Characterization of patients with dermatomyositis in a university hospital in Colombia

Introduction: Dermatomyositis is an idiopathic inflammatory myopathy characterized by the presence of skin lesions; it is considered a heterogeneous disease, due to its clinical presentation, course, and prognosis. In Colombia there are few records that describe the clinical characteristics of these patients. Methods: Cross-sectional study. Medical records of patients who consulted a university hospital in Colombia between January 2004 and December 2019 were reviewed. The records were obtained using databases from the dermatology, rheumatology, dermatopathology, and electrophysiology units, and CIE10 diagnostic codes. Results: Seventy patients with a dermatomyositis diagnosis were found, 63 (90%) fulfilled the Bohan and Peter diagnostic criteria and 7 (10%) had amyopathic dermatomyositis, with an average age of 43 years (SD ± 15.3). Forty-eight were women (68.5%). The most frequent clinical signs were Gottron's papules 80%, periorbital violaceous (heliotrope) erythema with edema 78.5% (n = 55) and poikiloderma 75.7% (n = 53). The most frequently found systemic manifestations were dysphagia (21.4%, n = 15), interstitial lung disease (11.4%, n = 8), and pulmonary hypertension (8.5%, n = 6). Cancer was documented in 8.5% (n = 6) of patients. Conclusion: We showed clinical information of patients with dermatomyositis in a referral hospital in Colombia. The data obtained is consistent with information from other case series worldwide.

Introducción: La dermatomiositis es una miopatía inflamatoria idiopática que se caracteriza por presentar lesiones en la piel; por su presentación clínica, su curso y su pronóstico, se la considera una enfermedad heterogénea. En Colombia existen pocos registros que describan las características clínicas de los pacientes afectados por esta enfermedad. Métodos: Estudio descriptivo de corte transversal, se revisaron las historias clínicas de pacientes que consultaron a un hospital universitario en Colombia entre enero del 2004 y diciembre del 2019. Los registros se obtuvieron utilizando bases de datos de las unidades de dermatología, reumatología, dermatopatología, electrofisiología y códigos diagnósticos CIE10 asociados con dermatomiositis. Resultados: Se obtuvieron 70 pacientes con diagnóstico de dermatomiositis, 63 (90%) de los cuales cumplían criterios de clasificación de Bohan y Peter, en tanto que 7 (10%) presentaban dermatomiositis amiopática. El promedio de edad fue de 43 arios (DS ± 15,3); 48 fueron mujeres (68,5%); los signos clínicos más frecuentes fueron: pápulas de Gottron (80%, n = 56), eritema heliotropo (78,5%, n = 55) y poiquilodermia (75,7%, n = 53). Las manifestaciones sistêmicas más comúnmente encontradas fueron: disfagia (21,4%, n = 15), enfermedad pulmonar intersticial (11,4%, n = 8) e hipertensión pulmonar (8,5%, n = 6). Se documentó cáncer en el 8,5% (n = 6) de los pacientes. Conclusión: Se presenta información clínica de pacientes con dermatomiositis en un centro hospitalario de referencia en Colombia; los datos obtenidos concuerdan con la información de otros estudios de series de casos a escala mundial.

Fever in the initial stage of IIM patients: an early clinical warning sign for AE-ILD

Abstract Background Fever is a common symptom of Idiopathic inflammatory myopathies (IIM). However, the exact correlation between fever and the prognosis of IIM is still unclear. This study aims to clarify if the IIM patients initiated with fever are associated with poorer outcomes. Methods This was a single-center retrospective cohort study. Data were collected from 79 newly diagnosed, treatment-naive IIM patients in the Affiliated Wuxi People's Hospital of Nanjing Medical University (Wuxi, Jiangsu, China) from November 2016 to June 2020. According to the presence or absence of fever at the onset, the IIM patients were divided into two groups(fever group n = 28, without fever group n = 51) Clinical characteristics, laboratory data, treatment, and outcomes were recorded. The Kaplan-Meier and log-rank tests were used to compare the all-cause mortality, relapse rate, and acute exacerbation of interstitial lung disease (AE-ILD) incidence. The association of fever with the outcomes was assessed in the unadjusted and adjusted forward logistic regression model. Results Compared with the non-fever group, the age at onset of the fever group was higher, and mechanic's hands (MH) and interstitial lung disease (ILD) were more common. Systemic inflammation (CRP and ESR) was significantly higher in the fever group, while the level of albumin(ALB) and muscle enzymes were lower. The fever group seemed to be received more aggressive treatment, with higher dose glucocorticoids and higher rates of intravenous immunoglobulins(IVIG) use. The all-cause mortality rate and the incidence rate of AE-ILD were higher in the fever group. Even adjusted for the age at onset and treatments, fever was significantly associated with AE-ILD and all-cause mortality. Conclusion Our study has demonstrated that fever at initial diagnosis is associated with AE-ILD and mortality. Fever should serve as an early clinical warning sign for poor outcomes in IIM patients.

