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Lung is a most commonly involved organ in systemic scleroderma ��SS��. As a predominate reason for the death of patients with SS, pulmonary hypertension may be a primary manifestation of SS or a secondary manifestations following interstitial pulmonary fibrosis. It is difficult to diagnose pulmonary hypertension at early stage since symptoms may be absent or just unobvious in patients, while pulmonary function test, which can reveal gas diffusion dysfunction at early stage, may benefit the early diagnosis of it. To understand the risk factors and pathogenesis of SS-associated pulmonary hypertension may facilitate the early recognization, diagnosis, and optimization of treatment of this entity.

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