Autoimmune Connective Tissue Diseases (AICTDs)

Apart from the brain, the body's immune system is probably the least understood ' body organ system' by people! The 'immune system' is difficult to conceptualise and learn about given it is a complicated 'fluid' organ system existing in the blood, lymphatics, bone marrow, spleen, thymus, gut lining and elsewhere and consists of interacting cells (e.g. lymphocytes) and molecules (e.g. cytokines, antibodies). Normally the main function of the immune system is to protect the body from micro-organisms like bacteria and viruses. The symptoms everyone gets as part of a cold or flu is a consequence of a successfully working immune system - it switches on to enable killing of the invading micro-organism when it needs to and switches off when the battle is won!


Autoimmune diseases occur when the immune system either switches on, or doesn't switch off, abnormally - simply put it 'thinks' there is something like a micro-organism invading the body to fight (but there isn't). So in a way the autoimmune system is 'failing' in certain respects. So as there can be: ….. kidney disease / kidney failure, heart disease / heart failure etc., there can also be (auto)immune disease / (auto)immune failure.


Autoimmune disease can affect a single organ in the body (organ specific, e.g. thyroid or type 1 diabetes [pancreas]) or can be 'non-organ specific'. Autoimmune connective tissue diseases (AICTDs) are non-organ specific. People affected by AICTDs can get symptoms and problems from many different tissues in the body such as:
•    joints and tendons,
•    skin (often UV light/sun sensitive rashes),
•    mucosal linings in mouth, lungs and eyes (typically dryness symptoms),
•    muscles,
•    internal organs can become inflamed (lung, kidney),
•    blood vessels (livedoid skin, vasculitis, Raynaud's Syndrome)
 

Rheumatologists have a unique role in diagnosing AICTDs because they know and can assess for the wide range of symptoms and features that can occur in the AICTDs.
The main AICTDs are: Systemic Lupus Erythematosus (SLE; 'lupus'), Primary Sjögren's Syndrome, Systemic Sclerosis (scleroderma), Polymyositis, Dermatomyositis and Antiphospholipid Syndrome


AICTDs are treated with immunosuppressant medications such as steroids (prednisolone or methylprednisolone) hydroxychloroquine, azathioprine, mycophenolate, methotrexate, rituximab, belimumab and cyclophosphamide. For information on any of these medicines please see the PILS document on the relevant drug at https://www.medicines.org.uk/emc/


Links
Patient run advisory and support organisation in The UK to support sufferers with Sjögrens syndrome: 
www.bssa.uk.net

Patient run advisory and support organisation for people with SLE ('lupus'):
www.lupusuk.org.uk

Information on the inflammatory muscle diseases (myositis):
https://www.versusarthritis.org/about-arthritis/conditions/polymyositis-and-dermatomyositis/

Patient run organisation giving advice and support to people diagnosed with either systemic sclerosis or Raynaud's disease: 
www.sruk.co.uk