Gastrointestinal Tract Involvement in Systemic Sclerosis (SSc)

The gastrointestinal (GI) tract is often affected in systemic sclerosis. Up to 90% of people will have some form of GI tract involvement, which can occur at any point and may progress over time.  Any section of the GI tract may be affected, and whilst symptoms can vary greatly, effective treatments are available to help keep these under control.

The GI tract runs from top (mouth) to tail (anus). It allows the intake, digestion and absorption of food and the disposal of waste in the form of faeces. Food is propelled along through co-ordinated contractions of the muscles in the gut wall (peristalsis). In systemic sclerosis, thickening of the gut wall and atrophy (thinning) of the muscles can lead to the muscles of the digestive system not working as they should. This is known as dysmotility.

On these pages you will find information on the following:

Symptoms

Individual symptoms will depend on which part of the GI tract is affected. Early signs may seem quite non-specific, such as heartburn or a reduced appetite. If you do start to notice changes, it is important to tell your doctor because if left untreated, your condition may get worse.

General symptoms include:

  • Reflux/heartburn
  • Difficulty swallowing
  • Loss of appetite
  • Bloating
  • Nausea
  • Diarrhoea
  • Constipation

If your intestines are affected, specific symptoms may be:

  • Nausea and vomiting
  • Bloating
  • Increased flatus (wind)
  • Pain
  • Diarrhoea
  • Constipation (colonic involvement)

If your anorectum is affected, specific symptoms may be:

  • Increased bowel frequency
  • Constipation
  • Difficulties in emptying
  • Faecal incontinence

How SSc can affect the GI tract

The mouth 

Thinning of the upper lip and reduction in mouth opening may affect what a someone is able to eat comfortably. Oral dryness may also affect dental health. Telangiectasia means red vascular mucosal lesions in the mouth that may occasionally bleed.

The oesophagus

The oesophagus (gullet) runs from the mouth to the stomach. It is affected in most patients with SSc (80-90%), although this will not always cause any symptoms.

Gastro-oesophageal reflux occurs when acid from the stomach slips back into the oesophagus, which can lead to other complications such as oesophagitis, strictures (narrowing due to fibrosis) and Barrett’s oesophagitis (mucosal changes that require surveillance as there is a risk of developing malignant changes). Typical symptoms include difficulty swallowing, heartburn and water brash/reflux. Severe reflux can also result in acid spilling over into the lungs, that can  aggravate any lung involvement.

The stomach

The stomach is less commonly involved in SSc. The two main features are:

  • Vascular lesions (e.g. gastric antral vascular ectasia), which can lead to bleeding (acute and chronic) and anaemia
  • Delayed emptying of the stomach with bloating due to dysmotility, that may contribute to reflux

The small and large intestines

The small intestine absorbs nutrients from food that is digested, whereas the main function of the large intestine (the colon) is to reabsorb water and salts that have been secreted by the rest of the gut, as well as the disposal of waste. Both the small and large intestines may be affected in several ways, including reduced movement, reduced absorption (of various minerals/vitamins/iron/fatty acids), and overgrowth of bacteria.

Anorectum

The rectum is designed to hold faeces until the bowels are emptied, maintaining continence with the help of the anal sphincters. If anorectal involvement in scleroderma is not managed effectively, it may lead to rectal prolapse and thinning of the anal sphincter, as well as incontinence.

Diagnosis

Testing is an essential part of the management of SSc, and this can detect signs of GI tract involvement at an early stage. Your doctor may diagnose your GI tract involvement in SSc by asking about your medical history to learn about signs and symptoms, as well as possible risk factors. Relevant tests are described below.

Gastroscopy

A procedure done usually under sedation where a flexible telescope is passed by the mouth down into the stomach. A paediatric scope, which is smaller, may be used to minimise discomfort caused by a smaller opening. It allows direct vision of the oesophagus, stomach and first part of the small intestine. Biopsy (extracting cells) of the mucosal surfaces may be taken to evaluate any changes.

Oesophageal physiology studies

This involves a small tube being passed from the nose to the stomach. The amount of acid reflux can be measured by a 24- hour study, again involving a small tube passed from the nose to the oesophagus. 

