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myositis-associated interstitial lung disease

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https://www.readbyqxmd.com/read/29665800/mda-5-associated-rapidly-progressive-interstitial-lung-disease-with-recurrent-pneumothoraces-a-case-report
#1
Safi Alqatari, Peter Riddell, Sinead Harney, Michael Henry, Grainne Murphy
BACKGROUND: Clinically hypomyopathic dermatomyositis is a rare disease that is important to recognize, investigate and treat early as it is associated with poor prognosis. In a proportion of patients, myositis specific antibodies could be negative, but with high clinical suspicion, myositis associated antibodies should be ordered. Anti-MDA-5 antibodies was reported in literature to be associated with severe and rapidly progressive interstitial lung disease, with few case reports of pneumothorax and/or pneumomediastinum...
April 17, 2018: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/29651121/classification-of-myositis
#2
REVIEW
Ingrid E Lundberg, Marianne de Visser, Victoria P Werth
The idiopathic inflammatory myopathies (IIMs; also known as myositis) are a heterogeneous group of disorders in which a common feature is chronic inflammation of skeletal muscle, leading to muscle weakness. Other organs are frequently affected in IIMs, such as the skin, joints, lungs, gastrointestinal tract and heart, contributing to morbidity and mortality. Currently, IIMs are most often subclassified into polymyositis, dermatomyositis and inclusion body myositis, but this subclassification has limitations as these subgroups often have overlapping clinical and histopathological features, and outcomes vary within the subgroups; additionally, subgroups without considerable myopathy are not included...
April 12, 2018: Nature Reviews. Rheumatology
https://www.readbyqxmd.com/read/29644083/acute-interstitial-pneumonia-as-first-presentation-of-anti-synthetase-syndrome-an-atypical-case
#3
Kalimullah Jan, Melonie Kannamma Sriranganathan
Anti-synthetase syndrome is characterized by myositis associated with interstitial lung disease (ILD), the usual pattern of ILD being non-specific interstitial pneumonia type or usual interstitial pneumonia. We report a case of anti-synthetase syndrome presenting as acute interstitial pneumonia which is reported only once before. With this case, we emphasize the need to consider anti-synthetase syndrome even in patients presenting with acute onset ILD. Physicians should raise their index of suspicion in this clinical context as timely diagnosis, early treatment, and a multidisciplinary approach is paramount for optimal care of these patients...
April 2018: Oxford Medical Case Reports
https://www.readbyqxmd.com/read/29596687/initial-predictors-of-poor-survival-in-myositis-associated-interstitial-lung-disease-a-multicentre-cohort-of-497-patients
#4
Shinji Sato, Kenichi Masui, Naoshi Nishina, Yasushi Kawaguchi, Atsushi Kawakami, Maasa Tamura, Kei Ikeda, Takahiro Nunokawa, Yoshinori Tanino, Katsuaki Asakawa, Yuko Kaneko, Takahisa Gono, Taro Ukichi, Shinjiro Kaieda, Taio Naniwa, Masataka Kuwana
Objective: To identify initial predictors of poor survival in patients with PM/DM-associated interstitial lung disease (ILD). Methods: We established a multicentre retrospective cohort of incident cases of PM/DM-associated ILD from 44 institutions across Japan (Multicentre Retrospective Cohort of Japanese Patients with Myositis-associated ILD, JAMI). Inclusion criteria were an onset age ⩾16 years; PM/DM or clinically amyopathic DM according to the published criteria; imaging evidence of ILD; and availability of serum samples for assays of autoantibodies such as anti-melanoma differentiation-associated gene 5 and anti-aminoacyl tRNA synthetase...
