Disease duration as an indicator of the efficacy of liraglutide in patients with type 2 diabetes mellitus.

Comment on the article of Usui et al. Retrospective cohort study of obese patients with type 2 diabetes mellitus (n = 69) demonstrates that the glucose-lowering effect of liraglutide as add on therapy to insulin relies on the remaining beta-cell function in type 2 diabetes. Shorter disease duration implies a more favourable prognosis for response on instantaneous substitution of insulin with liraglutide (HR 2.39 (95% CI: 1.20-4.76).

Pulmonary arterial hypertension, a novelty in idiopathic inflammatory myopathies: insights and first experiences with vasoactive therapy.

To characterise the different types of pulmonary hypertension (PH) among idiopathic inflammatory myopathy (IIM). A retrospective case series with assessment of PH by right heart catheterisation, extent of interstitial lung disease (ILD) and outcome of vasoactive therapy.The group of patients with IIM with PH (n=9) showed a median age at PH diagnosis of 62 years (IQR 48-71 years; eight women), seven diagnosed with polymyositis and two with dermatomyositis; median disease duration of 5.7 years and five patients with a positive anti-Jo1 antibody. We found one patient to be classified in PH WHO group 2 (left heart disease), five patients in WHO group 3 (lung disease) and three patients in WHO group 1 (pulmonary arterial hypertension (PAH)). During median observed follow-up of 24 months, mortality for the total group was 44%. Surprisingly, we found a relevant group (33%) of patients with IIM who suffered from non-ILD-PH, which reflects the presence of PAH phenotype. This result should lead to more awareness among treating physicians that complaints of dyspnoea among patient with IIM could be related to PAH and not only ILD. The role of vasoactive therapy remains to be defined in patients with IIM suffering from PAH or PH-ILD.

Patients with Systemic Sclerosis/polymyositis Overlap Have a Worse Survival Rate Than Patients Without It.

OBJECTIVE: Studies on mortality associated with patients with systemic sclerosis (SSc) and myopathy have been limited by heterogeneous definitions of muscle involvement. The objective of this study is to determine whether homogeneous-defined SSc/polymyositis overlap (SSc-PM overlap) is associated with a worse survival rate compared with SSc without PM. METHODS: Data from the Nijmegen Systemic Sclerosis cohort were used. Incidence rates were calculated from the observed number of deaths and followup time. Survival analysis using Cox proportional hazard modeling was performed to compare survival among patients with SSc and patients with SSc-PM overlap, including controlling for confounders. All patients with SSc-PM fulfilled the Bohan and Peter criteria for PM. RESULTS: There were 24 patients with SSc-PM (5.7%) and 396 patients with SSc (94.2%). The 5- and 10-year cumulative survival rates from diagnosis were 82% and 68% for the SSc-PM group and 93% and 87% for the SSc group, respectively. Multivariate survival analysis revealed an adjusted HR of 2.34 (95% CI 1.09-5.02) for SSc-PM compared with SSc, with age at diagnosis, modified Rodnan skin score, diffuse cutaneous subtype, and male sex included as confounders. The most common cause of death among patients with SSc-PM overlap was cardiopulmonary involvement (63%), which was similar to the patients with SSc (51%). CONCLUSION: Patients with SSc-PM overlap have a worse survival rate compared with patients with SSc.

Increased fascial thickness of the deltoid muscle in dermatomyositis and polymyositis: An ultrasound study.

INTRODUCTION: We investigated the use of ultrasound to detect fascial thickening of the deltoid, vastus lateralis (VL), and rectus femoris (RF) muscles in dermatomyositis (DM) and polymyositis (PM). METHODS: Fascial thickness of 7 DM and 5 PM patients was compared with that of healthy controls (n = 54). RESULTS: The deltoid fascial thickness was 2-fold greater in the DM/PM group (1.15 mm vs. 0.54 mm; P < 0.001) compared with healthy controls. Eight patients had markedly thickened deltoid fascia (>5 SD). Only 1 patient with a mild clinical presentation had normal deltoid fascial thickness. Four patients also had fascial thickening of the VL and/or RF. CONCLUSIONS: Deltoid fascial thickness was increased significantly in DM and PM patients, whereas the quadriceps muscle only showed thickened fascia in severely affected patients. This study suggests that a concomitant fasciitis may be present more often than previously believed.

Quantitative muscle ultrasonography in the follow-up of juvenile dermatomyositis.

INTRODUCTION: We explored the use of quantitative muscle ultrasonography (QMUS) for follow-up of juvenile dermatomyositis (JDM). METHODS: Seven JDM patients were evaluated at diagnosis and 1, 3, 6, 12, and 24 months using the Childhood Myositis Assessment Scale (CMAS) and QMUS. Muscle thickness (MT) and quantitative muscle echo intensity (EI) were assessed with QMUS in 4 muscles. RESULTS: Six patients experienced a monocyclic course. At diagnosis EI was slightly increased, and MT was relatively normal. After start of treatment MT first decreased and EI increased, with normalization of EI within 6-12 months (n = 4). One patient had higher EIs at diagnosis and slower normalization, indicating fibrosis, despite early normalization of CMAS. One patient experienced a chronic course, with high EIs and atrophy during follow-up. CONCLUSIONS: QMUS can provide additional information for follow-up of JDM regarding disease severity and residual muscle damage, particularly after normalization of CMAS.

Scleroderma-polymyositis overlap syndrome versus idiopathic polymyositis and systemic sclerosis: a descriptive study on clinical features and myopathology.

INTRODUCTION: The objective was to characterize the clinical and myopathologic features of patients with scleroderma-polymyositis (SSc-PM) overlap compared with a population of patients with systemic sclerosis (SSc) and polymyositis (PM). METHODS: A three-way comparison of patients with SSc-PM overlap (n = 25) with patients with SSc (n = 397) and PM (n = 40) on clinical and myopathologic features and causes of death. One neuropathologist blinded for the diagnosis evaluated all recent available muscle biopsies. Biopsies were scored for presence of inflammation, necrotic muscle fibers, rimmed vacuoles, fibrosis, and immunohistochemical staining. Clinical or myopathologic characteristics were compared by using the χ(2) test or one-way analysis of variance (ANOVA). RESULTS: The prevalence of SSc-PM overlap in the Nijmegen Systemic Sclerosis cohort was 5.9%. The mortality was 32% (eight of 25) in SSc-PM, of which half was related to cardiac diseases. The prevalence of pulmonary fibrosis was significantly increased in SSc-PM (83%) (P = 0.04) compared with SSc (49%) and PM (53%). SSc or myositis-specific antibodies were nearly absent in the SSc-PM group. In almost all biopsies (96%) of SSc-PM patients, necrotic muscle fibers were present, which was significantly increased compared with PM patients (P = 0.02). CONCLUSIONS: Patients with SSc-PM have increased prevalence of pulmonary fibrosis and cardiac disease as the cause of death compared with patients with SSc and PM . In addition, we found that necrotizing muscle fibers with inflammation characterize SSc-PM overlap in muscle biopsies. Further research should focus on underlying mechanisms causing necrosis, inflammation, and fibrosis and their relation to pulmonary involvement and mortality in patients with SSc-PM overlap.