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OBJECTIVE To establish predictive models for mortality in patients with polymyositis/dermatomyositis-associated interstitial lung disease (PM/DM-ILD) using a combination of initial serum biomarker levels. METHODS The Multicenter Retrospective Cohort of Japanese Patients with Myositis-Associated ILD (JAMI) database of 497 incident cases of PM/DM-ILD was used as a derivation cohort, and 111 cases were additionally collected as a validation cohort. Risk factors predictive of all-cause mortality were identified by univariate and multivariable Cox regression analyses using candidate serum biomarkers as explanatory variables. The predictive models for mortality were generated in patients with and those without anti-melanoma differentiation-associated gene 5 (MDA-5) antibody, using a combination of risk factors. Cumulative survival rates were assessed using Kaplan-Meier analysis, and were compared between subgroups using the Breslow test. RESULTS In the derivation cohort, C-reactive protein (CRP) and Krebs von den Lungen 6 (KL-6) levels were identified as independent risk factors for mortality in both anti-MDA-5-positive and anti-MDA-5-negative patients. We then developed a prediction model based on anti-MDA-5 antibody status, CRP level, and KL-6 level, termed the "MCK model," to identify patients at low (

Background Muscle enzymes are the major noninvasive diagnostic parameters useful in polymyositis/dermatomyositis (PM/DM). Few studies have yet correlated findings on 18F-FDG PET with disease activity in patients with PM/DM. Purpose We evaluated 18F-FDG muscle uptake in patients with PM/DM compared with non-muscular diseases and correlated the results with serum muscle enzymes. Methods A total of 28 patients with untreated PM/DM and 28 control patients with non-muscular diseases were examined with 18F-FDG PET/CT. 18F-FDG uptake was evaluated in 9 proximal skeletal muscle regions bilaterally. The uptake was scored as follows: 0 = less than that of the mediastinal blood vessels, 1 = greater than or equal to that of the mediastinal blood vessels, and 2 = greater than or equal to that of the liver. A score 1 or 2 was considered positive. The mean and maximum standardized uptake values (SUV) were calculated in each muscle and were averaged for all muscle regions. PET findings were correlated with serum muscle enzymes. Results 18F-FDG uptake was observed in 82% of patients with PM/DM and 7% of control patients. The number of positive regions, total score, mean SUVmean, and mean SUVmax in patients with PM/DM were significantly higher than those in the control patients (all P < 0.001). The total score of 2 was the best cut-off value that could discriminate patients with PM/DM from control patients. The total score, mean SUVmean, and mean SUVmax showed significant correlations with creatine kinase (P = 0.047, 0.002, 0.010, respectively) and aldolase (P = 0.036, 0.005, 0.038, respectively). Conclusion 18F-FDG PET/CT using visual and SUV methods demonstrated its usefulness by discriminating PM/DM from non-muscular diseases and correlating with serum muscle enzymes in patients with PM/DM.

We encountered a 30-year-old woman who developed dermatomyositis during pregnancy and was positive for anti-Mi-2 antibodies. She was successfully treated with prednisolone and tacrolimus and delivered a healthy child. We reviewed the cases of idiopathic inflammatory myositis (IIM) that developed during pregnancy that were published after the year 2000 to elucidate the profile of myositis-specific antibodies (MSAs) in them and to evaluate their obstetric outcomes. In cases with IIM that developed during pregnancy, anti-Mi-2, anti-TIF1-g, anti-Jo-1, and anti-EJ antibodies was detected in one case each. The obstetric outcomes of the IIM-complicated pregnancies were poor, especially when complicated with active maternal myositis. Further studies focusing on the possible causal relationships between MSAs and cases with IIM that developed during pregnancy are needed. For better obstetric outcomes, appropriate suppression of the maternal disease activity using immunosuppressants and vigilance regarding the patient's requirement of Caesarean section is important.

Objectives: Although intensive immunosuppressive treatment is necessary for the severe cases with polymyositis (PM)/dermatomyositis (DM), the prognostic factors or disease activity indices for PM/D...

