1. Case presentation Rheumatology R1 박현진

2. C.C C.C dysphagia onset) 2months ago dysphagia onset) 2months ago P. I. P. I. 7 년 전부터 비가오면 손바닥까지 파래지고, 손에 상처가 나면 잘 낫지 않는 증상으로 외부 병원 혈관외과 내원, vasodilator 복용중이던 환자로 2 개월 전 시작된 고기덩 어리 등을 삼킬 때 걸리는 느낌 있어 소화기 내과에서 상 부 위장관 내시경 시행, 역류성 식도염있었으며 신체 검 사상 전신성 경화증 의심되어 류마티스 내과 진료 의뢰 됨. 7 년 전부터 비가오면 손바닥까지 파래지고, 손에 상처가 나면 잘 낫지 않는 증상으로 외부 병원 혈관외과 내원, vasodilator 복용중이던 환자로 2 개월 전 시작된 고기덩 어리 등을 삼킬 때 걸리는 느낌 있어 소화기 내과에서 상 부 위장관 내시경 시행, 역류성 식도염있었으며 신체 검 사상 전신성 경화증 의심되어 류마티스 내과 진료 의뢰 됨. 00264265 조 O 호 M/49 Case presentation

3. PMHx. PMHx. DM/HTN/TB/Hepatitis(-/-/+/-) DM/HTN/TB/Hepatitis(-/-/+/-) TB : 20 대 때, medication 후 완치판정 TB : 20 대 때, medication 후 완치판정 Op. Hx. (-) Op. Hx. (-) Personal Hx. Personal Hx. Smoking (+) Smoking (+) 30 PYs (1pack/day * 30years) 30 PYs (1pack/day * 30years) Alcohol (-) Alcohol (-) 아크릴 용접 공장에서 12 년간 일함 아크릴 용접 공장에서 12 년간 일함

4. Review of Systems  G/A) fatigue (+) weight loss (-) : 1~2 년간 2kg wt.loss fever (-) chill (-) sweating (-) fever (-) chill (-) sweating (-)  Skin) Finger hardening(+) rash (-) itching (-) Bluish change of hand to cold Bluish change of hand to cold  H&N) headache (-) trauma (-) stiffness (+)  Eye/ ENT) visual disturbance (-) diplopia (-) hearing loss(-) vertigo(-) hearing loss(-) vertigo(-) nasal obstruction (-) dryness(-) nasal obstruction (-) dryness(-) soreness(-) hoarseness(-) soreness(-) hoarseness(-)  Respiratory) cough (+) sputum (+) : whitish dyspnea(-) hemoptysis(-) dyspnea(-) hemoptysis(-)  Cardiac) angina (-) palpitation (-) orthopnea(-) exercise limitation (-) orthopnea(-) exercise limitation (-)

5.  G-I) A/N/V/D/C(-/-/-/-/-) hematochezia (-) melena(-) hematochezia (-) melena(-) dysphagia(+): solid, progressive dysphagia(+): solid, progressive regurgitation(+) : postprandial, supine position regurgitation(+) : postprandial, supine position  Renal &Urinary) dysuria (-) hematuria (-) polyuria (-) incontinence (-) nocturia (-) incontinence (-) nocturia (-)  Musculoskeletal) myalgia (+) weakness (+) arthralgia(+) atrophy (-) joint pain (-) stiffness (-) atrophy (-) joint pain (-) stiffness (-)  Endocrine) weight change(-) polydipsia (-) polyuria(-)  Neurologic) syncope(-) seizures(-) dizziness(-)

6. Physical Examination Vital sign) 110/60mmHg – 62/min – 20/min – 36.7 ℃ General appearance General appearance Alert mentality Alert mentality Not so ill-looking appearance Not so ill-looking appearance Skin Skin Rash(-) Pigmentation(-) Jaundice(-) Rash(-) Pigmentation(-) Jaundice(-) Sclerotic finger tip, Puffy fingers Sclerotic finger tip, Puffy fingers Head and Neck Head and Neck Normocephaly Normocephaly Perioral wrinklings Perioral wrinklings LN enlargement(-/-) LN enlargement(-/-) Neck vein engorgement(-/-) Neck vein engorgement(-/-) Thyroid enlargement(-) Thyroid enlargement(-) Eyes and ENT Eyes and ENT Isocoric pupil with PLR(++/++), EOM : full Isocoric pupil with PLR(++/++), EOM : full Whitish conjunctiva, Pinkish sclera Whitish conjunctiva, Pinkish sclera

7. Chest Chest Coarse breathing sound with inspiratory crackles, Both. Coarse breathing sound with inspiratory crackles, Both. Regular heart beat without murmur Regular heart beat without murmur Abdomen Abdomen Soft and flat abdomen Soft and flat abdomen Normoactive bowel sound Normoactive bowel sound No abdominal tenderness, rebound tenderness No abdominal tenderness, rebound tenderness No palpable abdominal mass No palpable abdominal mass Back and extremities Back and extremities CVA tenderness(-/-) CVA tenderness(-/-) Peripheral pitting edema(-/-) Peripheral pitting edema(-/-) Neurology Neurology Motor : intact, Sensory : intact Motor : intact, Sensory : intact No pathologic reflexes No pathologic reflexes

