허리통증을 주소로 내원한 17세 남자 소화기내과 R1 최윤영/ Prof. 김효종

주 호소 양측 둔부 통증 onset ) 내원 9개월 전 환자이름: 장O영(M/17) 병록번호: 12349258 입원일자: 2013.06.10 현 병력 M/17, 특이병력 없는 자로 약 9개월여 전부터 특별한 외상 없이 양측 둔부 통증 및 조조강직, 양 무릎 통증, 오른쪽 어깨 통증 등 다발성 관절통, 간헐적 설사 발생해 한의원에서 물 리 치료 받았으나 증상 호전 없어 이에 대한 추가 평가 및 진 단 위해 류마티스 내과 외래 경유 입원함.

과거력 사회력 가족력 Alcohol (-) Smoke (-) DM/HTN/Tb/Hepatitis (- /- /- /-) OPHx (-) 사회력 Alcohol (-) Smoke (-) 가족력

계통문진 General Febrile sensation(-) Chill(-) Fatigue(+) Sweating(-) Back & extremity Buttock pain(+/+) morning stiffness(+) Rt shoulder pain(+) Knee pain(+/+)  swelling(+), redness(+) Skin Rash(-) Itching(-) Jaundice(-) Cardiac Chest Pain(-) Palpitations(-) Respiratory Cough(-) Sputum(-) Dyspnea(-) Gastrointestinal Anorexia(-) Nausea(+) Vomiting(-) Diarrhea(+) Diarrhea : loose stool, 3-4회/day, 5~6months ago intermittently blood tinged Genitourinary Flank pain(-) Dysuria(-) Urgency(-) Nervous Dizziness(-) Sensory loss(-) Motor weakness(-)

신체검진 Vital sign 110/70 mmHg – 70 /min – 20 /min – 36.1 ℃ Height : 175 cm Weight : 57 kg BMI : 18.6 kg/m2 General Alert mentality Chronic-ill looking appearance Skin Rash (-) Striae (-) Pigmentation(-) Petechiae(-) Chest Clear breathing sound s rale Regular heart beat s murmur Abdomen Soft & flat abdomen Td/rTd (-/-) Hyperactive bowel sound Back&Ext. CVA Td (-/-) Pitting edema (-)

Initial lab finding CBC Chemistry Urine analysis 6710 /uL – 13.3 g/dL – 41.9 % – 342,000 /uL (seg 56.2%) PT / INR 13.0 sec / 1.00% aPTT 39.7 sec Chemistry TB 0.53 mg/dL BUN/ Cr 18/0.8 mg/dL Prot/ Alb 8.0 / 4.1 g/dL Ca/ P/ Mg 9.4 / 4.4 / 2.2 mg/dL ALP/r-GT 208/ 312 IU/L Na /K /Cl 141 / 4.2 / 103 mEq/L AST/ALT 30 / 34 IU/L ESR 63 mm/hr Uric acid 7.7 mg/dL CRP 2.56 mg/dL Urine analysis pH 6.0 S.G 1.018 Glucose - / uribilinogen 0.1 mg/dL / Ketone - / protein - RBC / WBC 2~4 / 0~1

Chest X-ray No active lung lesion.

Initial EKG HR 74 normal sinus rhythm

Initial Problems #1. LBP, morning stiffness, both buttock pain, Rt shoulder pain #2. Elevated ESR, CRP #3. Diarrhea #4. Elevated ALP, γGT r/o Ankylosing spondylitis Chronic diarrhea r/o Enteropathic arthritis Biliary obstructive disease r/o cholangitis early onset 이상의 증상 및 검사 소견을 바탕으로 다음의 problem list를 설정하였습니다.

r/o Ankylosing spondylitis Initial Problems #1. LBP, morning stiffness, both buttock pain, Rt shoulder pain #2. Elevated ESR, CRP r/o Ankylosing spondylitis Diagnostic plan> Pelvic AP, CT. if needed, pelvis MRI ANA, ANCA, RF, anti-CCP, IgG, A, M, E Check HLA-B27 Therapeutic plan> DMARD Immunosuppressants, if needed early onset 이상의 증상 및 검사 소견을 바탕으로 다음의 problem list를 설정하였습니다.

r/o Enteropathic arthritis Initial Problems #2. Elevated ESR, CRP #3. Diarrhea Chronic diarrhea r/o Enteropathic arthritis Diagnostic plan> Stool WBC, stool culture, stool parasite exam Sigmoidoscopy or colonoscopy Abdominal CT Therapeutic plan> Symptomatic care : anti-spasmotics apply Antibiotics apply, if needed Mesalazine, azathioprine if needed early onset 이상의 증상 및 검사 소견을 바탕으로 다음의 problem list를 설정하였습니다.