Objectives To elucidate the serum cytokine profile and address the pathomechanism of interstitial lung disease (ILD) complicated with PM/DM. Methods Forty patients with PM/DM-ILD were enrolled, and principal components analysis and cluster analysis were performed to classify patients into subgroups. Additionally, we compared cytokine profiles between the survivors and dead patients and between anti-melanoma differentiation-associated gene 5 antibody- and anti-aminoacyl tRNA synthetase antibody-positive ILD patients. We also examined the association of various cytokines with disease activity indicators and prognosis of ILD. Results The principal components analysis data allowed classification of the cytokine profile into three groups: group 1, neutrophilic and M1-macrophage-driven cytokines; group 2, type 1 Th cell-driven and M2-macrophage-induced cytokines; and group 3, M2-macrophage-driven cytokines. Cluster analysis showed the presence of PM/DM-ILD patient groups with high or low levels of total cytokines. Ninety percent of patients who died of ILD were included in clusters with high cytokine levels. Serum cytokine levels of all groups were significantly higher in the anti-melanoma differentiation-associated gene 5 antibody-positive patients than in the anti-aminoacyl tRNA synthetase antibody-positive patients. Groups 1 and 2 significantly correlated with known factors for poor prognosis, such as serum ferritin levels and alveolar-arterial oxygen difference. Serum cytokine levels of patients in group 1 were significantly higher initially and at 2 and 4 weeks in those who died. Conclusion These findings suggested that the activation of monocytes, macrophages and type 1 Th cells, and neutrophils play roles in the pathomechanism of PM/DM-ILD, and group 1 cytokines could be useful biomarkers for predicting prognosis of PM/DM-ILD.

Objectives: We aimed to evaluate the usefulness of serum KL-6 for interstitial lung disease (ILD) with polymyositis/dermatomyositis (PM/DM).Methods: All consecutive and previously untreated adult p...

Objective: The serum myositis-specific autoantibodies has been considered to be relatively specific and be useful for diagnosis of polymyositis/dermatomyositis associated interstitial lung disease (PM/DM-ILD). The goal of our retrospective study was to identify clinical features and prognostic factors for PM/DM-ILD based on serological phenotypes.Methods: PM/DM-ILD patients were diagnosed in the Department of Respiratory Medicine, Nanjing Drum Tower Hospital. MSAs were measured by anti-myositis antibody profile IgG detection kit. Based on the results of MSAs, the patients were divided into three groups: anti-MDA5 group, anti-Jo-1 group and other anti-ARS group. Kaplan-Meier, log rank, Kruskal-Wallis test and chi-square tests were used for analysis.Results: We identified 30 patients (22.0%) with positive anti-MDA5, 42 patients (31.0%) with positive anti-Jo-1 and 64 patients(47.0%) with other anti-ARS. acute disease onset was more frequently observed in the anti-MDA5 group (P = 0.005). The highest mortality rate was in the anti-MDA5 group (66.7%, P P P = 0.002), DAD pattern for HRCT imaging (P P = 0.02), presence of Anti-MDA5 (P P P = 0.02), and the treatment with PSL pulse and IVIG were markedly correlated with high mortality (P P = 0.001, respectively).Conclusion: Anti–MDA5 antibody is significantly associated with associated with worse prognosis in PM/DM-ILD patients. The application of PSL pulse and IVIG are not necessarily an effective treatment for positive anti-MDA5 patients.

Corticosteroids are a class of steroid hormones that are produced in the adrenal cortex of the vertebrates, as well as the synthetic analogs of these hormones that are synthesized in the laboratories. Two main classes of corticosteroids, glucocorticoids, and mineralocorticoids, are involved in a wide range of physiologic processes, including stress response, immune response, and regulation of inflammation, carbohydrate metabolism, protein catabolism, blood electrolyte levels, and behavior. Corticosteroids have been used for almost 60 years in medicine and their roles in patients have always been discussed by researchers and clinicians dedicated in the related field. Currently, they are still used in the treatment of patients with neurological disorders. Usually, corticosteroids are used in the treatment of various inflammatory diseases and conditions. In this review, we present five key indications, i.e., neuromyelitis optica, acute spinal cord injury, chronic inflammatory demyelinating polyneuropathy, myasthenia gravis, polymyositis/dermatomyositis for the systemic use of corticosteroids in neurology based on a mix of quality of evidence, prevalence, and impact on disease management.

