Scleroderma— Know It All!

All you need to know about Scleroderma.

Know your ailment well, so you can manage it better!!

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What is Scleroderma?

Scleroderma is a rheumatic and autoimmune tissue that causes skin and other areas of the body to become inflammatory. When an immune response tricks tissues to believe they are wounded, the inflammation is triggered and the body produces too much collagen to contribute to scleroderma. Too much collagen creates tight, hard skin patches in your skin and other tissues. Scleroderma includes many of the body’s processes.

The following descriptions will help you understand how each of these mechanisms is affected by the disease.

Binary tissue dysfunction affects tissues including muscle, tendons and cartilage. Connective tissue helps preserves, and constructs other tissues and organs.

Autoimmune diseases arise when your immune system, which usually helps protect your body from infection and disease, attacks your own tissue.

Rheumatic disease refers to a category of disorders that are characterised by muscle, joint or fibrous tissue inflammation or pain.

What are the types of Scleroderma?

There are two kinds of scleroderma:

Localized scleroderma mainly affects your skin. It happens in one of two forms:

• Morphea. This involves hard, oval-shaped patches on your skin. They start out red or purple and then turn whitish in the centre. Sometimes, this type can affect blood vessels or internal organs. This is called generalized morphea.
• Linear. This kind causes lines or streaks of thickened skin on your arms, legs, or face.

Systemic scleroderma, also called generalized scleroderma, can involve many body parts or systems. There are two types:

• Limited scleroderma. This comes on slowly and affects the skin of your face, hands, and feet. It can also damage your lungs, intestines, or esophagus, the tube that carries food from your mouth to your stomach. It’s sometimes called CREST syndrome, after its five common signs:
• Calcinosis. This is when calcium salts form nodules under your skin or in your organs.
• Raynaud’s phenomenon. This is a lack of blood flow to parts of your body such as your fingers, toes, or nose, usually because of cold. Your skin might turn red, white, or blue.
• Esophageal dysfunction. This is when your esophagus doesn’t work the way it should.
• Sclerodactyly. This is when your skin becomes thin and shiny. It usually causes problems with moving your fingers and toes.
• Telangiectasia. This is when small blood vessels grow near the surface of your skin.
• Diffuse scleroderma. This comes on quickly. Skin on the middle part of your body, thighs, upper arms, hands, and feet can become thick. This form also affects internal organs like your heart, lungs, kidneys, and gastrointestinal tract.

What Happens in the disease:

It is not clear what causes scleroderma. Researchers nevertheless assume that the immune system overreacts and destroys the cells that line blood vessels. This leads to the formation of too many collagen and other proteins by connective tissue cells, especially cell type called fibroblasts. The fibroblasts live longer than average, causing a collagen build-up in the skin and other bodies, which contributes to sclerodermic signs and symptoms.

Who Gets The disease?

Scleroderma can occur to anyone, but some groups have a higher chance of developing the disease.

The following factors may affect your risk.

• Sex. Scleroderma is more common in women than in men.
• Age. The disease usually appears between the ages of 30 and 50 and is more common in adults than children.
• Race.

Scleroderma can affect people of all races and ethnic groups, but the disease can affect African Americans more severely.

For example:

• The disease is more common in African Americans than European Americans.
• African Americans with scleroderma develop the disease earlier when compared with other groups.
• African Americans are more likely to have more skin involvement and lung disease when compared with other groups.

What are the Causes and Risk Factors of Scleroderma?

Overproduction and deposition of collagen in body tissues is responsible for scleroderma. Collagen is a fibrous protein forming the connective tissues of your body, including your skin.

Doctors don’t know exactly what causes an irregular development of collagen, but the immune system of the body seems to play a part. Scleroderma is most likely triggered by a variety of factors, including immune issues, genetics and environmental causes.

Anyone can get scleroderma, but it happens in women much more often than in men. Various combined factors seem to affect the risk of scleroderma development:

• Genetics. People who have certain gene variations appear to be more likely to develop scleroderma. This may explain why a small number of scleroderma cases appear to run in families and why certain types of scleroderma are more common for certain ethnic groups. For example, Choctaw Native Americans are more likely to develop the type of scleroderma that affects internal organs.
• Environmental triggers. Research indicates that scleroderma symptoms in certain people may be caused by exposure to certain viruses, medicines or medicines. Repeated exposure — including at work — to any toxic substances or chemicals can also raise the risk of scleroderma.
• Immune system problems. It is assumed that scleroderma is an inflammatory disease. This means it happens partially because the body’s immune system starts destroying the connective tissues. In 15–20 % of cases, scleroderma patients have signs of other autoimmune disorders, including rheumatoid arthritis, lupus or Sjogren’s

What are the symptoms of Scleroderma?

The symptoms of scleroderma differ depending on the type of scleroderma you encounter.

