The licensing of belimumab for use in active systemic lupus erythematosus (SLE) has ushered in a new era for the connective tissue diseases (CTDs) based on a better understanding of disease pathogenesis. Genome-wide association studies have been used to identify a number of new therapeutic pathways. Anti-B-cell therapy is proposed for other CTDs such as primary Sjogren’s syndrome (PSS) and inflammatory myositis. Other biological therapies – for example targeted at interferons and the innate immune system may also be on the horizon for several of these conditions. Eculizumab, a complement inhibitor has been used successfully in cases of catastrophic anti-phospholipid syndrome.
One theme across the connective tissue diseases is to optimize existing therapies and reduce corticosteroid and cyclophosphamide usage. In SLE for example, the use of mycophenolate mofetil to replace cyclophosphamide in many cases of lupus nephritis and steroid-free regimes are also being evaluated. IVIG may also have a role in reducing steroid usage e.g. in myositis flare.
In scleroderma, regular screening has improved survival, particularly with regard to earlier intervention for lung fibrosis and pulmonary hypertension. Interstitial lung disease is also common in myositis. Careful attention to nutrition and other gastrointestinal complications have improved patients’ general health.
Another theme is improved classification of patients into more specific subgroups for targeted management. New antibodies with clinical relevance continue to be discovered: For example - in myositis anti-TIFF antibodies are associated with cancer associated dermatomyositis and anti-MDA5 antibodies with interstitial lung disease. In scleroderma anti-U11/U12 RNP antibodies identify patients with lung fibrosis.
International collaborations have been an important driver of new approaches to assessment and therapies. In SLE the SLICC and BILAG groups have been long-established. EUSTAR in scleroderma, EUMYONET in myositis and the UKPSSR and EULAR working group in Sjogren’s are all examples of this trend.
Acknowledgements With thanks to: Dr David D’Cruz, Prof Chris Denton, Prof Caroline Gordon, Dr Patrick Gordon and Dr Voon Ong.
Disclosure of Interest S. Bowman Consultant for: Merck-Serono