Case Presentation 류마티스 내과 R2 강경환

Chief Complaint Present Illness 정O운 F/18 ADM on 09-02-20 Chief Complaint both calf pain onset : 내원 6주전 Present Illness 내원 6주 전부터 both calf pain 및 both eye ball pain, sclera injection 있어 2주 전부터 한방병원 입원 치료 받던 환자로, 종아리 근육통 지속되고 양 하지의 피부병변 및 소변검사 이상소견 보여 류마티스 내과로 전과됨.

DM / HTN / Tb / Hepatitis ( - / - / - / - ) Past medical history DM / HTN / Tb / Hepatitis ( - / - / - / - ) Op Hx(-) Personal history Smoking (-) Alcohol (-) Family history No specific

Review of System General fatigue (-) febrile sense (-) weight change (-) Skin purplish mottling (+) itching (-) bruise (-) Head & Neck headache (-) dizziness (-) Eye & ENT visual disturbance (-) tinnitus (-) both eye ball pain (+) Respiratory cough (-) sputum (-) dyspnea (-) tachypnea (-) Cardiac chest pain (-) palpitation (-) orthopnea (-) Purpuric rash가 있다고 해서 받기는 했는데 실제로 보였던 피부 병변이 purpura 보다는 livedo reticularis에 가까워서 rash -> mottling으로 수정 Eyeball pain에 대해서는 시린 느낌인지 쓰린 느낌인지 눈 안이 아픈지 밖이 아픈지 통증인지 묘사를 ..

Review of System Gastrointestinal A/N/V/D/C (-/-/-/+/-) hematemesis (-) melena (-) hematochezia (-) abdominal pain (+) Urinary dysuria (-) frequency (+) nocturia (-) both flank pain (+) residual urine sense (-) Gynecologic amenorrhea(+): for 2 month vaginal discharge (-) Musculoskeletal myalgia(+): both calf region Neurologic syncope (-) dizziness (-) numbness (+) 배아프단 애기도 했을거에요

Physical Examination Vital sign 125/77 – 64회/min – 20회/min – 36.7°c General alert consciousness not-so-ill looking appearance Skin livedo reticularis (+) :under knee no pigmentation Head & Neck no neck vein engorgement no neck LN enlargement Eye & ENT isocoric pupil with PLR (++/++) pinkish conjunctiva both sclera injection (+) PI (-) PTH (-/-) Purpuric rash가 있다고 해서 받기는 했는데 실제로 보였던 피부 병변이 purpura 보다는 livedo reticularis에 가까워서 수정 -> 사진 넣어주세요 눈도 찍은 사진이 있을거에요 옆에 넣어주세요 그리고 fever 있었는지 다시 확인해주삼

Physical Examination Thorax clear breathing sound without crackle or wheezing regular heart beat without murmur Abdomen soft and flat abdomen normoactive bowel sound no tenderness / rebound tenderness no palpable mass no abdominal bruit no splenomegaly no hepatomegaly Back & Extremities CVA tenderness (+/+) pretibial pitting edema (-/-)

Initial Lab Findings CBC/DC Chemistry 7850/mm² - 8.4 g/dl – 25.8% - 543K (seg 74.4%) MCV:80.8fl MCHC:32.6% PT (INR) 1.16 aPTT 47.2 Chemistry TB/DB 0.24/0.10 mg/dL AST/ALT 14/6 IU/L ALP/GGT 45/21 IU/L Prot/Alb 7.3/3.6 g/dL BUN/Cr 4/0.6 mg/dL LD/CK 356/28U/L Na/K/Cl 140/3.1/99 mmol/L CRP 9.0mg/dL Ca/P/Mg 7.0/2.9/2.1 mg/dL ESR 67mm/hr T-chol/uric acid 135/2.4 mg/dL ESR , CRP 넣어주시고 MCV도 했을테니 넣어주세요 UA에서 nitrate (-), moderate bacteriauria 소견도 중요

Initial Lab Findings U/A Micro Occult Blood (+++) Protein (++) Glucose (-) Leukocytes Nitrite(-) Micro RBC many/HPF WBC 10-29/HPF ESR , CRP 넣어주시고 MCV도 했을테니 넣어주세요 UA에서 nitrate (-), moderate bacteriauria 소견도 중요

Chest x-ray

ECG 이건 바꿀거죠?

