Description Of The Disease
What is autoimmune disease?
Autoimmune diseases (AID) affect 30 million Europeans, including 10% of adults.
After cancer and heart disease, autoimmune diseases are the third most common category of disease in industrialised countries when it comes to morbidity and mortality. For unknown reasons, the prevalence of AID is increasing, and woman are more prone to develop one. AID have a huge impact on the life of people suffering from them, they also represent a significant health and economic burden.
The aetiology of AID is poorly understood and differs between pathologies; 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, their own immune system, which is supposed to protect against invaders, instead turns against itself, attacking organs, tissues and cells. We now have treatments to slow down the progression of AID but there is currently no cure.
Treatment includes medications that are generally immunosuppressive drugs and 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 need for these medications, and their side effects, has been raised as a key focus by patient advisory groups working with the PARADISE project.
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. Although these conditions are not passed down directly through families, there is an increase in incidence in family members. Depending on the pathology, outcomes may be variable and symptoms change with time. Patients can have limited or no symptoms, or the disease can get worse in the case of flare-up.
What autoimmune disease does PARADISE focus on?
The PARADISE project focuses on the multi-system AID ANCA Associated Vasculitis (AAV). This is a relapsing and remitting disorder that results in severe morbidity and excess mortality.
Nature Reviews Disease Primers volume 6, Article 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 above mentioned disease phenotypes.
What are some good sources of sound information on vasculitis?
European Reference Network RITA: https://ern-rita.org/disease-category/rare-diseases/autoimmune-diseases-ai/
Vasculitis Ireland Awareness (VIA): http://vasculitis-ia.org/ ➔
Vasculitis Foundation (types of vasculitis): https://www.vasculitisfoundation.org/ ➔
European Vasculitis Society: https://vasculitis.org/ ➔
Association France Vascularites (French): https://www.association-vascularites.org/accueil/presentation ➔
French group researching vasculitis: https://www.vascularites.org/ ➔
Vasculitis Information and Resources
Information on ANCA vasculitis (in multiple languages): https://www.myancavasculitis.com/ ➔
General vasculitis information: https://www.mayoclinic.org/diseases-conditions/vasculitis/symptoms-causes/syc-20363435 ➔
Vasculitis resources in Europe: https://www.vasculitisint.com/ ➔
Vasculitis resources United Kingdom: https://www.vasculitis.org.uk/ ➔
Vasculitis resources Germany: http://www.vaskulitis.org/➔
Vasculitis resources Spain: https://vasculitis.es/➔
Vasculitis resources Netherlands: https://vasculitis.nl/ ➔
Vasculitis resources Italy: https://apacs-egpa.org/ ➔