March 27, 2018: Rheumatology
https://www.readbyqxmd.com/read/29595279/-systemic-sclerosis-in-2017
#5
Tomáš Soukup, Tomáš Veleta
Systemic sclerosis is classed as a diffuse (systemic) disease of connective tissue. It is a heterogeneous disease significantly shortening life expectancy. Its etiology is unknown. Pathogenetic interplay is assumed to involve a triad of pathological autoimmune inflammation, vasculopathy and fibrosis. Clinical manifestations can be classed based on the preponderant pathogenetic process. Vasculopathy is manifested by secondary Raynauds phenomenon with abnormal findings on the nailfold capillaroscopy, skin telangiectasias, gastric antral vascular ectasia, life threatening scleroderma renal crisis, digital ulcerations and prognostically severe pulmonary arterial hypertension...
2018: Vnitr̆ní Lékar̆ství
https://www.readbyqxmd.com/read/29593187/a-case-of-interstitial-pneumonia-associated-with-anti-pl-7-antibody-in-a-patient-with-rheumatoid-arthritis
#6
Hiroyuki Kozai, Yuko Toyoda, Hisatsugu Goto, Jun Kishi, Makoto Tobiume, Yuya Yamashita, Haruka Nishimura, Mayo Kondo, Hiroshi Kawano, Yasuhiko Nishioka
A 65-year-old female had been treated rheumatoid arthritis (RA), interstitial pneumonia (IP) and nephrotic syndrome with prednisolone and cyclosporine. She was emergently admitted to our hospital due to the worsening exertional dyspnea and severe hypoxemia. Chest computed tomography (CT) showed new diffuse ground-glass opacities (GGOs) with slight consolidations along with bronchovascular bundle were observed in addition to pre-existing reticular shadows in both lungs with lower lobe-predominance. An acute exacerbation (AE) of pre-existing IP triggered by an infection was suspected, and the treatment with antibiotics and corticosteroid pulse therapy improved her general condition and chest radiological findings...
2018: Journal of Medical Investigation: JMI
https://www.readbyqxmd.com/read/29532710/myositis-specific-autoantibodies-in-japanese-patients-with-juvenile-idiopathic-inflammatory-myopathies
#7
Masahiro Ueki, Ichiro Kobayashi, Shunichiro Takezaki, Yusuke Tozawa, Yuka Okura, Masafumi Yamada, Masataka Kuwana, Tadashi Ariga
OBJECTIVES: The aim of our study is to clarify the association of myositis-specific autoantibodies (MSAs) with clinical and laboratory features in Japanese patients with juvenile idiopathic inflammatory myopathies (JIIMs). METHODS: We retrospectively analyzed the frequency of MSAs and their association with clinical or laboratory findings in 25 Japanese patients with JIIMs in Hokkaido district. RESULTS: Eighteen of 25 patients (72%) were positive for MSAs; seven with anti-melanoma differentiation associated gene (MDA) 5 (28%), 5 with anti-transcriptional intermediary factor (TIF)-1γ (20%), 4 with anti-MJ/nuclear matrix protein (NXP)-2 (16%), 2 with anti-Jo-1 (8%), 1 with anti- HMG-CoA reductase, 1 with anti-signal recognition peptide (SRP) antibodies (4% each), including co-existence and transition of MSAs in one patient each...
March 13, 2018: Modern Rheumatology
https://www.readbyqxmd.com/read/29530007/elevated-%C3%AE-defensin-levels-in-plasma-and-bronchoalveolar-lavage-fluid-from-patients-with-myositis-associated-interstitial-lung-disease
#8
Noriho Sakamoto, Hiroshi Ishimoto, Tomoyuki Kakugawa, Minoru Satoh, Tomoko Hasegawa, Shin Tanaka, Atsuko Hara, Shota Nakashima, Hirokazu Yura, Takuto Miyamura, Hanako Koyama, Towako Morita, Seiko Nakamichi, Yasushi Obase, Yuji Ishimatsu, Hiroshi Mukae
BACKGROUND: Interstitial lung disease (ILD) is a prognostic indicator of poor outcome in myositis. Although the pathogenesis of myositis-associated ILD is not well understood, neutrophils are thought to play a pivotal role. Neutrophils store azurophil granules that contain defensins, which are antimicrobial peptides that regulate the inflammatory response. Here, we evaluated levels of the human neutrophil peptides (HNPs) α-defensin 1 through 3 in patients with myositis-associated ILD to determine whether HNPs represent disease markers and play a role in the pathogenesis of myositis-associated ILD...
