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Dr. Shiel Gives Perspectives Of Interest On Vasculitis From 2001 Annual Scientific Meeting Of The American College Of Rheumatology

Below are perspectives on key reports presented at the recent national meeting of the American College of Rheumatology:

• Introduction
• Wegener's Graunolmatosis
• Bechet's Disease
• Kawasaki's Disease
• Henoch-Schonlein Purpura
• Takayasu's Arteritis
• Polyarteritis Nodosa
• Churg-Strauss Syndrome

Introduction

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

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.