Vasculitis

When blood vessels become inflamed for any reason, it  is called vasculitis. The diagnosis is usually insufficient by itself. As rheumatologists, we have to determine which size vessels are involved and what part of the body in addition to blood vessels are involved as it makes a considerable difference in diagnosis and treatment. For example, when large blood vessels are involved, it could be giant cell or temporal arteritis. Typically patients may have scalp tenderness and difficulty chewing. One of the blood test, a sedimentation rate is typically but not always elevated. If the diagnosis is considered at all likely, corticosteroids need to be started soon and the temporal arteries need to be biopsied by a surgeon. Time is of the essence in this diagnosis as blindness can rarely be a consequence. In addition sometimes the aorta may be involved with this disease and appropriate imaging studies can help determine the diagnosis.

Another type of vasculitis we see less commonly used to be called Wegener’s granulomatosis. It is now called granulomatosis with polyangiitis (quite a mouthful) after it was discovered it Wegener was a Nazi. In this disease, patients may initially have sinus infections or cellulitis around their eyes. Subsequently they may develop pneumonia and cough up blood. Joints can be involved and kidney involvement can also be severe. Making the diagnosis can be very difficult. Blood studies (i.e. ANCA) can help as can imaging studies of the sinuses and chest. Ultimately biopsies are often necessary. Treatment entails high dose corticosteroids and Cytoxan. More recently we have had significant success with Rituxan, a drug we “borrowed” from the hematologists. It was originally used for a type of lymphoma. We have found it to be quite successful in rheumatoid arthritis and now we know it can make a considerable difference in stopping the progression and even reversing much of the disease process of granulomatosis with polyangiitis.

The most common type of vascultis involves small blood vessels and can be caused by medications.  Usually stopping the offending medicine is sufficient although corticosteroids may also be needed.  Other diseases involving small blood vessels include Henoch-Schonlein purpura which may also present with abdominal pain, blood in the urine and blotchy rashes on the legs.  Steroids are often used in these cases as well.