Presented at the Royal Free Hospital 4^th March 2004

A dry mouth is a common problem due to the reduced production of saliva that can occur in scleroderma. This may lead to early gum disease and tooth decay. Therefore regular visits to the dental hygienist are helpful. A smaller mouth opening which often accompanies this disease may hinder treatment.

Most patients present with symptoms from the oesophagus, the tube joining the mouth to the stomach. Amongst these is the pain caused by acid from the stomach passing upward into the oesophagus (acid reflux) giving rise to heartburn or a recurrent cough particularly at night. Treatment with drugs which reduce acid production, such as Losec or Zoton, are often required to be taken for life. Resistant cases may require double the recommended dose for a few weeks. Ranitidine taken at night may be added.

Difficulty in swallowing solid foods is a common complaint. This may be helped by taking plenty of fluids with each meal. Domperidone which increases the movement of the oesophagus is sometimes prescribed. Avoiding large meals late at night is also helpful.

A stricture, a tight circumferential band of scar tissue in the oesophagus, often results from recurrent acid reflux and may cause vomiting. This is treated by dilatation or stretching, a procedure usually carried out under sedation as an outpatient by passing a fibreoptic tube down the oesophagus (an endoscopy) and using a balloon to open up the stricture.

Persistent acid reflux may change the lining of the oesophagus in a few patients to a degree that may predispose it to malignant changes. These patients may have to undergo repeat endoscopy every two years with a small biopsy of the gastro-oesophageal junction to make sure it is not becoming cancerous.

A common stomach problem is early satiety or a feeling of fullness occurring after a few mouthfuls of food. This is often associated with abdominal discomfort. Drugs such as domperidone, metoclopramide and erythromycin may be helpful to increase the movements of the bowel.

Bleeding sometimes occurs at the junction of the oesophagus and stomach, or from the stomach itself, from dilated blood vessels. The latter can be treated with a laser. If the bleeding occurs surreptitiously, without being noticed by the patient, the blood count will fall causing anaemia. This will lead to shortness of breath and a feeling of weakness particularly on exertion.

Problems of the small intestine may give rise to pain, bloating of the abdomen or diarrhoea due to stagnation of the contents of the gut allowing overgrowth of normal bacterial organisms. Treatment includes Imodium and antibiotics often given in rotation.

The small intestine is responsible for absorbing all valuable nutrients from the diet. Even if this organ fails completely, as it does in only a minute number of patients, feeding can still be continued using the intravenous route.

Large intestinal problems are also common. These include constipation, difficulty with evacuation, faecal urgency and incontinence. The latter is a particularly disturbing symptom and is often not admitted by the patient as they feel socially embarrassed.

It is important to investigate these problems as the more common condition of diverticular disease may present with similar symptoms and requires different treatment.

Cancer of the colon can present with these symptoms but it is often accompanied by a sudden change in bowel habit. This needs to be excluded by further tests including special Xrays and colonoscopy.

A few patients develop a rectal prolapse where the lining of the bowel is extruded through the anus. This condition can be treated with an operation but patients need to be screened for heart and lung disease before having an anaesthetic.

Surgical treatment of prolapse does not always involve opening the abdomen. Patients who are already incontinent, however, before their operation may not regain their continence after this procedure although they are usually cured of their prolapse symptoms.

Incontinence is sometimes resolved with simple remedies such as control of diarrhoea, anal plugs and occasionally various surgical procedures such as sacral nerve stimulation. This is a field where treatment is improving. Only rarely if the patients symptoms are intolerable will a colostomy be suggested.

Patients should be aware of a situation where they may suddenly develop signs of abdominal pain, vomiting and inability to pass a motion. This is referred to as pseudo-obstruction and should not normally be treated with an operation. The patient should be referred to a centre used to dealing with scleroderma where they will be given the appropriate medical treatment.

Occasionally scleroderma patients produce insufficient juice from the pancreatic gland. This may exacerbate their gut symptoms but can be treated by taking Creon, a pancreatic substitute.

Finally there is a small group of patients who develop an associated condition of the liver called primary biliary cirrhosis. In this the patient presents with tiredness and skin irritation.

Diagnosis is by a simple blood test and liver biopsy.This complication may contribute to malabsorption of vital food products and can lead to thinning of the bones. Very rarely this will require liver transplantation.

Gut problems in scleroderma are common. Symptoms are often only reluctantly admitted by the patient. They require early investigation and are amenable to treatment.