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[Renal crisis in scleroderma with normal blood pressure]
1Dept. of Medicine D, Soroka Medical Center, Beer Sheba.
Harefuah
|March 15, 1990
Summary
A patient with multi-organ scleroderma experienced fatal kidney failure despite normal blood pressure. This occurred after surgery for pneumatosis cystoides intestinalis, a condition involving intestinal air cysts.
Area of Science:
- Gastroenterology
- Nephrology
- Rheumatology
Background:
- Scleroderma is a chronic autoimmune disease causing hardening and tightening of the skin and connective tissues.
- Systemic scleroderma can affect multiple organs, including the skin, lungs, heart, and kidneys.
- Pneumatosis cystoides intestinalis is a rare condition characterized by gas-filled cysts in the intestinal wall.
Observation:
- A 58-year-old man with advanced scleroderma presented with fatal renal failure.
- The patient had normal blood pressure prior to the development of renal failure.
- Two months earlier, he underwent surgery for spontaneous rupture of pneumatosis cystoides intestinalis, revealing free air under the diaphragm.
Findings:
- The case highlights a potential, albeit unusual, link between pneumatosis cystoides intestinalis complications and subsequent renal failure in scleroderma patients.
- Renal failure in scleroderma can occur independently of hypertension, suggesting other pathogenic mechanisms may be involved.
Implications:
- This case underscores the complex and sometimes unpredictable manifestations of scleroderma.
- Further investigation may be warranted to explore potential associations between gastrointestinal complications and renal outcomes in scleroderma.
- Clinicians should maintain a high index of suspicion for diverse complications in patients with multi-organ scleroderma.