What Is Scleroderma
Scleroderma is a chronic, immune mediated condition that affect females more frequently than males. Although, no curative treatment is available, the symptomatology and risk of complications can be reduced by various therapeutic options.
Our skin is the most extensive organ of the body that protect the tissues from environmental forces, physical trauma as well as infectious and disease causing pathogens. However, there are several other benefits of skin, such as the flexibility and resilience that is essential to promote free movement of limbs and trunk muscles, bones and joints.
Certain connective tissue disorders such as scleroderma may affect the integrity of skin along with associated supporting tissues that may compromise the quality of life. According to a research report published in peer reviewed Arthritis Care and Research (1), for every 100,000 individuals there are 87 cases of scleroderma. This condition can cause swelling in the muscles and joints.
What are the risk factors that may increase your risk of scleroderma?
Scleroderma (also referred to as systemic sclerosis) is a chronic, relapsing- remitting and disabling condition that is marked by overproduction of collagen in response to chronic inflammation.
- Females are more prone to develop scleroderma as compared to males.
- The peak age of incidence is 30-50 (with most cases reported in early 30’s).
- The severity of symptoms and risk of terminal complications increases with advancing age.
What are the classic symptoms of scleroderma?
The extent and severity of symptoms vary considerably from person to person and virtually any organ or tissue that contains collagen (a structural protein that form the lining of tissues and organs) may get affected. Skin is involved in almost all the cases of scleroderma but in some individuals, the stiffening or hardening may involve deeper structures as well such as digestive tract, blood vessels and internal organs.
The classic symptoms include:
Skin changes are most common in the setting of scleroderma. Most patients develop bands of skin thickening especially around extremities that may interfere with free mobility and activity across limbs.
- Digestive system
Gut involvement directly affects the digestive functions that may present with symptoms like indigestion. Other gastrointestinal complications of scleroderma are:
- Low appetite
- Low salivary production that makes swallowing difficult
- Acid reflux due to tightening of gut musculature
- Constipation due to low gut motility
- Bowel obstruction (due to low gastrointestinal motility)
- Constipation alternating with diarrhea
- Involvement of extremities
A significant percentage of people with scleroderma develops Raynaud’s phenomena in which blood vessels become extremely sensitive to environmental temperature or emotional triggers. With mild changes in emotional distress or physical temperature, individuals may experience severe vasoconstriction that my present with (3):
- Painful digits or toes
- Discoloration of finger and toe nails
Most cases respond to rewarming, massage and other simple interventions. Untreated or poorly managed cases may culminate in complications such as:
- Ulcers due to poor blood supply to the edges
- Involvement of oral cavity
Facial skin tightening ultimately affects simple daily activities and interfere with normal facial expressions such as smiling, opening mouth and eating. Often time fibrosis affects the mouth opening (making it harder to brush teeth). In addition, fibrosis also affects the patency of salivary ducts that eventually affects secretion of saliva. Low saliva production increases the risk of tooth decay and dental caries.
- Involvements of major organs
In almost 10-15% cases, scleroderma may involve major organs like heart, lungs and kidneys. Risk of morbidity and mortality increases significantly with the involvement of major organs.
- In the involvement of lungs, patients may develop widespread pulmonary fibrosis that interferes with normal chest expansion, breathing and gaseous exchange during respiration. This ultimately affects exercise tolerance and may lead to pulmonary hypertension in poorly managed cases.
According to research conducted by Robert W. Battle (2) and associates, the overall prevalence of undiagnosed cases of pulmonary dysfunction is fairly high in scleroderma patients.
- In the involvement of kidneys, patients develop vascular dysfunction that leads to hypertension (elevation of blood pressure beyond normal recommended range). If left untreated, renal function is compromised that present as appearance of proteins in the urine, edema, puffiness, renal crisis or even death in extreme cases (3).
- Involvement of heart can increase the risk of arrhythmia and poor exercise tolerance. Some individuals can also develop fibrosis of pericardial sac (thickening of cardiac walls) that leads to pericarditis and right-sided congestive heart failure. In a study published in peer reviewed journal Chest (2), investigators identified that 97% of the study sample manifested the signs of tricuspid regurgitation.
- Sexual dysfunction
Males often experience varying degrees of erectile dysfunction (that responds poorly to ordinary remedies) in long standing scleroderma. Since multiple sclerosis also affects the secretion of female genital tract, a lot of females experience dyspareunia (painful intercourse) due to vaginal dryness and tightening of vaginal opening.
How To Manage Scleroderma
The diagnosis of scleroderma is usually made by blood investigations (that detects antibodies in the serum). Scleroderma is mainly classified in two varieties on the basis of extent and severity;
- Limited (only skin): Limited scleroderma has a good prognosis (the intensity of symptoms improve in most patients).
- Diffuse (multiple organs and organ systems are involved): Diffuse scleroderma on the other hand usually worsen over time due to widespread complications and involvement of internal organs.
In most cases, the rate of complications can be reduced by several treatment options.
Pharmacological preparations can be used to manage the symptoms of scleroderma. For example, vasodilators (drugs that increase the caliber of blood vessels) can be used to improve the symptoms of Raynaud’s phenomena. Other popular drug regimen that are helpful in managing the symptoms or complications of scleroderma are:
- Anti-hypertensive medications (ACE inhibitors are fairly helpful in preventing as well as managing renal crisis (a condition marked by acute renal failure and a permanent need for dialysis therapy)
- Pain-killers (non-steroidal anti-inflammatory drugs are helpful in mild cases of arthritis)
- Antacids (to reduce the symptoms of acidity, stomach pain, indigestion and ulcer pain)
- Immuno-suppressant agents to reduce the activity of hyper-functioning immune system
- Surgical Intervention
Surgeries are needed to improve the quality of life and manage complications. For example, in case of gangrene, amputation is needed to prevent infection and sepsis. Likewise, in case of pulmonary hypertension or severe pulmonary fibrosis, lung transplantation is needed to maintain the quality of life.