Local scleroderma generally produces thick, tough skin patches in one of two patterns.

Morphea causes skin patches to thicken into strong oval areas. These regions can look yellow and waxy with a reddish or bruise-like border. The patches can remain in one area or spread to other skin areas. The disease typically becomes dormant over time, but skin patches can still be darkened. Some people often develop tiredness (feel tired).

Linear scleroderma causes thickened or different coloured skin lines to run down your arms and legs and, occasionally, on your foreheads.

Systemic scleroderma also referred to as systemic sclerosis may occur rapidly or gradually, and can also cause internal organs as well as skin problems. Many people with scleroderma of this sort have fatigue.

Restricted skin scleroderma develops gradually and typically affects the skin under your fingertips, elbows, face, lower arms and legs. It may also cause blood vessel and oesophagus problems. The restricted shape has an internal organ involvement but is usually less than diffuse. People with restricted skin scleroderma sometimes experience all or some of the symptoms called CREST by some physicians, meaning:

• Calcinosis, the accumulation of connective tissue calcium deposits that can be detected by x-ray.
• Raynaud’s Phenomenon, in which the hands or feet of little blood vessels contract in reaction to cold or fear, causing finger and toes (white, blue and/or red) colour changes.
• Esophageal dysfunction which refers to the impaired function of the oesophagus (the throat and stomach tube) which occurs when smooth muscles lose normal movement in the oesophagus.
• Sclerodactyly, a dense, tight skin on the fingers caused by excessive collagen deposits on the skin.
• Telangiectasia, a disorder caused by irregular blood vessels that show on the hands and faces with tiny, red spots.

Diffuse skin scleroderma unexpectedly develops, usually by thickening the skin on your fingers or toes. The skin thickening then extends over the elbows and/or knees to the rest of your body. This form can damage your internal organs, for example:

• Any part of your digestive system.
• Your blood.
• Your kidneys.
• Your heart.

While CREST is traditionally limited scleroderma, CREST features can also be present in those with the diffuse shape of scleroderma.

How is Scleroderma diagnosed?

Diagnosing scleroderma is difficult for physicians since the signs differ from person to person and are similar to other diseases. No single test is required to diagnose the disease; instead, physicians use a mixture to diagnose scleroderma.

Your physician may:

• Ask about your experience with medicine.
• Ask about your symptoms now and in the past.
• Conduct a physical test.

Your doctor can suggest more tests, such as:

• Laboratory tests are ordered to search for certain antibodies that wrongly target and react to your tissues. Some antibodies can be normal in scleroderma patients. Anticorps may evolve because of other causes, so that scleroderma is not identified by the blood test alone.
• Doing a skin biopsy.

Your doctor will order additional tests if you have symptoms that indicate problems with your organ such as your heart, lungs, or kidneys. Early diagnosis of organ involvement aids physicians in treating and controlling the disorder.

Testing can contain:

Computerized tomography (CT) which uses a lung and other organ picture scanner.

Echocardiograms that produce moving images of your heart with sound waves.

What is the treatment for Scleroderma?

Your condition will depend on your form of scleroderma, symptoms and tissues and organs. Treatment can allow symptoms to be managed and harm reduced.

Medicines can be prescribed by your doctor includes:

• Anti-inflammatory medicines for pain relief and swelling reduction.
• Corticosteroid topical creams, including straightening and scratching, to combat skin changes.
• Corticosteroids are given through mouth, injection, or intravenous infusion (IV) to help regulate articular pain or inflammation. Since these are effective drugs, the doctor can prescribe the lowest possible dose to achieve the desired benefit and prevent side effects.
• Immunosuppressants that can suppress the overactive immune system and regulate the disease symptoms. Oral, IV, or topical immunosuppressants may be recommended by your doctor.
• Vasodilators are used to dilate (widen) blood vessels, avoid damage to the lung and kidney, and treat the phenomenon of Raynaud.

Many people benefit from physical or work therapy to the following:

• Relax pain.
• Develop muscle strength and agility, including the arms, legs and jaw muscles.
• Teach your strategies to assist with everyday life tasks. For example, a therapist may prescribe toothbrushes and devices that help promote flossing if hand pain and discomfort make it difficult to brittle your teeth.
• Regular dental care is crucial as scleroderma can make your mouth dry, damage your mouth’s connective tissues, accelerate tooth decay, loosen your teeth. Strengthening facial skin can also minimise the opening of your mouth, making it more difficult to care for your teeth. Here are several ways to prevent issues with tooth and gum:
• Brush your teeth and flow daily.
• Have daily dental checks. If you suffer oral sores, oral pain or lose teeth, contact your dentist immediately.
• Speak to your dentist and doctor about the best ways to keep your mouth moist.
• Using special mouth washing or dry mouth toothpaste. You should also discuss drugs to relieve dry mouth with your doctor.

