8 research outputs found
Polymyositis/Dermatomyositis: the current position
Polymyositis/dermatomyositis are a heterogeneous group of diseases characterised by
skeletal muscle inflammation and necrosis.' 2
Since an excellent clinical description in 1903 by
Steiner of dermatomyositis, which is essentially
still valid,3 much progress has been made
towards our understanding of this group of
diseases. The most widely used clinical classification of idiopathic inflammatory myopathies is
the one proposed by Bohan and Peter in 1975.4
That was a landmark, providing guidelines in
clinical practice to accurate diagnosis of inflammatory myopathies and standardisation of
studies. Nonetheless, the classification was
based on clinical data. In view of recent
histological and immunological studies the classification proposed by Karpati et al in 1987
seems better to fit our current view of such
diseases5 (table 1). In this review we focus on
recent developments in polymyositis/dermatomyositis, analysing separately the currently
considered third major form of inflammatory
myopathy-inclusion body myositis
The Use of Cyclosporine A in Rheumatology: a 2016 Comprehensive Review
Cyclosporine A, an inhibitor of calcineurin, exerts an immunomodulator action interfering with T cell activation. Even though novel therapeutic tools have emerged, CyA still represents a suitable option in several clinical rheumatology settings. This is the case of refractory nephritis and cytopenias associated with systemic lupus erythematosus. Furthermore, CyA is a valued therapeutic tool in the management of uveitis and thrombophlebitis in course of Behçet's disease. Topical CyA has been proven to be beneficial in the dry eye of Sjogren's syndrome, whereas oral treatment with CyA can be considered for the severe complications of adult onset Still's disease. CyA provides a therapeutic option in psoriatic arthritis, being rather effective in skin disease. CyA is currently regarded as a second-line option for patients with inflammatory myopathies refractory to standard regimen. CyA is used even in paediatric rheumatology, in particular in the management of juvenile dermatomyositis and macrophage activation syndrome associated with systemic juvenile idiopathic arthritis. Importantly, CyA has been shown to suppress the replication of HCV, and it can thus be safely prescribed to those patients with chronic hepatitis C. Noteworthy, CyA can be administered throughout the gestation course. Surely, caution should be paid to CyA safety profile, in particular to its nephrotoxicity. Even though most evidence comes from small and uncontrolled studies with few randomised controlled trials, CyA should be still regarded as a valid therapeutic tool in 2016 rheumatology