The patient was a 13-year-old girl. In August 2000, she presented with a
fever, together with
diarrhea,
vomiting,
arthralgia,
nasal bleeding and malaise, and was examined by another physician. Because her platelet count was low, and there were positive reactions for anti-nuclear
antibodies, anti-DNA antibodies and platelet-associated
IgG, idiopathic
thrombopenic purpura, and
systemic lupus erythematosus (SLE) was suspected. From January 2001, when she caught
measles, she reported
abdominal pain, and urinalysis indicated urinary
protein and occult blood, and the left kidney was found hydronephrotic. At the same time left ureter
stenosis and dilatation were demonstrated. Symptoms were disappeared by hydration and treatment with
NSAIDs, but 2 months later
fever and erythematous patches seen on both cheeks led to the proper diagnosis of SLE, and she was admitted to our hospital. Intravenous pyelography revealed
hydronephrosis on left kidney, constriction and dilatation of the left ureter, and intracystic endoscopy showed
erythema at the orifice of the left ureter. The pathological examination indicated the presence of
vasculitis, and finally lupus
cystitis was diagnosed. Intravenous
cyclophosphamide (IVCY)-pulse
therapy was introduced to a total of 8 times over the period of a year, and maintenance
therapy with predonisolone and azathioprin was also used. After completion of the IVCY-pulse
therapy, the
hydronephrosis and constriction of the ureter were disappeared. No side effects of IVCY-pulses were observed, and the patient is now in remission. We reported a case of childhood SLE complicated with lupus
cystitis and successfully treated by IVCY-pulse
therapy and maintenance predonisolone and azathioprin.