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Sjögren's disease (SjD) is a long-term autoimmune disease that primarily affects the body's exocrine glands, particularly the lacrimal and salivary glands. Common symptoms include dry mouth, dry eyes and often seriously affect other organ systems, such as the lungs, kidneys, and nervous system.
Rheumatology is a branch of medicine devoted to the diagnosis and management of disorders whose common feature is inflammation in the bones, muscles, joints, and internal organs. Rheumatology covers more than 100 different complex diseases, collectively known as rheumatic diseases, which includes many forms of arthritis as well as lupus and Sjögren's syndrome. Doctors who have undergone formal training in rheumatology are called rheumatologists.
Methotrexate, formerly known as amethopterin, is a chemotherapy agent and immune-system suppressant. It is used to treat cancer, autoimmune diseases, and ectopic pregnancies. Types of cancers it is used for include breast cancer, leukemia, lung cancer, lymphoma, gestational trophoblastic disease, and osteosarcoma. Types of autoimmune diseases it is used for include psoriasis, rheumatoid arthritis, and Crohn's disease. It can be given by mouth or by injection.
Antinuclear antibodies are autoantibodies that bind to contents of the cell nucleus. In normal individuals, the immune system produces antibodies to foreign proteins (antigens) but not to human proteins (autoantigens). In some cases, antibodies to human antigens are produced; these are known as autoantibodies.
Leflunomide, sold under the brand name Arava among others, is an immunosuppressive disease-modifying antirheumatic drug (DMARD), used in active moderate-to-severe rheumatoid arthritis and psoriatic arthritis. It is a pyrimidine synthesis inhibitor that works by inhibiting dihydroorotate dehydrogenase.
Palindromic rheumatism (PR) is a syndrome characterised by recurrent, self-resolving inflammatory attacks in and around the joints (rheumatism), and consists of arthritis or periarticular soft tissue inflammation. The course is often acute onset, with sudden and rapidly developing attacks or flares. There is pain, redness, swelling, and disability of one or multiple joints. The interval between recurrent palindromic attacks and the length of an attack is extremely variable from few hours to days. Attacks may become more frequent with time but there is no joint damage after attacks. It is thought to be an autoimmune disease, possibly an abortive form of rheumatoid arthritis.
Belimumab, sold under the brand name Benlysta, is a human monoclonal antibody that inhibits B-cell activating factor (BAFF), also known as B-lymphocyte stimulator (BLyS). It is approved in the United States and Canada, and the European Union to treat systemic lupus erythematosus and lupus nephritis.
Tocilizumab, sold under the brand name Actemra among others, is an immunosuppressive drug, used for the treatment of rheumatoid arthritis, systemic juvenile idiopathic arthritis, polyarticular juvenile idiopathic arthritis, giant cell arteritis, cytokine release syndrome, COVID‑19, and systemic sclerosis-associated interstitial lung disease (SSc-ILD). It is a recombinant humanized monoclonal antibody of the immunoglobulin IgG1 subclass against the interleukin-6 receptor (IL-6R). Interleukin 6 (IL-6) is a cytokine that plays an important role in immune response and is implicated in the pathogenesis of many diseases, such as autoimmune diseases, multiple myeloma and prostate cancer. Tocilizumab was jointly developed by Osaka University and Chugai, and was licensed in 2003 by Hoffmann-La Roche.
An overlap syndrome is a medical condition which shares features of at least two more widely recognised disorders. Examples of overlap syndromes can be found in many medical specialties such as overlapping connective tissue disorders in rheumatology, and overlapping genetic disorders in cardiology.
HLA-DR15 (DR15) is a HLA-DR serotype that recognizes the DRB1*1501 to *1505 and *1507 gene products. DR15 is found at high levels from Ireland to Central Asia. DR15 is part of the older HLA-DR2 serotype group which also contains the similar HLA-DR16 antigens.
Anti-topoisomerase antibodies (ATA) are autoantibodies directed against topoisomerase and found in several diseases, most importantly scleroderma. Diseases with ATA are autoimmune disease because they react with self-proteins. They are also referred to as anti-DNA topoisomerase I antibody.
Subacute cutaneous lupus erythematosus is a clinically distinct subset of cases of lupus erythematosus that is most often present in white women aged 15 to 40, consisting of skin lesions that are scaly and evolve as poly-cyclic annular lesions or plaques similar to those of plaque psoriasis.
Lupus, formally called systemic lupus erythematosus (SLE), is an autoimmune disease in which the body's immune system mistakenly attacks healthy tissue in many parts of the body. Symptoms vary among people and may be mild to severe. Common symptoms include painful and swollen joints, fever, chest pain, hair loss, mouth ulcers, swollen lymph nodes, feeling tired, and a red rash which is most commonly on the face. Often there are periods of illness, called flares, and periods of remission during which there are few symptoms. Children up to 18 years old develop a more severe form of SLE termed childhood-onset systemic lupus erythematosus.
Undifferentiated connective tissue disease (UCTD) is a disease in which the connective tissues are targeted by the immune system. It is a serological and clinical manifestation of an autoimmune disease. When there is proof of an autoimmune disease, but the disease does not correspond to any specific autoimmune disease, it will be diagnosed as UCTD. This is also the case of major rheumatic diseases whose early phase was defined by LeRoy et al in 1980 as undifferentiated connective tissue disease.
