![[Active problems]
• Lt. neck lymphadenitis,
leukopenia, anemia
• Suspect SLE flare up
• Significant body weight loss
• Diarrhea and vomiting, cause
to be determined
Basic information │ History │ Examination │ Impression │ Clinical course
Impression
22
[Chronic problems]
• SLE with nephritis
• Hypothyroidism
• Hypertension
• Anemia
• Uterine myoma with
hypermenorrhea](https://image.slidesharecdn.com/acasereportofnephropathy-190126073037/85/A-case-report-of-nephropathy-22-320.jpg)
A case report of nephropathy
- 1. A Case Report of Nephropathy Presenter │ M5 柯皓禎 VS 馮祥華 醫師 JAN 25, 2019 Basic information │ History │ Examination │ Impression │ Clinical course
- 2. General Data: Name: Gender: Age: Marital status: Education: Occupation: Date of history taking: 李O君 Female 37 y/o Married High school Housewife 2018.11.13 BH: 165 cm, BW: 76.7 kg, BMI: 28.17 Basic information │ History │ Examination │ Impression │ Clinical course Chief Compliant: General weakness and fatigue for two weeks. 2
- 3. Drug: Food: Allergy history Basic information │ History │ Examination │ Impression │ Clinical course ─ Personal history TOCC Travel history: Occupation history: Contact history: Cluster history: Others Special environmental exposure: Pet animal history: History of venereal disease: Using behaviors Cigarette smoking: ‒ socially before since 20 + years old Alcohol consumption: denied Betel nut chewing: denied Denied Denied NKA 3
- 4. Others Other systemic disease: ‒ Systemic lupus erythematosus ‒ Hypothyroidism ‒ Iron-deficiency anemia ‒ Uterine myoma Operation history: ‒ Status post cesarean section ‒ Uterine myoma, post myomectomy ‒ Uterine polyps, post laparoscopic operation Immunization history: denied Underline disease DM: Denied HTN: under medical control Basic information │ History │ Examination │ Impression │ Clinical course ─ Past history 4 Family history
- 5. Basic information │ History │ Examination │ Impression │ Clinical course ─ Past history Drug history Amoxicillin/Clavulanate 1g 1 粒 BID Mefenamic acid 500mg 1 粒 TID Alginic acid 200mg 1 粒 TID Levocetirizine 5mg 1 粒 HS Hydroxypropyl cellulose 1 滴 QID Hydroxychloroquine Sul. 200mg 1 粒 BID Strocain (複方) 1 粒 BID Labetalol 200mg 1 粒 BID Thyroxine 100mcg(對Eltroxin過敏病人使用) 2 粒 QD Ferrous citrate sodium 50mg 1 粒 QD Prednisolone 5mg tab 1 粒 QD 5 近期用藥 長期用藥
- 6. • Body weight lost 20 kilograms5 months This is a 37 years old woman who had the underlying disease of systemic lupus erythematosus(2016), presented with pitting edema, skin rash, oral ulcer, joint pain and proteinuria, and was treated with Prednisolone 1 粒 QD Basic information │ History │ Examination │ Impression │ Clinical course ─ Present illness • URI ‒ Sore throat, rhinorrhea and hoarseness were complained • A enlarged and painful lymph node at left neck was noted ‒ Went to ENT outpatient clinic ‒ Neck CT showed lymph nodes up to about 2*2 cm ‒ Empiric antibiotics with Augmentin was given and the size of lymph node shrinked 2 weeks 6 • Had frequent nausea, vomiting and diarrhea • General malaise, weakness and dizziness developed 2 months 2018/10/29 • Fever up to 39'C
- 7. • Body weight lost 20 kilograms5 months This is a 37 years old woman who had the underlying disease of systemic lupus erythematosus, presented with pitting edema, skin rash, oral ulcer, joint pain and proteinuria, and was treated with Prednisolone 1 粒 QD Basic information │ History │ Examination │ Impression │ Clinical course ─ Present illness • Neck infection ‒ Sore throat, rhinorrhea and throat hoarse were complained • A enlarged and painful lymph node at left neck was noted ‒ Went to ENT outpatient clinic ‒ Neck CT showed lymph nodes up to about 2*2 cm ‒ Empiric antibiotics with Augmentin was given and the size of lymph node shrinked 2 weeks 7 • Had frequent nausea, vomiting and diarrhea • General malaise, weakness and dizziness developed 2 months 2018/10/29 • Fever up to 39'C Due to persist symptoms, she came to our ER department and was admitted on 2018/11/13 for further evaluation and treatment.
