How does scleroderma affect the esophagus?

Patients with scleroderma of the esophagus typically lack this peristaltic contraction, which is called aperistalsis. When this occurs, foods, particularly solids, hang up in the esophagus and produce dysphagia (the sensation that food is stuck in the chest).

Does scleroderma cause esophageal dysmotility?

Hypotensive LES was encountered in more than 50% of patients, whereas esophageal body dysmotility was present in more than 60% of patients. Classic scleroderma esophagus was found in as many as 55% of patients [24-30].

Can scleroderma cause esophageal cancer?

An increased risk of esophageal carcinoma has been reported in systemic sclerosis (scleroderma). Periodic endoscopic or radiologic surveillance, or both, of systemic sclerosis patients with gastroesophageal reflux symptoms has been recommended for early detection of Barrett’s mucosa and esophageal adenocarcinoma.

How is scleroderma of the esophagus treated?

There is no cure for esophageal or other types of scleroderma, but your physician can provide relief with certain treatments, including: Medications: Over-the-counter and prescription medication can control stomach acid production. Other medications promote better muscle activity in the esophagus.

Can scleroderma affect the lungs?

The lungs are involved in around 80% of all patients with scleroderma. Lung involvement in all its forms has emerged to be the leading cause of death and disability.

What are the pathological changes associated with scleroderma?

The clinical and pathologic manifestations result from three distinct processes: 1) severe fibroproliferative vascular lesions of small arteries and arterioles, 2) excessive and often progressive deposition of collagen and other extracellular matrix (ECM) macromolecules in skin and various internal organs, and 3) …

How is scleroderma contracted?

Research suggests that, in some people, scleroderma symptoms may be triggered by exposure to certain viruses, medications or drugs. Repeated exposure, such as at work, to certain harmful substances or chemicals also may increase the risk of scleroderma. An environmental trigger is not identified for most people.

Can scleroderma turn into cancer?

Scientists have previously found that some patients with scleroderma have a higher incidence of cancer. In the most severe cases of scleroderma, patients with antibodies against RPC1 develop cancers around the time of their diagnosis more frequently than patients who have other antibodies.

What cancers are associated with scleroderma?

Squamous cell carcinoma has been reported in association with a rare form of localized scleroderma referred to as pansclerotic morphea. Patients with pansclerotic morphea are found to be at a higher risk for developing squamous cell carcinoma of the skin.

Can you get a lung transplant if you have scleroderma?

Abstract. Background: Due to its multisystemic nature, scleroderma is considered a relative contraindication to lung transplantation at many centers. However, recent studies suggest similar post-transplant outcomes in patients with scleroderma compared to those with other causes of interstitial lung disease (ILD).

What is pulmonary scleroderma?

Scleroderma-related interstitial lung disease (SSc-ILD) is a pulmonary fibrosing disorder characterized by systemic inflammation and progressive scarring of the lungs that leads to respiratory failure.

What are the possible complications of lung involvement in scleroderma?

Lung involvement occurs in both diffuse and limited scleroderma; thus all patients need to be concerned about this potential complication. Involvement of the lung causes shortness of breath or fatigue during physical activity.

What are the key elements of scleroderma lung disease?

The key elements of scleroderma lung disease involve 1) inflammation (potentially treatable); 2) lung scarring (not reversible but potentially preventable); and 3) blood vessel injury ( treatable – see Pulmonary Hypertension ). Inflammation and scarring of the lung tissue is called interstitial lung disease or ILD.

Is esophageal involvement in pulmonary disease associated with severity of pulmonary disease?

Compelling evidence has correlated esophageal involvement to the severity of pulmonary disease.

What is the esophageal involvement in systemic sclerosis (SSc)?

The esophagus is the most commonly affected part of the gastrointestinal system in patients with systemic sclerosis (SSc). Esophageal involvement may lead to a significant reduction in patient quality of life. The exact pathophysiology is complex and not yet fully elucidated.