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Connective Tissue Diseases: ENT Complications
Published in John C Watkinson, Raymond W Clarke, Louise Jayne Clark, Adam J Donne, R James A England, Hisham M Mehanna, Gerald William McGarry, Sean Carrie, Basic Sciences Endocrine Surgery Rhinology, 2018
Sore throat may be the presenting symptom in: Acute rheumatic feverSystemic onset juvenile idiopathic arthritis (JIA)Macrophage activation syndromes (MAS)/haemophagocytic lymphohistiocytosis (HLH), either primary or secondary to other autoimmune inflammatory statesPeriodic fever syndromes including periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome.
Bones, joints, muscles and tendons
Published in Kevin G Burnand, John Black, Steven A Corbett, William EG Thomas, Norman L Browse, Browse’s Introduction to the Symptoms & Signs of Surgical Disease, 2014
Kevin G Burnand, John Black, Steven A Corbett, William EG Thomas, Norman L Browse
Apart from other joint involvement, there may be generalized wasting and anaemia. Three systemic diseases are associated with rheumatoid joint disease: Still’s disease, a disease in which adults develop arthritis, splenomegaly and lymphadenopathy. A similar condition can occur in children, known as systemic onset juvenile idiopathic arthritis.Reiter’s syndrome, in which there is urethritis, conjunctivitis, skin rashes and arthritis.Systemic lupus erythematosus, a collagen disease in which there is a scaly, red rash on the face, debility and manifestations in all tissues of a small vessel arteritis.Other conditions, which may also present in a similar fashion to rheumatoid arthritis (Revision panel 6.3)
Immune system modulators
Published in Gabriel Virella, Medical Immunology, 2019
Richard M. Silver, Stephen Elmore
The cytokine interleukin-1β (IL-1β) provokes a number of immune and inflammatory responses, including its role in Th17 cell differentiation, promoting cartilage destruction, inducing fever, and stimulating production of other cytokines. IL-1β is initially produced in an inactivated form (pro-IL-1β) before becoming activated by the inflammasome, a multiprotein complex that forms in cells to cleave cytokines into their biologically active forms. Several agents have been developed to target IL-1β in order to impede its actions. Anakinra is a soluble IL-1 receptor antagonist, while canakinumab is a “human” monoclonal antibody produced in transgenic mice directed against IL-1. Both agents are able to prevent the interaction of IL-1 with its receptor, thus inhibiting the inflammatory response. Rilonacept, also known as IL-1 Trap, is a dimeric fusion protein consisting of the ligand-binding domains of the human IL-1 receptor (IL-1R1) and IL-1 receptor accessory protein (IL-1RAcP) linked to the Fc portion of IgG1 that binds and neutralizes IL-1. Side effects include infusion reactions, increased risk of infection, and a small increase in the risk of lymphoma. Anakinra and canakinumab are used in the treatment of Still's disease (both systemic-onset juvenile idiopathic arthritis [JIA] as well as the adult-onset form of Still's disease), cryopyrin-associated and other forms of periodic syndromes such as familial Mediterranean fever, hyperimmunoglobulin-D syndrome, and TNF receptor–associated periodic syndrome. Anakinra is also approved for use in rheumatoid arthritis and is being explored as a treatment for acute polyarticular gout.
Histone deacetylase inhibitors as a potential new treatment for psoriatic disease and other inflammatory conditions
Published in Critical Reviews in Clinical Laboratory Sciences, 2023
Jehan Mohammad Nazri, Katerina Oikonomopoulou, Elvin D. de Araujo, Dziyana Kraskouskaya, Patrick T. Gunning, Vinod Chandran
A subset of HDAC inhibitors have undergone rapid clinical development entering different phase II and III clinical trials for treatment of various hematological malignancies or solid tumors [175,193]. For immune- and/or inflammatory-related diseases, only the pan inhibitor Givinostat, or ITF2357, has entered a phase II clinical trial to treat systemic-onset juvenile idiopathic arthritis (JIA) in children (ClinicalTrials.gov Identifier: NCT00570661) [194,195]. Briefly, JIA is a type of arthritis seen in children that typically causes inflammation and joint pain in the hands, knees, ankles, elbows, and/or wrists [196,197]. Overall, Givinostat was found to be well-tolerated by 17 JIA patients with modest adverse events including nausea, vomiting, and/or fatigue [194,195]. Reduction in the number of joints with active disease or with limited range of motion was also reported. Clinical response at week 4, as measured by American College of Rheumatology Pediatric (ACR Pedi) 30, ACR Pedi 50, and ACR Pedi 70 criteria, improved significantly at 77.8%, 55.6%, and 22.2%, respectively [194]. By week 12, improvement was further increased with measurements shown to be 77.8%, 77.8%, and 66.7%, respectively.
Current treatment options and safety considerations when treating adult-onset Still’s disease
Published in Expert Opinion on Drug Safety, 2020
Giulio Cavalli, Nicola Farina, Corrado Campochiaro, Elena Baldissera, Lorenzo Dagna
IL-1 inhibitors are rapidly effective in controlling different clinical and laboratory manifestations of AOSD, especially in patients affected by systemic form of the disease [10]. Targeted blockade of IL-1 signaling typically leads to rapid and sustained therapeutic responses and steroid sparing effects. Across different studies, the effectiveness of IL-1 inhibitors in AOSD treatment ranged from 50% to 100% with a rate of remission ranging from 22% to 100% (median 70%) [61]. Studies from systemic-onset juvenile idiopathic arthritis (SOJIA), which is considered the pediatric counterpart of AOSD, indicate that two distinct clinical phenotypes can be identified based on the response to IL-1 inhibition. One is characterized by marked systemic inflammation, neutrophilia, elevated acute phase proteins, a lower number of inflamed joints, and dramatic clinical responses to IL-1 blockade. On the other hand, a clinical phenotype characterized by severe arthritis and limited systemic inflammation may not respond as brilliantly to IL-1 inhibition [62].
A Rare Ocular Manifestation of Adult Onset Still’s Disease: Purtscher’s-like Retinopathy
Published in Ocular Immunology and Inflammation, 2018
Cihan Buyukavsar, Ergenekon Karagoz, Murat Sonmez, Taner Kar, Abdullah Kaya, Eyup Düzgun, Yildiray Yildirim
Adult-onset Still’s disease (AOSD) is a rare multisystemic autoinflammatory disease with quotidian spiking fever, evanescent rash, arthralgia, and multiple organ involvement. Still’s disease was first described by a pediatrician, Sir Frederic George Still, in 1897. The initial description was in 22 children who had lymphadenopathy, arthritis, and systemic involvement—symptoms simulating arthritis. It was further described as a form of rheumatoid arthritis and named “systemic onset juvenile idiopathic arthritis”.1 In 1971 a rheumatologist, Eric Bywaters, described similar symptoms in 14 adults older than 16 years and named the entity “adult-onset Still’s disease”.2 Its prevalence is 0.16/100 000 and it is known to affect women slightly more often than men without race selectivity; however, the etiopathogenesis remains unclear.3 AOSD has a known predilection for young patients; three-quarters of patients are between the ages of 16 and 35. AOSD is also a multisystemic disease that affects various systems and tissues including the integumentary and musculoskeletal systems, upper respiratory tract, lymphoid tissues, lungs, cardiovascular, and central nervous systems.3,4 Ocular involvement is rarely seen in AOSD4 and data on ocular involvement in AOSD are scant and limited in the current literature. Here, we describe a case of Purtscher’s-like retinopathy without thrombotic microangiopathy (TMA) in an AOSD patient.