Scleroderma is a rare and chronic autoimmune disease that causes a build-up of collagen in various tissues. Because collagen is a major component of scar tissue, this causes patches of hardened and thickened scar-like skin seen in all types of scleroderma.

Systemic scleroderma is a more severe form of the disease, which affects various internal organs as well as the skin, including the gastrointestinal tract, or the digestive system.

A common problem associated with this is gastroesophageal reflux disease (GERD), where the seal connecting the stomach and the food pipe (or esophagus) weakens and does not close properly. This results in the contents of the stomach — as well as gastric acid — traveling back into the esophagus.

GERD is associated with frequent and painful heartburn, among other symptoms, as the stomach acid damages the esophagus. The esophagus becomes inflamed, and, if left untreated, can be permanently damaged by scarring.

Proton pump inhibitors, or PPIs, are one type of therapy used to manage scleroderma-associated GERD.

How do PPIs work?

PPIs work by reducing the amount of acid available in the stomach that causes damage.

Gastric acid is produced and secreted by specific cells called parietal cells that line the stomach wall. When these cells are stimulated, for example when eating food, they activate enzymes called H, K-ATPase, more commonly known as the proton pump. The action of the proton pump causes the gastric acid to be secreted into the stomach.

PPIs act by irreversibly binding to and blocking the proton pump, thereby preventing the secretion of stomach acid. Stomach acid production can restart only after new proton pumps are produced by the body.

PPIs are very effective at treating the symptoms of GERD, but do not provide immediate relief, unlike some therapies such as H2 blockers. It may take multiple doses of PPIs before the levels of proton pump in the stomach are consistently reduced.

Types of PPIs

PPIs can be purchased over the counter or by prescription, if stronger doses are needed. There are many types of PPIs available, sold under various brand names.

Some of the more common over-the-counter PPIs include Prilosec (omeprazole), Zegerid (immediate-release omeprazole with sodium bicarbonate), and Prevacid (lansoprazole).

Common prescription PPIs include Protonix (pantoprazole), Nexium (esomeprazole), Vimovo (esomeprazole and naproxen), Dexilant (dexlansoprazole), and Aciphex (rabeprazole).

Other information

Some of the more common side effects of PPIs include headaches, nausea, vomiting, diarrhea, constipation, light-headedness, rashes, and changes to the sensation of taste. Less common side effects include hair loss (alopecia), respiratory infections, increased liver enzymes, abnormal vision, and low magnesium levels in the blood.

Long-term use of PPIs can cause magnesium or vitamin B12 deficiencies, increased risk of bone fractures (osteoporosis), pneumonia, gastric infections, and the formation of gastric polyps.

But PPIs are often prescribed to scleroderma patients as the risk of permanent damage from GERD outweighs the possible long-term side effects of the medications.

Some patients may require a greater level of stomach acid suppression and may be prescribed other medications at the same time, such as H2 blockers. Prescribing H2 blockers alongside PPI therapy can reduce nocturnal acid breakthrough if the stomach acid increases at night despite taking PPIs.

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Scleroderma News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.