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Nephrology conference. 報告人: R3 王劭瑜 指導老師:鄭昌錡醫師. Case presentation. A 46 years old male presented as bilateral legs pain for 2 weeks. Patient profile. Name︰ 楊 X 州 Age : 46-year-old Gender : male Height/Weight: 170cm /75 kg Occupation: Worker Marriage status: married - PowerPoint PPT Presentation

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  • Nephrology conference

    R3

  • Case presentationA 46 years old male presented as bilateral legs pain for 2 weeks

  • Patient profileNameXAge46-year-oldGendermaleHeight/Weight: 170cm /75 kgOccupation: WorkerMarriage status: marriedHospitalization2011/01/26~2011/04/16Chart number21418544

  • Chief complaintBilateral legs progressive pain for 2 weeks

  • Present illnessThis 46 years old male has underlying of Diabetes mellitus, type 2; Hypertension for 5 years => medications controlChronic kidney disease? Etiology?

  • Present illnessBilateral legs progressive pain for 2 weeks => Rehab clinic and Chinese herb (pain killer?) Admission in H (2011/01/19) Acute kidney injury => Hemodialysis twice due to hyperkalemia and acute pulmonary edemaKidney echo: bilateral large renal hypertrophy with mild right hydronephrosisDyspnea persistent refractory to emergent hemodialysis, with sudden onset of hemoptysis on 1/25 & 1/26

  • Present illnessAssociated symptoms: Decreased urine outputProgressive edema Poor appetite Chest pain over left chestNegative findings: Recently surgical procedures/trauma/immobilizationFever/chills/Purulent cough Rhinorrhea/sorethroat

  • Past historyDiabetes mellitus, type 2, for 5 yearsHypertension for 5 yearsOperation history: Traffic accident 20 years agoLeft leg fracture status post interlocking nailDenied major systemic disease as coronary artery disease, asthma, hepatitis B/C, peptic ulcer diseaseMedications: DM, HTN, Pain controller?

  • Personal historyAllergy: no known allergyAlcohol: DeniedSmoking: 0.5PPD for 10+ yearsBetalnut: Denied

  • Family HistoryUnknown cancer historyDiabetes mellitus Heart disease

  • Physical examinationBT:36.5, PP:78/min, RR:18/min, BP:201/107mmHgAppearance: alert, ill-looking, E4V5M6Sclera: not icteric; Conjunctiva: paleNeck: no stiffness, no lymphadenopathyChest: bilateral diffuse crackles, wheezing, ronchiHeart: regular heart beat without audible murmursAbdomen: soft and flat, no tenderness, normoactiveExtremities: free movable, grade 2 pitting edema pulsation: symmetric

  • H/D on 1/24, 1/25

  • Arterial blood gas2011/01/28Temp37pH7.301PCO2mmHg33.1PaO2mmHg72.6HCO3mm/L16.0SBEmm/L-10.4Sat%93.3

  • 2011/01/26Marked interstitial/airspace infiltration of both lungs, with diffuse patchy opacities. Favor ongoing inflammationTortuous thoracic aorta and borderline cardiomegaly

  • ImpressionRapidly-progressing glomerulonephritisHemoptysis, suspect pulmonary hemorrhageBilateral lower legs pain, cause to be determinatedMicrocytic anemia

    Diabetes mellitus, type 2, HbA1c=6.6Hypertension

  • Rapidly-progressing glomerulonephritis

    2011/01/26ANANegativeC3mg/dL110 (90~180)C4mg/dL17.8 (10~40)A-DSDNAWHOunit/mL

  • Clinical courseHydrocortisone 100mg q8h Heparin

  • Clinical courseHydrocortisone 100mg q8h Heparin

  • Clinical courseHydrocortisone => PrednisoloneHeparin => WarfarinChannel ulcer bleeding (pyloric ring, GC site) PES failure => operation on 3/17Discharge with OPD follow up on 4/16

    1/264/5c-ANCA>400 u/ml29.55 u/mlp-ANCAnegativenegative

  • DiagnosisC-ANCA associated vasculitisDeep vein thrombosisChannel ulcer bleeding, status post endoscope and operationDiabetes mellitus, type 2, HbA1c=6.6Hypertension

  • Discussion

  • OutlinePulmonary-renal syndromeANCA-associated VasculitidesWegener's granulomatosisDiagnostic procedureCase correlation

  • Pulmonary Renal Syndrome1919, Ernest Goodpasture: A case of pulmonary hemorrhage and glomerulonephritis1955, Parkin: Lung hemorrhage and nephritis, absence of arteritis1958, Stanton and Tang: Pulmonary hemorrhage with glomerulonephritis1950s, Krakower and Greenspun: Identified GBM as the antigen1967, Lerner, Glassock, and Dixon: Anti-GBM antibodies => Pathogenesis

  • Pulmonary Renal SyndromeAnti-GBM antibody disease (Goodpasture)Systemic vasculitis: Wegener's granulomatosisAcute glomerulonephritis: Complicated with pulmonary edemaUremia-induced increase in pulmonary capillary permeability Pulmonary infection

    1 Disease or 2 Diagnosis

  • Specks U. Diffuse alveolar hemorrhage syndromes CURR OPIN RHEUMATOL 2001;13:12-17

  • Pulmonary Renal Syndrome

    The remaining patients had a variety of disorders including pulmonary emboli, infection, and lupusNiles JL, Bttinger EP, Saurina GR, et al. The syndrome of lung hemorrhage and nephritis is usually an ANCA-associated condition Arch Intern Med 1996; 156:440

