Systemic Sclerosis(scleroderma) is derived from the Greek term for "hard skin. Scleroderma describes a group of autoimmune diseases. Systemic sclerosis (SSc) is a clinically heterogeneous generalized disorder which affects the connective tissue of the skin and internal organs such as gastrointestinal tract, lungs, heart and kidneys. It is characterized by alterations of the microvasculature, disturbances of the immune system and by massive deposition. This disease may produce local or systemic symptoms. Raynaud phenomenon is usually the first symptom of systemic sclerosis. Patients experience episodes of vasospasm , which causes blood vessels in the fingers and toes to constrict. Environmental factors are thought to play a role in triggering the disease. These environmental factors are thought to include, organic solvents, several synthetic adhesive or coating substances, viruses etc. Women are more at risk than men at all ages, but by the age of 50 women are 12 times more likely than men to develop the disease.
Scleroderma affects the skin , and in more serious cases it can affect the blood vessels and internal organs. The most evident symptom is the hardening of the skin and associated scarring. General. Basic functions of various cell types (endothelial cells, T-lymphocytes, monocytes, fibroblasts, mast cells) as well as the production and effects of cytokines, growth factors, and adhesion molecules have been studied and animal models have been developed to give closer insights into the pathophysiology of this disease. Excess collagen deposits in the skin and other organs produce the symptoms. Anaemia, raised sedimentation rate (ESR) and increased gamma globulins (hypergammaglobulinaemia) and varying immune abnormalities are quite common especially positive rheumatoid factors.
Causes of Systemic Sclerosis
Common causes of Systemic Sclerosis
- Vibration injury.
- Organic solvents.
- Aliphatic hydrocarbons.
- Epoxy resin.
- Amino acid compound L-5-hydroxytryptophan
- Appetite suppressants.
- Cosmetic procedures
Symptoms of Systemic Sclerosis
Common Symptoms of Systemic Sclerosis
- Swelling of fingers and joints
- Skin thickening.
- Skin is hard
- Tight and mask-like facial skin
- Ulcerations on fingertips or toes
- Bloating after meals
- Weight loss
- Shortness of breath
Treatment of Systemic Sclerosis
Common Treatment of Systemic Sclerosis
- Vascular therapy is multifaceted and not necessarily pharmacologic.
- Avoidance of tobacco, cold exposure, and vasoconstricting medications.
- Biofeedback has been helpful in some patients with the development of tissue ischemia of digital tip ulcers. Calcium channel blockers (eg, nifedipine, nicardipine) are suggested.
- Nonsteroidal anti-inflammatory drugs (NSAIDs), but to a lesser extent than the arthritis associated with other connective tissue diseases.
- Interstitial lung disease associated with SSc is a major therapeutic challenge.
- Calcinosis may be treated with calcium channel blockers, anticoagulants, colchicine and intralesional steroids and possibly excision of deposits.