Ask the Expert: Dr. Robert Spiera, Rheumatologist, Answers Your Questions About Vasculitis

Dr. Robert Spiera, Rheumatologist

Q1: Is vasculitis only related to lupus or is it associated with other diseases/conditions?

Although vasculitis can occur in the context of lupus or other ‘connective tissue disorders’ such as Sjogren’s syndrome, it can also occur as a disease entity onto itself or in association with other diseases and conditions. Some examples of conditions include certain forms of cancers, exposures to medications and illegal drugs as well as infections such as hepatitis C.

Q2: Typically, does vasculitis only occur in older people?

Vasculitis can occur in any age group and is not at all confined to older patients. The one form of vasculitis that is more common in the elderly is temporal arteritis (also known as giant cell arteritis or cranial arteritis), where patients can develop severe headaches, systemic symptoms such as fevers and most importantly can develop loss of vision unless the condition is appropriately identified and treated. Most other forms of vasculitis occur at younger ages and are indeed, more common in younger age groups.

Q3: I recently was diagnosed with vasculitis. Does it ever go into remission? Anything I can do to encourage it to?

Different forms of vasculitis have potentially different courses. Most commonly, however, vasculitis can and does go into remission with recognition of the appropriate diagnosis and institution of therapy. The challenge is achieving remission with the safest medication regimen possible and then being vigilant in monitoring for relapse of the disease. Of course, healthy lifestyle habits are helpful for one’s general health, but in patients with vasculitis there are well recognized therapeutic interventions medically that can help in achieving remission.

Q4: My doctor recently did a tissue biopsy to test for vasculitis. If diagnosed, what does treatment entail?

Tissue biopsies are generally the most reliable way to make a diagnosis of vasculitis. The nature of the treatment, however, depends on what type of vasculitis is identified and the severity. Treatment often includes corticosteroids (such as prednisone), and may include other immunosuppressive drugs such as Cyclophosphamide (Cytoxan). These medications often reserved for patients with the most serious forms of vasculitis. More recently, a medication previously approved for treating lymphoma or rheumatoid arthritis called Rituximab (Rituxan) has been recognized as being an effective therapy for severe forms of certain small vessel vasculitic syndromes including Granulomatosis with polyangitis (GPA) and microscopic polyangiitis (at times referred to as ‘ANCA-Associated Vasculitis’).

Q5: I have lupus and am wondering what symptoms to look for when it comes to vasculitis? As someone living with lupus, I suffer from fatigue, joint pain, headaches and wonder what is specific to vasculitis?

Not all patients with lupus have vasculitis, and of course most patients with vasculitis can have it without having concurrent lupus. Some of the nonspecific symptoms that can be seen in lupus or vasculitis include fatigue, joint pain and headaches. Symptoms that would be more specific to vasculitis include certain forms of rashes (such as palpable purpura or raised red spots on the skin (which at times can break down); kidney inflammation; and neurologic problems including mononeuritis multiplex. This is when motor and sensory nerves can be damaged by the process in which the vascular supply to those nerves is disrupted by the inflamed and subsequently damaged vessels.

Q6: Are there different kinds of vasculitis? What makes vasculitis easy to manage? Anything that makes it more serious?

There are many different kinds of vasculitis. Vasculitis is not necessarily easy to manage and in many cases can be quite difficult. Corticosteroids (medications like prednisone) which are often the mainstay of therapy in vasculitis are effective but can be accompanied by significant side effects that can range from being bothersome to dangerous. Similarly, some of the more substantial immunosuppressive therapies used to treat vasculitis require knowledge on the part of the treating physician, as to how to use the medication and reliability on the part of the patient in terms of being compliant with recommendations regarding monitoring the medication. In the past two decades, rheumatologists have become much more skilled at being aware of the potential complications of these medications and how to mitigate against damaging side effects. The forms of vasculitis that are most serious are the ones that affect visceral organs such as kidneys, brain, heart, lung or nerves. Any organ system, however, could be involved and can be very limiting or damaging to the patient. In general these are considered fairly serious diseases.