Gathering patients and rheumatologists' perceptions to improve outcomes in idiopathic inflammatory myopathies

Abstract Objective Therapeutic targets in Idiopathic Inflammatory Myopathies (IIM) are based on the opinions of physicians/specialists, which may not reflect the main concerns of patients. The authors, therefore, assessed the outcome concerns of patients with IIM and compared them with the concerns of rheumatologists in order to develop an IIM outcome standard set. Methods Ninety-three IIM patients, 51 rheumatologists, and one physiotherapist were invited to participate. An open questionnaire was initially applied. The top 10 answers were selected and applied in a multiple-choice questionnaire, inquiring about the top 3 major concerns. Answers were compared, and the agreement rate was calculated. Concerns were gathered in an IIM outcome standard set with validated measures. Results The top three outcome concerns raised by patients were medication side effects/muscle weakness/prevention functionality loss. The top three concerns among rheumatologists were to prevent loss of functionality/to ensure the quality of life/to achieve disease remission. Other's outcomes concerns only pointed out by patients were muscle pain/diffuse pain/skin lesions/fatigue. The agreement rate between both groups was 41%. Assessment of these parameters guided the development of an IIM standard set which included Myositis Disease Activity Assessment Visual Analogue Scale/Manual Muscle Testing/fatigue and pain Global Visual Analogue Scale/Health Assessment Questionnaire/level of physical activity. Conclusion The authors propose a novel standard set to be pursued in IIM routine follow-up, which includes not only the main patients/rheumatologist outcome concerns but also additional important outcomes only indicated by patients. Future studies are necessary to confirm if this comprehensive approach will result in improved adherence and ultimately in better assistance.

Profile of various idiopathic inflammatory myopathies at two university hospitals in Yangon, Myanmar

@#Objective: to determine the distribution of various idiopathic inflammatory myopathies (IIM) and their profile at the largest university hospitals in Yangon, Myanmar. Method: It was a hospital based prospective study recruiting IIM patients admitted to Neurology and Rheumatology ward over a 1.5 year period from September 2017 to February 2019. Results: Among total 51 IIM patients recruited, 62.7% presented to Neurology ward and 37.3% to Rheumatology ward. Overlap myositis (OM) was the commonest (43%), followed by immune-mediated necrotizing myopathy (IMNM) 27%, dermatomyositis (DM) 24%, polymyositis (PM) 6%. Among OM, anti-synthetase syndrome (ASS) was 23%, and among IMNM, anti-SRP positive was 79%. IMNM and PM patients presented more to neurologists while OM/ASS and DM more to rheumatologists; 82% were females (F:M= 4.6:1). Mean age of onset of myositis was 40.2 + 17.8 years, and duration of symptoms before presentation was 10-3,600 days (shortest in anti-SRP and longest in anti-HMGCR myopathy). Myositis antibodies were positive in 67%. CK range was 40-25,690 U/l, highest in IMNM and lowest in DM. Associated connective tissue diseases among OM in order of descending frequency were 47% systemic lupus erythematosus, 24% Sjogren syndrome, 41% scleroderma and 12% rheumatoid arthritis. Associated cancer identified were one lung cancer in DM, one breast cancer in OM, one buccal cancer in IMNM cases. Conclusions: With recent availability of myositis antibody panel and MHC staining in Myanmar, we have applied current updated classification to describe the first Myanmar data on IIM cases.