Background:Impaired microcirculation is one of the leading factors in local and general pathogenesis of SSc. Widefield nail-fold video-capillaroscopy (NFVC) stands as the most informative and at the same time simple method used for evaluation of capillary circulation.Objectives:To identify characteristic and specific for SSc– PM/DM capillaroscopic features.Methods:Both hand II – V fingers of 68 pts with SSc-PM/DM were subjected to widefield NFC, evaluated using a binocular 20x magnification Olympus microscope and analyzed in view of specific skin lesions discriminating diffuse and limited SSc forms.Results:SSc-specific dilatations of capillary loops were the most common for SSc-PM/DM and were found in all pts; 50% of them had signs of active scleroderma pattern, such as capillary loss or “avascular areas” (50%) and hemorrhages (51.5%), associated with generalized microvascular spasm in early disease and capillary sclerosis in advanced disease. The morphological capillary abnormalities such as varying degrees of capillary loops tortuosity/vascular inhomogeneity were present in 63% of examined nailfolds, branching bushy behavior of capillary loops and mega-capillaries predominated; architectural disorientation/disarrangement of capillary loops with formation of subcutaneous plexus was seen in more than 50% of them. Capillaroscopic changes consistent with active scleroderma pattern were present in 54 % and were associated with lab signs of inflammatory muscle syndrome and immunological disorders: giant capillaries (pConclusion:Thus, widefield NFVC revealed a “mixed” nature of capillaroscopic changes, combining features specific for SSc (capillary dilation, avascular areas, hemorrhages) and for PM/DM (bushy and giant capillaries, disorientation of capillary loops of the nailfold with formation of subcutaneous plexuses.)Disclosure of Interests:None declared

The objective of this study is to elucidate predictors of relapse in patients with polymyositis and dermatomyositis (PM/DM).Fifty PM/DM patients who achieved disease stabilization at Okayama University Hospital in 2004-2014 were enrolled retrospectively. Candidate predictors such as demographic factors, clinical symptoms, laboratory data, and treatment status were compared.The mean age of enrolled patients was 58 years; 34 were female. The patient groupings were as follows: 21 with PM, 27 with DM, and two with clinically amyopathic DM. During a mean observation period of 685 d, 5 patients (10%) died and 20 (40%) relapsed. The relapsed patients displayed baseline muscle weakness less frequently (85% versus 100%, p = .03) and anti-SS-A/Ro antibody more frequently (65% versus 27%, p = .007). Anti-SS-A/Ro-positive patients exhibited a higher relapse rate than anti-SS-A/Ro-negative patients (log-rank test, p = .03). Anti-SS-A/Ro-positive patients also exhibited higher anti-Jo-1 antibody positivity and lower levels of serum complement. After adjusting anti-Jo-1 antibody positivity, age, sex, CK500 IU/L, and lung involvement, anti-SS-A/Ro positivity was still an independent risk factor for higher relapse-rate (odds ratio, 5.5; 95% confidence interval, 1.4-25.1).Anti-SS-A/Ro antibody positivity may be a useful biomarker for prediction of relapse.

Background We attempted to clarify whether the presence of anti-aminoacyl-transfer RNA synthetase antibody (anti-ARS Ab) or anti-melanoma differentiation-associated gene 5 antibody (anti-MDA5 Ab) is associated with the therapeutic response of polymyositis/dermatomyositis-associated interstitial lung disease (PM/DM-ILD). Methods We retrospectively investigated 22 patients with PM/DM-ILD (10 positive for anti-ARS Ab and nine positive for anti-MDA5 Ab) for whom antibody analysis of conserved serum was possible. We assessed mortality in the first three months as the therapeutic response in the acute phase and compared changes in clinical data for up to one year considered as the chronic phase. We classified the clinical changes over the year into three groups: Improvement (increased % vital capacity [%VC] or diffusing capacity of the lung for carbon monoxide [%D LCO ]≥10 or 15%), deterioration (decreased %VC or %D LCO ≥10 or 15%), and no change (remainder of the changes). The extent of abnormality demonstrated by high-resolution computed tomography (HRCT) was scored. Results Positivity for anti-MDA5 Ab, but not for anti-ARS Ab, was associated with mortality in the first 3 months. Evaluation of the therapeutic response in the first year showed that positivity for the anti-ARS Ab, but not for the anti-MDA5 Ab, was associated with an improvement in %D LCO and a decline in the serum KL-6 levels. Positivity for the anti-ARS Ab or negativity for anti-MDA5 Ab was associated with a greater decrease in bronchial dilatation as seen by HRCT. Conclusions Anti-ARS and anti-MDA5 Abs are associated with the therapeutic response of PM/DM-ILD.

