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lindagray

Scleroderma Symptoms 3

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Pulmonary (Lungs)

Some reduction of lung functioning occurs in almost all cases of scleroderma, both in the limited and diffuse forms. However, unless closely monitored, there may be no symptoms until later stages of the disease, at which point lung problems can become a major cause of death. The most common initial symptom is shortness of breath after exercise or other exertion. Later, a persistent non-productive cough can develop. Usually, there is no chest pain caused by the lung involvement, although chest pain can occur from other causes such as muscle pain or heartburn.

There are two different lung complications that are associated with scleroderma – interstitial lung disease (ILD) and pulmonary artery hypertension (PAH).  While these can both occur, there is a definite association between different subtypes of scleroderma and the likelihood of developing these specific complications.

Interstitial lung disease (ILD) is basically inflammation and scarring of the lung tissue caused by progressive fibrosis of the lungs.  Pulmonary artery hypertension means high blood pressure in the lungs.  Patients with limited scleroderma have the greater risk of developing progressive blood vessel narrowing in the lungs even in the absence of lung scarring and inflammation.

The main diagnostic tool for lung problems is a pulmonary function test (PFT), often done on a yearly basis.  Two different measures are specifically looked at when diagnosing lung problems: forced vital capacity (FVC) and diffusion capacity of the lung for carbon (DLCO).  With ILD, these two measures tend to decline simultaneously while with PAH the DLCO measure declines more rapidly.  High-resolution computerized axial tomography (CAT scan) is more useful for diagnosis of ILD than a standard x-ray.  For detection of PAH, Doppler echocardiography is useful and should also be done on an annual basis for patients who are likely to develop this complication (based on specific antibody subtypes).  An additional method of evaluating the severity of PAH, as well as the effectiveness of PAH treatments is a simple test called the 6-minute walk test (6MWT).  Basically, this test measures the distance that a patient can walk on a flat, hard surface in a period of 6 minutes.  This test is inexpensive and easy to perform and assesses distance walked, shortness of breath, and O2 saturation levels.  Several studies have shown that the score on the 6MWT is correlated with mortality rates over a one to three-year period.

While the course of lung involvement is highly variable, most patients have an early but limited decline in lung functioning and then either stabilize or improve. About one third of patients have a more severe progression for several years before stabilizing. While other lung problems can develop secondary to other complications, these are much less common. In addition, there is an increased risk of lung cancer with scleroderma.

Gastrointestinal

Some of the most common symptoms of scleroderma are various difficulties with the gastrointestinal tract. This occurs with both systemic forms of the disease. As fibrosis develops in the upper part of the gastrointestinal tract, moderate to severe heartburn commonly develops.  At later stages, the muscles that propel food from the mouth to the stomach function less efficiently leading to difficulty in swallowing.  In addition, the stomach may empty more slowly adding to heartburn symptoms and causing bloating, nausea, and vomiting.  Some scleroderma patients develop what is called “watermelon stomach” (GAVE syndrome), in which the stomach develops red streaked areas from widened blood vessels.  This can increase the risk of stomach cancer and can lead to anemia (low red blood cell counts).

There is some research that supports the idea that patients with severe reflux disease may breathe in tiny amounts of stomach acid that in turn may contribute to lung scarring and fibrosis.  Untreated or undertreated reflux can lead to erosion of the esophagus resulting in bleeding, strictures with narrowing of the esophageal opening, and Barrett’s esophagus, a pre-cancerous condition.

In the lower part of the GI tract, movement of the food through the intestines can be slowed, sometimes resulting in an increase of bacterial levels in the intestines and reduction in food absorption.  This can cause weight loss, cramping, constipation or diarrhea, and in severe cases, malnutrition.  An under-reported symptom that can develop with severe scleroderma is fecal incontinence (leakage) because of fibrosis and reduced muscle tone of the internal anal sphincter.

Two tests are often used to investigate the extent and severity of upper GI symptoms related to systemic scleroderma: upper endoscopy and esophageal manometry.

  • Upper Endoscopy involves using a thin scope with a tiny light and camera that is inserted through the mouth to examine the condition of the esophagus, stomach, and the first part of the small intestine. This is done under sedation in a hospital setting and allows the physician to detect a number of potential Scleroderma related complications, including esophageal erosion from frequent reflux, Barrett’s esophagus, and watermelon stomach.

  • Esophageal Manometry is a procedure for measuring how well the muscles of the esophagus work, especially the strength of the lower esophageal sphincter.  The procedure is performed by passing a thin plastic tube through one nostril, down the throat, and into the esophagus.  Once the tube is inserted, pressure readings can be done when the esophagus is resting or when the patient is swallowing.  The strength of contractions while the patient is swallowing can help to diagnosis a number of swallowing related problems, and the measure of LES pressure can be useful in determining appropriate treatments for the patient’s reflux problems.

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