Vasculopathy-related clinical and pathological features are associated with severe juvenile dermatomyositis.

OBJECTIVE: Outcome of JDM is highly heterogeneous. Our objective was to determine clinical and muscle biopsy features associated with poor outcome and response to treatment. METHODS: Clinical data and muscle biopsy were obtained from a monocentric cohort of 29 patients. Clinical subgroups were defined by latent class model analysis of initial and follow-up parameters. Myopathological features were analysed using validated scores. Capillary loss was determined on reconstructions of transversal sections and assessed in the different age groups to take into account variations of muscle capillarization during post-natal development. Regression models were used to identify initial predictors of therapeutic response. RESULTS: Two distinct homogeneous subgroups of patients were identified according to clinical severity and pathological findings. The smallest group of patients (7/29) presented with severe JDM. Compared with the other group (22/29), patients had more severe muscle weakness at disease onset, low remission rate at 12 months, frequent subcutaneous limb oedema or gastrointestinal (GI) involvement and higher myopathological scores (capillary dropout, perifascicular necrosis/regeneration, fibres with internal myonuclei and fibrosis subscores). Relevance of capillary dropout to JDM severity was substantiated by age-based analysis, confirming its major role in JDM pathophysiology. Most of these manifestations could be related to vasculopathy (limb oedema, GI involvement, capillary dropout). Furthermore, Childhood Myositis Assessment Scale <34 with either GI involvement or muscle endomysial fibrosis at disease onset were the best predictors of poor response to treatment. CONCLUSION: Vasculopathy is prominent in severe JDM. Simple criteria can be used at initial evaluation to identify patients requiring a more intensive therapy.

Effect of land cover and ecosystem mapping on ecosystem-risk assessment in the Little Karoo, South Africa.

Extinction-risk assessments aim to identify biological diversity features threatened with extinction. Although largely developed at the species level, these assessments have recently been applied at the ecosystem level. In South Africa, national legislation provides for the listing and protection of threatened ecosystems. We assessed how land-cover mapping and the detail of ecosystem classification affected the results of risk assessments that were based on extent of habitat loss. We tested 3 ecosystem classifications and 4 land-cover data sets of the Little Karoo region, South Africa. Degraded land (in particular, overgrazed areas) was successfully mapped in just one of the land-cover data sets. From <3% to 25% of the Little Karoo was classified as threatened, depending on the land-cover data set and ecosystem classification applied. The full suite of threatened ecosystems on a fine-scale map was never completely represented within the spatial boundaries of a coarse-scale map of threatened ecosystems. Our assessments highlight the importance of land-degradation mapping for the listing of threatened ecosystems. On the basis of our results, we recommend that when budgets are constrained priority be given to generating more-detailed land-cover data sets rather than more-detailed ecosystem classifications for the assessment of threatened ecosystems.