Idiopathic inflammatory myopathies (IIM) include the main subgroups polymyositis (PM), dermatomyositis (DM), inclusion body myositis (IBM) and juvenile DM (JDM). The mentioned subgroups are characterised by inflammation of skeletal muscles leading to muscle weakness and other organs can also be affected as well. Even though clinically significant heart involvement is uncommon, heart disease is one of the major causes of death in IIM. Recent studies show an increased prevalence of traditional cardiovascular risk factors in JDM and DM/PM, which need attention. The risk of developing atherosclerotic coronary artery disease is increased twofold to fourfold in DM/PM. New and improved diagnostic methods have in recent studies in PM/DM and JDM demonstrated a high prevalence of subclinical cardiac involvement, especially diastolic dysfunction. Interactions between proinflammatory cytokines and traditional risk factors might contribute to the pathogenesis of cardiac dysfunction. Heart involvement could also be related to myocarditis and/or myocardial fibrosis, leading to arrhythmias and congestive heart failure, demonstrated both in adult and juvenile IIM. Also, reduced heart rate variability (a known risk factor for cardiac morbidity and mortality) has been shown in long-standing JDM. Until more information is available, patients with IIM should follow the same recommendations for cardiovascular risk stratification and prevention as for the corresponding general population, but be aware that statins might worsen muscle symptoms mimicking myositis relapse. On the basis of recent studies, we recommend a low threshold for cardiac workup and follow-up in patients with IIM.

Only patients with JDM and no controls had subclinical left ventricular diastolic dysfunction; the patients with elevated E/E' also had high prevalence of pathological ECG and hypertension. High disease activity 1-year post diagnosis predicted high E/E' at follow-up. The findings suggest that subclinical heart disease is related to the systemic nature of JDM.

Long-axis strain was reduced in JDM patients compared with controls, suggesting systolic dysfunction. Impaired systolic and diastolic function was predicted by DAS skin 1 year. This indicates a common pathway to two different cardiac manifestations in JDM, perhaps with similar pathogenesis as skin affection.

ObjectiveTo compare cytokine profiles in patients with juvenile dermatomyositis (JDM) after medium to long-term follow-up with matched controls, and to examine associations between cytokine levels and disease activity, disease duration and organ damage.MethodsFifty-four JDM patients were examined median 16.8 years (2–38) after disease onset (follow-up) and compared with 54 sex- and age-matched controls. Cytokine concentrations in serum were quantified by Luminex technology. In patients, disease activity score (DAS), myositis damage index (MDI) and other disease parameters were collected by chart review (early parameters) and clinical examination (follow-up).ResultsSerum levels of eotaxin, monocyte chemoattractant protein-1 (MCP-1) and interferon-inducible protein 10 (IP-10) were elevated in JDM patients compared to controls (31.5%, 37.2% and 43.2% respectively, all p<0.05). Patients with active (n = 28), but not inactive disease (n = 26) had a higher level of MCP-1 than their respective controls. Levels of eotaxin and MCP-1 correlated with disease duration (r = 0.47 and r = 0.64, both p<0.001) and age in patients, but not with age in controls. At follow-up, MDI was associated with MCP-1(standardized β = 0.43, p = 0.002) after adjusting for disease duration and gender. High MDI 1 year post-diagnosis predicted high levels of eotaxin and MCP-1 at follow-up (standardized β = 0.24 and 0.29, both p<0.05) after adjusting for disease duration and gender.ConclusionPatients with JDM had higher eotaxin, MCP-1 and IP-10 than controls. High eotaxin and MCP-1 at follow-up was predicted by early disease parameters, and MCP-1 was associated with organ damage at follow-up, highlighting a role of these chemokines in JDM.

JDM patients had reduced HRV, which was associated with elevated inflammatory markers, active disease and reduced myocardial function. This suggests reduced vagal control of the heart; further studies are needed to determine whether this is also associated with cardiac morbidity or mortality.

In 12 weeks of active treatment, we compared the efficacy and safety of a new (micronized) formulation of fenofibrate (F) (200 mg/day) with that of simvastatin (S) (20 mg/day), an inhibitor of hydroxy-methyl-glutaryl coenzyme A (HMG-CoA)-reductase. Men and women with primary hyperlipoproteinemia (HLP) with low-density lipoprotein (LDL) cholesterol level 180-300 mg/dl and triglyceride level < 500 mg/dl had dietary treatment for 8 weeks, and 133 (2 of 3 type IIa, 1 of 3 type IIb HLP) were randomized. The decrease in total cholesterol differed between type IIa patients (F - 17.9 vs. S - 25.8%), the decrease in triglyceride levels between the type II b groups (F - 52.8 vs. S - 14%), whereas the degree of decrease in LDL cholesterol (F - 20.9 vs. S - 34.9%) differed among all patients. Despite the difference in LDL cholesterol decrease, no difference was noted in total apolipoprotein (apo) B lowering (F - 20.8 and S - 26.5%). Increases in high-density lipoprotein (HDL) cholesterol (F + 18.5 vs. S + 15%) differed specifically in type IIb patients (F + 33.6 vs. S + 11.4%), accompanied by a more pronounced increase in apo AI with fenofibrate (F + 10.5% vs. S no change). Improvement in the ratios of total cholesterol/HDL cholesterol and apo AI/apo B occurred similarly with both drugs. Only fenofibrate, not simvastatin, decreased both fibrinogen (-10.3 vs. + 3.6%) and uric acid (-25% vs. no change) in type IIa and type IIb patients. Safety parameters reflected drug-specific known side effects, underscoring the safety of both drugs in addition to their efficacy in lipid lowering. Besides its advantages in type IIb hyperlipidemia, micronized fenofibrate proved a potent drug in decreasing total and LDL cholesterol and in very effectively decreasing apo B-containing lipoproteins, which is a recommendation for its use in primary hypercholesterolemia.

Background Visceral leishmaniasis (VL) is a protozoal disease that may be aggravated by immunosuppression. In recent years, a growing number of patients with chronic diseases use biological treatment. When such immunosuppressed patients travel to endemic areas, they are facing the risk of VL. Increased incidence of leishmaniasis is reported in endemic areas like the Mediterranean basin, an area frequently visited by Norwegian tourists. This may lead to an increased number of patients, many presenting to health personnel unfamiliar with the disease, in their home countries. Methods We reviewed the files of seven immunosuppressed patients with VL, admitted to Oslo and Haukeland University Hospitals in Norway in the period 2009–2018. Results The patients were 41–83 (median 66) years of age; four had rheumatic disease all of whom used methotrexate; one had advanced HIV infection, one had inflammatory bowel disease and one had myelofibrosis. Leishmania infantum was confirmed in five patients by polymerase chain reaction (PCR) and sequencing. In the remaining two patients, a definite Leishmania species could not be identified. All patients had a history of recent recreational travel to Spain. Most patients underwent extensive diagnostic work-up before diagnosed with VL. All received treatment with liposomal amphotericin B and all were cured; albeit two after re-treatment due to relapse. Conclusions Visceral leishmaniasis is a potentially life-threatening but usually treatable condition. It is endemic in Southern Europe, including popular tourist destinations such as the Mediterranean basin. It is relatively unknown to most medical practitioners in non-endemic areas and clinical vigilance is required to identify those who are infected.

Submaximal exercise capacity was reduced in patients with JDM, particularly those with active disease. This reduction was associated with muscle and lung dysfunction and poorer HRQOL.