EFFECTS OF SYSTEMIC SCLERODERMA
Living with chronic illnesses can cause anxiety and depression. Some patients also experience chronic fatigue, which may result in cognitive impairment. Some medications also have the side effect of “brain fog.” Scleroderma patients must communicate with their physician about these issues.
Over 20% of systemic scleroderma patients have Sjögren’s Syndrome (“show-gren”), an autoimmune disease identified by its two most common symptoms: dry eyes and a dry mouth. This disease causes the immune system to attack the tear and saliva producing glands, as well as the mucous-secreting glands of the vagina. The unusual dryness of the eyes resulting from Sjögren’s Syndrome may lead to severe irritation and inflammation.
Frequent use of artificial tears and ophthalmic ointments may help lubricate dry eyes. Sipping fluids during the day (ex. a squirt bottle filled with water), chewing sugar-free gum, or sucking sugar-free sour candy may help keep the mouth lubricated. The latter two options stimulate salivary activity. Artificial saliva is also a possibility. Patients with Sjögren’s Syndrome must make regular visits to an ophthalmologist.
Scleroderma patients may experience a general tightening of skin over their whole face. In addition, the size of the mouth opening may decrease (microstomia: “small mouth). Lip and mouth movements as well as oral hygiene become difficult in patients with microstomia.
Facial grimacing and mouth stretching exercises, in which oral augmentation props may be placed between the upper and lower teeth, are used to treat microstomia. When performing mouth stretching exercises, patients must be careful to avoid further injury to the jaw bone and teeth. Particular care should be taken in using oral augmentation props. Consult with your dentist before beginning any exercises or using any augmentation props.
Dental care, including regular flossing and brushing of teeth and gums, and regular dental visits are extremely important for the prevention, early detection, and prompt treatment of any abnormalities. Your dentist can also recommend a good oral hygiene program. Floss holders, pump toothpaste, and toothbrushes with built-up handles may be helpful for people with hand impairment. For patients with Sjögren Syndrome, these measures are equally important.
The vascular system consists of the vessels and tissues responsible for circulating blood. Vascular diseases typically block/weaken blood vessels or damage valves in veins. Decreased or blocked blood flow can cause damage to organs. Raynaud’s phenomenon is the most common example of the effects of scleroderma on the vascular system.
The endothelium is the later of cells covering the interior surface of the blood vessels. Scleroderma damages the cells of the endothelium. The cause for this damage is still unknown.
In addition to causing vascular injury, scleroderma also results in inadequate vascular repair. Once blood vessels have been damaged, a scleroderma patient’s body will have difficulty repairing the damage. Pulmonary Arterial Hypertension (PAH) is attributed to this type of vascular system damage.
Muscle thickening and fibrous scar tissue accumulation in the heart muscle may decrease the force of heart contractions, possibly ultimately resulting in heart failure. Coronary arteries are the main blood vessels to the heart. Spasms in these arteries may cause chest pain (and rarely, lead to heart attacks). Such spasms seem similar to that involving the fingers/toes in Raynaud’s Phenomenon. In addition, pain and accumulation of fluid around the heart may result from inflammation of the outer heart lining (pericarditis). An irregular heartbeat may also develop. These conditions require careful evaluation and treatment by the patient’s physician.
Pulmonary (lung) involvement in systemic scleroderma can result from multiple factors; collagen build-up thickens lung tissue and causes fibrosis or scarring, thus oxygen transport into the bloodstream becomes more difficult. Symptoms include shortness of breath, decreased tolerance for exercise, and a persistent cough. To detect or confirm pulmonary involvement, the physician may order a chest X-ray, a CAT scan of the lungs, an echocardiogram (ultrasound of the heart), or special breathing tests (pulmonary function tests).
A type of Interstitial Lung Disease (ILD), pulmonary fibrosis is a buildup of scar tissue in the lungs that has progressed to the point that the patient’s breathing is affected. The scarring blocks the transfer of oxygen from the lungs to the bloodstream. The physician may give medication to decrease inflammation, which is thought to lead to the scarring. Many investigations are underway, but there currently are no proven medications to reverse lung damage once it has occurred. Therefore, scleroderma patients must take every possible measure to avoid further lung damage. In addition to avoiding smoking, a major cause of lung disease, patients should avoid exposure to air pollutants, which may worsen breathing problems. Your physician may suggest deep breathing exercises, a graduated aerobic exercise program, or medications to make breathing easier.