Necrotizing Autoimmune Myopathy: A Case Series

@#Necrotizing autoimmune myopathy (NAM) is considered a new subgroup of a rare autoimmune idiopathic inflammatory myopathies. Classically, NAM presented with sub-acute onset of proximal muscle loss of power with raised creatinine kinase and characteristic muscle biopsy showing muscle necrosis and regeneration with little inflammation. Statin use, connective tissue diseases, malignancy and HIV infection are the identified risk factors for NAM. The autoantibodies expected to be presented in NAM are anti-signal recognition particle (SRP) and anti-hydroxymethylglutaryl-coenzyme A reductase (anti-HMGCR) antibodies. In this article, we present three cases of NAM with different risk factors and autoantibodies which we believe to have impact on the clinical course and outcome of our patients

Classification of idiopathic inflammatory myopathies based on clinical manifestations and myositis-specific antibodies / 南方医科大学学报

OBJECTIVE@#To investigate the classification of idiopathic inflammatory myopathies (IIM) based on clinical manifestations and myositis- specific antibodies using cluster analysis.@*METHODS@#We retrospectively analyzed the data of patients with IIM admitted in Nanfang Hospital in 2015-2019. The clinical data of the patients including serum creatine kinase (CK), interstitial lung disease (ILD), cancer, and myositis-specific antibodies were collected for two-step cluster analysis to identify the distinct clusters of patients, whose clinical characteristics were subsequently analysed.@*RESULTS@#A total of 71 patients with IIM were included in this study, including 30 (42.3%) with polymyositis (PM), 20 (28.2%) with classic dermatomyositis (DM), 16 (22.5%) with amyopathic dermatomyositis (CADM), and 5 (7.0%) with immune-mediated necrotizing myopathy (IMNM). Two-step cluster analysis identified 3 distinctive subgroups: Cluster 1 of 15 (51.7%) patients characterized by rash, positive anti-MDA5 antibody and hypoproteinemia ( < 0.05) with normal or slightly elevated CK level, mainly corresponding to CADM; Cluster 2 of 4 (57.1%) patients with significantly elevated CK and positive anti-SRP antibody ( < 0.001) corresponding to IMNM; and Cluster 3 of 17 (48.6%) patients consisting primarily of patients with PM, characterized by positivity for anti- aminoacyl transfer RNA synthetases antibodies (=0.022) corresponding to antisynthetase syndrome (ASS).@*CONCLUSIONS@#Patients with IIM can be divided into 3 subgroups based on their clinical and serological characteristics (especially myositis-specific antibodies), and among them ASS may represent an independent IIM subgroup with unique clinical characteristics.

Idiopathic inflammatory myopathy / 中华神经科杂志

Idiopathic inflammatory myopathies (IIM) are a group of acquired immune myopathy,which mainly include polymyositis,dermatomyositis,amyopathic dermatomyositis,sporadic inclusion body myosistis (sIBM) and immune-mediated necrotizing myopathy,as well as some special types of antisynthetase syndrome,anti-signal recognition particle antibody positive necrotizing myopathy (NM),anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase antibody positive NM.The diagnosis of these different types of IIM mainly depends on clinical manifestations,antibody detection and muscle pathological techniques.Different types of IIM have different clinical manifestations,or overlapping manifestations.This article systematically describes the evolution of IIM types,the main antibodies to myositis,the pathological characteristics of muscles,the manifestations of various types and the treatment of myositis.In addition to sIBM,patients with most of the other types of IIM have good outcomes by early diagnosis,timely,correct and adequate drug treatment.

Autoanticuerpos en miopatías inflamatorias idiopáticas: enfoque en la práctica clínica / Autoantibodies in idiopathic inflammatory myopathies: focus on clinical practice

Las miopatías inflamatorias idiopáticas (MII) conforman un grupo diverso de en-fermedades caracterizadas por lesiones musculares inmunomediadas, que pue-den estar acompañadas de manifestaciones extramusculares.La identificación de autoanticuerpos específicos de miositis (AEM) y asociados a miositis (AAM) se ha convertido en una herramienta relevante en el diagnóstico, clasificación y pronóstico en este grupo de enfermedades.Adquieren relevancia en el diagnóstico y determinación de pronóstico de la Enfer-medad Pulmonar Intersticial (EPI), en el diagnóstico diferencial entre subgrupos de MII, especialmente en entidades de comportamiento atípico como la Miopatía Necrotizante Inmuno Mediada (MNIM) y la Miositis por Cuerpos de Inclusión, y permiten el enfoque y seguimiento estricto en pacientes con autoanticuerpos asociados a cáncer. En el último tiempo se ha planteado su utilidad tanto en la determinación de actividad de la enfermedad como en predecir la respuesta al tratamiento inmu-nosupresor.

