The extra-skin manifestations of progressive systemic sclerosis

Progressive systemic sclerosis（PSS） is a multisystem disorder. This article reported 56 cases of PSS with emphasis on the extra-skin manifestations, mainly the visceral and vascular lesions, as well as their signiticance to the lesion and prognosis of PSS. Ninety-five percent of the patients had one or multiple organ involvements in which the lungs were the most common one, followed by the digestive tract. Raynaud’s phenomenon was present in most cases. Fever, arthralgia and myositis frequently occurred one to five years after the onset of the disese. The patients with CREST syndrome had a long course and visceral involvements were not infrequent. All of the PSS patients with Sjogren’s syndrome were females of middle and old age and usually had multiple organ involuements. The prognosis was quite good in the patients who has no involvement of the heart, kedney or blood system. The recognition of extra-skin manifestations was of great significance to the early diagnosis, treatment and to the improvement of prognosis of this disease. Ultrasonic cardiogram is the most sensitive examination for demonstrating the mild multiple immunologic abnormalities.