1. SLE 환자에서 동반된 복통과 설사 고려대학교 안암병원 소화기내과 박 성 철 대한소화기내시경학회 월례집담회

2. Chief complaint F/16 Watery diarrhea, lower abdominal pain (onset : 2 days ago)

3. Present illness 내원 2 개월 전 SLE 로 진단 받고 본원 F/U 하는 환 자로, 최근 피부 병변 악화 등 SLE activity 가 증가 하여 azathioprine 100mg, prednisolone 20mg, HCQ 200mg 으로 유지하고 있던 중, 내원 2 일 전 부터 시작된 하루 10 여 차례의 watery diarrhea 와 좌하복부 통증을 주소로 내원

4. Past history Family, Social & Allergy history 4 년 전 chronic ITP 로 진단 후 steroid 반응 없 어 2 년 전 splenectomy Rt ovarian cystectomy Non specific

5. Review of system left lower abdominal pain(+) tenesmus(-) poor oral intake(-) weight loss(-) melena/hematochezia(-/-) nausea/vomiting(-/-) constipation(-) diarrhea(+) arthralgia(-) hair loss(-) cough/sputum/rhinorrhea(-/-/-) fever(+) chilling(-) chest pain/dyspnea(-/-) urinary Sx(-)

6. Physical examination V/S 100/60mmHg - 118/min - 20/min – 38.3°c G/A Acute ill-looking appearance Alert mentality HEENT Anicteric sclera Pale conjunctiva Oral ulcer(-)

7. Physical examination Chest Regular heart beat/murmur(-) Clear breath sound Crackle(-) wheezing(-) Abdomen Soft & flat abdomen Normoactive bowel sound Mild tenderness on left lower abdomen No rebound tenderness No organomegaly

8. Physical examination Extremity pitting edema(-/-) CVA tenderness(-) Skin erythematous rash on face & upper extremities

9. Laboratory findings (1) CBC - MCV 68.7(fl) MCH 21.5(pg) MCHC 31.3(g/Dl) - WBC 12700 (neut 69, lymp 16, mono 4%) LFT Other blood chemistries - CRP 3.7 mg/L ESR 4 mm/hr - BUN 19 mg/dL Cr 0.7 mg/dL - Na-K-Cl 146-4.4-111 mmol/L - PT(INR)/aPTT 0.99/45 sec - AST 40 IU/L ALT 19 IU/L - T.pro/Alb 5.0/2.7 g/dl T.bil 0.41 mg/dl - PLT 365k - Hb 9.1 g/dl Hct 29.0% RDW 32.6%

10. Laboratory findings (2) Connective tissue disease lab - C3 62.7 mg/dL (Ref 88-201 mg/dL) - C4 13.6 mg/dL (Ref 16-407 mg/dL) - anti-ds DNA Ab 134.7 IU/mL (Ref<5.3 IU/mL) - anti-Smith Ab(+) - ANA(+) (homogenous, 1:640) - antiphospholipid Ab 4.0 U/mL (Ref<10 IU/mL) - lupus anticoagulant(-) U/A - Non specific

11. Laboratory findings (3) Stool lab - Stool OB(+), WBC(-) - Stool culture(-) : no Salmonella, Shigella, S. aureus - C. difficile toxin assay(-) - C. difficile stool culture(-) - parasites(-)

12. Skin manifestations

13. Chest X-ray

14. Abdominal X-ray flat upright

15. Abdomen pelvis CT

16. Colonoscopic findings (1)

17. Colonoscopic findings (2)

18. Pathologic findings

19. Diagnosis Ischemic colitis associated with systemic lupus erythematosus (subtype of Lupus colitis)

20. Hospital course  fluids, intravenous antibiotics (ciprofloxacin)  intravenous steroid pulse therapy  intravenous cyclophosphamide Clinical improvement over the next 3 days  stopped diarrhea  decrease of abdominal pain Management

21. Hospital course FEVER SEIZURE ABD PAIN DIARRHEA

22. Brain MRI

23. 3 weeks later F/U

25. Gastrointestinal Vasculitis Gastrointestinal Vasculitis ► Gastrointestinal vasculitis of SLE (Lupus enteritis)  one of the most serious complications of SLE  occurrence of colonic lesions : rare (0.2%)  tissue damage from vasculopathy mediated by immune complexes  small vessels of the intestinal wall rather than medium-sized mesenteric arteries  immunohistochemistry of adventitia and media ► immune complex, C3 complement, fibrinogen deposition ► fibrinoid necrosis

26. Gastrointestinal Vasculitis Gastrointestinal Vasculitis ► Lupus colitis  complications of vasculitis in the large intestine  Classification vasculitis (size of blood vessels, anatomical features) 1) multiple ulcers 2) ischemic colitis 3) protein losing enteropathy ► lymphedema, widespread capillary leakiness 4) intestinal pseudo-obstruction ► smooth muscle dysmotility (vasculitis, autoantibody) 5) others (cystic emphysema, Crohn’s dz, UC)

27. ► Management of intestinal vasculitis of SLE  antibiotics  high dose steroid  i.v. cyclophosphamide  oral mesalazine