- 8. HEENT / Neck Palpable apical impulse at 5th intercostal space and left mid-clavicular line Heart Lung Abdominal Ext / Neuro Neck: lymphadenopathy(+) left neck level III, 1-2cm, movable, no tenderness Symmetric expansion Breathing sound: clear Soft and flat, Rebounding pain (-) Tenderness(-) Hepatomegaly (-), splenomegaly (-) Normal active bowel sound Skin rash over extrimity EXT: freely movable without pitting edema Consciousness: clear & oriented General appearance: fair GCS: E4V5M6 Vital sign BT：36.9℃, PR：78/min, RR：18/min, BP：117/71mmHg Basic information │ History │ Examination │ Impression │ Clinical course ─ Physical examination 8
- 9. Basic information │ History │ Examination │ Impression │ Clinical course ─ EKG 9
- 10. Basic information │ History │ Examination │ Impression │ Clinical course ─ CXR (PA view) 10 Clear bilateral costophrenic angle(s). Normal heart size. Prominent bilateral lung marking.
- 11. 2018/11/12 (CBC) Variable Reference Range On Admission Hb 12.0-16.0 7.5 Hct 36.0-46.0 22.8 RBC 4.2-5.4 3.12 RDW 11.5-14.5 16.7 WBC 4.0-10.0 2.48 Neutrophil Seg. 40.0-75.0 79.9 Lymphocyte 20.0-45.0 13.7 MCV 81.0-97.0 73.1 MCH 26.0-34.0 24.0 Plt 282 Basic information │ History │ Examination │ Impression │ Clinical course ─ Lab data 11 2018/11/12 (Plasma) Variable Reference Range On Admission Na 135-145 135 K 3.5-5.3 3.8 Glucose 70-140 108 BUN 8-25 23 GPT 5-35 14 LDH 95-215 106 Creatinine 0.44-1.00 0.80 GFR 80.7 APTT 23.9-35-5 23.6 CRP 0.010-0.500 0.990
- 12. 2018/11/14 (CBC) Variable Reference Range On Admission Hb 12.0-16.0 7.6 Hct 36.0-46.0 23.0 RBC 4.2-5.4 3.00 RDW 11.5-14.5 17.1 WBC 4.0-10.0 2.33 Neutrophil Seg. 40.0-75.0 78.1 Lymphocyte 20.0-45.0 13.7 MCV 81.0-97.0 76.7 MCH 26.0-34.0 25.3 Plt 329 Reticulocyte 1.28 Basic information │ History │ Examination │ Impression │ Clinical course ─ Lab data 12 2018/11/14 (Plasma) Variable Reference Range On Admission CD3(+)/WBC 8.80-27.40 8.85 CD14(+)/WBC 1.10-9.30 4.33 CD19(+)/WBC 0.20-7.00 1.08 CD4(+)/CD3(+) 44.40-66.80 45.38 CD8(+)/CD3(+) 23.40-49.20 50.29 CD25(+)/CD4(+) 13.50-20.80 5.59 DR(+)/CD4(+) 4.60-11.20 10.57 ESR 0-20 104 EB-VCA IgM Ab (-)0.09 CMV IgM Ab (-)0.32 TSH 0.25-4.00 0.76 Free T4 0.56-1.80 1.19
- 13. 2018/11/14 (Serum) Variable Reference Range On Admission IgA 82.0-453.0 445.0 IgM 46.0-304.0 58.2 IgG 751.0-1560.0 1830.0 C3 79.00-152.00 46.10 C4 16.00-38.00 13.70 Albumin 3.5 -8.5 3.2 A/G 1.2 -5.5 0.9 Ferritin 12-150 ng/mL 8.86 Fe 50-170 μg/dL 28 TIBC 260-445 μg/dl 279 Basic information │ History │ Examination │ Impression │ Clinical course ─ Lab data 13 2018/11/14 (Serum) Variable Reference Range On Admission Anti RNP Ab + Anti Sm Ab (+)124.9 Anti RNP Ab 44.40-66.80 131.6 Anti Ro(SSA) Ab 13.50-20.80 (-)13.1 Anti La(SSB) Ab 0-20 (-)3.6 ANA (+)5120 dDNA (+)1105 pANCA (+) cANCA (-)
- 14. Basic information │ History │ Examination │ Impression │ Clinical course ─ Lab data 14
- 15. Basic information │ History │ Examination │ Impression │ Clinical course ─ Lab data 15
- 16. Basic information │ History │ Examination │ Impression │ Clinical course ─ Lab data 16
- 17. Basic information │ History │ Examination │ Impression │ Clinical course ─ Lab data 17
- 18. Basic information │ History │ Examination │ Impression │ Clinical course ─ Lab data 18
