Vasculitis

Caused by inflammation of the blood vessels themselves, including arteries and veins. With this disease, the actual vessel walls themselves are damaged, including thickening, weakening, narrowing or scarring, all which can restrict blood flow and lead to organ and tissue damage. Vasculitides have the potential to be very serious illnesses, so prompt diagnosis and treatment is very important. Common types of vasculitis are:

Giant Cell Arteritis (GCA) is also known as temporal arteritis. This condition is caused by inflammation of the large arteries in the head and neck. Patients commonly present with symptoms such as temporal headache, scalp tenderness, jaw pain, vision problems, and it can even lead to blindness if left untreated. This condition only occurs in older adults, mainly those over age of 50. A biopsy of the temporal artery is most often done to make a definitive diagnosis, to look for “giant cells” under the microscope which is characteristic of this condition. Treatment for GCA should begin as soon as possible because of the risk of loss of vision. High dose corticosteroids have been the mainstay of treatment, but newer biologic medications have recently been approved to treat this condition.

This is a rare condition involving inflammation of the aorta that more often affects women than men. It characteristically affects young women starting in their mid-20s. The aorta is the largest artery of the body, and it supplies blood from the heart to the head, arms and legs, and internal organs. In Takayasu arteritis, the aorta and its main branches get inflamed and damaged. Chronic inflammation can cause thickened, narrowed, blocked, or weakened artery walls. Patients can present with arm or chest pain, high blood pressure, dizziness or fainting, heart attack or stroke. Treatment is geared toward reducing the inflammation of the artery and preventing permanent damage to the blood vessel wall. In certain cases, if the blood vessel is already too narrowed, a vascular surgeon evaluates to determine if surgery could help reopen it and improve blood flow.

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a classification term for several conditions, each distinctive but unified by their association with ANCA positivity. ANCA is a blood test. These conditions are granulomatosis with polyangiitis (GPA) formerly known as Wegener’s, eosinophilic granulomatosis with polyangiitis (EGPA) formerly known as Churg-Strauss Syndrome, and microscopic polyangiitis (MPO). These conditions are characterized by neutrophil cells attacking and causing inflammation, damage, and narrowing of the smaller and medium-sized blood vessels in the body. Symptoms depend on what organ system is involved, which can range from petechiae and palpable purpura (skin), mononeuritis multiplex (nerve), glomerulonephritis (kidney), diffuse alveolar hemorrhage (lung), just to name a few. ANCA-associated vasculitis can be a potentially very serious illness, so prompt diagnosis and initiation of treatment is very important. It is also a relapsing-remitting disease, which means it can alternate between disease flare-up then going into remission, then flaring back up, and going into remission, etc. Because of the variable course of this condition, it is prudent to have regular follow-up to watch for any warning signs before the disease becomes active again.

Also known as Henoch Schönlein Purpura (HSP), this form of vasculitis causes blood vessel inflammation primarily in the small capillaries of the skin, joints, intestines and kidneys due to abnormal immune system response. The characteristic symptom is a tender, burning, raised, reddish-purplish rash which looks like bruising that commonly appears on the legs and buttocks, called purpura. HSP can also manifest as arthritis, kidney failure, and intestinal ischemia. In children, symptoms can last a month or two with most patients recovering on their own, but in adults, the vasculitis has a higher likelihood to persist.z

Polyarteritis Nodosa (PAN) is a rare systemic vasculitis characterized by necrotizing inflammatory lesions primarily affecting small and medium-sized muscular arteries, which can result in microaneurysm formation, aneurysmal rupture with hemorrhage, and thrombosis. Necrotizing means that a segment of the blood vessel wall has died. An aneurysm is an abnormal bulge or ballooning of a blood vessel. PAN can affect nearly any organ system and therefore can present with a wide range of symptoms, including but not limited to decreased appetite and weight loss, abdominal pain and bloody stools, excessive fatigue, fever, muscle and joint aches, nerve problems and kidney failure. Moderate and severe PAN will typically require high-dose steroids at first followed by some type of immunosuppressive medication for maintenance treatment. In patients with PAN who have been found to have an associated hepatitis B or C infection, treatment is geared toward treating the underlying viral infection.

A rare condition characterized by recurrent inflammation of cartilaginous tissues throughout the body. This condition causes inflammation, pain, and swelling of cartilage in the ears, nose, trachea (windpipe), joints, heart valves, and eyes. The initial symptom is typically pain in the affected cartilage area, and if left untreated can progress to deformity, destruction, and collapse of the cartilage.

Cryoglobulins are abnormal antibody proteins in the blood that clump together and form immune complexes which are even bigger clumps of protein. When these gelatinous protein clumps form they can block blood circulation, which can damage the skin, joints, nerves, and internal organs, particularly the kidneys and liver. Cryoglobulinemia can be due to many different causes such as an underlying autoimmune disease, viral hepatitis, and cancers of the bone marrow and blood. Patients found with underlying chronic hepatitis C need to be treated for their infection.

This is a rare disorder that can cause blood vessel inflammation throughout the body. Behcet’s can lead to numerous signs and symptoms that may seem unrelated at first. Symptoms can include recurrent mouth and genital ulcers not due to an infection, eye inflammation, skin rashes and lesions, and in severe cases brain, spinal cord, and large blood vessel involvement. Behcet’s was historically identified in the Turkish population and surrounding areas of the ‘silk road’ and has been found to be associated with the gene HLA B51.

The approach to treatment of vasculitis is based on current evidence-based guidelines. Treatment is customized based on what body part is currently affected and symptomatic to the patient, prevention of serious organ involvement, and patient preference. Initial fast-acting treatments are typically oral or injection corticosteroids such as prednisone or NSAIDs. DMARDs and Biologics are used as ‘steroid-sparing agents’ and are more specific and targeted treatments for the disease. Depending on the patient’s needs, sometimes DMARDs and Biologics may be combined. Common DMARDs used for vasculitis include methotrexate, Cellcept, and Imuran. Subcutaneous Actemra has been recently approved for treatment of GCA. Cytoxan, an infusion chemotherapy treatment, is sometimes used for very severe refractory cases of vasculitis, administered in the hospital outpatient infusion clinic. The infusion biologic offered by Lomibao Rheumatology & Wellness Care for the treatment of vasculitis is Rituxan. Wellness services for vasculitis – coming soon!