The rheumatic connective tissue disorders are inflammatory (inflammatory) autoimmune states called in English "autoimmune connective tissue diseases / disorders". Typical examples are Systemic lupus erythematosus (SLE), Mixed connective tissue disease (MCTD), Systemic sclerosis / scleroderma, (Dermato-) myositis , Sjögren's syndrome and overlapping diseases like Skleromyositis and og undifferentiated systemic connective tissue disease which are mentioned on separate pages.
Autoimmune, systemic connective tissue disease (and Vasculitis diseases) may be mild or severe conditions within the consept of rheumatism ("Rheuma") and the profession Rheumatology. They are characterized by rheumatic, autoimmune inflammation of connective tissue and often attack internal organs. The connective tissue is located around the organs, but is also a part of them. The diseases are also referred to as collagenoses or connective tissue inflammation. They may include skin, joints, internal organs. The connective tissue is made of the proteins collagen ("Collagenosis") and elastin that bind the different organs of the body together.
Causes of connective tissue disease
Triggering reasons of connective tissue diseases are still unknown. Women get more frequent systemic connective tissue disease, which may be due to the fact that the X chromosome (women has two, men one) contains over 1000 genes while the human Y chromosome has about 100, and many of the genes on the X chromosome are associated with autoimmune diseases. Genetic disposition and a more complex immune system among women are just partial explanations.
Once a systemic connective tissue disease has started, the immune system will overreact with excessive activity. By mistake, the immune system attacks the body's own proteins and tissues (autoimmunity). Nobel prize winner in immunology, Paul Ehrlich, described in his time the significant problems that may occur as "Horror autotoxicus". The various connective tissue disorders (diagnoses) are differentiated because the immune system triggers different Antibodies and damage different organs.
It has been said about rheumatic diseases that "No other group of diseases causes so much suffering for so many over so long time". Fortunately, rheumatology has developed significantly since then. Newer medications and proper handling save many for serious disease development, pain and disability, but for some with systemic connective tissue disease, the statement is unfortunately still striking.
Vasculitis is inflammation of blood vessels
- Vasculitis is inflammation of blood vessels and is not considered among connective tissue disorders, but may have similar symptoms
- Vasculitis diseases are described here (Temporalis (giant cell-) arteritis, Polymyalgia rheumatica, GPA / Wegenes granulomatosis, Takayasu arteritis, Behcet's syndrome and several others
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Treatment of connective tissue disorders
Information on indication of the drugs and the risk of side effects are important for each treatment. The treatment should suppress the overactive immune system so that kidneys, lungs and other internal organs retain their function. At the same time, the symptoms should be reduced. Cortison (for example prednisolone) is still used in addition to many new drugs. Cortisone can be decisive in the first and most active, destructive disease stages, but must be avoided at Systemic sclerosis. Other medications (for example Plaquenil, Methotrexate, Azathioprine) are added to avoid much cortisone side effects and to keep the disease at bay. Against pain is paracetamol formulations usually the first choice, followed by NSAIDs. The treatment goal is often that the disease goes in remission.
Research on connective tissue disease
Rheumatology Department at Oslo University Hospital (OUS), Rikshospitalet researches
autoimmune connective tissue disease. For research tasks, patients are included in a disease register: Norwegian systemic connective tissue disease and vasculitis register (NOSVAR). In recent years, the register has contributed to more than 70 published works in international medical journals. PhD (doctorate) work has been completed for the following diseases: MCTD, Systemic sclerosis, SLE, Myositis, Antisynthetase syndrome, Takayasu arteritis and more new work is underway. The good collaboration between patients, doctors and researchers is crucial for obtaining new knowledge for the benefit of the treatment.
For doctors and other health workers
Hereditary (genetic) non-inflammatory connective tissue disease
- The symptoms often include different types of growth disorders and usually start in childhood or adolescence. These diseases are most commonly handled by pediatricians or specialist in physical medicine. Marfan's syndrome characterized by long, thin arms, legs, fingers and toes. Often hypermobile joints. Ehlers-Danlos syndrome usually causes hypermobile joints, stretchable skin and some developing aneurysms (aneurysms) on the main pulmonary artery. Loeys-Dietz syndrome have Marfan-like symptoms and aneurysms on the main artery. Osteogenesis imperfecta causes weakened bones and increased risk of bone fractures. Stickler's syndrome may have striking facial shape, vision problems, hearing loss and arthritis. Alport syndrome characterized by non-infectious renal infections (glomerulonephritis) and hearing loss. Congenital contractural arachnodactyly (Beals syndrome) may resemble Marfan's syndrome, but causes bent hips, knees, elbows and ankles (contractures)
- The content is based on own notes from meetings, international congresses, textbooks and input from skilled colleagues through 30's work on rheumatic diseases
- The purpose is to describe the inflammatory rheumatic connective tissue diseases and related conditions that may be confused with these (differential diagnoses)
- Information is needed to understand the diseases and the treatment, and making patients able to contribute to the best solutions
- Knowledge reduces risk failure in follow-up, which is a collaboration between patient, general practitioner and specialist in rheumatology / rheumatology department
- The pages are closely linked to Vaskulitt.no which mainly describes vascular diseases
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