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We report a case of systemic sclerosis (SSc) complicated with benign pneumoperitoneum without apparent pneumatosis cystoides intestinalis (PCI). A 43-year-old woman was admitted to our hospital because of prominent abdominal distension in April 1997. Raynaud's phenomenon has been detected since 1991. She was suffering from recurrent diarrhea, constipation, and subileus. The diagnosis of SSc was made in 1996 based on the sclerosis in her face, forearms, and chest, and hypomotility of the esophagus. On admission, she presented no signs of peritoneal irritation. The laboratory data revealed that white blood cell count was 7,400/mm3 and C-reactive protein was 0.1 mg/dl. Chest and abdominal roentgenograms showed massive free air under the diaphragm, dilatation of small and large intestine, and air-fluid level. PCI was not apparent. Pneumoperitoneum was improved after four weeks with intravenous hyperalimentation. But she presented recurrent severe diarrhea and high fever whenever she tried to take food orally. Klebsiella pneumoniae was proved in her jejunal juice by bacteriologic examination. Intravenous prostaglandin F2 alpha and oral fosfomycin calcium intake made her condition better. Benign pneumoperitoneum without PCI is rarely reported in the patients with SSc. In her condition, weakness of intestinal wall, hypomotility of intestine, unusual bacterial overgrowth, and elevated intraluminal pressure made intraluminal gas go through the wall of the fragile intestine of SSc. As operation of intestine of SSc usually cause miserable outcome, pneumoperitoneum accompanied with SSc even if PCI is apparent or not must be treated with conventional manner while there is no signs of peritoneal irritation.

A 41-year-old woman was admitted to Tokyo Women's Medical College Aoyama Hospital in January 1994. She presented cough and dyspnea in September 1991. The diagnosis of interstitial pneumonia was made based on TBLB. Interstitial pneumonia was responsive to initial prednisolone of 40 mg daily. When dose of prednisolone was reduced to 15 mg daily, she complained of cough and dyspnea again. She was referred to Institute of Rheumatology, Tokyo Women's Maedical College in November 1993. She was diagnosed as systemic sclerosis associated with interstitial pneumonia based on proximal scleroderma and digital pitting scar. Double immunodiffusion and immunoprecipitation assay revealed the presence of anti-Ki, anti-Wa, and anti-RNA polymerases antibodies in the serum. On admission in March 1994, she was treated with intravenous cyclophosphamide therapy at 500 mg/day once a mouth. After the third infusion, respiratory symptoms, pulmonary function test values, findings of chest X-ray and CT scan were improved without adverse drug effects. Intravenous cyclophosphamide therapy seems to be useful in this case. The efficacy of Intravenous cyclophosphamide therapy in the treatment of the interstitial pneumonia associated with systemic sclerosis was discussed.

Forty six patients (41 female and 5 male) with progressive systemic sclerosis were analysed through X-ray photographs of feet. Age ranged from 33 to 74 (mean: 48 +/- 13) years old, and durations of the disease were one to 28 (mean: 9.8 +/- 8) years. Abnormal radiological findings of feet were noted as following; bone absorption of the terminal tufts of the first phalanx (6 out of 46 cases: 13%), bone absorption of metatarsals and/or mid, basal phalanges (2 cases: 4.3%), bone erosion (6 cases: 13.0%), calcification (7 cases: 15.2%), diffuse osteoporosis (2 cases: 4.3%) and cystic lesion (one case: 2.2%). All patients with abnormal findings on X-ray photographs of feet simultaneously showed radiological changes of hands. It took 13.3 +/- 7 years until the radiological findings appeared in feet. On the other hand, it took much shorter 8.8 +/- 6 years in hands. Patients with radiological changes in feet tended simultaneously to have pulmonary and digestive tract involvements, and widespread skin lesions such identified as diffuse scleroderma. Anti-Scl 70 antibody were positive in a half of these patients.