Acevedo JL, Oliva-Candela JA, García-Hjarles MA, Domínguez-Huarcaya L Tags: Allergol Immunopathol (Madr) Source Type: research

Safety and efficacy of PD-1/PD-L1 blockade in patients with preexisting antinuclear antibodies
AbstractPurposeImmune checkpoint inhibitors (ICIs) show promising clinical activity in advanced cancers. However, the safety and efficacy of PD-1/PD-L1 blockade in patients with preexisting antinuclear antibodies (ANA) are unclear.Methods191 patients treated with nivolumab, pembrolizumab, atezolizumab, or durvalumab for unresectable advanced cancers between September 2014 and December 2018 were identified retrospectively. Patients were divided into positive (ANA titers  ≥ 1:160) and negative ANA groups (ANA titers 
Source: Clinical and Translational Oncology - October 1, 2019 Category: Cancer & Oncology Source Type: research

P.20Expanding the myasthenia-myositis association spectrum: clinical, morphological and immunological data form a large case series
Myastenia-inflammatory myopathy (MG-IM) association has been described in less than 50 cases, as isolated reports or in few case series. In most cases, MG and IM onset occur simultaneously even if the overlapping clinical manifestations could lead to delay the diagnosis in the early stage of disease. In these cases, thymic pathology is present in more than 50% of cases. Pathological findings can be consistent of polymyositis (63%), dermatomyositis (25%) or granulomatosis (12%). MSA and muscle MRI involvement have not been systematically investigated. (Source: Neuromuscular Disorders)
Source: Neuromuscular Disorders - October 1, 2019 Category: Neurology Authors: M. Garibaldi, L. Fionda, F. Vanoli, L. Leonardi, E. Bucci, S. Morino, G. Merlonghi, M. Lucchini, M. Mirabella, F. Andreetta, E. Pennisi, A. Petrucci, G. Antonini Source Type: research

P.06Muscle ultrasound in patients with inclusion body myositis: differentiating from mimics
In this study we aimed to further evaluate the ability of US to differentiate between IBM and Polymyositis/Dermatomyositis (PM/DM) and neuromuscular disorders using two separate cohorts. (Source: Neuromuscular Disorders)
Source: Neuromuscular Disorders - October 1, 2019 Category: Neurology Authors: K. Leeuwenberg, L. Christopher-Stine, J. Paik, E. Tiniakou, C. Mecoli, N. van Alfen, J. Doorduin, C. Saris, J. Albayda Source Type: research

Hemophagocytic Lymphohistiocytosis : A Skeleton in the Cupboard of Adult Idiopathic Inflammatory Myopathies.
CONCLUSION: In this study, secondary HLH was a fatal complication with higher on-admission disease activity, AE-ILD and infection working as risk factors. The underlying role of infection and autoimmune abnormality in HLH in connective tissue disease (CTD) was subsequently brought up. Clinical factors influencing the short-term outcome of patients with secondary HLH required further exploration. PMID: 31575703 [PubMed - as supplied by publisher] (Source: J Rheumatol)
Source: J Rheumatol - October 1, 2019 Category: Rheumatology Authors: Lianga J, Xu D, Sun C, Chen W, Cao H, Lina J Tags: J Rheumatol Source Type: research

Genetics of idiopathic inflammatory myopathies: insights into disease pathogenesis
Purpose of review To review the advances that have been made in our understanding of the genetics of idiopathic inflammatory myopathies (IIM) in the past 2 years, with a particular focus on dermatomyositis and polymyositis. Recent findings Fine-mapping studies in the major histocompatibility complex region in Caucasian and Korean populations have identified novel human leukocyte antigen (HLA) variants that are associated with autoantibody subgroups in IIM. Differences in HLA associations have been identified between Caucasian adult-onset and juvenile-onset patients with anti-TIF1 autoantibodies, suggesting distinct ae...
Source: Current Opinion in Rheumatology - September 25, 2019 Category: Rheumatology Tags: MYOSITIS AND MYOPATHIES: Edited by Andrea Doria, Anna Ghirardello, and Mariele Gatto Source Type: research

Using the tools of proteomics to understand the pathogenesis of idiopathic inflammatory myopathies
Purpose of review One of the most important advances in medical research over the past 20 years has been the emergence of technologies to assess complex biological processes on a global scale. Although a great deal of attention has been given to genome-scale genetics and genomics technologies, the utility of studying the proteome in a comprehensive way is sometimes under-appreciated. In this review, we discuss recent advances in proteomics as applied to dermatomyositis/polymyositis as well as findings from other inflammatory diseases that may enlighten our understanding of dermatomyositis/polymyositis. Recent findings ...
Source: Current Opinion in Rheumatology - September 25, 2019 Category: Rheumatology Tags: MYOSITIS AND MYOPATHIES: Edited by Andrea Doria, Anna Ghirardello, and Mariele Gatto Source Type: research

