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GI involvement challenging in systemic sclerosis: surgery is 'not a good way to go' for scleroderma patients, as it often compounds their GI problems.

SNOWMASS, COLO. -- Patients with systemic sclerosis are prone to a panoply of common GI problems that are unusually resistant to treatment, Dr. Daniel E. Furst said at a symposium sponsored by the American College of Rheumatology.

The drugs routinely used to treat these disorders--antisecretory agents, promotility drugs, and antibiotics--are usually effective. But scleroderma patients tend to require higher doses and much longer durations of treatment than do patients with the same GI disorders who don't have scleroderma, according to Dr. Furst, Carl M. Pearson Professor of Medicine at the University of California, Los Angeles.

GI surgery is often a last-ditch measure in patients with medically refractory erosive esophagitis, strictures, or colonic motility problems. But it is "not a good way to go" with scleroderma patients, he said. It often results in extensive fibrosis, which only compounds their GI problems.

Although some may see GI involvement in scleroderma as a field of study ripe for research, the less positive view is that it's a field in disarray--and always has been. It is less data driven and more dependent on anecdotal experience than are many other areas of medical research. Dr. Furst recalled a colleague who was fond of saying that no drug could be considered completely worthless until it had been tried in scleroderma, because everything works the first time in patients with this disease.

Nevertheless, some things that can be said with near certainty about GI involvement in systemic sclerosis. Symptoms start at the proximal end of the GI tract and progress distally. In one series of 226 patients, the prevalence of heartburn was 71%; other symptoms included dysphagia (53%), diarrhea (20%), constipation (19%), and bloating (10%).

The pathogenesis of GI disease in scleroderma is believed to involve arteriolar changes in the vaso vasorum resulting in smooth-muscle atrophy and fibrosis. "Most of the symptoms we see are the result of progressively weak gut muscular motion," explained Dr. Furst, who is also the director of the rheumatology clinical research center at UCLA.

He offered tips on the problems doctors can expect to encounter all along the GI tract in scleroderma patients, and on managing such patients. From the top down:

* Esophagus. Erosive esophagitis in scleroderma patients is not associated with the classic symptom of heartburn, although it is associated with dysphagia and abnormal peristalsis. Early esophageal involvement in scleroderma is characterized by diminished peristalsis and reduced function of the lower-esophageal sphincter, while esophageal emptying and pressure on the upper-esophageal sphincter are preserved.

Late expressions of scleroderma-related esophageal disease are marked by absent peristalsis and lower-esophageal sphincter function, delayed esophageal emptying, and decreased upper-esophageal sphincter function. The risk of esophageal cancer is elevated in scleroderma patients, but is not as high as many doctors think, Dr. Furst said. In a series of 262 patients who were followed for 11 years, there was one case of esophageal cancer. Esophageal stricture occurs in 7%-10% of scleroderma patients.

The standard dosage of a proton pump inhibitor, such as esomeprazole (Nexium), for erosive esophagitis is 20-40 mg once daily for 4-8 weeks, but that's often inadequate in patients with superimposed scleroderma. Symptomatic relief in these patients is no indication of healing on endoscopy. "Some of these patients need a lot more than you'd think. Three times per day [of proton pump inhibitor] therapy is something to consider, along with combination therapy with the addition of sucralfate and/or an [H.sub.2] blocker," he said.

* Stomach. Watermelon stomach, also known as gastric antral vascular ectasia, is "remarkably common" in scleroderma patients and is often asymptomatic. Treatment involves repeated endoscopic argon plasma laser coagulation sessions every 4-6 weeks until the lesions are completely healed. Periodic touch-ups are required.

* Small intestine. As the physician moves further down a scleroderma patient's GI tract, problems become a little more complicated. Malabsorption is often due to impaired gastric motility secondary to small-bowel fibrosis and may be accompanied by ischemia and bacterial colonization.

The most frequent cause of diarrhea and bloating in scleroderma patients is over-growth of the small-bowel bacteria by organisms that normally reside in the distal colon. Suppression using antibiotics often reduces these symptoms. It's a good idea to rotate antimicrobials; for example, a month of ciprofloxacin might be followed by a month of ampicillin, then a month of doxycycline, followed by metronidazole.

In addition to antibiotics, affected patients require promotility agents. Meeting this requirement can be a challenge. Metoclopramide, the most widely used drug, often is effective only at doses high enough to cause significant extrapyramidal side effects. Erythromycin at 250-333 mg three or four times daily is effective, but few scleroderma patients can tolerate it.

As an alternative, Dr. Furst said he has had good success using subcutaneous octreotide at 50 [micro]g in the evening. "This is a drug that can be used very effectively to keep your patients with frequent pseudoobstruction out of the hospital."

A fallback treatment for scleroderma patients who are deteriorating because of refractory malabsorption is home central venous hyperalimentation. In the short term, it produces significant weight gain and welcome increases in hematocrit and serum albumin levels. In the long term, however, significant problems--such as sepsis, hypoglycemia, catheter-related complications, thromboembolism, and premature death--eventually emerge.

* Colon. Colonic activity is abnormal in scleroderma patients. The conduit is stiff and doesn't tolerate much volume. Fecal incontinence and constipation are common, and are responsive to the usual measures.

But late-stage motility disorders in scleroderma patients are myopathic in nature and may require enterostomy. Resection is to be avoided because of the resultant (often severe) fibrosis, Dr. Furst said.

BY BRUCE JANCIN

Denver Bureau
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Title Annotation:gastrointestinal
Author:Jancin, Bruce
Publication:Internal Medicine News
Geographic Code:1U8CO
Date:Jun 1, 2004
Words:928
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