Objective This study aims to detect serum levels of monocyte chemoattractant protein-1 (MPC-1) and transforming growth factor-β1 (TGF-β1) in polymyositis/dermatomyositis (PM/DM) patients complicated with interstitial lung disease (ILD), to reveal the significance of the changes in these levels in the pathogenesis of PM/DM complicated with ILD. Methods Serum MCP-1 and TGF-β1 levels in PM/DM patients complicated with ILD, patients with pulmonary infections and normal controls (n = 30, each) were detected using enzyme-linked immunosorbent assay (ELISA), and the correlation between PM/DM complicated with ILD and serum MCP-1 and TGF-β1 levels was analyzed. Results Serum MCP-1 and TGF-β1 levels were both higher in PM/DM patients complicated with ILD compared with patients with pulmonary infections and normal controls. Conclusion Serum MCP-1 and TGF-β1 levels increased in PM/DM patients, and were closely correlated to the complication of ILD. This finding can be used for distinguishing between pulmonary infections and ILD, providing a new diagnostic method for the early prediction of DM/PM complicated with ILD.

Objective We aimed to clarify the clinical significance of serum levels of MMPs in interstitial lung disease (ILD) complicated with PM/DM (PM/DM-ILD). Methods We retrospectively analysed serum levels of seven subsets of MMPs in 52 PM/DM-ILD patients diagnosed at Kyoto University Hospital or Tenri Hospital from January 2005 to December 2014. The patients were sub-grouped based on the presence of anti-amimoacyl-tRNA synthetase antibody (anti-ARS antibody), anti-melanoma differentiation-associated protein 5 antibody (anti-MDA5 antibody) or lack of the antibodies (ARS-ILD, MDA5-ILD and other-ILD groups, respectively) and independently analysed. Eighteen PM/DM patients without ILD and 55 healthy control were also analysed. Associations between serum levels of MMPs and clinical findings including mortality were analysed. Results Among the MMPs analysed, MMP-7 serum levels in the ARS-ILD group were significantly higher compared with those in any of the other groups of PM/DM patients or in healthy controls. On the other hand, in the MDA5-ILD group, serum MMP-7 levels >5.08 ng/ml were associated with worse overall survival both in univariate (P = 0.017; odds ratio 18.0; 95% CI 1.69, 192.00) and multivariate (P = 0.027; odds ratio 14.60; 95% CI 1.11, 192.00) analyses. Immunohistochemical analysis suggested that MMP-7 was expressed in type II alveolar epithelial cells adjacent to the fibrotic lesions. Conclusion Serum MMP-7 levels were higher in anti-ARS antibody-positive PM/DM-ILD patients, while higher serum MMP-7 levels among anti-MDA5 antibody-positive PM/DM-ILD patients were associated with a worse prognosis. Fibrotic processes may be associated with the elevation of serum MMP-7 levels.

Bei konsequentem Screening weisen ca. 40 % der Patienten mit einer Polymyositis/Dermatomyositis (PM/DM) Zeichen einer interstitiellen Lungenkrankheit auf. In der Mehrzahl der Falle handelt es sich dabei um eine „non-specific interstitial pneumonia“, einen histopathologischen Typ, der initial durch ein Entzundungsbild charakterisiert ist und einer immunsuppressiven Therapie zuganglich ist. Die Diagnose der interstitiellen Lungenkrankheit (ILD) im entzundlichen Fruhstadium wird unterstutzt durch die Autoantikorperbestimmung (mehrheitlich Antisynthetaseantikorper), die differenzierte Spirometrie und die Dunnschicht-Computertomographie. Die Wertigkeit dieser Techniken fur die Fruherkennung und Prognoseabschatzung wird dargestellt, und die derzeit ausschlieslich empirischen Therapieoptionen werden erlautert.

