SCLERODERMA: signs and symtoms plus therapy and surgery info

Scleroderma (systematic sclerosis) is a generalized connective tissue disorder of unknown cause characterized by hardenig or thickening of the skin, blood vessels, synovium, skeletal muscles, and internal organs. Fibrotic, degenerative, and inflammatory changes including vascular insufficiency are most likely caused by overproduction of collagen by fibroblasts. The disorder affects three to four times as many women as men.

Complications include skin ulcers, malasorption, esophageal adenocarcinoma, pulmonary hypertension, renal failure, heart failure, and death.


Symptoms:

a. Skin related manifestations
- Bilateral symmetric swelling of the hands and sometimes the feet.
- Hardening and thickening of skin after edematous phase.
- Skin changes may regress after several years.
- Raynaud's phenomenon.
- Areas of calcinosis in late disease

b. GI manifestations
- Esophageal dysmotility resulting in reflux and esophagittis
- Distal esophageal dilation and esophagitis
- Duodenal atrophy and dilation may cause postprandial abdominal pain
- Diarrhea
- Abdominal distention
- Colonic hypomotility resulting in constipation

c. Musculoskeletal manifestations:
- Joint pain
- Polyarthritis
- Carpal tunnel syndrome
- Flexion contractures
- Inflammation muscle atrophy

d. Cardiac manifestations
- Left ventricular dysfunction
- Heart failure and atrial and ventricular arrhythmias
- Right ventricular involvement secondary to pulmonary disease

e. Pulmonary manifestation
- Interstitial fibrosis
- Restrictive lung disease
- Pulmonary hypertension

f. Renal manifestations
- Scleroderma renal crisis - rapid malignant hypertension with encephalopathy


Diagnosing scleroderma:

Scleroderma may be difficult to diagnose because it can develop gradually and present itself in several different forms. Experts say that there is no single test that can make a definitive diagnosis. The doctor needs to look at the whole person, taking into account signs and symptoms, the results of a physical examination and some tests. Diagnosis often requires the expertise of a rheumatologist, a doctor who specializes in joint and connective tissue diseases.

The following tests may be ordered:

Fingernails - a skin microscope is used to look at the tiny blood vessels (capillaries) in the nail area. Changes associated with systemic sclerosis may be detected.

Skin biopsy - a small sample of the affected skin is extracted and examined under a microscope.

Blood test - patients with scleroderma usually have high levels of certain antibodies.

Other tests - other tests to detect any lung, heart or gastrointestinal complications may also be ordered.
What are the treatment options for scleroderma?

There is currently no treatment that can cure scleroderma. No medication can halt the overproduction of collagen. The localized variety of scleroderma may resolve on its own. Some medications may help control scleroderma symptoms and also help prevent complications.

The doctor's aim will be to:

Relieve symptoms
Try to prevent the condition from progressing, or at least slow down its speed of progression

Detect and treat complications as soon as possible

Minimize possible disabilities

Medications

Dilating blood vessels - blood pressure medications that dilate the blood vessels may help prevent some organ problems (e.g. kidney or lung). They may also help treat Raynaud's disease.

Immune system - some medications, known as immunosuppressants, may suppress the immune system (calm it down). These are the same medications organ transplant recipients use to stop their immune systems from rejecting the new organ.


Therapy


Physical therapy (UK: physiotherapy) or occupational therapy - the patient may be helped to manage pain, improve mobility, gain more strength. Certain strategies may be learnt to help the patient perform essential daily tasks. An occupational therapist may advise on certain aids, such as splints, which support the joints and help the patient carry out daily tasks more successfully.

Skin appearance - exposure to ultraviolet light may help improve the appearance of certain skin lesions.

Dental checkups - if the patient has dry mouth symptoms regular dental checkups are essential.

Surgery

Amputation - if ulcers on the fingers have developed gangrene it may be necessary to amputate (more common with severe Raynaud's disease).

Lung transplant - if the patient has developed pulmonary hypertension (high blood pressure in the arteries to the lungs), a lung transplant may help.

Laser surgery may be used to help with tight skin, eliminate lesions, or camouflage them.
What are the possible complications of scleroderma?

Scleroderma complications may be mild, severe and even life-threatening.

Raynaud's complications - restricted blood flow may permanently damage the fingertips, resulting in pits or ulcers in the flesh. Gangrene can occur, leading to amputation.

Lung complications - pulmonary fibrosis (scarring of lung tissue) can lead to reduced lung function, making it harder to breathe properly. The patient may also develop pulmonary hypertension - high blood pressure in the pulmonary artery that conveys blood from the right ventricle (of the heart) to the lungs. The lungs can become permanently damaged, and there may be failure of the right ventricle.

Kidneys - kidney damage can result in hypertension (high blood pressure) and excess urine protein levels. In some cases there may be a sudden increase in blood pressure and rapid kidney failure (renal crisis) - symptoms include headache, vision problems, seizures, breathlessness, swelling of the legs and feet, or reduced urine production.

Heart - arrhythmias (abnormal heartbeats) and congestive heart failure may result from scarring of heart tissue. The patient may develop pericarditis - inflammation of the lining around the heart (the pericardium); this causes chest pain and fluid build-up around the heart (pericardial effusion).

Teeth - the facial skin may tighten so much that the mouth becomes smaller, making it harder to brush one's teeth; even having one's teeth professionally cleaned may be difficult. Dry mouth is common in patients with scleroderma - this increases the risk of tooth decay. Acid reflux may destroy tooth enamel. Changes in gum tissue may result in loose teeth (they may even fall out).

Sexual function - male erectile dysfunction is common. Women's vaginal opening may be constricting, making sexual function harder. Females with scleroderma may also experience decreased sexual lubrication.

Thyroid - the thyroid gland can become underactive (hypothyroidism).

Joints and muscles - if tight skin limits the movement of a joint, muscles and joints may become affected.

Intestines - the intestines may become underactive, resulting in bloating, constipation, and some other problems.