Dr. Robert Spiera is a rheumatologist and Director of the Vasculitis and Scleroderma Program at Hospital for Special Surgery. He is the principal investigator in several clinical trials and observational studies focusing on scleroderma and vasculitis.

Topics: Rheumatology
The information provided in this blog by HSS and our affiliated physicians is for general informational and educational purposes, and should not be considered medical advice for any individual problem you may have. This information is not a substitute for the professional judgment of a qualified health care provider who is familiar with the unique facts about your condition and medical history. You should always consult your health care provider prior to starting any new treatment, or terminating or changing any ongoing treatment. Every post on this blog is the opinion of the author and may not reflect the official position of HSS. Please contact us if we can be helpful in answering any questions or to arrange for a visit or consult.


  1. I have PMR. I have had it for over 3 years. Treatment is prednisone. Current dosage is 5 mg. unable to get below that dosage. I read recently Dr. Robert Spiera study using Actemra as a new treatment for PMR. Is there a new study to be undertaken to investigate Actemra in longer term pmr patients???

    1. Hello- Our study was an “open label” trial addressing whether tocilizumab may have a role in treating PMR, and the results were encouraging, as were the results of an “open label” European trial also recently published. These studies however were not definitive proof that tocilizumab is helpful- that would require a “double blinded randomized placebo controlled trial”, in which one group of patients are treated with tocilizumab and a second group is treated with placebo, and we see whether the group treated with tocilizumab does better in terms of ability to come off steroids, less flares, etc. To my knowledge, no such study is ongoing at this time. There are other medications with a similar mechanism of action (IL-6 blockers) which are going to be looked at in trials of PMR, and the related condition, Giant Cell Arteritis. None of the upcoming trials however are enrolling patients with later disease as far as I know.

  2. I am a 66 year old woman in very good health and no history of any serious diseases. Occasionally, I have had borderline high BP, but it generally normal. I eat mostly a vegan diet and exercise every day. About a year ago, I thought I had ringworm on my lower trunk. I had no symptoms other than the circular red rash-like entities. My doctor ordered a biopsy and sure, enough , it came back as “low-level vasculitis” but the blood work was negative with respect to any serious disease or condition. Both my GP and dermatologist said this happens and often there is never a definitive answer as to what caused and that it would eventually go away. They have shown absolutely no concern and not suggested that I seek other opinions.

    Now, it has been a year and I still have a number of spots in the same area. They have not spread. They have not worsened and several have faded considerably.I have no other symptoms. I feel fine in all respects, but find the spots unsightly (Thankfully, I can hide them under my clothes)
    Should I just stay the course or see a vasculitis specialist?

    1. Hi Patricia, thank you for writing. Dr. Lindsay Lally, Rheumatologist, says: “What you are describing sounds like what physicians would call ‘cutaneous vasculitis’ or inflammation of blood vessels confined to the skin. There are several causes of vasculitis in the skin including medications (even over-the-counter drugs), infection and even some cancers. It sounds like your doctors made sure there was no infection or chronic disease causing the vasculitis and that none of your internal organs were involved. Most forms of cutaneous vasculitis should resolve on its own, especially if there is a known trigger that is removed. Sometimes as a rash heals, excess pigment can be deposited in the skin, which may have a dark red or brownish appearance depending on your skin tone. This hyperpigmentation is typically harmless, but may be cosmetically unappealing. A detmatologist can help you determine whether the spots you still have on your skin are related to ongoing vasculitis and inflammation in the skin or if they are healed areas with hyperpigmentation.”

