Scleroderma - thoracic and gastrointestinal manifestations

Case contributed by Fabien Ho , 9 Jan 2018
Diagnosis certain
Changed by Daniel J Bell, 12 Jan 2018

Updates to Case Attributes

Title was changed:
SclerodermiaScleroderma: thoracic and oesophago-gastrointestinal findings
Presentation was changed:
Acute sepsis, acute and diffuse abdominal pain. Patient has been followedSerial follow-up for several years inby internal medicine for a disease with abnormal thoracic and abdominal findings.
Age changed from 30 to 30 years.
Body was changed:

Patient got better under probabilist antibiotherapy (C3G+with broad-spectrum antimicrobial therapy (third generation cephalosporins and metronidazole). Infectious samplingsSeptic screen (sputum, blood, urine, stools) werewas negative, and the etiologyaetiology of the sepsis is unclear.

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

This case shows an associationa combination of thoracic (NSIP) and gastrointestinal findings (oesophageal dilation; smalldilatation, small bowel: dilation dilatation, reduceddecreased peristalsis, duodenal diverticulum; large, large bowel: dilation, dilatation with loss of haustrationshaustra) inconsistent with scleroderma.

  • -<p>Patient got better under probabilist antibiotherapy (C3G+ metronidazole). Infectious samplings (sputum, blood, urine, stools) were negative, and the etiology of the sepsis is unclear.</p><p>This patient has been followed up in internal medicine for years for scleroderma.</p><p>This case shows an association of <a href="/articles/scleroderma-pulmonary-manifestations-1">thoracic</a> (NSIP) and <a href="/articles/scleroderma-gastrointestinal-manifestations-1">gastrointestinal findings</a> (oesophageal dilation; small bowel: dilation, reduced peristalsis, duodenal diverticulum; large bowel: dilation, loss of haustrations) in <a href="/articles/scleroderma">scleroderma</a>.</p>
  • +<p>Patient got better with broad-spectrum antimicrobial therapy (third generation cephalosporins and metronidazole). Septic screen (sputum, blood, urine, stools) was negative, and the aetiology of the sepsis is unclear.</p><p>This patient has been followed up in internal medicine for years for scleroderma.</p><p>This case shows a combination of <a href="/articles/scleroderma-pulmonary-manifestations-1">thoracic</a> (NSIP) and <a href="/articles/scleroderma-gastrointestinal-manifestations-1">gastrointestinal findings</a> (oesophageal dilatation, small bowel dilatation, decreased peristalsis, duodenal diverticulum, large bowel dilatation with loss of haustra) consistent with <a href="/articles/scleroderma">scleroderma</a>.</p>

Updates to Study Attributes

Caption was changed:
Lung CT a: one year ago
Findings was changed:

Institial lungInterstitial pneumonia on baseline 1 year ago with subpleural reticulations in both basal and apical regions, thickenings in both apexesapices. NSIP pattern.

Updates to Study Attributes

Caption was changed:
On admissionPresentation to emergencyED
Findings was changed:

Institial lungInterstitial pneumonia on baseline 1 year ago with subpleural reticulations in basal and apical regions,thickenings and thickenings on both apexes. NSIPapices; NSIP (non-specific interstitial pneumonitis) pattern.

Findings that may be related to current acute sepsis: bilateral groungground glass, consolidation and ill-defined centrolobularcentrilobular nodules.

OesogastrointestinalStable gastrointestinal findings, stable on successive CT exams: Oesophageal dilation. Pyloricserial CTs: oesophageal dilatation, pyloric, duodenal and jejunal wall thickeningsmucosal thickening, with a D1 diverticulum and well-visible-seen valvulae conniventes. Jejuno-ileal dilation (stable)Stable panenterocolic dilatation without transition point, followed by whole colon dilation, identical identical to previous CT scansCTs (not shown): most likely adynamic bowel.

CurrentConcurrent acute sepsis brought, coupled with dilated bowel of 6cm and mild free fluid, raised suspicion for toxic megacolon, due to the diameter of the dilated bowels of 6cm and a slight amount of free peritoneal fluid. Normal bowel enhancement.

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