- 19. Basic information │ History │ Examination │ Impression │ Clinical course ─ Lab data 19
- 20. Basic information │ History │ Examination │ Impression │ Clinical course ─ Lab data 20 2018/11/14 (Urine analysis) Variable Reference Range On Admission Urine Protein/Creatinine <150 3272.7 Microscopic RBC 0-3 Microscopic WBC 6-9 Ep.cell 0-5 Cast - Crystal - Cast Waxy cast: 0-2/LPF Bacteria 4+ 2018/11/14 (Urine analysis) Variable Reference Range On Admission Ketone Body - Sp.gr 1.020 OB +/- PH 6.0 Protein 100 mg/dL 2018/11/14 (Stool) Variable Reference Range On Admission OB 2+ WBC 0-5/HPF
- 21. Basic information │ History │ Examination │ Impression │ Clinical course ─ Lab data 21
- 22. [Active problems] • Lt. neck lymphadenitis, leukopenia, anemia • Suspect SLE flare up • Significant body weight loss • Diarrhea and vomiting, cause to be determined Basic information │ History │ Examination │ Impression │ Clinical course Impression 22 [Chronic problems] • SLE with nephritis • Hypothyroidism • Hypertension • Anemia • Uterine myoma with hypermenorrhea
- 23. Basic information │ History │ Examination │ Impression │ Clinical course Plan 23 1. Symptomatic management for GI symptoms 2. Increased steroid dose (solumedrol 0.5 amp BID) 3. Consult ENT for lymphadenopathy 4. Iron supplement
- 24. Basic information │ History │ Examination │ Impression │ Clinical course ─ TPR monitor 24
- 25. Basic information │ History │ Examination │ Impression │ Clinical course ─ TPR monitor 25
- 26. • Symptomatic treatment for gastrointestinal discomfort was given, and the symptoms of nausea, vomiting and diarrhea improved • Arranged neck echo for thyroid nodule survey, and ENT was consulted for left neck lymphadenopathy ‒ Multiple lymphadenopathy over left neck level II-III was noted ‒ Fine-needle aspiration was done, with some serous fluid drained ‒ Inadquate spacement with few lymphoid cell 2018/11/16 This is a 37 years old woman who had the underlying disease of systemic lupus erythematosus, hypothyroidism, hypertension, and iron-deficiency anemia under regular medical treatment. 26 Basic information │ History │ Examination │ Impression │ Clinical course ─ POR#
- 27. • Symptomatic treatment for gastrointestinal discomfort was given, and the symptoms of nausea, vomiting and diarrhea improved • Arranged thyroid scan for thyroid nodule survey, and ENT was consulted for left neck lymphadenopathy ‒ Bilateral thyroid nodules were noted, with macro- calcification in the rt. lobe one, favor benign process ‒ Multiple lymphadenopathy over left neck level II-III was noted ‒ Fine-needle aspiration was done, with some serous fluid drained ‒ Suspected necrotizing lymphadenitis, but lymphoma could not be ruled out 2018/11/16 This is a 37 years old woman who had the underlying disease of systemic lupus erythematosus, hypothyroidism, hypertension, and iron-deficiency anemia under regular medical treatment. 27 Basic information │ History │ Examination │ Impression │ Clinical course ─ POR#
- 28. 28 • Symptoms resolved (rash, neck pain, lymphedenopathy) • Tapper steroid to 16 mg BID • Consult nephrologist for proteinuria→kidney biopsy 2018/11/17 Urine analysis UPCR - Microscopic RBC 0-3 Microscopic WBC 6-9 Ep.cell 0-5 Cast - Crystal - Cast - Bacteria +/- Ketone Body - Sp.gr 1.010 OB 1+ PH 6.0 Protein 300 mg/dL Urobilinogen 0.2 Nitrite - Leukocyte - • Plans: ‒ Keep methylprednisolone 20mg bid ‒ Oral PPI therapy (EGD at other hospital: esophagitis) ‒ Pain control: ancogen 1# tid, ultracet prn ‒ IV ferric for iron-deficiency anemia ‒ Keep plaquenil 1# bid for SLE ‒ Keep thyoxine 100mcg 2# qd for hypothyroidism