10. Lt. Rt.

13. scleroderma The combination of skin induration plus one or more of the following clinical features Digital infarctions and or digital tip pitting Characteristic facial, lip, or hand telangiectasia Heartburn and/or dysphagia of new onset Dyspnea on exertion associated with interstitial pulmonary changes on radiography Pulmonary hypertension Diarrhea with malabsorption Acute onset of hypertension and renal insufficiency

14. Impression CREST syndrome CREST syndrome R/O combined scleroderma Plan CBC/DC, Chemistry CBC/DC, Chemistry Acute phase reactant Acute phase reactant Serologic test Serologic test Radiographic study of joint Radiographic study of joint EGD with Esophageal manometry EGD with Esophageal manometry PFT and HRCT PFT and HRCT Echocardiogrphy Echocardiogrphy

15. Laboratory finding CBC/DC CBC/DC 11100 /mm3 – 10.6g/dL – 31.7% - 277k (seg : 77.0%) 11100 /mm3 – 10.6g/dL – 31.7% - 277k (seg : 77.0%) Chemistry Chemistry Prot/Alb 6.2/3.5 g/dL Prot/Alb 6.2/3.5 g/dL AST/ALT 13/8 IU/L AST/ALT 13/8 IU/L ALP/GGT 139/40 IU/L ALP/GGT 139/40 IU/L TB/DB 0.3/0.1mg/dL TB/DB 0.3/0.1mg/dL Glucose 127 mg/dL Glucose 127 mg/dL BUN/Cr 9/0.8 mg/dL BUN/Cr 9/0.8 mg/dL Na/K/Cl 140/3.7/108 mmol/L Na/K/Cl 140/3.7/108 mmol/L Mg/Ca/P 2.0/8.9/3.6mg/dL Mg/Ca/P 2.0/8.9/3.6mg/dL T-Chol/TG/HDL/LDL 108/117/35/77 T-Chol/TG/HDL/LDL 108/117/35/77 ESR 22mm/hr CRP 1.26mg/dL RF 7IU/mL ESR 22mm/hr CRP 1.26mg/dL RF 7IU/mL Urinalysis Urinalysis Protein(-) Occult blood(-) Nitrate(-) Protein(-) Occult blood(-) Nitrate(-) Microscopy : WBC 0-1/HPF RBC 0-1/HPF Microscopy : WBC 0-1/HPF RBC 0-1/HPF MCV 85.0fL MCH 28.5pg MCHC 33.5g/dL

16. Serologic markers Serologic markers ANA (Titration) ANA (Titration) S1+(1:80) S1+(1:80) Nu1+(1:160) Nu1+(1:160) H1+(1:640) H1+(1:640) Anti-Scl-70 Ab Positive Anti-Scl-70 Ab Positive Anti-Centromere Ab (EIA) Negative Anti-Centromere Ab (EIA) Negative ANCA ( 정성 ) ANCA ( 정성 ) P ANCA (anti-MPO) Negative P ANCA (anti-MPO) Negative C ANCA (anti-PR3) Negative C ANCA (anti-PR3) Negative

17. Hand AP ( 07-4-23 ) Rt. Hand AP Lt. Hand AP

18. Rt. Hand Lt. hand

19. EGD (07-4-23)

20. Esophageal manometry

21. Chest PA (07-5-8)

22. Chest CT (07-4-26)

23. PFT (07-4-26) Spirometry Spirometry FVC 3.57L (84%) FVC 3.57L (84%) FEV1 2.67L (83%) FEV1 2.67L (83%) FEV1/FVC 75% FEV1/FVC 75% FEF25-75% 2.00L/sec(59%) FEF25-75% 2.00L/sec(59%) Diffusing Diffusing DLco 12.6mL/mmHg/min (68%) DLco 12.6mL/mmHg/min (68%) Lung volume Lung volume TLC 5.16L (88%) TLC 5.16L (88%) VC 3.57L (84%) VC 3.57L (84%) IC 1.35L (47%) IC 1.35L (47%) FRC 3.74L (99%) FRC 3.74L (99%) RV 1.59L (82%) RV 1.59L (82%) RV/TLC 31% RV/TLC 31% 0 4 8 -4 -8 Flow Vol -2 0 2 4 6

24. Echocardiography (07-5-7) 287cm/s Systolic PAP = RA Pr + 4* 2.87 2 ≈ 40mmHg

25. Diagnosis and problem list Scleroderma with Scleroderma with Dysphagia with Reflux esophagitis Dysphagia with Reflux esophagitis d/t esophageal motility disorder d/t esophageal motility disorder Emphysematous change Emphysematous change with Interstitial lung disease with Interstitial lung disease Mild pulmonary hypertension with TR Mild pulmonary hypertension with TR

26. Final diagnosis Systemic sclerosis, diffuse type Systemic sclerosis, diffuse type Therapeutic plan Disease activity control Disease activity control Symptomatic treatment Symptomatic treatment

27. Disease course Disease activity control Disease activity control Colchicine, NSAIDs, PDL Colchicine, NSAIDs, PDL Symptomatic treatment Symptomatic treatment 1. Sclerodactyly 1. Sclerodactyly colchicine, NSAIDs, PDL colchicine, NSAIDs, PDL 2. Interstitial lung disease 2. Interstitial lung disease MPD pulse therapy MPD pulse therapy 3. Reflux esophagitis combined esophgeal motility disorder 3. Reflux esophagitis combined esophgeal motility disorder Proton pump inhibitor Proton pump inhibitor Prokinetics Prokinetics 4. Mild pulmonary hypertension 4. Mild pulmonary hypertension Close observation Close observation