  3. Hello,

    Back in the beginning of October I was walking with my parents to relieve some stress. It was getting cold out and I power walked 5 miles on the track. During this walk i did experience some discomfort in the ankles trying to keep up with my dad. Its the most I have ever walked on the track. When I got home, my legs were cold and I thought my raynaud’s was acting up because I had that hot feeling you get in your legs when you start to warm up. The next day I had what appeared to me as bloodshot marks on my ankles. I assumed this was from over doing it during the power walking. As the days went on they disappeared but more came up when I started working as a runner at a local restaurant. This time however it was starting to go up my legs and I went to the doctors and she tested me for Coxsackie, mono, strep and symphilis. All of these test came out clean. I mentioned something I researched myself, HSP vasculitis. 2 weeks later I was hit by a car and the rash got out of hand. It was all over my legs, feet, and butt. Plus i had some discomfort in my knees and ankles. I was seen by the dermatologist and he tested me for hepatitis A, B, and C, mono, strep, syphilis, ANA, CBC w/ Diff, and a few others. He said my blood looked clean. He also took 2 skin biopsies and the result was HSP vasculitis with a histologist differential diagnosis of leukocytoclastic vasculitis. My urine also shows a little bit of blood but everything else is showing normal. I was also bitten by many mosquitoes and had a insect bite that looked infected and i washed and disinfected and it cleared up shortly before the vasculitis occurred. My bites usually swell when I get them. Is it possible that this was caused by the exposure to the cold or stress through my raynaud’s or that an insect caused this?

    1. Hi Michelle, thank you for your note. Dr. Lindsay Lally, Rheumatologist, says: “HSP, also known as lgA vasculitis, is a common form of vasculitis in children and young adults. A raised red rash on the legs and buttocks is the most common manifestation of HSP. A biopsy of the skin showing lgA deposits in the small blood vessels in the skin can confirm a diagnosis of HSP. In addition to the skin, the gastrointestinal tract, joints and kidneys can also be involved. The underlying cause of HSP is unknown. However, many triggers associated with HSP have been identified. In almost 50% of cases, the onset of HSP is proceeded by an upper respiratory infection. Other infections such as strep have also been associated with development of HSP. HSP is also more common in the fall and winter, which may be related to higher frequency of infections during these months. There have been reports of HSP developing after insect bites or vaccinations. HSP is typically a self-limited disease. Most people with HSP need only ‘supportive’ care, meaning rest, hydration and symptomatic relief (for example, non-steroidal anti-inflammatory medication for joint or abdominal pain). In the first several months after a diagnosis of HSP has been made, monitoring for kidney involvement with regular blood pressure measurements and urinalysis to test for blood is important. To answer your question directly, it is possible that either the cold or the insect bites you experienced could have triggered the HSP. There is no known relationship between Raynaud’s and HSP.” It is best for you to consult with your treating physician so they can determine the best course of treatment. If you wish to receive care at HSS, please contact our Physician Referral Service at 877-606-1555 for further assistance.

  4. I have read the above, but really need to ask my questions of a Dr. I have had biopsies which show I have lymphocytic vasculitis. Now they have found white spots on my lungs, which they can’t identify, and a low white blood cell count. I don’t know where to turn. Please help

    1. Hi Catherine, thank you for your question. Dr. Robert Spiera, Rheumatologist, says: “Lymphocytic vasculitis can occur in a variety of systemic vasculitic syndromes. I suspect these findings were from a skin biopsy. There is a spectrum of conditions that can result in lymphocytic vasculitis in the skin. In the context of having ‘white spots’ on your lungs, however, this could raise concerns that it is a potentially more serious systemic vasculitis. The low white blood cell count generally is not seen in primary vasculitic syndromes, but can be seen in patients with connective tissue disorders and secondary vasculitis, such as in patients with Sjgren’s syndrome or systemic lupus who can have low white counts and can have vasculitis often on the skin. Addressing these issues adequately would involve a formal evaluation by your treating internist and preferably consultation with a rheumatologist.” If you are interested in care at HSS, please check with our Physician Referral Service at 877-606-1555.

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