Scleroderma or Mixed Connective Tissue Disease Information

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What is Scleroderma 

-  is a chronic connective tissue disorder, which literally means "hard Skin" It

 is often a life threatening disease.

Causes

unknown, some evidence of a genetic predisposition in some studies, autoimmunity, an infectious cause and enviromental triggers.

Attacks

*the immune system, *body tissue - skin, muscles, ligaments, * internal organs - lungs, heart, kidneys, esophagus and intestines. * blood vessels - vascular system

Results in

* Over production of collagen

* thickening, hardening and scarring of the skin

* Blood vessels unable to supply sufficient blood to many parts of the body.

*scarring of internal organs

Symptons

*muscle weakenss and pain

*stiffness and pain in joints

*swelling and thickening of the skin

*swallowing difficulties

*fingers and toes show sensitivity to cold

*severe fatigue

*breathing difficulties

*joint and bone acheing

*Not all of the above symptons would indicate scleroderma

Treatments

* no known cure

* treatments target specific symptons

*treatments may include, steriods, nonsteroidal anti-inflamatory drugs, immunosuppresants, antibiotics, some alternative and complementary therapies.

Who is affected

* far more prevalent than muscular or multiple sclerosis

* 4 out of 5 affected are women between 30-50 years old

*2 to 3 out of 10,000 persos is estimated to be affected.

Please contact the scleroderma society of Canada for furhter information regarding membership

www.sclerodermaontario.ca

The face of sclerodema

Test to be performed annually

ENA TEST

Enthrocyte Sedimentation Rate (SED) Determines the presence and level of inflamation.

Chest Xrays can determine abnormal heart and lung activites, thus determine the extent of inflamation and fibrosis caused by scleroderma

Electrocardiogram (ECG/EKG) uses graphic to record hearts electrical activity to determine heart involment.

Echocardiogram Uses ultrasound waves to create images of the heart to evaluate heart involvement.

Pulmonary Function Test (PFT) is a series of 5 test that measure how effective the lungs are functioning and assist the physician to determine extent of lung involvement.

Other Test

Barium Studies of the gastrointestinal tract which may be done every five years.

Skin and renal biopsy are very invasive and may not be necessary.  but are used to determine disease complications.

Antibody Test are often used to differenciate between diffuse and limited scleroderma

 

What is Scleroderma

Types of Scleroderma

Localized Scleroderma

*Morphea * Linear Scleroderma

Systemic Scleroderma

*Limited * Diffuse * Since

Morphea the most common form of localized scleroderma, and is chaterized by oval patches of inflamed, often discolored skin.  The trunk,face and extremities are involved.

Linear scleroderma

A band or bands of skin hardens and thickens on the trunk or extremeties.

Systemic Scleroderma

This form of scleroderma involves not only the skin but also internal organs, especailly the digestive, circulatory,pulmonary, and muscular systems.  Systemic scleroderma is divided into three froms: limited, diffuse and sine

Limited Scleroderma is often referred to as CREST.  This is an acronym that stands for a combination of symptons.

C- Calcinosis- small white calcium lumps forming under the skin

R-Raynaud's Phenomenon - poor circulation in the fingers and or toes. small vessels in the fingers tend to narrow and decrease blood flow casuing patients to be very sensitive to cold temperatures. Thiscauses fingers to turn white then blue during cold temperatures or a stressful episode.

E- Esophageal dysfunction - difficulty swallowing, heartburn, or regurgitation.

S -Sclerodactyl- skin of fingers and sometimes toes become thick and shiny.  Affected digits may be difficult to move and become fixed in a bent position.

T- Telangiectasia- small clusters of dillated blood vessels in the skin especially on the face, fingers and palms of hands.

 

Diffuse

The most serious is the internally involved form of the diesease.  It involves many of the internal organs of the body, esophagus, the digestive tracr, kidney, heart and or/ lungs.  The skin involvement includes the face, neck,torso, and both hands, arms, feet and legs.

 

Sine may resemble either limited of diffuse systemic sclerosis casuing damage in lungs and or blood vessels.  However unlike other forms of systemic sclerosis in sine the skin is not involved.

