Sine sclerosis is a rare autoimmune condition that affects only about 5 percent of people with systemic scleroderma. Patients with sine scleroderma do not have the hard, thick skin that is characteristic of systemic scleroderma, but still experience an abnormal accumulation of scar tissue in their internal organs.
Because this condition is so rare, some researchers think it may be a part of the systemic scleroderma disease spectrum rather than a separate entity. However, some studies have found it to be a distinct type of systemic scleroderma that is very similar to limited scleroderma.
Symptoms of sine sclerosis
Besides the absence of skin thickening, patients may develop Raynaud’s phenomenon, a condition in which fingers turn white, blue and red in response to emotional stress or exposure to cold.
Organs that may be damaged in sine sclerosis include the lungs (causing shortness of breath), kidneys (causing high blood pressure), the heart, or gastrointestinal tract (causing symptoms of heartburn, difficulties swallowing, diarrhea, or constipation).
Diagnosing sine sclerosis
Sine sclerosis is difficult to diagnose because there are no external skin manifestations typically seen in systemic scleroderma. Patients may test positive for certain autoantibodies, or antibodies typically produced in autoimmune diseases that mistakenly attack healthy tissues in the body. If sine sclerosis is suspected, patients may need to undergo more extensive blood tests, urine tests to assess kidney function, and chest X-rays, or CT scans of the lung.
Treatment and prognosis of sine sclerosis
Because sine sclerosis does not have any obvious signs, there may be a delay in diagnosis, which may lead to poorer outcomes. As with other types of systemic scleroderma, sine sclerosis is incurable. However, several treatments are available to manage and slow the progression of the disease. Each patient may need different types of treatment regimens depending on the damaged organs.
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