Interferon-signature in idiopathic inflammatory myopathies
Purpose of review The present review describes the interferon (IFN)-signature currently emerging as a tool for the diagnosis of idiopathic inflammatory myopathies (IIMs), and aims at presenting the interests and limitations of this recent tool for the clinics and the research. Recent findings Recent in-vivo and in-vitro transcriptomic studies have evidenced the involvement of IFNs in the pathogenesis of IIMs. A correlation between the IFN-signature and the clinical severity of IIMs has been established. Moreover, studies pointed out differences in the IFN-signature regarding the IIM subgroup (dermatomyositis, polymyos...
Source: Current Opinion in Rheumatology - September 25, 2019 Category: Rheumatology Tags: MYOSITIS AND MYOPATHIES: Edited by Andrea Doria, Anna Ghirardello, and Mariele Gatto Source Type: research

Imaging of Thoracic Wall Abnormalities.
Authors: Semionov A, Kosiuk J, Ajlan A, Discepola F Abstract Identification of certain abnormalities of the chest wall can be extremely helpful in correctly diagnosing a number of syndromic conditions and systemic diseases. Additionally, chest wall abnormalities may sometimes constitute diagnoses by themselves. In the present pictorial essay, we review a number of such conditions and provide illustrative cases that were retrospectively identified from our clinical imaging database. These include pentalogy of Cantrell, Klippel-Feil syndrome, cleidocranial dysplasia, Poland syndrome, osteopetrosis, neurofibromatosis ...
Source: Korean Journal of Radiology - September 24, 2019 Category: Radiology Tags: Korean J Radiol Source Type: research

Tertiary lymphoid organs in the inflammatory myopathy associated with PD-1 inhibitors
ConclusionsIn inflammatory myopathy associated with PD-1 inhibitors, CD8+ cells appear to predominantly destruct muscle fibers. The presence of lymphoid follicle-like structures and expression of PNAd and CCL21 on the endothelial cells suggest the tertiary lymphoid organs are formed, and involved in the leakage of lymphocytes. Thus, in the three cases examined, formation of the tertiary lymphoid organs is likely to play an important role in genesis of the PD-1 myopathy. (Source: Journal for Immunotherapy of Cancer)
Source: Journal for Immunotherapy of Cancer - September 18, 2019 Category: Cancer & Oncology Source Type: research

High-Dose Facilitated Subcutaneous Immunoglobulin in a Patient with Refractory Polymyositis and Severe Interstitial Lung Disease.
Authors: Giovanna Danieli M, Menghini D, Mezzanotte C, Gelardi C, Pedini V, Monteforte F PMID: 31507128 [PubMed - in process] (Source: The Israel Medical Association Journal)
Source: The Israel Medical Association Journal - September 12, 2019 Category: General Medicine Tags: Isr Med Assoc J Source Type: research

Clinical characteristics and prognosis of polymyositis and dermatomyositis associated with malignancy: a 25-year retrospective study
The objective of this study was to identify factors predictive of malignancy in patients with polymyositis (PM) and dermatomyositis (DM) in Japan. We conducted a retrospective study of PM and DM patients who were admitted to our hospital between January 1992 and September 2017. Among 134 patients, 29 (21.6%) were diagnosed with cancer in the 3  years prior to and 3 years after the initial diagnosis of PM or DM. According to multivariate analyses, male sex [odds ratio (OR) = 3.65,p = 0.03], old age (OR = 1.05,p = 0.02), and a past history of diabetes mellitus...
Source: Rheumatology International - September 11, 2019 Category: Rheumatology Source Type: research

Prevalence of other connective tissue diseases in idiopathic inflammatory myopathies
AbstractWe sought to determine the prevalence of additional connective tissue diseases (CTDs) in patients with idiopathic inflammatory myopathies (IIM), and to study the muscle biopsy patterns in various clinico-serologic subsets of myositis. We undertook a retrospective cohort study of 648 patients with a histological diagnosis of IIM. The following was determined from the South Australian Myositis Database: presence of associated CTDs, histological details and presence of myositis-specific (MSA) or myositis-associated (MAA) antibodies. Among patients with IIM, a significantly greater proportion had systemic sclerosis 32/...
Source: Rheumatology International - September 11, 2019 Category: Rheumatology Source Type: research