    ANCA48: pauci-immune glomerulonephritis with pulmonary symptoms8: good evidence for Wegener's granulomatosisAnti-GBM Ab6Both7

  • ANCA-associated Vasculitides

  • ClassificationsPrimary:Immune complex mediated ANCASecondary:Connective tissue disordersViral infection

    Large-vessel Vasculitis Giant cell (temporal) arteritis Takayasu arteritisMedium-sized-vessel Vasculitis Polyarteritis nodosa Kawasaki diseaseSmall-vessel Vasculitis Wegeners granulomatosis Churg-Strauss syndrome Microscopic polyangiitis Henoch-Schnlein purpura Essential cryoglobulinemic vasculitis Cutaneous leukocytoclastic angiitis

  • Diagnostic criteriaThe American College of Rheumatology (ACR) Vessel size, Histopathology, Clinical symptoms study criteria rather than diagnostic criteriaChapel Hill Consensus Conference (CHCC) Definitions, but not Diagnostic criteriaEuropean Medicines Agency algorithm: Watts et al. 2007 ANCA: permit the diagnosis of WG in the absence of biopsy

  • CSS criteriaWG criteriaPrognositc significanceResponse to therapyPorpensity for relapsePatient outcome

  • Churg-Strauss syndromeAsthma (a history of wheezing or the finding of diffus high pitched wheezes on expiration)Eosniophilia of > 10 %Mononeuropathy (including multiplex) or polyneuropathyMiugratory or transient pulmonary opacities detected radiographicallyParanasal sinus abnormalityBiopsy containing a blood vessel showing the accumulation of eosinophils in extravascular areas

  • Wegeners criteria Nasal or oral inflammation (painful or painless oral ulcers or purulent or bloody nasal discharge)Abnormal chest radiograph showing nodules, fixed infiltrates, or cavitiesAbnormal urinary sediment (microscopic hematuria or red cell casts)Granulomatous inflammation on biopsy of an artery or perivascular area> 2 / 4 => Sensitivity: 88%; Specificity: 92%

  • PathogenesisAbbreviations: CD, cluster of differentiation; CTLA4, tyotoxic T-lymphocyte antigen4 gene; PTPN22, protein tyrosine phosphagase, non-receptor type 22 gene

  • ANCAs in the pathogenesis of AAVNeonate glomerulonephritis and pulmonary hemorrhage: Transplacental of ANCA IgG from the mother who had anti-MPO-antibody-positive MPAANCAs: serological markerscANCA: proteinase-3 (PR3) / cytoplasmicpANCA: myeloperoxidase (MPO) / perinuclear

  • PathogenesisAbbreviations: CD, cluster of differentiation; CTLA4, tyotoxic T-lymphocyte antigen4 gene; PTPN22, protein tyrosine phosphagase, non-receptor type 22 gene

  • PathogenesisAbbreviations: CD, cluster of differentiation; CTLA4, tyotoxic T-lymphocyte antigen4 gene; PTPN22, protein tyrosine phosphagase, non-receptor type 22 gene Caucasians African-Americans German Netherlands Japan

  • PathogenesisAbbreviations: CD, cluster of differentiation; CTLA4, tyotoxic T-lymphocyte antigen4 gene; PTPN22, protein tyrosine phosphagase, non-receptor type 22 geneRespiratory tract: Silica exposure Accdelerated apoptosis of PolyMorphoNuclear and Macrophages: a trigger in the development of AAV

  • PathogenesisAbbreviations: CD, cluster of differentiation; CTLA4, tyotoxic T-lymphocyte antigen4 gene; PTPN22, protein tyrosine phosphagase, non-receptor type 22 geneStaphylococcus aureus: strongest association Superantigens from S. aureus: stimulate B & T cells, leading to AAV Directly prime neutrophils => membrance expression of PR3 Gram-negative bacteria: E. coli & K. pneumoniae LAMP-2 antibiotis: homologous to the bacterial adhesion protein FimH (Lysosomal membrane protein 2)

  • PathogenesisAbbreviations: CD, cluster of differentiation; CTLA4, tyotoxic T-lymphocyte antigen4 gene; PTPN22, protein tyrosine phosphagase, non-receptor type 22 geneAnti-Plasminogen antibodies correlates with both venous thromboembolic events and with characteristic glomerular histologic lesions and reduced renal function

  • Granulomatosis with polyangiitis (Wegeners granulomatosis)BackgroundClinical manifestationsDiagnosisTreatment

  • Background1897, Peter McBride: the first written description1931, Klinger: 70-year-old physician with constitutional symptoms, joint symptoms, proptosis, widespread upper respiratory tract inflammation leading to saddle nose deformity, glomerulonephritis and pulmonary lesions1936, Dr. Frederich Wegener: Distinct clinical and histopathologic findings1954, Goodman and Churg: triadSystemic necrotizing angiitisNecrotizing granulomatous inflammation of the respiratory tractNecrotizing glomerulonephritis2011: Granulomatosis with polyangiitis (Wegeners)

  • Epidemiology Frequency: rare disease, indeterminate incidence Prevalence in United States: 3/100,000Mortality: disease severity, intensity of TxUntreated: mean survival=5months 1-year mortality rate: 11% (2.2~25%) 5-year survival rate: 74~79%Cause of death: infection, respiratroy & renal failure, malignancy and cardiovascular eventsMorbidity: currently treatment relatedRace: White individualsSex: European populations male-to-female=1.5:1Age: occur at any age, typically 35~55 years oldPhillip R, Lu