- 29. 29 • No skin rash, no lower leg edema • Foamy urine • Lt neck LAP: less tender, decreasing size 2018/11/18 2018/11/19 • Mild pitting edema 1+ • Occasional dizziness, esp when changing posture to standing. CBC Hb 8.0 Ht 24.7 RBC 3.24 RDW 18.6 WBC 3.62 Neutrophil Seg. 77.7 Lymphocyte 14.6 MCV 76.2 MCH 24.7 Plasma ESR 60 PT 9.3 PT control 10.3 INR 0.90 APTT 21.6 APTT control 26.4 Na 136 K 4.4 Glucose BUN 23 GPT 16 LDH 106 Creatinine 0.61 eGFR 110.4
- 30. 30 2018/11/21 • Kidney echo was performed CBC Hb 7.7 Ht 24.0 RBC - RDW - WBC 6.11 Neutrophil Seg. 85.0 Lymphocyte 8.5 MCV - MCH - Urine analysis Microalbumin 599.9 Urine Alb/Cr 1333.1 Microscopic RBC 4-9 Microscopic WBC 20-29 Sp.gr 1.014 OB 2+ PH 6.0 Protein 100 mg/dL Urobilinogen 0.2 Nitrite - Leukocyte 1+
- 31. 31 2018/11/21 • Kidney echo was performed CBC Hb 7.7 Ht 24.0 RBC - RDW - WBC 6.11 Neutrophil Seg. 85.0 Lymphocyte 8.5 MCV - MCH - Urine analysis Microalbumin 599.9 Urine Alb/Cr 1333.1 Microscopic RBC 4-9 Microscopic WBC 20-29 Sp.gr 1.014 OB 2+ PH 6.0 Protein 100 mg/dL Urobilinogen 0.2 Nitrite - Leukocyte 1+ Bilateral increased kidney size with smooth outline and normal echogenicity
- 32. 32 Dx: Lupus nephritis • Diffuse membranous nephropathy • Focal proliferative glomerulonephritis Glomerular tissue • Segmental sclerosis: 10/31 • GBM changes: ‒ Rigid ‒ Thickening ‒ Double-contour • Proliferation: ‒ Segmental ‒ Endocapillary ‒ Mesangial • Necrosis: + • Inflammatory changes: + • Microangiopathy:-, (-) thrombosis • C3 • C4 • Kappa • Lambda Immunofluorescent • IgG • IgM • IgA • C1q Electron-dense material • (++) subepithelial areas • (+, some) subendothelial areas • (++) mesangial areas • (+) segmental expansion of mesangial matrix • (+) segmental proliferation of mesangial cells • (++) tubuloreticular inclusions in endothel ial cells • (+) mesangial interposition
- 33. 33 • Hard stool, less general arthritis2018/11/22 2018/11/21 • Kidney echo was performed CBC Hb 7.7 Ht 24.0 RBC - RDW - WBC 6.11 Neutrophil Seg. 85.0 Lymphocyte 8.5 MCV - MCH - Urine analysis Microalbumin 599.9 Urine Alb/Cr 1333.1 Microscopic RBC 4-9 Microscopic WBC 20-29 Sp.gr 1.014 OB 2+ PH 6.0 Protein 100 mg/dL Urobilinogen 0.2 Nitrite - Leukocyte 1+ Urine analysis Microscopic RBC 4-9 Microscopic WBC 6-9 Ep.cell 0-5 Ketone Body - Sp.gr <=1.005 OB 2+ PH 6.5 Protein 100 mg/dL Urobilinogen 0.2 Nitrite - Leukocyte +/- • No flank pain, no gross hematuria2018/11/23 Urine analysis Microscopic RBC 0-3 Microscopic WBC 20-29 Ep.cell 0-5 Ketone Body - Sp.gr 1.010 OB 2+ PH 6.5 Protein 100 mg/dL Urobilinogen 0.2 Nitrite - Leukocyte +/-
- 34. 34 Basic information │ History │ Examination │ Impression │ Clinical course Final Diagnosis 1.Bilateral neck lymphadenopathy ‒ ruled in necrotizing lymphadenitis or lymphoma 2.Significant body weight loss ‒ ruled in malignancy or autoimmune disease activity 3.Leukopenia ‒ suspected viral infection related, lupus disease activitiy, or azathioprine related 4.Systemic lupus erythematosus ‒ with nephritis
