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Description Of The Disease

Autoimmune diseases (AID) are affecting 30 million Europeans, including 10% of adults. AID is the third most common category of disease in industrialized countries in terms of morbidity and mortality, after cancer and heart disease. For unknown reasons, the prevalence of autoimmune diseases is increasing, and woman are more prone to develop one. AID have a huge impact on the life of people suffering from one of them and they represent a significant health and economic burden.

 

The aetiology of AID is poorly known and differs between pathologies, but possible interaction between genetic, environmental factors and personal health may play a role in the development of the disease. When somebody is affected by an autoimmune disorder, its own immune system supposes to protect against invader, turns against itself and attacks the organs, tissues and cells. Thanks to biomedical research, we have treatments to slow down the progression of autoimmune diseases but there is currently no cure. These medications called immunosuppressive drugs, act by suppressing the immune system, leaving the patient exposed to severe infection and at risk of cancer. Affected individuals receive standard treatment regimens for long periods of time, even though, in some cases, the autoimmune response may no longer be detectable. The general requirement for these medications, and their side effects, has been raised as a key target for research by the PARADISE consortium patient groups.

Autoimmune diseases have different degrees and can affect any organ. Most common are type 1 diabetes, multiple sclerosis, rheumatoid arthritis, lupus, Crohn’s disease, psoriasis and scleroderma. AID generally affecting different members of the same family and people can be affected by more than one. Depending on the pathology, outcomes may be variable and symptoms are changing with time. Patients can have limited or no symptoms, or the disease can get worse in case of flare-up.

PARADISE project focuses on ANCA Associated Vasculitis (AAV).

Nature Reviews Disease Primers volume 6, Articule number: 72 (2020) 

 

Granulomatosis with Polyangiitis (GPA, formerly called Wegener’s granulomatosis), Microscopic Polyangiitis (MPA) and Eosinophilic Granulomatosis with Polyangiitis (EGPA, formerly called the Churg-Strauss syndrome), are primary systemic vasculitides (a group of uncommon but serious multisystem autoimmune disorders characterized by blood vessel inflammation), mainly affecting small and medium vessels. Because of similarities in the clinical and pathological features among these diseases and the strong association of anti-neutrophil cytoplasmic antibodies (ANCA) with GPA, MPA and EGPA, these diseases are usually grouped and studied as one group referred to as ANCA-associated vasculitis (AAV). There are two main ANCA serotypes associated with AAV, the proteinase-3 (PR3) and myeloperoxidase (MPO). Some authors prefer to classify the AAV into PR3 and MPO-associated disease rather than the traditional classification based on the three abovementioned disease phenotypes.

 
What are some good sources of sound information on vasculitis?
 

https://ern-rita.org/disease-category/rare-diseases/autoimmune-diseases-ai/  Website of European Reference Network RITA

http://vasculitis-ia.org/  Website of our consortium partner Vasculititis Ireland Awareness (VIA)

https://www.vasculitisfoundation.org/  Website about all types of vasculititis

https://vasculitis.org/   Website of the European Vasculitis Society

https://www.myancavasculitis.com/  Website focusing on ANCA vasculititis available on multiple langues

https://www.mayoclinic.org/diseases-conditions/vasculitis/symptoms-causes/syc-20363435   Website providing information on vasculititis

https://www.vasculitisint.com/  Website of ressources on vasculititis in Europe

https://www.association-vascularites.org/accueil/presentation  Website of the French patients association France vascularites

https://www.vascularites.org/  Website of the French Group studying vasculititis

https://www.vasculitis.org.uk/  Website of ressources on vasculititis in United Kingdom 

http://www.vaskulitis.org/  Website of ressources on vasculititis in Germany

https://vasculitis.es/  Website of ressources on vasculititis in Spain 

https://vasculitis.nl/  Website of ressources on vasculititis in Netherland

https://apacs-egpa.org/  Website of ressources on EGPA in Italy

 

 

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