March 12, 2018: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/29453582/the-clinical-phenotype-of-patients-positive-for-antibodies-to-myositis-and-myositis-related-disorders
#9
S G Gofrit, H Yonath, M Lidar, Y Shoenfeld, S Kivity
Inflammatory myopathies are a clinically diverse group of diseases, in which the detection of particular autoantibodies may facilitate diagnosis, treatment, and prognosis. The aim of this report is to summarize our experience with specific autoantibody testing in patients with inflammatory myopathies. Data were collected over the last decade in the Autoimmune Center of the Sheba Medical Center, a tertiary referral hospital. Data regarding patients' positive for autoantibodies against Jo-1, PL-7, PL-12, SRP, Mi-2, Ku, and PM-Scl antigens were retrospectively collected...
May 2018: Clinical Rheumatology
https://www.readbyqxmd.com/read/29359173/immune-myopathies-with-perimysial-pathology-clinical-and-laboratory-features
#10
Robert C Bucelli, Alan Pestronk
Objective: Immune myopathies with perimysial pathology (IMPP) have a combination of damage to perimysial connective tissue and muscle fiber necrosis, more prominent near the perimysium. We studied the clinical and laboratory correlates of patients with pathologically defined IMPP. Methods: This is a retrospective chart and pathology review of 57 consecutive patients with IMPP myopathology and, for comparison, 20 patients with dermatomyositis with vascular pathology (DM-VP)...
March 2018: Neurology® Neuroimmunology & Neuroinflammation
https://www.readbyqxmd.com/read/29287518/anti-mda5-positive-dermatomyositis-complicated-with-rapidly-progressive-interstitial-lung-disease-a-case-report
#11
Eva De Backer, Félix Gremonprez, Guy Brusselle, Pieter Depuydt, Jo Van Dorpe, Carole Van Haverbeke, Pieter C Goeminne, Eric Derom
CASE PRESENTATION:  We present a case of a 55-year-old Caucasian male with manifestations of dermatomyositis complicated with rapidly progressive interstitial lung disease (RP-ILD). Diagnosis of anti-MDA5 positive dermatomyositis was made. DISCUSSION:  Myositis specific antibodies (MSA) can be used for diagnosis and predicting prognosis in patients with polymyositis and dermatomyositis. Anti-MDA5 positive dermatomyositis should be considered in patients presenting with dermatomyositis and a disease course resembling antisynthetase syndrome in the absence of antisynthetase autoantibodies, especially if a remarkably high ferritin is noted...
December 29, 2017: Acta Clinica Belgica
https://www.readbyqxmd.com/read/29237090/distinctive-cutaneous-and-systemic-features-associated-with-specific-antimyositis-antibodies-in-adults-with-dermatomyositis-a-prospective-multicentric-study-of-117-patients
#12
M Best, M Jachiet, N Molinari, F Manna, C Girard, V Pallure, A Cosnes, D Lipsker, T Hubiche, J-L Schmutz, Y Le Corre, N Cordel, M Dandurand, O Dereure, B Guillot, A Du-Thanh, C Bulai Livideanu, F Chasset, J-D Bouaziz, C Francès, D Bengoufa, T Vincent, D Bessis
BACKGROUND: Identification of myositis-specific autoantibodies (MSAs) for dermatomyositis (DM) could allow the characterization of an antibody-associated clinical phenotype. OBJECTIVE: We sought to define the clinical phenotype of DM and the risk of cancer, interstitial lung disease (ILD) and calcinosis based on MSA. METHODS: A 3.5-year multicentre prospective study of adult DM patients was conducted to determine the clinical phenotype associated with MSAs and the presence of cancer, ILD and calcinosis...