Pulmonary hypertension (PH) is high blood pressure in the blood vessels of the lungs. Pulmonary arterial hypertension (PAH) is pulmonary hypertension caused by the narrowing of pulmonary arteries. When the pulmonary vessels have high blood pressure, the right side of the heart pumps harder to move blood into the lungs and pick up oxygen; this can lead to heart failure on the right side of the heart. People with scleroderma have an increased likelihood for developing PH from several mechanisms, and they often have multiple causes of their PH. Those with PAH may be given special medications that dilate or open up the pulmonary vessels, possibly changing the disease’s underlying nature. This is one part of scleroderma for which recent medications have proven successful; now, treatment possibilities include three different classes of medications.
Renal (kidney) involvement in systemic scleroderma patients varies from mild to extremely serious. Early signs include mild hypertension (high blood pressure), blood test abnormalities, or protein in the urine. A sudden rise in blood pressure is the most important warning sign of renal crisis, a very dangerous complication of systemic scleroderma that may occur quickly. Other symptoms include headaches, visual disturbances, chest pain/discomfort, shortness of breath, and mental confusion. Without prompt treatment, renal crisis develops into kidney failure, in which the kidneys lose the ability to eliminate waste products from the body. Treatment involves anti-hypertensive drugs that belong to the category of ACE inhibitors (angiotensin-converting-enzyme inhibitors). ACE inhibitors control blood pressure and stabilize or improve kidney function. Dialysis may be required for cases of severe kidney failure. Successful recovery from renal crisis is possible, but only with quick recognition and treatment. Scleroderma patients should have their blood pressure and kidney function monitored at regular intervals.
The overproduction of collagen that is typical of scleroderma can cause thickening and fibrosis (scarring) of the tissues of the digestive system and gastrointestinal tract. The resulting weakening of muscles may lead to dysmotility (abnormally slow movement of food) in the digestive process.
The esophagus connects the mouth and throat to the stomach. The lower esophageal sphincter acts as a gate, opening to allow food to enter the stomach and closing to prevent food from coming back up. The “gate” of systemic scleroderma patients does not close properly, resulting in a backwash of acid. As the food and acid return into the esophagus, the patient feels a burning sensation called heartburn. The acid may cause scarring and a narrowing (stricture) of the esophagus by injuring the lining of the lower portion of the esophagus.
Acid production can be reduced by avoiding greasy or fatty foods, chocolate, alcohol, spicy foods, tobacco, and caffeine. Antacids, especially those in liquid form, can help neutralize acids. These methods can be used to treat problems of acid reflux and heartburn. However, some antacids can cause constipation or diarrhea. Before choosing over-the-counter products, consult your physician or pharmacist. Your physician may also prescribe antacid medications (ex. proton pump inhibitors or H-2 blockers) to decrease stomach acid production or drugs (ex. metoclopramide) to promote muscular activity and improve esophagus function.
Gravity helps keep food and acid in the stomach; thus, staying in an upright position after eating is helpful. Other common-sense measures to prevent acid reflux include eating smaller and more frequent meals, avoiding eating for several hours before bedtime, and elevating the head of the bed with wooden blocks (6-8 inches). Being overweight and wearing girdles or other tight-fitting clothing can aggravate this problem.
Dysmotility and narrowing of the esophagus may cause patients to have difficulty swallowing. It is important to eat slowly and chew thoroughly. Eating softer foods (ex. preparing food in a blender) and avoiding foods that tend to stick in the throat may make swallowing and digesting easier. The physician may periodically dilate the esophagus if it has narrowed significantly.
If damage to muscles of the small intestine occurs, the weakened muscles will not effectively push food through. One possible consequence is the overgrowth of bacteria, leading to diarrhea. If the intestine is stretched, patients may experience a bloated, distended feeling and pain. Malabsorption, in which nutrients remain in the intestine rather than being absorbed into the body, may lead to stool abnormalities and weight loss. The physician may suggest reducing the amount of fatty foods in the diet and increasing carbohydrate intake, or he/she may prescribe antibiotics, supplementary fat-soluble vitamins, and/or iron.
If muscles of the colon wall become weak or scarred, bowel function can be reduced; this results in constipation or other colon abnormalities. High fiber intake and at least six to eight glasses of fluids daily, especially water, will help prevent constipation. Fresh fruits and vegetables act as natural laxatives, and exercise can help maintain regular bowel movements. The physician may also recommend stool softeners or bulking agents such as Metamucil.
