Below are perspectives on key reports presented at the recent national meeting of the American College of Rheumatology:
- Wegener's Graunolmatosis
- Bechet's Disease
- Kawasaki's Disease
- Henoch-Schonlein Purpura
- Takayasu's Arteritis
- Polyarteritis Nodosa
- Churg-Strauss Syndrome
Vasculitis is a general term for a group of uncommon diseases that feature inflammation of the blood vessels. The blood vessels of the body are referred to as the vascular system. The blood vessels are composed of arteries, which pass oxygen-rich blood to the tissues of the body, and veins, which return oxygen-depleted blood from the tissues to the lungs for oxygen. Vasculitis is characterized by inflammation in and damage to the walls of the various blood vessels.
Each of the vasculitis diseases (also, as a group, referred to as vasculitides) is defined by certain patterns of distribution of blood vessel involvement, particular organ involvement, and laboratory test abnormalities. The diagnosis of vasculitis is confirmed by a biopsy of involved tissue or angiography. The treatment is directed toward decreasing the inflammation of the arteries and improving the function of affected organs.
Below are perspectives on key reports on vasculitis diseases presented at the recent national meeting of the American College of Rheumatology:
Wegener's granulomatosis classically involves inflammation of the arteries that supply blood to the tissues of the lungs, the nasal passages (sinuses), and the kidneys. There are "incomplete" forms of the disease that only involve one of these areas. Symptoms of Wegener's granulomatosis include fatigue, weight loss, fevers, shortness of breath, bloody sputum, joint pains, and sinus inflammation (sinusitis). Nasal ulcerations and even blood nasal discharge can occur. Other areas of the body that can also become inflamed in patients with Wegener's granulomatosis include the eyes, the nerves (neuropathy), the middle ear (otitis media), and the skin, which results in skin nodules or ulcers.
Cytoxan (cyclophosphamide) that is taken by mouth with prednisone until the disease is in remission and then switched to methotrexate for 2 years and tapered off was effective and less toxic than the traditional long-term Cytoxan treatment.
Dr. Shiel's Perspective: This was an important paper presented at this meeting. Methotrexate has recently been introduced as a drug for Cytoxan treatment failures. I have several patients doing very well on it for this purpose in my practice. The significance of this paper is twofold. First, while Cytoxan is very effective, it is also toxic. It now appears that Cytoxan will not be necessary in order to maintain long-term remission and that doctors can convert to the less toxic methotrexate for maintenance. Secondly, the report also demonstrates that methotrexate can eventually be tapered off entirely after 2 years. This is good news for patients with Wegener's granulomatosis.
Behcet's syndrome is classically characterized as a triad of symptoms that include recurring crops of mouth ulcers (called apthous ulcers), genital ulcers, and inflammation of a specialized area around the pupil of the eye, the uvea. The inflammation of the area of the eye that is around the pupil is called uveitis. Behcet's syndrome is also sometimes referred to as Behcet's disease.
Dr. Shiel's Perspective: Trental also seemed to maintain the healed ulcers for up to the 29 months of the study. The effectiveness of Trental, the researchers said, seemed to be enhanced by the combination with colchicine in some patients. I felt this paper was significant because, to date, colchicine has been the mainstay of treatment of these often terribly painful sores. Now, it appears that we have other options.
Kawasaki's disease is a syndrome of unknown cause that mainly strikes young children. Signs of Kawasaki's disease include fever and red eyes, hands, feet, mouth, and tongue. Kawasaki's disease can be treated with high doses of aspirin (salicylic acid) and gamma globulin. Kawasaki's disease usually resolves on its own within a month or two. Some children with Kawasaki's disease suffer damage to the coronary arteries.
Plasma Exchange (Plasmapheresis) was reported as effective in patients who were not responding to aspirin and gamma globulin.
Dr. Shiel's Perspective: Plasmapheresis is a procedure whereby the patient's plasma is removed from the blood and replaced with protein-containing fluids. By taking out portions of the patient's plasma, the procedure also removes antibodies and proteins that are felt to be part of the immune reaction that is causing the inflammation of the disease. Kawasaki's disease that is not responding to the traditional aspirin and gamma globulin treatments can be deadly. This research offers hope to those who are failing treatment.