Barium Swallow

A barium swallow is an examination of the oesophagus and the stomach. This test will be conducted by a radiologist (a doctor who uses x-rays to diagnose and treat illnesses), and a radiographer (a health professional that produces the images used to diagnose and treat illnesses).

The procedure uses a type of X-ray called fluoroscopic imaging to view images in real time. These areas of your body cannot normally be seen on X-ray images, so you will be required to swallow contrast material. This enables your doctors to evaluate the surface changes as the contrast material passes through the oesophagus and stomach.  The images are taken as you swallow the liquid and as it passes into your stomach. The radiologist can check the oesophagus and stomach and see how well the liquid moves through them.

Endoscopy

An endoscopy is a procedure where organs inside your body are looked at using an instrument called an endoscope. An endoscope is a long, thin, flexible tube that has a light and camera at one end. Images of the inside of your body are shown on a television screen. This test can also detect and help manage strictures (narrowing of the esophagus).

Oesophageal Manometry

Oesophageal manometry is a test used to measure the pressure and functioning of the muscles of the oesophagus and the sphincter leading to the stomach. This indicates how well the muscles are performing as food and liquids pass from mouth to the stomach

MRI (magnetic resonance imaging) scan

This test uses magnetism to create a detailed image of areas of your body. This scan may be used to assess whether the GI tract is involved in SSc.  Sometimes people are injected with a dye (contrast medium) to help make images show up more clearly. The procedure (carried out by a radiographer) is painless, however you may find it a little uncomfortable as you have to lie in a long tube for about 30 minutes. Importantly, you can only have the scan if you do not have any metal within your body (i.e. dental wires or metal plates). With MRI, patients do not have any radiation exposure.

Hydrogen or Lactulose breath test

This requires ingestion of a sugary solution and testing for serial breath samples over several hours. This may be considered in cases of suspected bacterial overgrowth.

Anorectal physiology studies

These are tests that check the function and structure of the anal sphincters and rectum and involve a small probe inserted at the tail end.

Treatment

There are several treatments available for GI tract involvement in SSC, and the best option will always depend on your individual situation. 

Types of treatment

You may suffer from poor oral intake and weight loss. Consuming small, but frequent meals, and nutritional and vitamin supplements can help many patients. Sometimes more extreme approaches are needed if you suffer from severe weight loss and malnutrition and are unable to eat adequately by mouth, such as having a small tube passed into the stomach, nasogastric tube (where a tube is inserted through the nose and throat into the stomach), percutaneous endoscopic gastrostomy (PEG; where a flexible feeding tube is placed through the abdominal wall and into the stomach) or intravenous feeding (tube inserted into veins).

Reflux can be treated with anti-reflux medications (proton pump inhibitors and antacids e.g. Gaviscon Advance, Rennies). If you suffer from dysmotility (swallowing difficulties, nausea, vomiting), this can be helped by prokinetic medications, such as domperidone, metoclopramide, and prucalopride.

Gastric antral vascular ectasia (GAVE or bleeding from stomach) can be alleviated with iron supplements (tablets or intravenous formulation) or endoscopic treatment with thermal/radiofrequency methods to stem the bleeding points.

Small bowel bacterial overgrowth can be managed with antibiotics. Either single course or cyclical courses may be required, and this largely depends on frequency and severity of your symptoms. Adjusting your diet can also help to alleviate symptoms of bacterial overgrowth.

Constipation and diarrhoea can be managed symptomatically but judicious use is required as some individuals may experience both constipation and diarrhoea.

Bowel rest may be required on occasion for severe cases of non-mechanical bowel obstruction. This often requires medical evaluation with inpatient admission.

Faecal incontinence is often a challenge, and this requires careful evaluation with your gastroenterologist. Obstetric history with forceps delivery may be relevant in some cases. In some selected cases, nerve stimulation to the anorectal apparatus can be considered. Pelvic floor physiotherapy can also be helpful.

Treatment for your anorectum is tailored to your individual’s symptoms and the abnormalities found. Many different treatments can be used and are often very effective. Practical changes, such as raising the head of the bed, can be very helpful to give immediate relief. You may suffer from diarrhoea and incontinence, treatment for this include loperamide and opiates. More specialised treatments maybe required such as trans-anal irrigation, sacral nerve stimulation, surgery (e.g. rectal prolapse repair).

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