Swelling and Puffiness:
Another typical early symptom of scleroderma, swelling or puffiness of the hands may be especially conspicuous upon waking up in the morning; this is because of the muscle inactivity at night. The skin of the fingers may become fat and sausage-like, and closing the hand into a fist may be difficult. Finger and toe exercises, as well as medications recommended by your physician, can help.
Joint Pain and Stiffness:
Pain, stiffness, swelling, warmth, or tenderness may occur along with the arthritis-like joint inflammation commonly observed in scleroderma patients. Other symptoms include weakness and muscle pain. Aspirin or other non-steroidal, anti-inflammatory prescription drugs can help.
The name “scleroderma” (“hard skin”) comes from the characteristic hardening and thickening of the skin. Although many medications and treatments are being tested, there are currently no proven treatments for preventing or altering the course of the skin changes in people with scleroderma. Sometimes, the skin will spontaneously soften over time.
Scleroderma patients typically have sores, especially on the fingertips. They may also occur on the knuckles, elbows, toes, or other areas where the skin is especially tight or stretched. Due to poor circulation, skin ulcerations may be slow or difficult to heal. Keep affected areas warm and clean to increase blood flow and avoid infection. If infection does, occur, soaking the area in warm water and applying an antibiotic ointment or antiseptic such as Betadine may be helpful. If these measures do not work, the physician may prescribe oral antibiotics.
Hard lumps or nodules of calcium deposits just below the skin surface may be painful. If they break through the skin, the chalky white deposits become visible and may become infected. In case of infection, antibiotics may be prescribed. Patients with calcinosis should make sure to avoid bumping or injuring affected areas. Although there are currently no good established calcinosis treatments, some patients with severe cases have the deposits surgically removed.
Dilation of small blood vessels near the skin’s surface causes the vessels to become visible as small red spots, called telangiectasias. This generally not-harmful abnormality typically occurs on the fingers, palms, face, and lips. Telangiectasias usually fade with pressure; however, when the pressure is released, the spots become red again. Patients with telangiectasia may use special make-up to cover the spots.
Excessively dry skin can cause skin breakdown and ulcers. To avoid complications from dry skin, it is important to avoid excessive bathing and handwashing or direct contact with household detergents (ex. wear rubber gloves). Rather than using harsh soaps that dry out the skin, use bath oils and moisturizing soaps. In addition, patients should keep their skin moist and well-lubricated such as through the use of moisturizing skin creams containing lanolin. Humidifiers may help during the winter.
The underlying inflammatory process associated with scleroderma may result in pruritus (itchy skin). If moisturizing creams prove unsuccessful, the physician may prescribe a topical cortisone cream. Some people find antihistamines effective.
Other skin symptoms:
Other potential skin symptoms include a decrease in hair and the ability to sweat in affected areas. Furthermore, patients may experience spotty pigment-loss or an increase in pigment (similar to a skin tan).
Around 40% of systemic scleroderma patients will develop at least one digital ulcer. These painful ulcers result from skin breakdown at or near the fingertip due to decreased circulation to the fingers. Usually, these ulcers last for weeks and sometimes months. Initially, these sores develop “spontaneously” due to lack of blood supply and not typically because of an infection. However, the area can become secondarily infected. Treatment of digital ulcers includes using medicine (vasodilators) to dilate or open up the small blood vessels to optimize circulation; in this treatment method, the patient must be carful to keep the area clean and protected from injury.
Raynaud’s Phenomenon, the most common early symptom of systemic scleroderma, is present in 95% of systemic scleroderma patients. Although most obvious in the fingers and toes, it can also involve the ears, nose, or tip of the tongue. In Raynaud’s Phenomenon, blood vessels will constrict (narrow) in response to cold, emotional upset, or stress; constriction decreases blood circulation, causing a series of changes in skin color. During reduced circulation, the skin will be white, blanched, or pale. As the affected area loses oxygen from decreased blood flow, the color turns blue. When blood flow returns and the area warms up, the skin becomes flushed or red. After the attack subsides and normal circulation returns, the usual skin color is restored. Tingling, numb, and cold sensations may accompany the “blue” and “white” stages. Warmth, burning, or throbbing may accompany the “red” stage. Some patients find Raynaud’s Phenomenon painful.
