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Unexplained Fever Associated With Hypersensitivity and Auto-Immune Diseases
Published in Benedict Isaac, Serge Kernbaum, Michael Burke, Unexplained Fever, 2019
The diagnosis of adult Still’s disease is either easy to establish if the three major symptoms are present, or very difficult if one of them is lacking.76 In the latter circumstance one must think of it and, when an infectious process is certainly discarded, a therapeutic corticosteroid trial (0.5 to 1 mg/kg/d) must be initiated for a few days: its dramatic and lasting efficacy is evocative of the diagnosis, but not specific.
Therapeutic Apheresis in Children
Published in James L. MacPherson, Duke O. Kasprisin, Therapeutic Hemapheresis, 2019
In a survey of pediatric rheumatologists, seven of 23 had used apheresis, treating a total of 20 patients.66 Ten patients received some improvement, but the remainder did not. The diseases treated were not specified but the majority of rheumatologists considered it a research tool or for use in patients with life-threatening complications. Wallace et al. reported three patients with JRA who were 16 to 21 years old. One patient with Still’s disease did not respond to apheresis, while one of two with the polyarticular form of the disease improved. All three patients were receiving medications concurrently. The type of apheresis was not specified.67
Rheumatology
Published in Shibley Rahman, Avinash Sharma, MRCP Part 2 Best of Five Practice Questions, 2018
Shibley Rahman, Avinash Sharma
Which of the following clinical features is unusual in adult onset Still’s disease? arthralgialung fibrosisfeverpericarditissplenomegaly
First-line treatment of multiple myeloma in both transplant and non-transplant candidates
Published in Expert Review of Anticancer Therapy, 2023
Albert Oriol, Laura Abril, Gladys Ibarra
Still, disease control is no longer the aim of frontline treatment. The achievement of deeper and faster responses may decrease the chances of the emergence of resistant clones and, ultimately, may actually cure a subset of patients. Up to 20% of patients treated with the currently available triplet induction therapy and high-dose melphalan are free of progression at 10 years. MoAbs are successfully being incorporated into initial treatment, and provide significant improvement in terms of depth of response and PFS. The next generation of induction combinations is going to include one agent of each of the major drug classes proven to be effective in multiple myeloma in order to obtain sustained MRD negativity. Moreover, a fast achievement of a negative MRD may allow to modulate subsequent therapy according to risk or toxicity.
Interleukin-6 inhibition: a therapeutic strategy for the management of adult-onset Still’s disease
Published in Expert Opinion on Biological Therapy, 2022
Adult-onset Still’s disease, a systemic inflammatory disease of unknown etiology, is characterized by high fever, arthritis, evanescent skin rashes, lymphadenopathy, hepatosplenomegaly, neutrocytosis, and hyperferritinemia [1]. It was first described by Dr. Eric Bywaters in 1971 as a systemic inflammatory condition similar to that seen in childhood-onset Still’s disease [2], which is now called systemic juvenile idiopathic arthritis. Although there is still debate over whether adult-onset Still’s disease and systemic juvenile idiopathic arthritis are the same disease at different ages of onset, recent studies have shown homology between the two diseases in terms of their pathogenesis and treatment strategies [3]. Because of the rarity of adult-onset Still’s disease, it is less intensively studied compared with other rheumatic diseases, and its pathogenesis and optimal treatment have still not been fully elucidated despite the difficulty in its diagnosis and the poor outcomes in some affected patients [4,5].
Unexpectedly lower proportion of placental transferred tocilizumab relative to whole immunoglobulin G: a case report
Published in Scandinavian Journal of Rheumatology, 2020
M Moriyama, Y Wada, T Minamoto, M Kondo, M Honda, Y Murakawa
TCZ is an IgG1 monoclonal antibody that can be transferred to the foetus via the neonatal Fc receptor on the placenta after the second trimester, similarly to other IgG1-type biologics such as adalimumab and infliximab. The median levels of infliximab and adalimumab in the cord are 160% and 153%, respectively, of those in the mother (7). In our case, although the IgG level in the cord was 145% of that in the mother’s serum, the concentration of TCZ in the cord was unexpectedly only 7.6% of that in the mother’s serum at delivery. During the preparation of this report, another group published a case report of adult-onset Still’s disease, with similar observations (6). Therefore, this is the second report to demonstrate a lower proportion of placental transport of TCZ. However, our case is still important in confirming that the relatively low level of TCZ placental transport is a common characteristic of TCZ.