Scleroderma - thoracic and gastrointestinal manifestations

Case contributed by Fabien Ho
Diagnosis certain


Acute sepsis and diffuse abdominal pain. Serial follow-up, for several years, by internal medicine for a disease with abnormal thoracic and abdominal findings.

Patient Data

Age: 30 years
Gender: Female

Lung CT: one year ago


Interstitial pneumonia on baseline 1 year ago with subpleural reticulations in both basal and apical regions, thickenings in both apices. No volume loss. NSIP pattern. Esophagus also dilated. Combination of NSIP pattern and dilated esophagus raises suspicion for scleroderma.

Interstitial pneumonia on baseline 1 year ago with subpleural reticulations in basal and apical regions and thickenings on both apices; NSIP (non-specific interstitial pneumonitis) pattern.

Findings that are likely related to superadded acute sepsis: bilateral ground glass, consolidation and ill-defined centrilobular nodules.

Stable, on serial CTs, gastrointestinal findings: esophageal dilatation, pyloric, duodenal and jejunal mucosal thickening, with a D1 diverticulum and well-seen valvulae conniventes. Stable enteric and proximal colonic (cecum, ascending and transverse colons) dilatation without discrete transition point, identical to previous CTs (not shown). Descending and sigmoid colons and the rectum are not dilated. Overall picture most likely an adynamic bowel.

Concurrent acute sepsis, coupled with dilated bowel of 6cm and mild free fluid, raised suspicion for toxic megacolon. Normal bowel enhancement.

Case Discussion

Patient got better with broad-spectrum antimicrobial therapy (third generation cephalosporins and metronidazole). Septic screen (sputum, blood, urine, stools) was negative, and the etiology of the sepsis is unclear.

This patient has been followed up in internal medicine for years for scleroderma.

This case shows a combination of thoracic (NSIP) and gastrointestinal findings (esophageal dilatation, small bowel dilatation, decreased peristalsis, duodenal diverticulum, large bowel dilatation with loss of haustra) consistent with scleroderma.

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