Those who experience Raynaud’s Phenomenon can take many common-sense measures to prevent attacks. Minimizing exposure to cold (ex. outdoor weather, air conditioning, reaching into a refrigerator or freezer) and keeping your extremities/body warm is extremely important. Patients should wear mittens or gloves, especially when touching refrigerated or frozen items. In addition, many warming devices are available to protect the hands. Thick socks, hates, ear muffs, and warm clothing made from silk, cotton, wool, or down can help people maintain their body temperature. At home, electric heaters, comforter, and electric blankets can also be used. Keeping the entire body warm helps prevent Raynaud’s Phenomenon attacks. Although avoidance of emotional upset and stress isn’t always possible, various relaxation techniques (self-taught or learned in training courses) have proven effective for some. One example, biofeedback, has been used to increase finger temperature; however, its benefit is still unclear.
Smoking worsens Raynaud’s Phenomenon; people with scleroderma should avoid smoking. Concurrent carpal tunnel, estrogen, and stimulants may also aggravate the condition. To make sure that you are not taking something that may worsen Raynaud’s Phenomenon, your physician should review all of your current medications.
When a Raynaud’s Phenomenon attack does occur, carefully waving the arms in an underhand, circular motion (similar to a softball pitch) may restore blood circulation. Rubbing or massaging the affected area may also work. Other possible options for relieving an attack include a warm bath/shower, heating pads, or hot water bottle on the back.
Diagnosis of Raynaud’s Phenomenon may occur through a capillaroscopy, in which the physician assesses severity by examining the patient’s nailfolds under a microscope. The presence of Raynaud’s Phenomenon should not be proved by provocation testing (ex. placing the hands in ice water).
Physicians prescribe a variety of different medications for Raynaud’s Phenomenon. Most of these medications work by dilating (opening up) the blood vessels. Common drugs include vasodilators such as prazosin and calcium channel blockers such as aldipine and nifedipine. Drugs that decrease the stickiness of platelets as well as mild blood thinners such as aspirin may ask improve circulation. In addition, medication for pulmonary arterial hypertension (PAH) may also help prevent and treat Raynaud’s Phenomenon attacks.
People without scleroderma can also have Raynaud’s Phenomenon. This condition also appears in people with lupus, rheumatoid arthritis, and other connective tissue diseases. Many otherwise healthy people experience Raynaud’s Phenomenon without any other illness; this is called Primary Raynaud’s Disease.
Distinct from digital ulcers, chalky white deposits may occur in the soft tissues of the hand. These deposits can drain from the skin’s surface and can commonly be inappropriately treated with antibiotics. Although there are currently no good established calcinosis treatments, some patients with severe cases have the deposits surgically removed.
Swelling and Puffiness:
Another typical early symptom of scleroderma, swelling or puffiness of the hands may be especially conspicuous upon waking up in the morning; this is because of the muscle inactivity at night. The skin of the fingers may become fat and sausage-like, and closing the hand into a fist may be difficult. Finger and toe exercises, as well as medications recommended by your physician, can help. At this point, referral to an occupational therapist may help with range of motion education.
Joint Pain and Stiffness:
Pain, stiffness, swelling, warmth, or tenderness may be experienced along with the arthritis-like joint inflammation commonly observed in scleroderma patients. Other symptoms include weakness and muscle pain. Aspirin or other non-steroidal, anti-inflammatory prescription drugs cab help. To determine if any additional therapies are needed, your physician may perform joint ultrasounds or hand radiographs.
Sclerodactyly and Joint Contractures:
Sclerodactyly means “hard skin of the digits,” especially the fingers and toes, generally occurs after initial swelling subsides. Characterized by shiny, tight skin on the fingers, sclerodactyly may also result in difficulty in moving the affected digits. Furthermore, they may become fixed in a vent or flexed position; this is called a “contracture” or “flexion contracture.” Decreased motion of the elbows, wrists, and other joints can also occur due to tightening and hardening of the skin and tissues surrounding the joints.
Daily range of motion exercises are key to maintaining limber joints and preventing or slowing down the development of such contractures. Such exercises may also increase circulation in the tissues. Range of motion exercises are typically simple and can easily be performed at home. For example, one exercise consists of laying the hand as flat as possible on a table and gently pressing down with the heel of the other hand to straighten the fingers. After consulting with your physician, an occupational therapist can develop your exercise plan. Occupational therapists may also provide devices that make performing personal care and household tasks easier.