December 13, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/29229575/anti-melanoma-differentiation-associated-gene-5-mda5-dermatomyositis-a-concise-review-with-an-emphasis-on-distinctive-clinical-features
#13
REVIEW
Drew J B Kurtzman, Ruth Ann Vleugels
Melanoma differentiation-associated gene 5 (MDA5) is a recently described autoantigen target in a subset of patients with dermatomyositis. Anti-MDA5 dermatomyositis is characterized by a unique mucocutaneous and systemic phenotype that includes cutaneous and oral ulceration, painful palmar papules, alopecia, panniculitis, arthritis, a lower incidence of myositis, and, importantly, an elevated risk of interstitial lung disease with a potentially fatal course. Because the clinical features can differ substantially from those typically observed in cutaneous dermatomyositis, the diagnosis is often overlooked, which might negatively affect patient outcomes...
April 2018: Journal of the American Academy of Dermatology
https://www.readbyqxmd.com/read/29206633/exploration-of-the-muc5b-promoter-variant-and-ild-risk-in-patients-with-autoimmune-myositis
#14
Cheilonda Johnson, Paul Rosen, Thomas Lloyd, Maureen Horton, Lisa Christopher-Stine, Chester V Oddis, Andrew L Mammen, Sonye K Danoff
Interstitial lung disease (ILD) is common in patients with autoimmune myositis but factors that determine susceptibility are unknown. Familial and sporadic idiopathic pulmonary fibrosis (IPF) are strongly associated with a single nucleotide polymorphism in the promoter region of MUC5B (rs35705950). We sought to determine the relationship between MUC5B polymorphism expression and myositis-ILD. The MUC5B minor allele frequency (MAF) was examined in 402 European American participants; 60 with idiopathic interstitial pneumonia (IIP), 208 with myositis-ILD, and 134 unaffected controls...
September 2017: Respiratory Medicine
https://www.readbyqxmd.com/read/29168054/cardiac-function-in-patients-with-polymyositis-or-dermatomyositis-a-three-dimensional-speckle-tracking-echocardiography-study
#15
Yue Zhong, Wenjuan Bai, Qibing Xie, Jianhong Sun, Hong Tang, Li Rao
Cardiac event is a major cause of death in patients with idiopathic inflammatory myopathies (IIM). The most frequent IIMs are polymyositis (PM) and dermatomyositis (DM). The purpose of this study was to analyze cardiac involvement by three-dimensional speckle-tracking echocardiography (3D STE) in patients with PM or DM, and to identify the relationship of cardiac injury with clinical characteristics and disease-specific parameters. 60 PM/DM patients with preserved left ventricular ejection fraction and 30 matched healthy controls were assessed by conventional echocardiography, 3D STE with biventricular strain analysis and electrocardiogram...
November 22, 2017: International Journal of Cardiovascular Imaging
https://www.readbyqxmd.com/read/29159696/reflex-testing-of-speckled-cytoplasmic-patterns-observed-in-routine-ana-hep-2-indirect-immunofluorescence-with-a-multiplex-anti-synthetase-dot-blot-assay-a-multicentric-pilot-study
#16
Maria Infantino, Boaz Palterer, Roberta Biagiotti, Fabio Almerigogna, Maurizio Benucci, Arianna Damiani, Valentina Grossi, Annalisa Azzurri, Patrizia Casprini, Giovanni Bacci, Maria Grazia Giudizi, Mariangela Manfredi
Immunofluorescence on HEp2-cells is the standard diagnostic assay for the detection of anti-nuclear antibodies (ANA). Cytoplasmic speckled patterns are a common finding, and are associated with various antibodies, including anti-synthetase antibodies. However, classic ENA testing generally identifies only anti-Jo-1. Moreover, anti-synthetase syndrome is increasingly recognized as a pleomorphic entity, possibly presenting as isolated arthritis or interstitial lung disease. Sera referred for routine ANA testing were selected on the basis of the presence of a fine dense speckled cytoplasmic pattern (254 samples) and compared to control sera with negative cytoplasm (239 samples)...
