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

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

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

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

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(-)

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(-)

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

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

Laboratory findings (1) CBC - MCV 68.7(fl) MCH 21.5(pg) MCHC 31.3(g/Dl) - WBC (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 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%

Laboratory findings (2) Connective tissue disease lab - C mg/dL (Ref mg/dL) - C mg/dL (Ref mg/dL) - anti-ds DNA Ab 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

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

Skin manifestations

Chest X-ray

Abdominal X-ray flat upright

Abdomen pelvis CT

Colonoscopic findings (1)

Colonoscopic findings (2)

Pathologic findings

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

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

Hospital course FEVER SEIZURE ABD PAIN DIARRHEA

Brain MRI

3 weeks later F/U

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

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)

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