A 24-year-old female patient experienced rash and oral ulcers with arthralgia at the age of 19 years, when antinuclear antibodies and double-stranded DNA antibodies were positive. The patient was diagnosed with SLE based on her clinical manifestations and relevant immune test results. She was initially treated with prednisone 50 mg/day and hydroxychloroquine orally. She returned regularly with reasonable disease control, followed by a gradual reduction to prednisone 10 mg for long-term maintenance therapy.
In August 2018, the patient began to experience repeated diarrhea with abdominal pain and occasional fever, with a body temperature up to 40 C, accompanied by nausea and anorexia. The stools varied from several to ten times a day and were yellow watery stools. No treatment was given. On October 17, 2018, the patient was admitted to the hospital. Blood routine: blood cell analysis (five-part differential): white blood cell count (WBC) 8.31G/L, neutrophil granulocyte% (NEUT%) 85%, red blood cell (RBC) 2.99T/L, hemoglobin (Hb) 74.00 g/L, hematocrit (HCT) 24.2%, platelet count (PLT) 295.00G/L. Urinalysis: RBC 1321.00 cells/ul, WBC 1636.00 cells/ul, urine protein + stool routine + occult blood test (OB): positive fecal occult blood. The stool smear revealed fungal spores and hyphae. High-sensitivity C-reactive protein 103.29 mg/L. The erythrocyte sedimentation rate was 94 mm/h. Coombs' test was positive. ANA antibody spectrum (containing anti-dsDNA) antinuclear antibody 1:1000 positive, anti-double-stranded DNA antibody negative, anti-Smith antibody weakly positive, anti-ribosomal P protein antibody strongly positive. Complement3 0.61 g/l, Complement4 0.14 g/l. T-cells enzyme linked immunospot (T-SPOT) was positive. Chest CT: interstitial change in both sides. For the patient. Considering the initial treatment by a variety of antibiotics was ineffective, and the fundamental disease is SLE, for which lupus associated intestinal vasculitis is a common complication, when obvious gastrointestinal symptoms occurred, relevant physician of the hospital related the diarrhea and lupus-associated intestinal vasculitis to intestinal fungus infection, and gave methylprednisolone pulse therapy, 500 mg/d, for 3 days, twice in total; and gave fluconazol 200 mg/d, po (10.21-11.04), fluconazol 200 mg/d, ivgtt (11.04-11.06) for antifungal therapy. After the above treatment, the patient's diarrhea was not significantly improved.
On November 6, 2018, the patient was transferred to our hospital for continuous treatment. Blood cell analysis (five-part differential) showed WBC 7.73G/L, RBC 2.68T/L, Hb 70.00 g/L, HCT21.9%, PLT 61.00G/L. Urine examination showed RBC 68.0 cells/ul, WBC 456.00 cells/ul, Blood 3 +. Stool routine + OB: positive fecal occult blood. Liver and kidney function and electrolytes showed no significant abnormalities. ANA antibody profile (containing anti-dsDNA) antinuclear antibody: 1:3200 positive, anti-Smith antibody negative, anti-double-stranded DNA antibody negative. Anti-ribosomal P protein antibody was strongly positive. Complement3 0.398 g/l, Complement4 0.124/l. High-sensitivity C-reactive protein 103.29 mg/L. The erythrocyte sedimentation rate was normal. QuantiFERON (R)-TB Gold In-Tube (QFT) was negative. Coombs' test was positive. Thoracoabdominal CT showed interstitial changes in both sides (the main manifestations are left upper lung nodule and right lower lung calcification), edema and thickening of the intestinal wall of the ascending colon, and multiple surrounding exudates. During the course of the disease, the patient had no arthralgia, alopecia, oral ulcers, or new rash, and the SLEDAI score was 3. Methylprednisolone (40 mg/d) was given to inhibit immunity, micafungin (100 mg/d) for antifungal treatment, levofloxacin (0.3 g/d) and metronidazole (0.2 g Tid) for anti-infection, and nutritional support and other treatments. After the above treatment, the diarrhea was not significantly relieved. During hospitalization, we found that: the patient had repeated diarrhea, yellow watery stools, water content, floating fecal residue on the surface, and most of the time was regular, with an interval of 3-5 hours; in addition, the patient's daily intake was about 2000mL, urine volume was significantly reduced (about 100mL/d), and there were many floccules in the urine, but the patient's multiple examinations of renal function showed no abnormality (creatinine fluctuating between 28.00-41.01 umol/l), and there was no significant systemic edema. According to the character and regularity of urine and defecation, the presence of urinary fistula was considered. Therefore, to confirm the diagnosis, we performed the following verification: First, we obtained the stool of this patient, measured its urinary creatinine value after centrifugation of the stool (2504 umol/L), and obtained the urine of another patient with normal renal function to measure its urinary creatinine value (3442 umol/L); then cystography revealed that contrast agent was exuding from the bladder (see Figure 1A and B); thereafter, renal dynamic imaging was performed, and we found that over time, the contrast agent was gradually visualized in both kidneys and bladder (see Figure 1C), and the stool sample of the patient also shows this visualization (Figure 1D). In summary, the diagnosis of urinary fistula was confirmed in this patient. To understand the cause of urinary fistula, a colonoscopy was conducted and showed multiple ulcers in the large intestine: Crohn's disease? (Figure 2), and colonic tissue biopsy revealed chronic inflammation and granulation tissue hyperplasia (Figure 3). Because Crohn's disease and intestinal tuberculosis are similar in clinical manifestations and pathological findings, considering that this patient had fatigue, anorexia, weight loss, recent night sweats, and a history of long-term use of hormones and immunosuppressive agents, chest and abdomen CT revealed multiple thoracic, abdominal, and retroperitoneal lymphadenopathy with calcification, positive T-SPOT, and elevated high-sensitivity C-reactive protein, although the predilection site for intestinal tuberculosis was the ileocecum, it could also occur throughout the intestine, so intestinal TBC still could not be excluded. Therefore, we further conducted the relevant examinations: gastroscopy showed chronic non-atrophic gastritis, QFT was positive, the results of detecting mycobacterium TBC in colon biopsy tissues using PCR technique were positive twice, and acid-fast staining revealed positive bacilli. Combined with the patient's medical history, clinical manifestations and relevant examinations, the diagnosis of vesicorectal fistula caused by intestinal TBC was confirmed. Quadruple anti-tuberculosis treatment (isoniazid + rifampicin + pyrazinamide + ethambutol) was given, and conservative treatment (indwelling catheter to observe whether the fistula could close spontaneously) was given based on the patient's underlying disease and poor nutritional status; for SLE, prednisone (20 mg/d) and hydroxychloroquine (0.2 g bid) were given.
More than 1 month later, the patient was re-admitted to our hospital due to acute low intestinal obstruction, and was given gastrointestinal decompression and drainage with small intestinal drainage tube. The patient continued to receive anti-tuberculosis treatment and was discharged with improved condition. During this period, the patient returned to the outpatient department regularly, and enteroscopy showed ulcer healing in November 2020 (Figure 4). During follow-up until January 7, 2022, the patient had gradually reduced prednisone to 7.5 mg/d and hydroxychloroquine to 0.2 g/d, and discontinued anti-tuberculosis drugs for 1 year. At present, the patient's condition is stable, urine and defecation are normal, and lupus is fairly controlled.