Idiopathic inflammatory myopathies (IIM) comprise a heterogenous group of ill-nesses characterized by immune mediated muscular injuries that may be accom-panied by extra-muscular manifestations.The identification of myositis-specific autoantibodies MSAs and myositis-associ-ated autoantibodies (MAA) has become a relevant tool in the diagnose, classifica-tion, and prognosis in this group of illnesses.They become relevant in the diagnose and determination of a prognosis for Inter-stitial Lung Disease (ILD), in the differentiated diagnose among sub-groups of MII, especially in entities of an atypical behavior, such as Immune Mediated Necrotiz-ing Myopathy (IMNM) and Inclusion Body Myositis, and they enable a strict focus and follow-up on patients with cancer-associated autoantibodies. Lately, its usefulness has been stated both to determine disease activity and to forecast the response to the immunosuppressive treatment.

Polimiositis secundaria a tuberculosis en un niño de 11 años / Polymyositis secondary to tuberculosis in an 11-year-old boy

Resumen:La polimiositis es una de las miopatías inflamatorias idiopáticas. Tiene una incidencia mundial estimada en 4 casos por cada millón de habitantes al año. Es considerada un diagnóstico de exclusión y se ha establecido una asociación con la infección por micobacterias. Se reporta el caso de un niño de 11 años de edad con polimiositis secundaria a tuberculosis. La polimiositis tuvo una adecuada respuesta a los corticosteroides, pero estos no fueron necesarios después del diagnóstico de una infección pulmonar por Mycobacterium tuberculosis. El tratamiento antifímico, sin la terapia con esteroides, permitió una resolución de ambas patologías y la evolución favorable del paciente.

Abstract:Polymyositis is one of the idiopathic inflammatory myopathies. It has an estimated worldwide incidence of 4 cases per million population per year. It is considered an exclusion diagnosis and a relationship with mycobacterial infection has been established. This article reports the case of an 11-year-old boy with polymyositis secondary to tuberculosis. Polymyositis had an adequate response to corticosteroids, but these were not needed after the diagnosis of a Mycobacterium tuberculosis lung infection. Anti-tuberculosis treatment without steroid therapy, allowed a resolution of both conditions and the favorable outcome of the patient.

Pregnancy investigation in idiopathic inflammatory myopathy in China / 中华风湿病学杂志

Objective We investigated female patients with idiopathic inflammatory myopathy (ⅡM),including polymyositis (PM) and dermatomyositis (DM) to understand if the activity and treatment of ⅡM affected the fetus outcomes as well as the correlation between pregnancy and the pathogenesis of ⅡM.Methods Questionnaires were designed,including patients' fertility,the activity and treatment of ⅡM during pregnancy and after childbirth,and the fetal outcomes.The questionnaires were conducted on 75 female patients (22-53 years old),who once hospitalized at Peking Union Medical College Hospital from 2007 to 2013.The mutual effect between the ⅡM disease activity,pregnancy and the fetus were compared by Chi square.Results Seventy-five patients (19 PM,56 DM) had 144 pregnancies,but only 18 of them became pregnant after the onset of the disease.There were two patients had onset of DM during pregnancy,and two patients suffered from DM after delivery or abortion within one month.The rates of abnormal pregnancy in patients with pregnancy before the onset of ⅡM was significantly lower than those with pregnancy after the onset of ⅡM (x2=10.21,P=0.001).Of the 18 pregnancies after the onset of ⅡM,7 had active ⅡM during pregnancy,3 of them flareddue to pregnancy.The 5 abnormal pregnancies (5/7) were seen in seven pregnancies totally,including two spontaneous abortion,one embryo diapause and two premature births.While of the other 11 pregnanciesin stable condition had only 2 abnormal pregnancies (2/11),including one fetus ended prematurely and one embryo diapause.The fetal outcomes were significantly related to the status of illness (x2=5.103,P=0.024).Two patients became pregant after the onset of the disease,both of them had stable disease before pregnancy.However,the disease became active during the pregnancies.One of them flared with rash during two pregnancies.The fetal end in embryo diapause and premature birth (33+4 weeks),respectively.Another flared with muscle weakness at five months of gestation.The fetal ended in premature birth (36+3 weeks).Two patients had onset of DM within one month following the delivery and voluntary abortion.Patients with active disease had taken medium to large doses of prednisone (30-60 mg) orally,in combination with hydroxychloroquine (0.2 bid) or without.In addition,two patients administrated with intravenous gamma globulin (IVIG) at low dose,whose illness condition had been well controlled.Conclusion Pregnancies after the onset of ⅡM tend to end with spontaneous abortion,embryo diapause,premature birth.The fetal outcomes are significantly related to disease activity.When ⅡM flared during pregnancy,IVIG might be the most effective and safe treatment,combined with corticosteroids.

