Systemic sclerosis lung disease in limited (C.R.E.S.T.) and diffuse subset

Fibrosing alveolitis and arterial pulmonary hypertension are the main cardiopulmonary findings in patients with systemic sclerosis. Clinically significant interstitial lung disease occurs in about 50% of the cases while 20% of the cases has pulmonary arterial hypertension. The onset of interstitial lung disease is characterized by cellular infiltration (alveolitis) and microvascular injury. Nonspecific interstitial pneumonia is the most common outcome of this pathological process. The extent of damage by means of high resolution CT and of disease severity by measuring the reduction of forced vital capacity with co-existing or isolated reduction of diffusing capacity, at the onset of the disease, are predictive of pulmonary fibrosis or pulmonary arterial hypertension. A case report with a limited cutaneous scleroderma leading to a fatal pre-capillary pulmonary arterial hypertension is presented.