February 2018: Immunologic Research
https://www.readbyqxmd.com/read/29151520/dropped-head-syndrome-and-the-presence-of-rimmed-vacuoles-in-a-muscle-biopsy-in-scleroderma-polymyositis-overlap-syndrome-associated-with-anti-ku-antibody
#17
Yoshida Takeshi, Yoshida Mai, Mitsuyo Kinjo, Jonosono Manabu, Higuchi Itsuro
A 66-year-old woman with a history of interstitial lung disease presented with a 3-month history of dropped head syndrome (DHS), followed by camptocormia and extremity weakness. A clinical examination revealed Raynaud phenomenon, arthralgia, distal skin sclerosis, and microbleeds in the nailfold capillaries. An anti-Ku antibody test was positive. A muscle biopsy revealed inflammatory myopathy with rimmed vacuoles (RVs). The diagnosis of scleroderma-polymyositis (SSc-PM) overlap syndrome was made. RVs on a muscle biopsy in a patient with inflammatory myositis involving axial muscles may be seen either in inclusion body myositis or SSc-PM overlap syndrome...
November 20, 2017: Internal Medicine
https://www.readbyqxmd.com/read/29062359/recovery-from-severe-dysphagia-in-systemic-sclerosis-myositis-overlap-a-case-report
#18
Keith J Chinniah, Girish M Mody
BACKGROUND: Dysphagia is common in inflammatory myopathies and usually responds to corticosteroids. Severe dysphagia requiring feeding by percutaneous endoscopic gastrostomy is associated with significant morbidity and high mortality. CLINICAL CASE: A 56-year old African Black woman initially presented with systemic sclerosis (SSC) - myositis overlap and interstitial lung disease. She responded to high dose corticosteroids and cyclophosphamide followed by azathioprine, with improvement in her lung function and regression of the skin changes...
June 2017: African Health Sciences
https://www.readbyqxmd.com/read/28866745/mortality-and-prognostic-factors-in-idiopathic-inflammatory-myositis-a-retrospective-analysis-of-a-large-multicenter-cohort-of-spain
#19
Laura Nuño-Nuño, Beatriz Esther Joven, Patricia E Carreira, Valentina Maldonado-Romero, Carmen Larena-Grijalba, Irene Llorente Cubas, Eva Gloria Tomero, María Carmen Barbadillo-Mateos, Paloma García De la Peña Lefebvre, Lucía Ruiz-Gutiérrez, Juan Carlos López-Robledillo, Henry Moruno-Cruz, Ana Pérez, Tatiana Cobo-Ibáñez, Raquel Almodóvar González, Leticia Lojo, María Jesús García De Yébenes, Francisco Javier López-Longo
The present study was undertaken to assess mortality, causes of death, and associated prognostic factors in a large cohort of patients diagnosed with idiopathic inflammatory myositis (IIM) from Spain. A retrospective longitudinal study was carried out in 467 consecutive patients with IIM, identified from 12 medical centers. Patients were classified as primary polymyositis, primary dermatomyositis (DM), overlap myositis, cancer-associated myositis (CAM), and juvenile idiopathic inflammatory myopathies. A total of 113 deaths occurred (24%) after a median follow-up time of 9...
November 2017: Rheumatology International
https://www.readbyqxmd.com/read/28864644/myositis-associated-interstitial-lung-disease-predictors-of-failure-of-conventional-treatment-and-response-to-tacrolimus-in-a-us-cohort
#20
Niharika Sharma, Michael S Putman, Rekha Vij, Mary E Strek, Anisha Dua
OBJECTIVE: Patients with myositis-associated interstitial lung disease (MA-ILD) are often refractory to conventional treatment, and predicting their response to therapy is challenging. Recent case reports and small series suggest that tacrolimus may be useful in refractory cases. METHODS: A retrospective cohort study of patients with MA-ILD comparing clinical characteristics between those who responded to or failed conventional treatment. In those who failed conventional treatment and received adjunctive tacrolimus, response to tacrolimus was measured by the improvement in myositis, ILD, and change in the dose of glucocorticoids...
November 2017: Journal of Rheumatology
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