Lungs Involvement
It is the involvement of lungs that is usually life threatening in these patients.[9] There are 2 types of expression that frequently appear together:
- Pulmonary Fibrosis. The lungs slowly shrink, producing progressive difficulty in breathing, initially only with exercise or exertion and later even at rest. May be accompanied by a dry cough.
- Pulmonary Hypertension: Damage to the lung tissue leads to the appearance of signs of respiratory failure (shortness of breath, feeling short of breath). The pressure in the pulmonary artery rises and a condition called pulmonary hypertension develops. This significantly increases the stress on the heart and leads to its dysfunction. The symptoms are similar to those of fibrosis.