Polymyositis-dermatomyositis is a rare systemic autoimmune disease which belongs to the class of idiopathic inflammatory myopathies. The disease exhibits high inter-individual variability, but chronic myositis is a common feature. As different manifestations often appear in atypical forms, establishing the precise diagnosis can be rather complicated. The prognosis and the patient’s life expectations highly depend on whether the clinician considers this possibility in the diagnostic process or not. The authors present the case of a 50-year-old woman who was referred to hospital with suspected myopathy by her general practitioner. The history of the patient, the overall clinical picture and some marked laboratory abnormalities raised the possibility of polymyositis-dermatomyositis, which was unequivocally confirmed by immunological tests. Drug therapy was started immediately with the administration of high dose corticosteroid (1–2 mg/kg/day methylprednisolone), which was found to be an effective strategy leading to fast and remarkable improvement in the patient’s condition. From the first hospital day, the patient also received insulin therapy in order to prevent any potential corticosteroid-induced imbalance in her carbohydrate metabolism. The long-term patient management was provided by an interdisciplinary team the members of which (both clinicians and other health care professionals) worked according to a co-ordinated, complex care plan, and managed not only the “physiological functions” but the different psychological and social problems as well, which are usually associated with the disease. The follow-up period of this polyphase disease process lasted for 4.5 years, during which only two relapses occurred, and muscle strength typically varied between 3 and 4 on a five grade scale with the exception of the relapse periods. Good outcome was attributed to the strict follow-up and individualized therapy/care. Orv. Hetil., 2012, 153, 467–474.

Idiopathic inflammatory myopathies are a heterogeneous group of autoimmune disorders predominantly affecting skeletal muscles, resulting in muscle inflammation and weakness. The 3 most common inflammatory myopathies are polymyositis (PM), dermatomyositis (DM), and inclusion body myositis. This review details the clinical findings noted in PM, DM, and the emerging entity of autoimmune necrotizing myopathy.

Rheumatische Erkrankungen oder Kollagenosen sind eine heterogene Gruppe von Systemerkrankungen, bei denen die Lunge ein haufig mitbeteiligtes Organ ist. Die Beteiligung der Lunge kann vielfaltige Ursachen haben, wie Infektionen, Medikamententoxizitat und spezifische Manifestationen des der Grunderkrankung zugrunde liegenden immunologischen Prozesses. Dabei kann die Lunge in all ihren Gewebskompartimenten von einer Schadigung betroffen sein. Aufgrund ihrer auserordentlich breiten Differerenzialdiagnose stellen pulmonale vaskulitische Syndrome noch immer eine Herausforderung fur den Pathologen dar. Die Lunge ist haufig betroffen bei den mit ANCA-assoziierten primar systemischen Vaskuliden (PSV); die ubrigen PSV fuhren nur selten zu einer Mitbeteiligung der Lunge. Histomorphologisch konnen eine Kleingefasvaskulitis mit neutrophiler Alveolitis und diffuser alveolarer Hamorrhagie sowie extravaskulare intraparenchymale oder peribronchiale Granulome auf eine PSV hinweisen. Haufig sind in einer einzelnen Biopsie nicht alle diagnostischen Kriterien nachweisbar; daher ist die Wahl geeigneten Biopsiematerials sowie eine Korrelation mit den klinischen, serologischen und radiologischen Parametern unerlasslich. Nahezu alle Formen der idiopathischen interstitiellen Pneumonien finden sich auch als Muster der Lungenbeteiligung bei Kollagenosen, wobei sich in vielen Fallen nicht nur ein morphologisches Muster, sondern mehrere nebeneinander finden.

In genetically predisposed individuals, viruses, bacteria, or parasitic infectious agents are suspected of inducing autoimmunity and/or exacerbating autoimmune rheumatic diseases (ARD) once self tolerance is broken. Although direct evidence for this association is still lacking, numerous data from animal models as well as from humans support the hypothesis of a direct contribution of pathogens to the induction of several ARD.This review focuses on the possible role of infectious agents as triggers of autoimmunity in systemic lupus erythematosus, polymyositis-dermatomyositis, antiphospholipid antibody syndrome, and primary vasculitis. Indeed, vasculitis may be a clinical manifestation of tin infectious disease (secondary vasculitris).In addition, immune response abnormalities and immunosuppressive medications may be responsible for the high percentage of infectious complications in ARD patients. Recent therapeutic approaches aimed at lowering doses of cytotoxic agents and shortening duration of treatment with the most toxic drugs, have proved to be as effective as conventional regimens. New drugs and strategies aimed at preventing infections could further improve the outcome of ARD patients.