- 35. Discussion of Lupus Nephritis Presenter │ M5 柯皓禎 VS 馮祥華 醫師 JAN 25, 2019
- 36. 36 10:1: SLE+Severe Nephritis Gender ratio Children Male HLA Genotype HLA-B8 HLA-DR3 HLA-DR2 →Deficiencies in complement components C1q, C2, C4 At least 20 types of gene mutation can lead to increased risk of SLE Epidemiology │ Pathogenesis │ Classification │ Treatment
- 37. 37 Definition of SLE＋ Renal Involvement Pathogenesis Persist Proteinuria: 500 mg/dl/day ( >+3 ) Cellular urinary cast T cells • Polyclonal activation if B cells • Defective B cell tolerance • Tc, Ts↓ • Th↑ • T cell signal abnormal • Th1, Th2, Th17 cytokine prduction B cells Apoptosis • Failure of apoptotic mechanisms to delete autoreactive B cell and T cell clones Epidemiology │ Pathogenesis │ Classification │ Treatment
- 38. 38 Epidemiology │ Pathogenesis │ Classification │ Treatment
- 39. 39 Epidemiology │ Pathogenesis │ Classification │ Treatment
- 40. 40 又分成A (active)、A/C、C (chronic) LN Class III Active的特徵 • Cellular crescents • Fibrinoid necrosis • Pyknosis or karyorrhexis (核分裂) • Rupture of GBM • Hematoxylin bodies • Wire loop ( glomerular capillary 變厚 或 intraluminal mass: hyaline thrombi) Epidemiology │ Pathogenesis │ Classification │ Treatment
- 41. 41 Epidemiology │ Pathogenesis │ Classification │ Treatment
- 42. 42 • Immune deposits沈積在subepithelial，形成membranous pattern • 和Primary Membranous GN(MGN)區分，Primary MGN 同時存在 Mesangial deposits Mesangial hypercellularity • Glomerular capillary wall變厚 • 在Subepithelial 有Spike formation • 可能合併LN Class III, IV Epidemiology │ Pathogenesis │ Classification │ Treatment
- 43. 43 Epidemiology │ Pathogenesis │ Classification │ Treatment
- 44. 44 Epidemiology │ Pathogenesis │ Classification │ Treatment
- 45. 45 螢光染色：IgG普遍存在，IgM, IgA, C3, C1q常見，全都有即為 Full house 電顯：電顯下的沈積是dense, granular，有些呈指紋狀，平行的線條，直徑約 10-15 nm (Churg’s thumbprints)，也可能會有Tubuloreticular inclusions Epidemiology │ Pathogenesis │ Classification │ Treatment
- 46. 46 CLASS Treatment Class I, II 腎臟預後好，不需要針對LN特別治療 Class III Few mild proliferative lesion, no necrotizing feature, crescent預後好、 短時間高劑量corticosteroid反應好 Class IV Prevent irreversible kidney injury from develope to ESRD Initial regimens: corticosteroid、cyclophosphamide、 mycophenolate mofetil、cyclosporin、tacrolimus +- rituximab • IV cyclophosphamide>Pulse corticosteroids • severe LN standard Therapy：pulse IV methylprednisolone + pulse IV cyclophosphamide (or immune medication + short course oral cyclophosphamide, less SE) • KDIGO：MMF or cyclophosphamide→severe LN first line therapy ‒ High risk for poor renal outcome→MMF> IV cyclophosphamide Maintenance Therapy： • MMF or azathioprine>IV cyclophosphamide ‒ less CKD, motality↓, less toxin Class V (Membranous LN) MMF consider the First Line theray Steroid + Azathioprine: highly effective Epidemiology │ Pathogenesis │ Classification │ Treatment
- 47. 47 • 嚴重的LN進展到透析或換腎： 5~50% • 透析時間延長→ clinically active patients發生率也下降 • 因為LN導致ESRD，最初幾個月死亡率的增加是因為使用免疫抑制 劑引發感染死亡 • 不管PD or HD，死亡率和非LN的病人沒有差異，死亡原因主要都 是CV problem • Before transplantation： • 先透析3-12個月，讓clinical和serologic活性在換腎前降低 • Transplantaiton後 LN recurrent rate低(4%) • 如果LN病人有antiphospholipid Ab→給anticoagulation therapy Epidemiology │ Pathogenesis │ Classification │ Treatment
- 48. Thank You! Presenter │ M5 柯皓禎 VS 馮祥華 醫師 JAN 25, 2019