Systemic Sclerosis

Systemic Sclerosis

The most common manifestation of systemic sclerosis is Scleroderma (hard skin). The thickening and hardening of the skin-and possibly of other organs, is caused by the production of too much collagen. Early indications of the disease include joint pain and swelling of the hands, tightening skin and sensitivity to cold. This reaction to temperature changes occurs when the blood vessels in the fingers and toes narrow and may spasm when exposed to extremes of cold or heat, resulting in color change (Raynaud's phenomenon). The fingers become swollen and small blood vessels may be prominent; gradually the skin over the face and fingers becomes thickened and tight. In severe cases the lungs, heart and kidneys can be affected.

Treatment of Scleroderma is difficult and the condition tends to progress gradually. However, rapid progression of the disease is seen in more severe cases.

There are two kinds of Scleroderma: The systemic form, which affects the internal organs as well as the skin, and the localized form, which affects a specific area of skin. Possibly as many as 300,000 people in the United States have Scleroderma with about four times as many women as men being affected.

Risk factors: In most cases, the cause of Systemic Sclerosis is unknown.

Symptoms: Generalized symptoms can include: Raynaud's phenomenon, swollen, painful and or stiff joints; thickening of skin, difficulty swallowing, weight loss, fatigue, muscle aches and joint weakness, shortness of breath.

Diagnosis: A diagnosis may be very difficult in the early stages because many of the symptoms overlap with other diseases such as Rheumatoid Arthritis, Lupus, and Polymyositis. Confirmation may depend on blood studies and specific organ function tests. Diagnosis is aided by the presence of auto-antibodies in the blood.