The idiopathic inflammatory myopathies, polymyositis (PM) and dermatomyositis (DM), are evaluated as systemic autoimmune diseases without the pathology determined. Past immunohistochemical findings suggested that the effector response is driven predominantly by CD4 T cells and by humoral immunity in DM, and by cytotoxic T cells in PM. However, histological observations of muscle tissue do not necessarily distinguish DM and PM. Thus, the two diseases including amyopathic DM might represent a spectrum of illness in which some patients suffer only from a muscle disease or from a skin disease. In comparison with research studies on other rheumatic diseases, there are much fewer research studies conducted on PM/DM. The relationship between PM and DM is not clear yet. We reviewed past clinical and basic research on the pathology of PM/DM, including research on relevant T cells, B cells and cytokines.

Pneumomediastinum is a rare complication of dermatomyositis (DM) and Polymiositis (PM). We report here three cases of PM/DM who developed pneumomediastinum. First case was 61 years old woman with amyopathic dermatomyositis (aDM). Her aDM was complicated with skin ulceration due to vasculopathy, but complicated interstitial pneumonia was not severe. She developed subcutaneous emphysema and pneumomediastinum. Second case was 57 years old woman with DM, who had intractable skin phenomena and mild interstitial pneumonia. The patient became subcutaneous emphysema and pneumomediastinum following severe vasculopathy of skin. The last case was 63 years old man with PM. His PM was complicated with interstitial pneumonia. He had intractable respiratory symptom. Ten years later, he became subcutaneous emphysema and pneumomediastinum following pneumothorax. First and second cases suggest that their pneumomediastinum were due to vasculopathy. On the other hand, pneumomediastinum of the last patient seemed to be associated with interstitial pneumonia and steroid.

In genetically predisposed individuals, viruses, bacteria, or parasitic infectious agents are suspected of inducing autoimmunity and/or exacerbating autoimmune rheumatic diseases (ARD) once self tolerance is broken. Although direct evidence for this association is still lacking, numerous data from animal models as well as from humans support the hypothesis of a direct contribution of pathogens to the induction of several ARD. This review focuses on the possible role of infectious agents as triggers of autoimmunity in systemic lupus erythematosus, polymyositis-dermatomyositis, antiphospholipid antibody syndrome, and primary vasculitis. Indeed, vasculitis may be a clinical manifestation of tin infectious disease (secondary vasculitris). In addition, immune response abnormalities and immunosuppressive medications may be responsible for the high percentage of infectious complications in ARD patients. Recent therapeutic approaches aimed at lowering doses of cytotoxic agents and shortening duration of treatment with the most toxic drugs, have proved to be as effective as conventional regimens. New drugs and strategies aimed at preventing infections could further improve the outcome of ARD patients.

The study aims to comprehensively assess the profiles of myositis-specific autoantibodies (MSAs) in Chinese patients with polymyositis (PM)/dermatomyositis (DM) and compare them with a Japanese cohort. One hundred forty-five Chinese patients (68 classic DM, 25 clinically amyopathic DM [CADM], and 52 PM) and 165 Japanese patients (56 classic DM, 52 CADM, and 57 PM) were recruited. MSAs were measured with immunoprecipitation, enzyme-linked immunosorbent assay, or immunoprecipitation-immunoblotting. MSA frequencies were compared. The overall frequency of anti-melanoma differentiation-associated gene 5 (MDA5) antibodies was significantly higher in the Chinese patients than in the Japanese cohort (36.6 % [53/145] versus 15.8 % [26/165], respectively, P 0.001), whereas the frequencies of anti-signal recognition particle (SRP) antibodies (1.4 % [2/145] versus 7.9 % [13/165], respectively, P = 0.008) and anti-aminoacyl-transfer RNA synthetase (anti-ARS) antibodies (27.6 % [40/145] versus 40 % [66/165], respectively, P = 0.02,) were significantly lower. The significantly lower frequency of anti-ARS antibodies and significantly higher frequency of anti-MDA5 antibodies in the Chinese patients were observed in the classic DM subset (14.7 % [10/68] versus 46.4 % [26/56], respectively, P 0.001, and 45.6 % [31/68] versus 5.4 % [3/56], respectively, P 0.001) and CADM subset (8.0 % [2/25] versus 28.8 % [15/52], respectively, P = 0.04, and 88.0 % [22/25] versus 44.2 % [23/52], respectively, P = 0.0002), but not in the PM subset. The first detailed profile of MSAs in Chinese patients with PM/DM was established. The differences in MSA frequencies in the Chinese cohort and Japanese cohort suggest underlying genetic and/or environmental differences between these two populations. Key Messages • A significantly higher frequency of anti-melanoma differentiation-associated gene 5 (MDA5) antibodies was observed in Chinese patients with polymyositis/dermatomyositis (PM/DM) than in Japanese patients. • Our findings suggest that distinct genetic and/or local environmental factors affect Chinese and Japanese patients with PM/DM, who have been considered a "homogeneous" population in previous studies.