Biliary obstructive disease Initial Problems #4. Elevated ALP, r-GT Diagnostic plan> Check viral markers ERCP, MRCP, if needed Abdominal US or CT F/U LFT Therapeutic plan> Symptomatic care : Hepatotonics Antibiotics apply, if needed ERCP, if needed Biliary obstructive disease r/o cholangitis early onset 이상의 증상 및 검사 소견을 바탕으로 다음의 problem list를 설정하였습니다.

Clinical course

Pelvis AP(6/10) Ankylosing spondylolitis, involving S-I joint.

Pelvis CT (6/11) <PELVIS 3D CT> Ankylosing spondylitis R/O IBD related spondylopathy. a/w sacroiliitis, both(Modified New York criteria 4). a/w subchondral erosions, subchondral sclerosis, and joint space narrowing with nearly complete bony ankylosis. a/w drooping osteophyte, head-neck junction of both femurs. R/O Old avulsion injury(enthesophyte), right anterior superior iliac spine. Disc bulging, L4-S1.

Other lab finding(6/10) 2013.06.10 Results Reference C-ANCA Positive 2+ Negative P-ANCA Ig G 2030 mg/dL 694 ~ 1618 mg/dL Ig A 193 mg/dL 68 ~ 378 mg/dL Ig M 256 mg/dL 60 ~ 263 mg/dL Ig E 178 mg/dL ~ 100 mg/dL C3 147 mg/dL 88 ~ 201 mg/dL C4 54.4 mg/dL 16 ~ 47 mg/dL ANA (정성) Non-reactive ANA (정량) HLA-B27 Positive

Ankylosing spondylitis Clinical course Ankylosing spondylitis 6/10 #1. Both buttock pain Both knee pain Rt. Shoulder pain #2. Elevated CRP, ESR #3.Diarrhea #4. Elevated ALP, rGT

CFS(6/13) Observed up to terminal ileum and random biopsy was performed, (Bx-A, x3) Longitudinal ulcer was noted at the Cecum and biopsy was performed. (Bx-B, x4) Aphtous ulcers were noted at the cecum. Random biopsy was performed at the rectum. (Bx-C, x3) Otherwise normal. 1. Crohn's disease

Small Bowel CT (6/13) SI joint margin irregular Liver reveals midlly dilated duct including peripheral duct but no solid mass nor hydronephrosis. EHD and GB shows no gross dilatation. Multiple and small periaortic nodes less than 1.1 cm in long diameter are noted. Cecum and terminal ileum shows milt wall thickening. Pancreas, adrenal glands, both kidneys and spleen show no gross lesions. No ascites are noted. Others unremarkable ====== [Conclusion] ====== Mildly dilated IH duct for age may sugggest primary sclerosing cholangitis or clonorchiasis of liver. Mildly thickened cecum and terminal ileum suggestives of IBD such as Crohn's disease Rec;clinical matching and MRCP

Ankylosing spondylitis Clinical course 6/10 6/11 6/13 #1. Both buttock pain Both knee pain Rt. Shoulder pain #2. Elevated CRP, ESR #3.Diarrhea #4. Elevated ALP, rGT Ankylosing spondylitis Crohn’s disease 크론병의 진단과 함께 소화기 내과로의 전과를 하였습니다 환자 지속적인 ALP와 rGT 의 상승으로 인하여 MRCP 를 시행하게 되었습니다 IG 로 전과

Primary Sclerosing Cholangitis MRCP (6/19) Primary Sclerosing Cholangitis MRCP 상 좁아진 duct 사이로 segmental dilatation을 동반. 환자의 나이에 고려할 때 stricture 군데 군데 심하게 나타나며 Clinical information: Crohn's disease, R/O primary sclerosing cholangitis IHD에 primary, secondary confluence와 peripheral branchy까지 multiple focal stricture가 있음. 좁아진 duct 사이로 segmental dilatation을 동반함. CBD에는 stricture보이지 않음. GB, pancreas에 이상 소견 없음. ====== [Conclusion] ====== Suggestive of primary sclerosing cholangitis involving IHD.

Clinical course 6/10 6/19 6/27 7/10 7/31 8/21 9/25 10/10 MPD60mg 30mg Adalimumab Ankylosing spondylitis Mesalazine 3000mg Azathioprine 50mg Crohn’s disease UDCA 1200mg Primary Sclerosing cholangitis 약물자가중단 CDAI 291 278 206 327.1 56

Final diagnosis #1. Ankylosing spondylitis #2. Crohn’s disease #3. Primary sclerosing cholangitis