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A Case of Progressive Systemic Sclerosis (PSS) M. Tanaka 1 , K. Tsutsumi 1 , K. Nagasako 1 1The Institute of Gastroenterology, Tokyo Women's Medical School pp.667-672
Published Date 1979/5/25
DOI https://doi.org/10.11477/mf.1403107679
  • Abstract
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 A 45-year-old man was admitted to the hospital on July 1977. At the age of 35, he noted heart burn, and at the age of 42, tightness and thickness of his skin and Raynaud's phenomenon began. Meanwhile, he developed dysphagia and epigastralgia. In Dec. 1976, gastric ulcer was found and subtotal gastrectomy (B-1) was performed, with relief of epigastralgia. Five months before admission, skin biopsy was done and diagnosis of PSS had been made. Present chief complaints were nausea, vomiting and abdominal distension.

 Physical Examination-The patient was thin. The skin was thin, taut and atrophic. The hands appeared claw-like with loss of extension and flexion of the fingers. The remainder of the physical examination was noncontributory.

 Laboratory Data-Hb 13.6 g/100 ml, WBC 5,300, with normal differential count; blood chemistry study was essentially normal, ESR 35 mm in the first hour. EKG showed low voltage. The 4-days faecal out put of 131I-Triolein was 5.9% of the parentally administered dose.

 Roentgenologic Studies-Examination with barium showed dilatation of the esophagus and small intestine. Peristalsis was diminished.

 Intraluminal pressures of Esophagus-Disappearance of a peristaltic wave and low pressure (+2~3 cm H2O) at the lower esophageal sphincter were demonstrated.

 Endoscopic studies-Mucosal redness was observed in the lower part of esophagus and duodenum.

 Biopsy on the duodenum-Atrophy of the villi and capillary dilatation were recognized.

 Course-Treatment was performed symptomatically. The patient became progressively weaker and died eight month after entry.

 Autopsy-The esophagus and the duodenum were slightly dilated, and the jejunum was makedly dilated. The mucosa of the lower esophagus and the duodenum was hyperemic.

 Histological Examination-All sections from all parts of the alimentary tract showed diffuse atrophy of the muscularis and increased deposition of collagen in the submucosa and muscular layer. Vascular abnormality, such as fibrosis and concentric intimal proliferation, was not recognized in all parts of the gastrointestinal tract.

 Comment-PSS is recognized as a member of the so-called collagen group of diseases. Involvement of the gastrointestinal tract in PSS has received increasing attention. The pathological changes consist of a systemic alteration in the connective tissue and vascular system. This patient's gastrointestinal symptoms was severe. Abdominal distension, disphagia, nausea and vomiting dominated the clinical picture. Dilatation of the esophagus and small intestine, diminution of peristalsis and malabsorption were demonstrated by the clinical examination. Histological study showed atrophy and partial replacement of the muscular coat by collagenous tissue in all part of the alimentary tract. But vascular alteration of the G-I tract was not recognized. Autonomic nervous system dysfunction was suggested to be one of the pathogenesis of Raynaud's phenomenon in PSS. The same phenomenon without morphological changes of the blood vessel may exist at the G-I tract.


Copyright © 1979, Igaku-Shoin Ltd. All rights reserved.

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電子版ISSN 1882-1219 印刷版ISSN 0536-2180 医学書院

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