Anti-histone antibodies are autoantibodies that are a subset of the anti-nuclear antibody family, which specifically target histone protein subunits or histone complexes. They were first reported by Henry Kunkel, H.R. Holman, and H.R.G. Dreicher in their studies of cellular causes of lupus erythematosus in 1959–60. Today, anti-histone antibodies are still used as a marker for systemic lupus erythematosus, but are also implicated in other autoimmune diseases like Sjögren syndrome, dermatomyositis, or rheumatoid arthritis. Anti-histone antibodies can be used as a marker for drug-induced lupus.
Atacicept is a recombinant fusion protein designed to inhibit B cells, thereby suppressing autoimmune disease. The designer protein combines the binding site for two cytokines that regulate maturation, function, and survival of B cells - B-lymphocyte stimulator (BLyS) and A proliferation-inducing ligand (APRIL), with the constant region of immunoglobin. Atacicept blocks activation of B cells by the tumor necrosis factor receptor superfamily member 13B, a transmembrane receptor protein found predominantly on the surface of B cells. Like the monoclonal antibody belimumab, atacicept blocks the binding of BLyS, but it also blocks APRIL. Binding of these TACI ligands induces proliferation, activation, and longevity of B cells and thus their production of autoantibodies. Atacicept is thought to selectively impair mature B cells and plasma cells with less impact on progenitor cells and memory B cells.
Anti-SSA autoantibodies are a type of anti-nuclear autoantibodies that are associated with many autoimmune diseases, such as systemic lupus erythematosus (SLE), SS/SLE overlap syndrome, subacute cutaneous lupus erythematosus (SCLE), neonatal lupus and primary biliary cirrhosis. They are often present in Sjögren's syndrome (SS). Additionally, Anti-Ro/SSA can be found in other autoimmune diseases such as systemic sclerosis (SSc), polymyositis/dermatomyositis (PM/DM), rheumatoid arthritis (RA), and mixed connective tissue disease (MCTD), and are also associated with heart arrhythmia.
Blisibimod is a selective antagonist of B-cell activating factor, being developed by Anthera Pharmaceuticals as a treatment for systemic lupus erythematosus. It is currently under active investigation in clinical trials.
Anthera Pharmaceuticals, Inc. is an American biopharmaceutical company focused on developing and commercializing products to treat serious conditions associated with cystic fibrosis, inflammation and autoimmune diseases. Liprotamase (Sollpura), Anthera's leading drug candidate which is being developed for exocrine pancreatic insufficiency (EPI) is currently in Phase 3 clinical trials, and A-623 (Blisibimod) for the treatment of IgA nephropathy is currently in Phase 2 clinical trial.
Daniel Jeffrey Wallace is an American rheumatologist, clinical professor, author, and fellow. Wallace has published 500 peer reviewed publications, 9 textbooks, and 28 book chapters on topics such as lupus, Sjögren syndrome, osteoarthritis, and fibromyalgia. He has the largest cohort of lupus patients in the United States (2000). A full professor of medicine, he is associate director of the Rheumatology Fellowship Program at Cedars-Sinai. His seminal contributions to research include being an author of the first paper to demonstrate vitamin D dysfunction and the importance of interleukin 6 in lupus, conducting the first large studies of apheresis in rheumatoid arthritis and lupus, and insights into the mechanisms of action of antimalarials. Wallace's research accomplishments also include conducting many clinical rheumatic disease trials, examining the role of microvascular angina and accelerated atherogenesis in lupus, and work on anti-telomere antibodies which have garnered him 6 papers in The New England Journal of Medicine. Wallace's monograph, The Lupus Book, has sold over 100,000 copies since 1995.
References
- ↑ World Health Organization (2017). "International Nonproprietary Names for Pharmaceutical Substances (INN). Proposed INN: List 117" (PDF). WHO Drug Information. 31 (2).
- ↑ "Ianalumab - MorphoSys/Novartis". AdisInsight. Springer Nature Switzerland AG.
- ↑ van den Hoogen LL, van Laar JM (February 2020). "Targeted therapies in systemic sclerosis, myositis, antiphospholipid syndrome, and Sjögren's disease". Best Practice & Research. Clinical Rheumatology. 34 (1): 101485. doi: 10.1016/j.berh.2020.101485 . PMID 32067925.
- ↑ Narain S, Berman N, Furie R (November 2020). "Biologics in the treatment of Sjogren's syndrome, systemic lupus erythematosus, and lupus nephritis". Current Opinion in Rheumatology. 32 (6): 609–616. doi:10.1097/BOR.0000000000000754. PMID 33002950. S2CID 222150641.
- ↑ Clinical trial number NCT03574545 for "Study Comparing Two VAY736 Drug Products in Patients With Rheumatoid Arthritis" at ClinicalTrials.gov
- ↑ Clinical trial number NCT03656562 for "Study the Efficacy and Safety of VAY736 and CFZ533 in SLE Patients" at ClinicalTrials.gov
- ↑ "Risk Adjusted Net Present Value: What is the current valuation of Novartis's Ianalumab". Pharmaceutical Technology. 30 June 2023.
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