Autoimmune
Disorders

Inflammation is sparked by some type of trigger, whether it is caused by an underlying infection (virus, microbe) or another environmental agitatent. 

The tissue of the blood vessel’s inner lining called the endothelium become inflamed when immune cells become hyperactive and attack native tissue. This attack is perceived as a defensive maneuver to destroy a foreign intruder like a virus, but the body’s immune system is tricked and the immune cells actually attack the “self”.  This is the definition of autoimmune. When the site of attack is a blood vessel, we call this a vasculitis. While most vasculitis is due to inflammation and autoimmune disorders, occasionally cancers such as lymphoma and leukemia can be the root cause. There are even cases of drug-induced vasculitis.

Problems really occur when distal organs or systems are affected.  A severely narrowed blood vessel or even occluded due to severe inflammation will not allow the passage of oxygen containing RBCs and anything downstream suffers hypoxia.  This leads to necrosis or death of tissue starved of oxygen. End organs such as kidneys and lung tissue can be destroyed. When the vessel wall becomes weakened to the point of dilating or expanding, the integrity may be so stressed the vessel ruptures.  Surgical intervention is sometimes necessary to repair a damaged vessel before it ruptures.

There are different types of vasculitis and different end organs affected.  While the pathological and histological appearance may remain the same, the variance occurs with the size of the vessels involved and the end organs involved.  

For example when large vessels are involved you have conditions such as Behcet’s disease, which involve painful ulcers in the mouth and genital area with the uvia of the eyes often involved.  It is usually specific to men in their 30’s and of Mediterranean descent. Another is Cogan’s syndrome which is more systemic and can lead to skin changes and eye inflammation and even hearing loss.  Giant Cell arteritis occurs in the artery of the temple of our head and usually affects older folks. Headaches, scalp tenderness and pain are harbingers of this condition and without treatment can lead to sudden vision changes or loss.

Polymyalgia Rheumatica (PMR) affects the joints and muscles causing pain, stiffness and weakness, mostly of the proximal muscle groups of the shoulder and pelvic girdle.  Takayasu’s arteritis (KA) while rare usually affects Asian teenagers and women. KA can cause destruction of the aortic arch. Malaise, fever, weight loss and sore joints usually precede vessel involvement.

Medium sized vessels are affected in a few other disorders such as Buerger’s disease and Raynaud’s phenomenon.  Buerger’s is also known as thromboangitis obliterans which can cause disruption of blood flow to fingers and toes with necrosis and loss of those affected limbs.  Seen very often in men in their 30’s and associated with cigarette smoking and elevated serum homocysteine levels. Raynaud’s is a commonly seen malady where extremities such as fingers get cold and turn “white” with decreased blood flow when exposed to a trigger (like touching a cold object).

A rare childhood disease known as Kawasaki disease (KD) where the walls of vessels systemically are inflamed and all sizes are involved and can be damaged with aneurysms.  Also known as muscocutaneous lymph node syndrome as a child presents very often with red patches on mucous membranes and a red rash on the skin. Some serious cardiac issues may occur in these children if not treated early.  It is successfully treated with medication if caught early and rarely do surgeons get involved to repair enlarged weak blood vessels.

Polyarteritis nodosa can present with lace like rash, bumps under the skin, anemia is yet another manifestation of a vasculitis.  Other conditions such as Eosinophilic granulomatosis with polyangiitis (EGPA) aka Chung-Strauss Syndrome, affecting skin, lungs, kidneys and nervous system.  

Cryoglobulinemic vasculitis, IgA vasculitis (Henoch-Schonlein Purpura), hypersensitivity vasculitis and microscopic polyangitis are others. Wegener’s granulomatosis (GPA) is still another variety that affects the upper airway, lung and kidney.

Signs and symptoms can include fever, loss of appetite, weight loss, fatigue and generalized pain.  Very often there are skin manifestations. Organs involved can include skin, joints, the lung, the GI tract, upper airway tree (nose ears), eyes, the brain and nervous system tissue.  It can be quite disturbing for a parent to find their child with a very high fever, mucous membrane lesions and peeling skin. These are some classic signs of Kawasaki Disease, a rare type of vasculitis in children.

Tests that are used to diagnose vasculitis are:  CBC to rule out anemias; an antineutrophilic cytoplasmic antibodies test (ANCA) where certain antibodies are produced in some vasculitis.  The Erythrocyte Sedimentation Rate (ESR, aka Sed Rate) is a very common and important test in evaluations. Another inflammatory marker often ordered by doctors is the C-reactive Protein (CRP).  As an inflammatory disease, inflammatory biomarkers are important tests for diagnosing these disorders. Sometimes biopsy of an affected vessel is important as histologic evaluation can help make a diagnosis.  Other studies such as an EKG and ECHO to measure cardiac function, a Chest x-ray for pulmonary issues and a urinalysis to check for renal function and involvement are ordered. Ultrasound, CT and MRI scans may be helpful as well as angiography which visualizes blood vessels that may be narrow, swollen, deformed or obstructed.

Treatments vary according to what type and the severity of the disease.  Also considered in a medical regimen is what end organs are involved. Most treatment is by medication, but on occasion surgery is called for.  

Many can be treated with over-the-counter pain medications in the class called NSAIDS (Motrin, Aleve or aspirin). Other choices are corticosteroids like prednisone.  Some physicians may prescribe cytotoxic medications. These types of medications will suppress or kill cells that cause inflammation. Some examples are cyclophosphamide, methotrexate and azathioprine.  In pediatric cases of Kawasaki disease, high doses of aspirin are used in combination with intravenous immune globulin. To repair bulging vessels, vascular surgeons may have to go in and remove or repair aneurysms.

There are no set guidelines for prevention of vasculitis, however, keeping a healthy immune system, proper diet, avoiding environmental toxins and stimulants such as tobacco smoking and doing what it takes in higher risk patients to avoid triggers can help lower the risk of getting this disorder.  As fitness professionals, being observant with clients when they are ill appearing and may present with skin manifestations, should be directed to their healthcare providers if they have not been evaluated.