Bronchiolitis obliterans organizing pneumonia (BOOP) is defined by buds of granulation tissue within lung distal airspaces. The diagnosis requires the histopathologic evidence of organizing pneumonia along with a suggestive clinical and radiographic pattern. This disorder is characterized by a good response to corticosteroids and an excellent prognosis. It can occur in association with a broad spectrum of clinical conditions or can be isolated, in this last case named cryptogenic organizing pneumonia. We searched for BOOP in patients with autoimmune rheumatic diseases (ARD) in the literature, and we found 32 well-documented cases. We reported here demographic features, manifestations, treatment and outcome of patients with BOOP associated with ARD. Notably, BOOP can be the presenting feature in some patients with ARD; thus, a close follow-up of patients with BOOP is recommended.

In SLE, scleroderma, and PM/DM, infections are important causes of morbidity and mortality. This increased risk for developing infections is the result of immune abnormalities and of organ system manifestations associated with these diseases and their treatments. Common bacteria are responsible for most mild and lethal infections; however, opportunistic microorganisms cause death in some patients, particularly in those receiving high doses of corticosteroid and immunosuppressive therapy. Various viral and fungal infections also contribute to the morbidity and mortality associated with these diseases. Regardless of the cause of infections, adequate and prompt recognition and proper treatment of the infected patient are imperative. Patients who have these diseases, especially when receiving high doses of corticosteroids and immunosuppressive therapy, need to be monitored closely for these infections. This care and concern is necessary to ensure optimal patient outcomes, both in morbidity and mortality.

Objective.To identify early echocardiographic abnormalities at the time of diagnosis of polymyositis (PM) and dermatomyositis (DM) and follow the echocardiographic findings during the first 3 months of therapy.Methods.We included 30 PM/DM patients (23/7) with a mean age of 42.3 ± 1.6 years and without cardiovascular symptoms. Age-matched healthy patients served as controls. Clinical characteristics were recorded. Traditional echocardiography and tissue Doppler imaging (TDI) were performed to measure systolic [ejection fraction, right ventricular fractional area change (RV FAC), lateral and tricuspid annulus s velocities] and diastolic echocardiographic variables (mitral inflow velocities: E, A; deceleration time: DT; lateral and tricuspid annulus e′, a′ velocities, lateral E/e′).Results.The left and right ventricular systolic dysfunction detected by TDI at the time of the PM/DM diagnosis improved, and characteristic values at the end of the followup period were comparable to those of the controls (lateral s: 10.6 ± 0.2, 8.7 ± 0.4, 9.6 ± 0.3, 11.3 ± 0.2 cm/s; RV FAC: 45.2 ± 2.3, 36.9 ± 1.5, 42.2 ± 1.3, 46.9 ± 1.2%; tricuspid s: 13.3 ± 0.2, 9.5 ± 0.4, 10.3 ± 0.3, 11.6 ± 0.5 cm/s; control, 0, 1, and 3 mos, respectively). Measurements indicated the development of diastolic dysfunction at 3 mos (E/A: 1.4 ± 0.1, 1.29 ± 0.05, 1.03 ± 0.05, 0.92 ± 0.05; DT: 148.6 ± 3.6, 157.3 ± 5.7, 168.3 ± 6.0, 184.3 ± 6.2 ms; lateral e′: 12.8 ± 0.3, 12.1 ± 0.5, 10.2 ± 0.6, 10.8 ± 0.8 cm/s; E/e′: 5.6 ± 0.1, 5.0 ± 0.22, 6.92 ± 0.46, 7.64 ± 0.47; control, 0, 1, and 3 mos, respectively).Conclusion.TDI is a useful method to detect early cardiac abnormalities complementing the conventional echocardiographic measurements. LV and RV systolic dysfunction found in the acute phase significantly improved during the first 3 months of therapy; however, deterioration of diastolic dysfunction was also observed.

