SSc - A Multisystem Fibrotic Disease

SSc is a multisystem fibrotic disease characterized by fibrosis and a cellular autoimmune response. It is a chronic disease that affects any age and race, but is more common in African-Americans.

It is classified into two major types: limited cutaneous SSc and diffuse cutaneous SSc. Limited SSc is characterized by thickened skin in the arms, feet, or hands, while diffuse SSc involves both proximal and distal extremities.

Patients with SSc may also experience other symptoms, such as muscle weakness, bloating, and problems with the absorption of nutrients. In addition, some individuals with SSc have a decreased heart muscle mass. In these cases, they may also develop abnormal cardiac rhythms.

The initial testing for SSc should include a complete blood count (CBC), rheumatoid factor, ESR, and metabolic panel. In addition, serologic testing of autoantibodies is recommended. These tests can detect a rheumatoid factor or anti-DNA-topoisomerase I (ATA) antibody.

Several medications are under investigation as potential new therapies for SSc. Currently, the most widely used treatments are immunosuppressant therapies, including cyclophosphamide and mycophenolate mofetil. These treatments should be administered with caution, however, as they can result in serious adverse outcomes.

Some of the more severe forms of SSc may require lung transplantation. However, this is only possible in a minority of patients. In addition, it is contraindicated in certain cases, such as gastroparesis, increased risk of aspiration due to oesophageal dysmotility, or active systemic disease.

In addition, some patients with SSc can also suffer from neuropathies. This can lead to numbness, digital ulcers, and gangrene of the fingers.