Scleroderma and Lung CHAPTER 15

Scleroderma is a chronic systemic disease of unknown cause, affecting around 15 persons in one million inhabitants in all parts of the world, but most frequently women between 40–60 years of age. Until recently, scleroderma was considered to be a disease with gloomy prognosis and no cure. The general attitude has changed during last decades due to successful treatment of scleroderma renal crisis with ACE inhibitor drugs with consequent dramatic decrease of mortality. Today the pulmonary complications, interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH) are the leading cause of death.1 Several studies showed that the disease modifying drug, cyclophosphamide, can reduce the progression of scleroderma ILD in patients with early symptomatic disease with stabilization and also improve the extent of skin indurations. Pulmonary vasodilators have been shown to improve exercise tolerance and slow the rate of clinical deterioration in PAH. Improved survival in scleroderma is associated also with better ascertainment of internal organ disease.2 All these data combined, contribute to the changing attitude towards scleroderma patients, with certain groups already having moderately better prognosis and other emerging towards the same goal. The lung disease has the worst prognosis and governs the destiny of patients with scleroderma.