2012/09/03

Scleroderma: treatment options

Scleroderma is a multisystem disease characterized by a relatively common inflammatory changes, and sclerotic vascular diseases affecting the skin and internal organs, especially lungs, heart and gastrointestinal tract. The mean age of disease onset is 30-50 years with a female predominance of 4:1.

 


Two forms of the disease are recognized:

    * Systemic Scleroderma located (60%) in women and generally occurs in people older than those who diffuse systemic scleroderma. In this case the skin damage is limited to hands, feet, face and forearms, and organ damage occurs late;
    * Sclerodemia systemic spread. Onset is quick and is characterized by extensive involvement of the trunk early systemic and determination.
A symptom is present in all people with Raynaud’s phenomenon, characterized by ischemia in the fingers to cold exposure, which may precede the disease for years. Also, people complain of pain and reduced mobility in the fingers and knees and may have arthritis.

Skin Damage

1. Effect on the hands and feet. Initial: Raynaud’s phenomenon occurs (hands become pale when exposed to cold, and cyanotic), sclerosis (thickening and reduced skin elasticity), finger pulp sores that heal with scars;
2. Impaired face
    * Initial: periorbital edema occurs;
    * Late: skin edema and fibrosis leading to the appearance of a mask layout, with the disappearance of wrinkles, thinning lips, oral aperture reduction, narrowing the nose, so that eventually the patient looks younger than it really is.

Organ damage

    * Esophageal Damage: dysphagia occurs (the patient swallows hard) initially solid and then liquid;
    * Effect on Gastrointestinal: constipation, diarrhea, bloating, malabsorption (decreased absorption of nutrients);
    * Impairment of pulmonary alveoli, pulmonary fibrosis and inflammation, decreased oxygenation of the lungs;
    * Impaired Cardiac conduction disturbances, heart failure, pericarditis;
    * Renal impairment: occurs in approximately 45% of cases, can lead to kidney failure and malignant hypertension;
    * Impaired musculoskeletal disorders: muscle weakness, carpal tunnel syndrome.

Treatment Options

The corticosteroids can be useful early in the disease. Other drugs (Capro amino acid, D-penicillamine, colchicine) have not proven effective. Immunosuppressive drugs (cyclosporine, methotrexate, cyclophosphamide, mycophenolate mofetil) have improved skin symptoms, but had a limited benefit on organ damage.