Henoch-Schonlein purpura (HSP or anaphylactoid purpura) is a form of blood vessel inflammation, or vasculitis. There are many different conditions that feature vasculitis. Each of the forms of vasculitis tends to involve characteristic blood vessels. HSP affects the small arterial vessels, called capillaries, in the skin and frequently in the kidneys. HSP results in skin rash (most prominent over the buttocks and behind the lower extremities) that is associated with joint inflammation (arthritis) and cramping pain in the abdomen.
Steroids (prednisone or prednisolone) early on in treating HSP were not helpful in reducing kidney damage.
Dr. Shiel's Perspective: It seems that the effects of HSP on the kidneys are the same whether or not steroids are used. It is still possible that the bowel disease in these patients, which causes blood in the stools and belly pain, might be responsive to the steroid medications.
Takayasu's disease, or Takayasu's arteritis, is a chronic inflammation of the large blood vessel that distributes blood from the heart, called the aorta. It is most common in women (90%) of Asian descent. The disease usually begins between 10 and 30 years of age and its cause is unknown. The inflammation of the aorta and its branch arteries can lead to a poor blood supply to the tissues of the body in patients with Takayasu's disease. This can cause painful, cool or blanched (white color) extremities, dizziness, headaches, chest pain, and abdominal pain. Other early symptoms can include fatigue, weight loss, and low-grade fever. Takayasu's disease has also been referred to as "pulseless disease" and "aortic arch syndrome."
Symptoms and Signs in 117 patients with Takayasu's arteritis were described.
Dr. Shiel's Perspective: This report was important for doctors to review. One of the key highlights that the researcher pointed out to me was that magnetic resonance angiography (MRA) could be used as a non-invasive method of diagnosing patients with this illness. Also, that doctors who treat children should be alert to the combination of findings of elevated blood sedimentation rate (sed rate, a test for inflammation) and elevated blood pressure (hypertension, which is very unusual in the young).
Polyarteritis nodosa (PAN) is a rare autoimmune disease (immune system attacking its own body) that is characterized by spontaneous inflammation of the arteries (arteritis) of the body. Because arteries are involved, the disease can affect virtually any organ of the body. The most common areas of involvement include the muscles, joints, intestines (bowels), nerves, kidneys, and skin. Poor function or pain in any of these organs can be a symptom. Poor blood supply to the bowels can cause abdominal pain, local bowel death, and bleeding. Fatigue, weight loss, and fever are common. Polyarteritis nodosa is most common in middle-aged persons. Its cause is unknown, but it has been reported after Hepatitis B infection.
Dr. Shiel's Perspective: Traditionally, PAN that is not associated with hepatitis virus infection is treated with high doses of steroids and Cytoxan (cyclophosphamide). Cytoxan can suppress the immune system. Thus, if a patient's PAN is associated with hepatitis infection, the virus infection could worsen with Cytoxan. These researchers report that removing immune factors in the blood with plasmapheresis combined with antivirus drug (lamivudine) can be beneficial in the situation of Hepatitis B-related PAN. This is very helpful information for doctors and those patients affected by this condition.
Churg-Strauss syndrome is a rare disease and is one of many forms of vasculitis. Vasculitis diseases are characterized by inflammation of blood vessels. Churg-Strauss syndrome, in particular, occurs in patients with a history of asthma or allergy and features inflammation of blood vessels (also referred to as angiitis) in the lungs, skin, nerves, and abdomen. The blood vessels involved in Churg-Strauss syndrome are the small arteries and veins. The cause of the syndrome is unknown, but it involves an abnormal over-activation of the immune system. Treatment usually includes high doses of cortisone-related medication (such as prednisone or prednisolone) to calm the inflammation and suppression of the active immune system with cyclophosphamide (Cytoxan). Churg-Strauss syndrome is also called allergic granulomatosis and angiitis.
Churg-Strauss Syndrome patients did equally well if treated with Cytoxan (cyclophosphamide) for 6 or for 12 months.
Dr. Shiel's Perspective: This study suggests that doctors might now be able to recommend a shorter (and, therefore, less toxic) course of Cytoxan for patients with Churg-Strauss Syndrome.