Special Feature


Vascular dilemma




Posted on February 25, 2017


IN 1908, Leo Buerger, an assistant adjunct surgeon at The Mount Sinai Hospital, in New York City, published a study in The American Journal of the Medical Sciences describing a disease which had first been reported by the physician Felix von Winiwarter in 1879. In one case, the said disease caused the obliteration of almost every artery in the leg of a patient.

  
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“We usually find it occurring in young adults between the ages of 20 and 35 or 40 years, and it is because the gangrenous process may begin at an early age that the names presenile and juvenile gangrene have been employed,” he wrote. Gangrene is the death of body tissues due to loss of blood supply.

He observed that patients complained of indefinite pains in their feet, in the calves of their legs or in their toes and numbness or coldness. The feet looked pale, “almost cadaveric.” A blister, hemorrhagic bleb or ulcer developed near the tip of one of the toes and under the nail, resulting in intense pain. “Even before the gangrene, at the ulcerative stage, amputation may become imperative because of the intensity of the pain,” Dr. Buerger said.

The disease has come to be known as Buerger’s disease or thromboangiitis obliterans, the inflammation of and clotting in the arteries and veins of the hands and feet. It is a rare form of vasculitis. According to an article by Perttu Arkkila that appeared in the Orphanet Journal of Rare Diseases, it is found worldwide, but its incidence is highest in the Middle and Far East. He added, “The prevalence of the disease among all patients with peripheral arterial disease ranges from values as low as 0.5 to 5.6% in Western Europe to values as high as 45 to 63% in India, 16 to 66% in Korea and Japan, and 80% among Jews of Ashkenazi ancestry living in Israel.”

The most popular face of Buerger’s disease in the Philippines is no other than President Rodrigo Duterte himself. In a speech before business leaders at Malacañang last December, he said, “And I have, might as well tell you about my medical history. Don’t believe in cancer. What I have is really Buerger’s disease. It’s an acquired thing that you get from smoking because of nicotine.”

More than a hundred years after the publication of Buerger’s findings, no one knows what causes the disorder. But the connection between Buerger’s disease and smoking is unarguable. “Most patients with Buerger’s are heavy smokers, but some cases occur in patients who smoke “moderately”; others have been reported in users of smokeless tobacco,” the US-based Johns Hopkins Vasculitis Center said. “It has been postulated that Buerger’s Disease is an “autoimmune” reaction (one in which the body’s immune system attacks the body’s own tissues) triggered by some constituent of tobacco.”

Males aged 20 to 40 are the most commonly affected by the disease, but cases involving females and people aged 50 and above have been reported. The center said these people often experience pain induced by insufficient blood flow during exercise in the hands and/or feet (claudication) or even while at rest, and this pain can spread to other parts of the body. “Other signs and symptoms of this disease may include numbness and/or tingling in the limbs and Raynaud’s phenomenon (a condition in which the distal extremities -- fingers, toes, hands, feet -- turn white upon exposure to cold). Skin ulcerations and gangrene of the digits (fingers and toes) are common in Buerger’s disease,” the center said. “Pain may be very intense in the affected regions.”

It is possible to confuse Buerger’s disease with other disorders, such as atherosclerosis, the build-up of cholesterol plaque in the arteries, and endocarditis, an infection of the lining of the heart, since diminished blood flow to extremities can be caused by more than one condition. “These other disorders must be ruled out with an aggressive evaluation, because their treatments differ substantially from that of Buerger’s Disease,” Johns Hopkins Vasculitis Center said, adding that angiograms of the upper and lower extremities is helpful in making a diagnosis of the disease.

Buerger’s disease has no cure. The center said anti-inflammatory agents like steroids and anticoagulation strategies such as thinning the blood with aspirin have not been beneficial and effective. “It is essential that patients with Buerger’s disease stop smoking immediately and completely. This is the only treatment known to be effective in Buerger’s disease,” the center said. “Patients who continue to smoke are generally the ones who require amputation of fingers and toes.” -- Francis Anthony T. Valentin