Systemic Autoimmune Rheumatic Diseases (SARDs) are just one subset of diseases caused when immunological tolerance of our own tissue is somehow lost and our immune system begins to attack our tissues in the same manner that it would a foreign antigen. Autoimmune diseases can be divided into two general groups: Organ specific, where the autoantibodies attack a specific organ, and Non-organ specific (or systemic), where the autoantibodies attack multiple organ systems. An example of an organ specific autoimmune disease is Hashimoto’s thyroiditis where autoantibodies damage the thyroid gland. An example of a systemic autoimmune disease is Systemic Lupus Erythematosus (SLE) where the autoantibodies can attack any organ in the body. Why our immune system malfunctions is not completely understood.
SARDs include: Rheumatoid Arthritis (RA), Systemic Lupus Erythematosus (SLE) (and subsets of Lupus), Sjögren’s syndrome (SjS), Systemic Sclerosis (SSc), Polymyositis (PM) Wegener’s Granulomatosis (WG) and Dermatomyositis (DM). For a complete list of SARDs and other autoimmune diseases please click here.
Wegener’s granulomatosis (WG), also called granulomatosis with polyangiitis (GPA), is a rare disorder that causes inflammation of your blood vessels. This inflammation restricts blood flow to various organs. WG often affects the kidneys, lungs and upper respiratory tract. The restricted blood flow to these organs can damage them. WG can affect other organs, but this isn’t as common, and generally isn’t as serious. WG can produce a type of inflammatory tissue known as a granuloma that’s found around the blood vessels. Granulomas can destroy normal tissue.
There is no cure for WG, but early diagnosis and treatment may lead to a full recovery. Without treatment, it can be fatal, most commonly from kidney failure.
It is estimated that 1 in 50,000 people suffer from WG. It is more common in middle-aged people of Northern European descent, but can occur in young and old people of any ethnicity. The prevalence of WG and other autoimmune diseases seems to be on the rise.
As mentioned, there is no cure for SARDs. The goal of treatment is to reduce symptoms and control the autoimmune response. Treatments vary but most SARDs conditions require some form of immune suppression. This can be achieved through the use of corticosteroids (prednisone) and nonsteroid drugs such as azathioprine, cyclophosphamide, mycophenolate, sirolimus, or tacrolimus. More recently newer drugs like Rituamab have been used to treat some conditions with some success. In additions to medication, patients may receive other treatments like plasma exchange, blood transfusions, or hormone replacement.
The outlook depends on the specific disease and the extent at which it affected the patient at diagnosis. In general, the survival rate for most SARDs conditions has improved dramatically over the past 50 years. Most conditions can be controlled with treatment and people can return to a fairly normal life. Returning symptoms, or “flare-ups”, are quite common and requires that the patient continues with regular blood testing and doctor visits.