We herein report the clinical and laboratory characteristics of two anti-OJ (anti-isoleucyl-tRNA synthetase) autoantibody-positive interstitial lung disease patients with polymyositis/dermatomyositis (PM/DM). We compared these characteristics with previously published findings. Previous reports and our present cases show that anti-OJ autoantibody-positive interstitial lung disease (ILD) patients with PM/DM lack the manifestations of Raynaud's phenomenon and sclerodactyly and show good prognoses and responses to glucocorticoid therapy. These results indicate that the presence of anti-OJ autoantibodies may be useful for predicting the prognosis of ILD and its clinical course in PM/DM patients.

The aim of this study is to determine whether serum KL-6 and surfactant protein D (SP-D) levels predict the prognosis of patients with interstitial pneumonia (IP) in cases of polymyositis (PM) and dermatomyositis (DM).Fifty consecutive patients with PM (n = 17) or DM (n = 33) and active IP, 6 of whom died of respiratory failure, were enrolled in this study. Serum KL-6 and SP-D levels were measured every 2-4 weeks. Medical records were reviewed retrospectively. Univariate analyses and multivariate analyses with a logistic regression model were conducted.Serum KL-6 and SP-D levels were elevated in patients with active IP. At the time of diagnosis of active IP, the serum KL-6 level was within the normal range in 28 % of patients and the SP-D level was within the normal range in 46 % of patients. Serum KL-6 level increased up to 3 months after starting treatment and then decreased gradually to baseline, whereas SP-D level peaked within the first 4 weeks after treatment and decreased rapidly to normal levels. Patients with poor prognosis showed increases in KL-6 and SP-D levels during the first 4 weeks after treatment, which was confirmed by uni- and multivariate analyses. Comparing the marker levels at 2-4 weeks after treatment with those at 0 weeks, an increase in the ratio over 1.70 for KL-6 and over 1.75 for SP-D, and an increase in KL-6 over 850 U/ml during the first 4 weeks after treatment, were poor prognostic factors.Increases in serum KL-6 and SP-D levels during the first 4 weeks after starting therapy, but not their levels at any one time point, predict poor prognosis in patients with PM/DM. When marked increases of KL-6 and SP-D levels during the first 4 weeks are found or are predicted by serial measurement of the markers, patients have risks of poor prognosis and additional therapy should be considered.

Objective. Recent case reports have raised the possibility that use of injectable bovine collagen may be associated with polymyositis/dermatomyositis (PM/DM). Because the number of collagen users is high, PM/DM would be expected to occur in some for reasons unrelated to the collagen use. A central issue is whether the number of observed cases exceeds the number expected on the basis of background rates alone. The present study was undertaken to investigate this. Methods. The number of observed cases was determined by review of the medical records of collagen users who had reported illnesses consistent with PM/DM. Because of the uncertainty about diagnosis of PM/DM, population incidence rates, number of patients treated with collagen, and duration of followup after treatment, we examined a range of estimates of each of these factors that would affect the expected number of cases. Results. From reports among collagen users, 7 probable or definite cases of PM/DM were confirmed. In contrast, 13 cases would be expected based on the best estimates of relevant factors. Under the most conservative estimates for factors that influence the number of expected cases, 12 cases would be expected, while worst-case assumptions would yield an expected 130 cases. Conclusion. The consistent finding of fewer-than-expected PM/DM cases among collagen users suggests that collagen use is not associated with the development of PM/DM.

The increased incidence of pulmonary hypertension and its association with decreased survival is well-recognised in patients with systemic sclerosis. This association is not widely appreciated in patients with polymyositis-dermatomyositis. We report clinical and hemodynamic characteristics and response to vasoactive therapy in three patients with polymyositis-dermatomyositis and pulmonary hypertension and discuss them in light of the available literature.

THE polymyositis and dermatomyositis complex encompasses a heterogeneous group of acquired muscle diseases called inflammatory myopathies because muscle weakness and inflammatory infiltrates within the skeletal muscles are the principal clinical and histologic findings.1 2 3 4 5 6 7 8 9 10 11 12 Traditionally, polymyositis and dermatomyositis have been viewed as pathogenetically similar and part of the spectrum of "idiopathic" inflammatory myopathies,9 , 10 , 13 , 14 despite the variability in their clinical and laboratory characteristics, prognosis, and response to therapy. With the evolution over the past 10 years of rather well defined clinical, demographic, histologic, and immunopathological criteria and the identification of inclusion-body myositis as a distinct type of polymyositis,15 the inflammatory myopathies now . . .