- Lifestyle And Home Remedies
Often time, lifestyle modification helps in reducing the severity of symptoms and minimizing the risk of complications. Most popular home or lifestyle remedies are:
- Keep your extremities (limbs, both upper and lower) warm and fully covered especially in winter season.
- Adopt a more physically active lifestyle to reduce stiffness and rigidity of joints.
- It has been observed that smoking can increase the risk of Raynaud’s phenomena several folds.
- Heart burn and gastric issues can be reduced by diet modification. Avoid spicy, fatty and heavy meals late at night. Sit in prop up position for at least 45 minutes post meal and increase your intake of water.
Complementary And Alternative Medicine
A lot of people also utilize different types of complementary and alternative therapies in order to improve the mental and physical well-being in the setting of advanced or chronic systemic sclerosis or scleroderma.
According to a report published in The Journal of Science and Healing (5), investigators suggested that patients of scleroderma who utilize CAM are more motivated than those of non-CAM users. Almost all patients reported higher physical activity and perceived functional independence after receiving CAM therapy.
Report also suggested that CAM therapy especially target symptoms like bodily pain, dysfunctional gastric motility (constipation alternating with diarrhea symptoms), arthritis or arthralgia and fatigue
Most common CAM therapies that are used in scleroderma management are:
- Herbal or nutraceutical therapy
- Mind-body therapy (this includes a variety of interventions such as relaxation, meditation and positive imagery).
- Sacral nerve stimulation
- Transcutaneous electrical neural stimulation
In another study published in The Journal of Science and Healing (6), investigators identified that acupuncture can help in managing esophageal and gastric dysmotility in patients with scleroderma.
Pregnancy In Scleroderma
A lot of female patients are concerned about getting pregnant after being diagnosed with scleroderma. It is true that scleroderma can affect pregnancy and may also lead to complications, but the risk can be minimized significantly by:
- Seeking help from a registered healthcare professional.
- Initiating prenatal vitamins (such as folic acid, multi-vitamin complex, calcium supplements and Iron).
- Keeping up with regular antenatal visits.
According to a research report compiled by Merav Lidar (7), scleroderma does not generally interfere with normal pregnancy and maternal as well as fetal survival is excellent as per recent medical data. In some cases, the risk of some fetal complications increases (such as premature birth, low birth weight and small sized baby for gestational age).
Some maternal complications that may worsen during pregnancy are:
- Hypertension (as it may increase the risk of pre-eclampsia and eclampsia during pregnancy)
- Placental insufficiency (due to construction or narrowing of blood vessels)
- Higher risk of worsening of cardiac, renal and pulmonary functioning
What else should you know about getting pregnant with scleroderma?
- It has been observed that drugs like cyclophosphamide (Cytoxan) can affect fertility and may decrease your chances of getting pregnant due to ovarian failure.
- A lot of scleroderma drugs during pregnancy can increase the risk of fetal anomalies and therefore you should seek alternative options to control scleroderma during pregnancy.
- Pregnancy should be avoided for 3 years of onset of scleroderma symptoms. This is mainly because the risk of complications is highest within the first few years of initial diagnosis.
- If you are consuming immune-suppressant drugs, the risk of infection during pregnancy (or after childbirth) increases.
- Avoid non-steroidal anti-inflammatory drugs (NSAIDs) during pregnancy that directly increases the risk of bleeding during active gestation and at the time of childbirth.
However, the positive points are; despite the fact that scleroderma interferes with your ability of skin to undergo stretch or expansion. Most females experience no problem during their pregnancy in regards to expansion of abdominal skin. Moreover, normal vaginal delivery is also possible in the setting of diffuse scleroderma.
1. Bernatsky, S., Joseph, L., Pineau, C. A., Belisle, P., Hudson, M., & Clarke, A. E. (2009). Scleroderma prevalence: Demographic variations in a population‐based sample. Arthritis Care & Research, 61(3), 400-404.
2. Battle, R. W., Davitt, M. A., Cooper, S. M., Buckley, L. M., Leib, E. S., Beglin, P. A., & Tischler, M. D. (1996). Prevalence of pulmonary hypertension in limited and diffuse scleroderma. CHEST Journal, 110(6), 1515-1519.
3. Guiducci, S., Giacomelli, R., & Cerinic, M. M. (2007). Vascular complications of scleroderma. Autoimmunity reviews, 6(8), 520-523.
4. Cameron, A. J., & Payne, W. S. (1978, September). Barrett’s esophagus occurring as a complication of scleroderma. In Mayo Clinic proceedings. Mayo Clinic (Vol. 53, No. 9, pp. 612-615).
5. Hunnicutt, S. E., Grady, J., & McNearney, T. A. (2008). Complementary and alternative medicine use was associated with higher perceived physical and mental functioning in early systemic sclerosis. Explore: The Journal of Science and Healing, 4(4), 259-263.
6. Sallam, H. S., McNearney, T. A., & Chen, J. D. (2014). Acupuncture-Based Modalities: Novel Alternative Approaches in the Treatment of Gastrointestinal Dysmotility in Patients with Systemic Sclerosis. EXPLORE: The Journal of Science and Healing, 10(1), 44-52.
7. Denton, C. P., Lapadula, G., Mouthon, L., & Müller-Ladner, U. (2009). Renal complications and scleroderma renal crisis. Rheumatology, 48.