Myositis-specific and myositis-associated autoantibody profiles and their clinical associations in a large series of patients with polymyositis and dermatomyositis

OBJECTIVE: To analyze the prevalence of myositis-specific and myositis-associated autoantibodies and their clinical correlations in a large series of patients with dermatomyositis/polymyositis. METHOD: This cross-sectional study enrolled 127 dermatomyositis cases and 95 polymyositis cases. The disease-related autoantibody profiles were determined using a commercially available blood testing kit. RESULTS: The prevalence of myositis-specific autoantibodies in all 222 patients was 34.4%, whereas myositis-associated autoantibodies were found in 41.4% of the patients. The most frequently found autoantibody was anti-Ro-52 (36.9%), followed by anti-Jo-1 (18.9%), anti-Mi-2 (8.1%), anti-Ku (4.1%), anti-SRP (3.2%), anti-PL-7 (3.2%), anti-PL-12 (2.7%), anti-PM/Scl75 (2.7%), and anti-PM/Scl100 (2.7%). The distributions of these autoantibodies were comparable between polymyositis and dermatomyositis, except for a higher prevalence of anti-Jo-1 in polymyositis. Anti-Mi-2 was more prevalent in dermatomyositis. Notably, in the multivariate analysis, anti-Mi-2 and anti-Ro-52 were associated with photosensitivity and pulmonary disorders, respectively, in dermatomyositis. Anti-Jo-1 was significantly correlated with pulmonary disorders in polymyositis. Moreover, anti-Ro-52 was associated with anti-Jo-1 in both diseases. No significant correlation was observed between the remaining autoantibodies and the clinical and/or laboratory findings. CONCLUSIONS: Our data are consistent with those from other published studies involving other populations, although certain findings warrant consideration. Anti-Ro-52 and anti-Jo-1 were strongly associated with one another. Anti-Ro-52 was correlated with pulmonary disorders in dermatomyositis, whereas anti-Jo-1 was correlated with pulmonary alterations in polymyositis. .

Skeletal muscle major histocompatibility complex class I and II expression differences in adult and juvenile dermatomyositis

OBJECTIVE: To analyze major histocompatibility complex expression in the muscle fibers of juvenile and adult dermatomyositis. METHOD: In total, 28 untreated adult dermatomyositis patients, 28 juvenile dermatomyositis patients (Bohan and Peter's criteria) and a control group consisting of four dystrophic and five Pompe's disease patients were analyzed. Routine histological and immunohistochemical (major histocompatibility complex I and II, StreptoABComplex/HRP, Dakopatts) analyses were performed on serial frozen muscle sections. Inflammatory cells, fiber damage, perifascicular atrophy and increased connective tissue were analyzed relative to the expression of major histocompatibility complexes I and II, which were assessed as negatively or positively stained fibers in 10 fields (200X). RESULTS: The mean ages at disease onset were 42.0±15.9 and 7.3±3.4 years in adult and juvenile dermatomyositis, respectively, and the symptom durations before muscle biopsy were similar in both groups. No significant differences were observed regarding gender, ethnicity and frequency of organ involvement, except for higher creatine kinase and lactate dehydrogenase levels in adult dermatomyositis (p<0.050). Moreover, a significantly higher frequency of major histocompatibility complex I (96.4% vs. 50.0%, p<0.001) compared with major histocompatibility complex II expression (14.3% vs. 53.6%, p=0.004) was observed in juvenile dermatomyositis. Fiber damage (p=0.006) and increased connective tissue (p<0.001) were significantly higher in adult dermatomyositis compared with the presence of perifascicular atrophy (p<0.001). The results of the histochemical and histological data did not correlate with the demographic data or with the clinical and laboratory features. CONCLUSION: The overexpression of major histocompatibility complex I was an important finding for the diagnosis of both groups, particularly for juvenile dermatomyositis, whereas there was lower levels of expression of major histocompatibility complex II than major histocompatibility complex I. This finding was particularly apparent in juvenile dermatomyositis.

