I would like to thank the ORA for your support to attend the 2018 ACR State of the Art. This workshop was an information packed, clinically focused weekend of lectures from well recognized experts in the rheumatology community.
I have no personal conflicts of interest but most speakers did have their own disclosures in their presentations. The references below are taken from their talks and I have not attempted to verify their accuracy. Some of the key take home points for me from the perspective of a fellow-in-training, included:
Dr. Michelle Petri gave an excellent case-based lecture on hematologic challenges in SLE. There is an increasing movement to group atypical hemolytic uremic syndrome, HELLP syndrome, catastrophic antophospholipid antibody syndrome (APS, CAPS), and paroxysmal nocturnal hemoglobinuria (PNH) into a category of diseases with similar treatment options unified by a mechanism of complementopathy. A modification of the Ham test for PNH has been used to distinguish between CAPS, APS, and normal serum (p=0.033-0.037, unpublished figure; Brodsky, RA Blood 2015; 126:2459-65). There is a potential role for eculizumab in these diseases (Shapira et al. Arth Rheum 2012 ;64:2719). She also mentioned that G-CSF can flare SLE given mature neutrophil activation, nettosis, and inhibition of apoptosis (Vasilu IM et al. J Rhem 2006; 33:1878-1880).
Dr. James T. Rosenbaum reviewed the literature and recent guidelines for hydroxychloroquine from the American Academy of Ophthalmology. The major risk factors for retinopathy are: hydroxychloroquine dose >5.0 mg/kg real weight, chloroquine >2.3 mg/kg real weight, >5 years duration of use, subnormal GFR, concomitant tamoxifen use, and maybe underlying macular disease (Marmor MF et al. Opth 2016; 123:1386). A baseline screen is recommended within the first year with visual fields and SD OCT if there is maculopathy and annual screens after 5 years or sooner with risk factors.
Dr. Jill Buyon reviewed neonatal lupus risks, pathophysiology, and types of conduction blocking lesions on pathology, and their management. Current data suggest there is no role for fluorinated steroids in isolated neonatal 3rd degree congenital AV block (AVB) without hydrops as it is not reversible nor is there difference in outcomes with regards to need for pacemakers, survival, or extranodal disease (n=394 with 156 isolated 3⁰ AVB; Izmirly. Ann Rheum Dis 2016). However, she anecdotally has a few personal cases where high risk patients do home fetal doppler checks using off the shelf monitors to identify bradycardia and she has had some success reversing damage providing the condition is rapidly treated. (Cuneo. J Perinatology 2017). In first degree, second degree AVB, or third degree AVB with hydrops there is a role for dexamethasone (Izmirly. Curr Opin Rheum 2012). With regards to outcomes, cardiac dysfunction persists in 17% in the first year of life but normalizes in the majority in later childhood (Saxena et al. ACR 2017). An analysis pooling over 600 SLE anti-SSA/Ro+ patients who were either treated or not treated with hydroxychloroquine during pregnancy suggests that hydroxychloroquine decreases the risk of 3rd degree AVB about 3x fold (6/254 (2.4%) vs 29/374 (7.8%) p=0.004; pooled from Martinez-Sanchez 2017, Tunks 2013, Gayed 2014, Barasalou 2017, Barasaloou 2016). The PATCH study is ongoing an open label prospectively evaluating this in patients with +anti-SSA/Ro antibodies and prior neonatal lupus. Finally, 50% of mothers that develop congenital AVB in pregnancy and are found to have positive anti-Ro antibodies will develop symptoms of rheumatic disease (unreferenced).
Dr. Lianne Gensler discussed imaging and treatment options in spondyloarthritis. Dr. Inman and colleagues in Toronto published an interesting study comparing AP pelvis and Fergusson views grading sacroiliitis and found general agreement with no clear superiority, challenging the traditional view that extra dedicated SI joint films are more sensitive (n=266; Omar A et al. Rheum 2017; 56:1740-1745).
Dr. Philip Seo’s lecture on vasculitis mimics presented humbling cases of diagnosing and treating RCVS vs. PACNS, Behcet’s disease, fibromuscular dysplasia vs. GCA and Takayasu’s arteritis that would be difficult to briefly summarize here.
Dr. Yusuf Yazici presented the new EULAR 2018 guidelines for the treatment for manifestations of Behcet’s disease (Hatemi G et al. Ann Rheum Dis 2018 http://ard.bmj.com/content/early/2018/04/06/annrheumdis-2018-213225 ) and contrasted neurological involvement to MS, gastrointestinal involvement to Crohn’s disease, and reinforced that psoriasis, sacroiliitis and spinal involvement are not typical features of this disease.
Finally, Dr. Janet Pope gave a talk and workshop on scleroderma, evidence for treatment, and a review of the literature. Dr. Pope presented the results of a survey of 62 experts on real-life scleroderma treatment algorithms and changes to the published 2012 algorithms (Fernandez-Codina A et al. EULAR 2017 poster; publication).
Dr. Konstantin Jilkine