Pediatric Rheumatology Online Journal → July 2003 → Childhood Lupus→ Case Reports → Abstract #111

LUPUS CYSTITIS IN ASSOCIATION WITH SEVERE GASTROINTESTINAL MANIFESTATIONS IN AN ADOLESCENT

P. Dancey,¹ E. Constantin,¹ C. M. Duffy,^1,2 R. Scuccimarri.^1,2

¹Rheumatology, Montreal Children's Hospital, Montreal, Canada; ²McGill University, Montreal, Canada

Background: Lupus cystitis is a rare interstitial inflammatory disease of the bladder, unique to systemic lupus erythematosus (SLE) that usually occurs in association with gastrointestinal manifestations. There are very few reports of this entity in pediatric SLE.
Case: We describe a previously well 16 year old girl who presented with abdominal pain and rebound tenderness. An ultrasound (US) examination of the abdomen was normal, aside from some free fluid. A laparotomy to rule out appendicitis revealed a normal appendix. Postoperatively she developed vomiting, severe diarrhea, urinary frequency, incontinence, tachypnea with pleural effusions, ascites, pancreatitis, and hepatitis. A repeat US showed bilateral ureterohydronephrosis and bladder wall thickening. CT scan, endoscopy and colonoscopy showed profound generalized gastric and intestinal edema. Work up for serositis revealed lymphopenia, Coombs' positive hemolytic anemia, decreased C3 and C4, ANA titre 1:640, DNA binding 43%, and antibodies to Sm. During the course of her six week admission she developed proteinuria and hematuria with class III lupus nephritis on biopsy. A diagnosis of SLE was made with evidence of severe multisystem disease. Treatment with pulse methylprednisolone for 3 days, was followed by daily prednisone. Resolution of diarrhea occurred within 1 wk. Imuran and plaquenil were added. Urinary symptoms improved progressively. A repeat CT scan at 11 weeks showed only mild hydronephrosis with resolution of all other prior findings. At 5 months follow-up all symptoms have effectively resolved. Conclusion: While many reports of lupus cystitis describe resistant disease with poor prognosis, this patient responded well to treatment. Early recognition of lupus cystitis and its frequent association with gastrointestinal manifestations may facilitate timely diagnosis and appropriate treatment.