1 / 72

Nephrology conference

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

miracle
Download Presentation

Nephrology conference

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript

  1. Nephrology conference 報告人：R3 王劭瑜 指導老師：鄭昌錡醫師

  2. Case presentation A 46 years old male presented as bilateral legs pain for 2 weeks

  3. Patient profile • Name︰楊X州 • Age：46-year-old • Gender：male • Height/Weight: 170cm /75 kg • Occupation: Worker • Marriage status: married • Hospitalization：2011/01/26~2011/04/16 • Chart number：21418544

  4. Chief complaint • Bilateral legs progressive pain for 2 weeks

  5. Present illness • This 46 years old male has underlying of • Diabetes mellitus, type 2; Hypertension for 5 years=> medications control • Chronic kidney disease? Etiology?

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

  7. Present illness • Associated symptoms: • Decreased urine output • Progressive edema • Poor appetite • Chest pain over left chest • Negative findings: • Recently surgical procedures/trauma/immobilization • Fever/chills/Purulent coughRhinorrhea/sorethroat

  8. Past history • Diabetes mellitus, type 2, for 5 years • Hypertension for 5 years • Operation history: Traffic accident 20 years ago • Left leg fracture status post interlocking nail • Denied major systemic disease as coronary artery disease, asthma, hepatitis B/C, peptic ulcer disease • Medications: DM, HTN, Pain controller?

  9. Personal history • Allergy: no known allergy • Alcohol: Denied • Smoking: 0.5PPD for 10+ years • Betalnut: Denied

  10. Family History Unknown cancer history Diabetes mellitusHeart disease

  11. Physical examination • BT:36.5, PP:78/min, RR:18/min, BP:201/107mmHg • Appearance: alert, ill-looking, E4V5M6 • Sclera: not icteric; Conjunctiva: pale • Neck: no stiffness, no lymphadenopathy • Chest: bilateral diffuse crackles, wheezing, ronchi • Heart: regular heart beat without audible murmurs • Abdomen: soft and flat, no tenderness, normoactive • Extremities: free movable, grade 2 pitting edema pulsation: symmetric

  12. H/D on 1/24, 1/25

  13. 2011/01/26 Marked interstitial/airspace infiltration of both lungs, with diffuse patchy opacities. Favor ongoing inflammation Tortuous thoracic aorta and borderline cardiomegaly

  14. Impression • Rapidly-progressing glomerulonephritis • Hemoptysis, suspect pulmonary hemorrhage • Bilateral lower legs pain, cause to be determinated • Microcytic anemia • Diabetes mellitus, type 2, HbA1c=6.6 • Hypertension

  15. Rapidly-progressing glomerulonephritis

  16. Clinical course Hydrocortisone 100mg q8h Heparin

  17. Clinical course Hydrocortisone 100mg q8h Heparin

  18. Clinical course • Hydrocortisone => Prednisolone • Heparin => Warfarin • Channel ulcer bleeding (pyloric ring, GC site)PES failure => operation on 3/17 • Discharge with OPD follow up on 4/16

  19. Diagnosis • C-ANCA associated vasculitis • Deep vein thrombosis • Channel ulcer bleeding, status post endoscope and operation • Diabetes mellitus, type 2, HbA1c=6.6 • Hypertension

  20. Discussion

  21. Outline Pulmonary-renal syndrome ANCA-associated Vasculitides Wegener's granulomatosis Diagnostic procedure Case correlation

  22. Pulmonary Renal Syndrome • 1919, Ernest Goodpasture: A case of pulmonary hemorrhage and glomerulonephritis • 1955, Parkin:Lung hemorrhage and nephritis, absence of arteritis • 1958, Stanton and Tang: Pulmonary hemorrhage with glomerulonephritis • 1950s, Krakower and Greenspun: Identified GBM as the antigen • 1967, Lerner, Glassock, and Dixon: Anti-GBM antibodies => Pathogenesis