Systemic scleroderma can affect and cause damage to the internal organs. Doctors decide which organ is damaged by the best care.

Lung Problems:

Almost every person with systemic scleroderma has some lung function loss. Some people experience serious pulmonary disease in two forms:

Pulmonary fibrosis, lung tissue hardening or scarring due to excess collagen.

Pulmonary hypertension, high blood pressure in the artery that carries blood from the heart to the lungs.

Treatment differs for these two conditions:

• Pulmonary fibrosis may be treated with medications that suppress the immune system, including a recently approved kinase inhibitor, which can help counter fibrosis.
• Pulmonary hypertension may be treated with medications that dilate the blood vessels or with newer medications that are prescribed specifically for treating pulmonary hypertension.

To help minimize lung complications, work closely with your doctor.

Watch for signs of lung disease, including fatigue, shortness of breath, dry cough, or difficulty breathing, and swollen feet. Report these symptoms to your doctor.

Follow up regularly with your doctor for evaluation of your lung function. This may include standard lung function tests, which measure your lung volumes to monitor the course of lung fibrosis. Checking for pulmonary hypertension early helps doctors manage and treat the condition, even before you may notice symptoms.

Get regular flu and pneumonia vaccines as recommended by your doctor.

Heart Problems:

Some people may develop complications that cause heart problems, including:

• Cardiomyopathy, scarring and weakening of the heart.
• Myocarditis, inflamed heart muscle.
• Arrhythmia, abnormal heartbeat.
• Treatments for heart complications can range from medications to surgery and vary depending on the nature of the condition.

Kidney Problems:

Renal crisis is uncommon but can be serious for people with systemic scleroderma. Renal crisis happens when blood pressure levels rise suddenly to dangerous levels, which can quickly lead to kidney failure. Side effects of certain medications, such as corticosteroids, can also trigger renal crisis. It is important that you and your doctor work together to monitor your blood pressure, including:

• Check your blood pressure regularly, and let your doctor know if you have any new or different symptoms such as a headache or shortness of breath. If your blood pressure is higher than usual, call your doctor right away.
• If you have kidney problems, remember to take your medications as prescribed. In the past two decades, medications known as ACE (angiotensin-converting enzyme) inhibitors have made scleroderma-related kidney failure a less threatening problem than it used to be.

How to cope up:

Depending on the form of scleroderma and symptoms, it can be difficult to live with the disease. Try to engage regularly in your scleroderma therapy to support. The following tips and suggestions may be of assistance.

• Keep warm. Your body controls your skin‘s temperature. Dress up in layers, wear gloves and socks, and avoid where necessary cold rooms and weather.
• Try to avoid damp or cold conditions that may cause symptoms of the Raynaud phenomenon.
• If you smoke, stop smoking. Nicotine and smoking cause blood vessels to contract, which can worsen some symptoms and cause lung problems.
• Apply sunscreen to protect against more damage from sunlight before going outside.
• Using moisturisers on your skin to minimise rigidity.
• Using humidifiers to humidify the air in the colder winter climate in your house. Clean humidifiers also prevent the production of bacteria in the water.
• Stop hot baths and showers as the skin gets hot water.
• Stop harsh soaps, home cleaning materials, and caustic chemicals. Wear rubber gloves if you use such products.
• Exercise regularly. Exercise, especially swimming, stimulates blood circulation to affected areas.
• Visit the dentist regularly for check-ups.
• Reach out to online and community support groups.
• Keep the lines of communication open. Talk to your family and friends to help them understand the disease.
• Talk to a mental health professional for help with coping with a chronic illness.
• Some types of scleroderma can affect parts of the digestive system. Doctors may prescribe heartburn, constipation, and motility medications to help manage these symptoms.

Here are some tips to help if you have digestive symptoms:

• Eat small, frequent meals.
• After meals, stay upright for 3 hours. Try to avoid reclining or slouching.
• Eat moist, soft foods, and chew them well. If you have difficulty swallowing or if your body doesn’t absorb nutrients properly, your doctor may prescribe a special diet.
• Drink less alcohol and caffeine.
• Stay hydrated.
• When it is time to sleep, raise the head of your bed with blocks. Using several pillows is not as helpful as raising the head of the bed by using blocks or special wedges.

References:

https://www.scleroderma.org/site/SPageNavigator/patients_whatis.html#.X3vlQGgzbIU

https://www.mayoclinic.org/diseases-conditions/scleroderma/diagnosis-treatment/drc-20351957

https://www.niams.nih.gov/health-topics/scleroderma/

https://www.webmd.com/skin-problems-and-treatments/scleroderma#2-5

By,

Gopala Krishna Varshith,

Content Developer & Editor,

Clipo.