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[Scleroderma with an unusual complication]

H C Langberg1, H P Aarseth

  • 1Medisinsk klinikk Ullevål sykehus, Oslo.

Tidsskrift for Den Norske Laegeforening : Tidsskrift for Praktisk Medicin, Ny Raekke
|September 20, 1994
PubMed
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A rare intestinal complication, pneumocystosis cystoides intestinalis, was diagnosed in a patient with progressive systemic sclerosis. Prompt treatment led to symptom improvement and no recurrence after four years.

Area of Science:

  • Gastroenterology
  • Rheumatology
  • Infectious Diseases

Background:

  • Progressive systemic sclerosis (PSS) is a chronic autoimmune disease affecting connective tissues.
  • Gastrointestinal manifestations are common in PSS, but rare complications like pneumocystosis are significant.
  • This case highlights a severe, yet treatable, intestinal complication in a long-standing PSS patient.

Observation:

  • A 30-year-old woman with a 7-year history of PSS presented with abdominal distension, peripheral edema, and weight loss.
  • Clinical examination revealed edema, ascites, and radiological evidence of distended bowels with pneumoperitoneum.
  • The constellation of symptoms and imaging findings suggested a diagnosis of pneumocystosis cystoides intestinalis.

Findings:

  • Pneumocystosis cystoides intestinalis was diagnosed as a complication of progressive systemic sclerosis.

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  • The patient's symptoms significantly improved following appropriate medical treatment.
  • No recurrence of the intestinal complication was observed during a four-year follow-up period.

    • Implications:

    • This case underscores the importance of considering rare gastrointestinal complications in patients with systemic sclerosis.
    • Early diagnosis and management of pneumocystosis cystoides intestinalis can lead to favorable outcomes.
    • Further research into the pathogenesis and management strategies for such rare complications in autoimmune diseases is warranted.