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Interstitial cystitis and ileus in pediatric-onset systemic lupus erythematosus
1Department of Pediatrics, Hirosaki University, School of Medicine, Japan. hirotana@cc.hirosaki-u.ac.jp
Insights
This study reports an uncommon case of pediatric systemic lupus erythematosus (SLE) presenting with paralytic ileus and chronic interstitial cystitis. High-dose prednisolone improved symptoms, but relapses occurred upon dose reduction.
Area of Science:
- Pediatric Rheumatology
- Nephrology
- Gastroenterology
Background:
- Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that can affect multiple organ systems.
- Pediatric-onset SLE often presents with unique clinical manifestations and disease progression.
- Gastrointestinal and genitourinary complications, while known, can be rare and challenging to diagnose.
Observation:
- An 11-year-old girl with autoimmune hemolytic anemia and thrombocytopenia developed severe abdominal pain, vomiting, and pollakiuria.
- Imaging revealed paralytic ileus with intestinal thickening and bilateral hydroureter with hydronephrosis.
- Bladder pathology showed interstitial cystitis with immune complex deposition in blood vessel walls.
Findings:
- The patient was diagnosed with systemic lupus erythematosus (SLE) complicated by paralytic ileus and chronic interstitial cystitis.
- Treatment with high-dose prednisolone led to clinical improvement.
- Lowering prednisolone dosage resulted in disease relapse, indicating the need for sustained immunosuppression.
Implications:
- This case highlights the diverse and uncommon gastrointestinal and genitourinary manifestations of pediatric SLE.
- It underscores the importance of considering SLE in children presenting with unexplained abdominal and urinary symptoms.
- The findings suggest that sustained immunosuppression may be crucial for managing complex SLE cases with multiple organ involvement.
Abstract:
A girl aged 11 years presented with autoimmune hemolytic anemia with thrombocytopenia, and subsequently developed severe abdominal pain, vomiting, and pollakiuria. X-ray findings of her abdomen demonstrated paralytic ileus with intestinal wall thickening. Intravenous pyelography revealed bilateral hydroureter with mild hydronephrosis and contracted bladder. Pathological examination of her bladder revealed interstitial cystitis, with evidence of focal deposition of IgG and C3 in a granular pattern on small blood vessel walls. She was diagnosed as having systemic lupus erythematosus (SLE) associated with paralytic ileus and chronic interstitial cystitis. Although initiation of high-dose prednisolone therapy resulted in a gradual improvement in clinical symptoms, reducing the dosage of prednisolone caused a relapse. To our knowledge, the combination of paralytic ileus and chronic interstitial cystitis is quite uncommon in pediatric-onset SLE.