Myopathy With Anti Signal Recognition Particle Antibody

Myopathies associated with anti-signal-recognition particle (SRP) antibodies usually present with severe muscle weakness and exhibit necrotizing myopathy with little inflammation pathologically. Here we report a case of a 61-year-old man who presented with subacute progressive proximal muscle weakness, dysarthria, and dysphagia. Although polymyositis was expected clinically, muscle biopsy revealed myopathic changes with degenerating fibers without definite inflammation. Further laboratory study revealed that the patient was positive for anti-SRP antibodies.

Miopatias inflamatórias idiopáticas: relato de caso / The idiopathic inflammatory myopathies: case report

JUSTIFICATIVA E OBJETIVOS: As miopatias inflamatórias idiopáticas, também conhecidas como síndrome de miosite, são da família de doenças sistêmicas adquiridas do tecido conjuntivo, caracterizadas por efeitos clínicos e patológicos de inflamação muscular crônica de causa desconhecida. As formas mais comuns são a polimiosite e a dermatomiosite, que tem seu diagnóstico com base em uma combinação de achados clínicos, laboratoriais e histopatológicos. O objetivo deste estudo foi abordar a característica clínica da dermatopolimiosite e demonstrar a evolução e o tratamento da doença. RELATO DO CASO: Paciente do sexo feminino, 66 anos, apresentando quadro de dor no abdômen e nos membros superiores e inferiores, associada com diminuição da força muscular. Presença de dispnéia acompanhada de tosse produtiva. Foi submetida à avaliação reumatológica, levantando-se a hipótese de dermatopolimiosite, concluindo-se o diagnóstico após avaliação clinica e laboratorial. CONCLUSÃO: O tratamento da doença ocorreu de acordo com o padrão evolutivo que a paciente se encontrava.

BACKGROUND AND OBJECTIVES: The idiophatic inflammatory myopathies - or syndrome myositis - are a group of acquired systemic diseases of connective tissue with characteristic of clinical and pathological effect of chronic inflammatory of muscle whose your pathogenesis is strange. The types habitual are the polymyositis and the dermatomyositis. For diagnosis is necessary a arrangement of clinical, laboratory and histopathologic founds. In this article we approach the characteristics of idiopathic inflammatory myopathies, aiming to demonstrate the evolution and treatment of disease. CASE REPORT: Female patient, 66 years, presenting chart of pain in abdomen and lower and upper members, associated with diminution of the muscular force. Presence of dyspnea accompanied of productive cough. It was submitted to Rheumatologic clinical evaluation being raised the hypothesis of dermatopolymyositis, concluding the diagnosis after evaluation is in clinical practice and laboratory. CONCLUSION: The handling of the illness occurs according to the developing standard that the patient is found.

Increased Interleukin-17 Expression in Patients with Idiopathic Inflammatory Myopathies / 대한류마티스학회지

OBJECTIVE: Idiopathic inflammatory myopathies (IIMs) are systemic autoimmune diseases characterized by infiltration of T lymphocytes, monocytes, and macrophages in muscle tissues. Interleukin-17 (IL-17), a Th17 cytokine, has potent pro-inflammatory actions and plays a role in autoimmune diseases. We investigated the expression of IL-17 in muscle tissues of patients with IIMs. METHODS: We measured the IL-17 mRNA level of muscle tissues from 14 patients with IIMs (9 patients with dermatomyositis and 5 patients with polymyositis) by real-time RT-PCR and compared with controls. We also performed an immunohistochemical stain to detect IL-17 expression. RESULTS: The expressions of IL-17 were significantly enhanced in IIMs than controls. In immunohistochemistry, IL-17 was expressed in perimysial, endomysial and perivascular infiltrating inflammatory cells. CONCLUSION: These results suggest that IL-17 plays a role in the immunopathogenesis of IIMs.