Problem list 1. Both calf pain with numbness 2. Livedo reticularis, both lower extremities 3. Both eye sclera injection 4. Hematuria with proteinuria 5. Anemia Prolblem list를 먼저 만들고 assess도 처음에는 neuropathy도 vascultitis의 증상으로 보았었고 Hypothyroid myopathy 는 TFT 했다 우연히 발견된 거라서 처음 workup한 순서대로 가는 게 좋을 것 같아요 배아프단 이야기도 했는데 좀 nonspecific해서 problem list에 넣어야하나 말아야 하나 고민, 우선 빼고 내일 이야기 해보죠

Initial assessment 1. Myopathy 2. Livedo reticularis r/o nonspecific skin change 3. Scleritis r/o episcleritis 4. Glomerulonephritis 5. Iron deficiency anemia 1~4. Vasculitis with muscle, skin, eye and renal involvement

Plans 1~4. Serologic test ANCA, ENA profile, anti-GBM Ab, anti-dsDNA C3/C4, ANA, ASO titer, viral marker CBC, ESR, CRP f/u 1. Myopathy Muscle enzyme, EMG & NCS Bone scan, TFT 2. Livedo reticularis Skin biopsy

Plans 3. Scleritis r/o episcleritis ophthalmologic evaluation 4. Glomerulonephritis 24hr urine collection, urine MCV urine RBC morphology, kidney US, renal biopsy 5. Anemia s-iron,TIBC, ferritin, PBS, folate,vit B12, reticulocyte count + gastroscopy, colonoscopy

Muscle enzyme Muscle enzyme Anti-Jo-1 negative Anti-RNP negative ANA negative Normal range AD#1 AD#6 LD 218~472 U/L 356 U/L 342 U/L CK 20~220 U/L 38 U/L 21 U/L Aldolase ~7.6 U/L 4.1 U/L

EMG

NCS

NCS

NCS

Bone scan

Thyroid function test TFT fT4: 0.58 ng/dl T3: 72 ng/dl TSH: 54 μU/dl Thyroid Ab thyroglobulin Ab 26.9 U/ml TSH receptor Ab 11.0 % TPO Ab 1118 U/ml

Thyroid US Hypothyroidism d/t Hashimoto thyroiditis Thyroid gland의 echo가 decreased되어있고 heterogeneity를 보임. Definite nodule or cyst보이지 않음. Thyroid gland의volume이 정상또는 약간 감소된 것으로 추정됨. Vascularity증가의 소견없음. Enlarged cervical LN보이지 않고 작은 size의 LN만 양쪽 upper neck에서 몇개 보임. Submandibular and parotid gland에 특이소견없음. Suggesting thyroiditis without nodule or cyst within thyroid gland. No evidence of enlarged cervical LN along cervical lymphatic chain. Hypothyroidism d/t Hashimoto thyroiditis

2. Livedo reticularis 3. Nodular episcleritis

Urine study 24hr urine collection Urine MCV 77.5fl Dysmorphic RBC 65% 24hr urine protein 1077 mg /day 1473 mg/g 24hr urine creatinine 731 mg/day CrCl 85 ml/min Urine MCV 77.5fl Dysmorphic RBC 65% Urine culture no growth

Serology HbsAg (-) anti-HCV (-) Viral marker ANA negative anti-HIV (-) VDRL (-) ANA negative ENA profile all negative anti-dsDNA 6.3 IU/ml RA factor <10 IU/mL anti CCP <25 units/mL

Serology IgG 2060mg/dL (694~1618mg/dL) IgA 265mg/dL (68~378mg/dL) IgM 97mg/dL (60~263mg/dL) IgE 474IU/mL (~100IU/mL) P-ANCA (+) (~150AAU) C-ANCA (-) (MPO Ab(+):413AAU) (Pr III Ab (-):119AAU) C3/C4 115/24mg/dl Anti GBM Ab (-) ASO titer 146mg/dL

PNS series

Kidney US

LM X 400 HE

Focal necrotizing glomerulonephritis

IF X400,IgA

EM X3500

p-ANCA associated GN with mesangial IgA deposit

Anemia study S-iron 9 TIBC 150 Ferritin 148.1 ng/ml Peripheral BS normocyti normochromic Gastroscopy mild erythematous gastritis Colonoscopy no specific abnormal finding Iron deficiency anemia combined with anemia of chronic disease

Final Diagnosis #3. Iron deficiency anemia combined with #1. Hypothyroid myopathy d/t Hashimoto thyroiditis #2. Microscopic polyangiitis Livedo reticularis Nodular episcleritis Focal necrotizing GN p-ANCA (+) #3. Iron deficiency anemia combined with anemia of chronic disease

Clinical course-I Synthyroxine 50 10 9.4 (g/dl) 40 8.4 8.5 8 7.8 30 5 20 (μU/dl) Myalgia 증상 호전 10 AD#3 AD#5 AD#7 AD#9 AD#11 AD#13 AD#15 Feroba 50mcg Synthyroxine

Clinical course-II PDL MPD 10.9 9.0 100 8.3 10 Sclera injection, Livedo reticularis Sx 호전 50 5 (mm/hr) 1.7 0.5 (mg/dL) AD#15 AD#5 AD#7 AD#9 AD#11 AD#13 PDL MPD 10mg 30mg 500mg U/A 상 protein(-)