 

 

Information on Scleroderma is not meant to be used as a diagnostic tool or to suggest treatment medications.  Always consult your physician regarding details of synptons, diagnosis and treatment.

Medical Tests for Scleroderma

Scleroderma is a very difficult diease to diagnose.  Specailist as well as General pratictioners order test at the discreation of the attending pyhsicans.  Commuicating honestly with your doctor is always important.

Information for accurate medical records

*list of doctors, * list and description of medical conditions * list of medication which should be updated as medications or doses change. * date, description of procedures.

Test

Anti nuclear antibodies (ANA) autobodies that attact the core body cells, scleorderma patients test positive.

Extractable Nuclear Antibodies (ENA) helps the doctor to accurately diagnose patient with suspected scleroderma who has a positve ANA.

*careful observation of the skin to determine the extent of the changes in skin thickening. 

*careful clinical observation of the heart, lungs and joints.

*Other Specialised test depending on the organs involved.

Ongoing test to monitor diesease progression

* monitor progression * determine response to pescribe medication * monitor side effects of pescribed medications which may cause serious damage.

Test performed every six months

CBC( complete blood count) includes test for red blood cells, WBC (white blood cell count) platelets.

Muscle Enzymes test (CPK,dldolase) measure the amount of muscle damage in some rheunatic diseasr, damaged muscles may release cetain enzymes into the blood.

Liver Enymes (bilirubin) and Alkaline Phosphate indicates the presence and amount of liver damage.

Creatine and Blood Urea Nitrogen (BUN) test measure overall kidney function, A high level indicates that the kidneys are not working well enough to remove waste products from the body.

Urinalysis

Urine test detects blood cells , protein or a variety of abnormal substances which may indicate kidney damage.

 

symptons

Scelroderma is highly individualized, so it affects patients in differnet ways. Symtons and severity are different.

Skin- The most common identified sympton of Scleroderm is the gradual thickenin and tigthening of the skin.  Ulcers, expecially on the fingertips are common.  These ulcers are slow to heal due to poor circulation.

Raynaud's Phenomenon- This is a very common sympton, and is generally beleived that 98% of scleorderma patients have raynauds.

Muscle Weakness- Muscles weaken and becoem painful.

Joints- joints becoem stiff and sore, simillar to arthiritis.

Digestive System and Gastrointestinal Track - Swallowing difficulties can result from the narowing of the esophagus.  Digestive diffulties range from poor absorption of nutrients to slow movement of food. 

Sjogrens Syndrome - sjogren's syndrome is dry eyes and mouth due to decrease in secretions of the tear ducts and salivary.

Dental-  due to tigteninf of the facial skin, dental health may be comprimised.  Dry mouth causes difficulties in swallowing, which in turn may lead to tooth decay.

Lungs: A build up of fibrosis ( scarring) in the lungs and or weaknedrespiratory muscles lead to shortness of breath and persistent coughing.  The fibrosis affects oxygen abosorption and may lead to pulmonary Arterial Hypertension.

Kidney -Early signs of kidney damage may include high blood pressre and excess protein in the urine.  Renal crisis is a severe complication and may lead to kidney failure.

Non - specific symptons, these include extreme fatigye, general weakness, weightloss, hair loss and vague aching of the muscles, bones and joints.

Causes of Scleroderma

Whilst the cause of scleroderma is not known, there are several theories being studied which involve different systems of the body, the immune system, the vascular system, and the connective tissues.  Some reasearchers indicate an infections component like a virus organisim acting as a trigger. There is an indication of a genetic predisposition to the diease with an enviromental trigger.

Scleroderma is not hereditary or contagious.

Who gets Scleorderma

Although scleroderma strikes every sex age and ethnic background, more than 80% are women between the ages of 30 -80 (child earing years).

Treatment

Although there is no known cure for scleroderma, symptons can be moderated with medication and lifestyle changes.  Some medications are aimed at specific symptons, while others are aimed at decreasing the activity of the immune system.  Because of the advances in treatment, patient survival has improved a great deal over the past years.