Connective tissue disorders include inflammatory autoimmune Conditions that are extensively described on these pages. Typical examples are Systemic lupus erythematosus (SLE), Mixed connective tissue disease (MCTD), Systemic sclerosis / scleroderma, (Dermato-) myositis og Sjögren's syndrome.
Rarer, hereditary diseases of connective tissue without signs of rheumatic inflammation also occur. Example is Marfan's syndrome og Ehlers-Danlos syndrome. Hereditary connective tissue disorders are shortly described at the bottom of this page.
Autoimmune, systemic connective tissue disease
Autoimmune, systemic connective tissue disease (and Vascular diseases) are potentially severe conditions covered by the term rheumatism ("Revma") and the profession Rheumatology. The autoimmune connective tissue disorders are characterized by rheumatic inflammation of connective tissue. 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 and the surroudning tissues . The connective tissue is made of the proteins collagen ("Collagenoser") and elastin that bind the different organs of the body together.
The triggering disease causes 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) 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. If a systemic connective tissue disease has first started, the immune system will overreact with excessive activity. By mistake, our immune system attacks our body's own proteins and tissues (autoimmunity). The different connective tissue diseases (diagnoses) are differentiated because the immune system triggers different Antibodies and damage different organs.
It is said about rheumatic diseases that "No other disease group is in so much suffering for so many over so long. " Fortunately, rheumatology has developed significantly. Proper handling and current therapies save a lot from serious disease development, pain and disability, but for some people, the statement is unfortunately still striking.
- Vasculitis is inflammation of blood vessels and is not considered among connective tissue disorders
- Vasculitis diseases are described here (Temporalis (giant cell) arteritis, Polymyalgia rheumatica, GPA / Wegenes granulomatosis, Takayasu arteritis, Behcet's syndrome And several others
Answers to frequently asked questions
- What does diet influence the disease course?
- Can or should I take vaccines?
- Where can I find information about current medication?
- Do I get free dental treatment?
- What happens during pregnancy?
- What is fatigue (exhaustion), Fibromyalgia, ME (Chronic Fatigue Syndrome)?
- What is meant by arthritis or synovitis?
- How do I prepare for medical consultation?
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 medications. Cortisone can be crucical in the first and most active, destructive disease stages. Other medications (for example Plaquenil, Methotrexate, Azathioprine) is added to avoid cortisone side effects and to keep the disease at bay. More about drugs here.
Rheumatology Department at Oslo University Hospital, Rikshospitalet do research on autoimmune connective tissue disease. For research tasks, patients are included in a disease register: Norwegian Systemic Connective Disease and Vascular Registry (NOSVAR). In recent years, the register has contributed to more than 50 published works in international medical journals. PhD (doctorate) work has been completed for the following diseases: MCTD, Systemic sclerosis, SLE og Myositis and more new work is under way. The good cooperation between patients, doctors and researchers is crucial to obtaining new knowledge for the benefit of the treatment.
For doctors and other health workers
Hereditary, 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 is characterized by long, thin arms, legs, fingers and toes. Often hypermobile joints
- Ehlers-Danlos syndrome usually causes excessive joints, stretchable skin and some developing aneurisms (aneurysms) of the main artery (aorta)
- Loeys-Dietz syndrome has Marfan-like symptoms and aneurysms on the main artery
- Osteogenesis imperfecta causes impaired skeletal and increased risk of bone fracture.
- Stickler's syndrome may have striking facial shape, impaired sight, hearing loss and arthritis
- Alport syndrome characterized by non-infectious renal inflammation (glomerulonephritis) and hearing loss
- Congenital contractural arachnodactyly (Beals syndrome) may resemble Marfan's syndrome, but causes bent hips, knees, elbows and ankles (contractures)
- Building on own notes from meetings, international congresses, textbooks and input from skilled colleagues through 30 years' work on rheumatic diseases
- Describes inflammatory rheumatic connective tissue disease and related conditions that may be confused with these (differential diagnoses)
- The pages are closely linked to Vaskulitt.no which describes vascular diseases