Healthcare utilization and unmet needs of patients with antisynthetase syndrome: An international patient survey.

Antisynthease syndrome (ASSD) is a rare, complex and understudied autoimmune disease. Internet-based studies can overcome barriers of traditional on-site research and are therefore very appealing for rare diseases. The aim of this study was to investigate patient-reported symptoms, diagnostic delay, symptoms, medical care, health status, working status, disease knowledge and willingness to participate in research of ASSD patients by conducting an international web-based survey. The multilingual questionnaire was created by an international group of rheumatologists and patients and distributed online. 236 participants from 22 countries completed the survey. 184/236 (78.0%) were female, mean age (SD) was 49.6 years (11.3) and most common antisynthetase antibody was Jo-1 (169/236, 71.6%). 79/236 (33.5%) reported to work full-time. Median diagnostic delay was one year. The most common symptom at disease onset was fatigue 159/236 (67.4%), followed by myalgia 130/236 (55.1%). The complete triad of myositis, arthritis and lung involvement verified by a clinician was present in 42/236 (17.8%) at disease onset and in 88/236 (37.3%) during the disease course. 36/236 (15.3%) reported to have been diagnosed with fibromyalgia and 40/236 (16.3%) with depression. The most reported immunosuppressive treatments were oral corticosteroids 179/236 (75.9%), followed by rituximab 85/236 (36.0%). 73/236 (30.9%) had received physiotherapy treatment. 71/236 (30.1%) reported to know useful online information sources related to ASSD. 223/236 (94.5%) were willing to share health data for research purposes once a year. Our results reiterate that internet-based research is invaluable for cooperating with patients to foster knowledge in rare diseases.

[Getting older with rheumatoid arthritis-is there a burnout of the disease?] / Älterwerden mit rheumatoider Arthritis ­ brennt die Erkrankung aus?

Rheumatoid arthritis (RA) is a chronic inflammatory disease. Synovitis is the main pathology and can lead to a progressive destruction of the joints. It is often said that RA "burns out", implying that the inflammation decreases spontaneously in the long term, mostly severe course of RA and reaches a stage with a stable absence of joint inflammation, even without treatment. To test this concept we analyzed the published evidence. Data of historic long-term inception cohorts of patients who have never been treated with antirheumatic drugs and patients who received conventional disease-modifying antirheumatic drugs (DMARD), show that the disease stays active with sustained radiological progression in the majority of patients. At best, the disease can show a milder course with time or a stage of absence of joint inflammation can be reached if patients responded very well to initial drug treatment. Terminating DMARD treatment in this situation bears the risk of a latent progressive joint destruction, the appearance of extra-articular manifestations and an increase in the cardiovascular risk. Hence there is no evidence for the existence of a "burnt out" RA with stable inactive disease without drug treatment in the long-term course. In a modern treatment strategy of RA following the treat-to-target principle and aiming at remission, the term "burnt out" RA should no longer be used.