Scleroderma is a chronic condition, characterized by excessive production of a protein, collagen, in response to the immune system mistakenly attacking healthy tissues. This leads to fibrosis or the formation of scar tissue, causing the skin to tighten and harden.
In more severe cases, scarring can damage organs in ways that lead to the patient developing one or more scleroderma-related conditions.
These scleroderma-associated comorbidities, and their treatments, are listed below.
Therapies for pulmonary arterial hypertension (PAH)
Pulmonary arterial hypertension (PAH), or high blood pressure in the arteries of the lungs, is a result of the blood vessels narrowing and making it more difficult for blood to flow through the lungs. Typically, this results from the combination of scar tissue building in the arteries, causing them to become smaller and stiffer, and from the damage response that causes blood vessel walls to contract, and the arteries becoming narrower.
Most approved treatments for PAH focus on dilating the arteries to allow for better blood flow. They can be categorized based on the biological pathway they target to dilate blood vessels. These therapies include:
- Endothelin receptor antagonists, such as Letairis (ambrisentan) and Opsumit (macitentan)
- Prostaglandin equivalents, such as Flolan (epoprostenol), trepostinil, and Ventavis (inhaled iloprost)
- PDE5 inhibitors, such as Revatio (sildenafil) and Adcirca (tadalafil)
- IP receptor agonists, such as Uptravi (selexipag)
Therapies for kidney problems
Scleroderma can cause serious damage to internal organs, including the kidneys, which if not treated can lead to renal crisis and kidney failure. Kidney problems are normally treated using angiotensin-converting enzyme (ACE) inhibitors. In extreme cases, the patient may require dialysis to filter their blood, or a kidney transplant.
Therapies for muscle and joint problems
Inflammation caused by scleroderma can lead to chronic arthritis-like pain in the muscles, joints, and tendons. Discomfort can be managed by pain-relief medications, such as Ultram (tramadol). In extreme cases, narcotic/opioid analgesics may be prescribed, including Vicodin (acetaminophen/hydrocodone), Percocet (acetaminophen/oxycodone), and OxyContin (oxycodone hydrochloride). However, these medications can be addictive, and should be prescribed and used with caution.
Medications to reduce inflammation can also help to manage muscle and joint pain. Examples include non-steroidal anti-inflammatory drugs (NSAIDs), such as ibuprofen, COX-2 inhibitors, such as Celebrex (celecoxib), and corticosteroids, such as prednisone and Medrol (methylprednisolone).
Therapies for interstitial lung disease (ILD)
Interstitial lung disease (ILD) can develop due to a buildup of scar tissue in the lungs, causing shortness of breath, fatigue, and other problems related to difficulties breathing. Several therapies work to improve lung function in patients with ILD.
These include therapies to suppress the immune system, like CellCept (mycophenolate mofetil), cyclophosphamide, and Imuran (azathioprine), or anti-fibrotic therapies that aim to slow or prevent further scar tissue accumulation, such as Esbriet (pirfenidone) and Ofev (nintedanib).
Therapies for Sjögren’s syndrome
- Eye drops, which can help reduce dryness in the eyes. If inflammation occurs, prescription drops containing steroids may be required.
- Saliva substitutes or stimulants, which may reduce dryness in the mouth and help with swallowing. However, long-term use can affect dental health and hygiene. These medications include Salagen (pilocarpine) and Evoxac (cevimeline).
- Vaginal dryness can be treated using lubricants, specialized moisturizers, or hormone replacement therapy.
Therapies for sexual dysfunction
Scleroderma can impact sexual relations and sexual well-being in some cases.
The condition, for example, can lead to erectile dysfunction in men due to problems with blood flow to the penis. This may also be treated with PDE-5 inhibitors, such as those used to treat PAH, and include Viagra (sildenafil), Levitra (vardenafil), Cialis (tadalafil), and Spedra (avanafil).
Lichen sclerosus is a related condition causing fragile, white patches of cracked, sore, and itchy skin around the genitals and anus. It may be treated with a topical corticosteroid cream. Other treatments options may include therapies to suppress the immune system, such as Protopic (tacrolimus) or Elidel (pimecrolimus).
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.