  23. Pulmonary Renal Syndrome • Anti-GBM antibody disease (Goodpasture) • Systemic vasculitis: Wegener's granulomatosis • Acute glomerulonephritis: • Complicated with pulmonary edema • Uremia-induced increase in pulmonary capillary permeability • Pulmonary infection 1 Disease or 2 Diagnosis

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

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

  26. ANCA-associated Vasculitides

  27. Classifications • Primary: • Immune complex mediated • ANCA • Secondary: • Connective tissue disorders • Viral infection

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

  29. Proteinase 3 ANCA disease Myeloperoxidase ANCA disease Seronegative ANCA disease • CSS criteria • WG criteria Prognositc significance Response to therapy Porpensity for relapse Patient outcome

  30. Churg-Strauss syndrome • Asthma (a history of wheezing or the finding of diffus high pitched wheezes on expiration) • Eosniophilia of > 10 % • Mononeuropathy (including multiplex) or polyneuropathy • Miugratory or transient pulmonary opacities detected radiographically • Paranasal sinus abnormality • Biopsy containing a blood vessel showing the accumulation of eosinophils in extravascular areas

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

  32. Pathogenesis Abbreviations: CD, cluster of differentiation; CTLA4, tyotoxic T-lymphocyte antigen4 gene;PTPN22, protein tyrosine phosphagase, non-receptor type 22 gene

  33. ANCAs in the pathogenesis of AAV Neonate glomerulonephritis and pulmonary hemorrhage:Transplacental of ANCA IgG from the mother who had anti-MPO-antibody-positive MPA ANCAs: serological markers cANCA: proteinase-3 (PR3) / cytoplasmic pANCA: myeloperoxidase (MPO) / perinuclear

  34. Pathogenesis Abbreviations: CD, cluster of differentiation; CTLA4, tyotoxic T-lymphocyte antigen4 gene;PTPN22, protein tyrosine phosphagase, non-receptor type 22 gene

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

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

  37. Pathogenesis Staphylococcus aureus: strongest association Superantigens from S. aureus: stimulate B & T cells, leading to AAV Directly prime neutrophils => membrance expression of PR3Gram-negative bacteria: E. coli & K. pneumoniae LAMP-2 antibiotis: homologous to the bacterial adhesion protein FimH (Lysosomal membrane protein 2) Abbreviations: CD, cluster of differentiation; CTLA4, tyotoxic T-lymphocyte antigen4 gene;PTPN22, protein tyrosine phosphagase, non-receptor type 22 gene

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

  39. Granulomatosis with polyangiitis (Wegener’s granulomatosis) Background Clinical manifestations Diagnosis Treatment

  40. Background • 1897, Peter McBride: the first written description • 1931, Klinger: 70-year-old physician with constitutional symptoms, joint symptoms, proptosis, widespread upper respiratory tract inflammation leading to saddle nose deformity, glomerulonephritis and pulmonary lesions • 1936, Dr. Frederich Wegener: Distinct clinical and histopathologic findings • 1954, Goodman and Churg: triad • Systemic necrotizing angiitis • Necrotizing granulomatous inflammation of the respiratory tract • Necrotizing glomerulonephritis • 2011: Granulomatosis with polyangiitis (Wegener’s)

  41. Epidemiology • Frequency: rare disease, indeterminate incidencePrevalence in United States: 3/100,000 • Mortality: disease severity, intensity of Tx • Untreated: mean survival=5months1-year mortality rate: 11% (2.2~25%)5-year survival rate: 74~79% • Cause of death: infection, respiratroy & renal failure, malignancy and cardiovascular events • Morbidity: currently treatment related • Race: White individuals • Sex: European populations male-to-female=1.5:1 • Age: occur at any age, typically 35~55 years old Phillip R, Luqmani R. Mortality in systemic vasculitis: a systematic review. Clin Exp Rheumatol. September-October 2008;26:S94-S104

  42. Clinical presentation • Constitutional symptoms: fever, migratory arthralgias, malaise, anorexia and weight loss • Prodromal symptoms: weeks to months without specific organ involvement • Ear, Nose and Throat • Pulmonary • Renal • Cutaneous

  43. ENT • Nasal crusting, sinusitis, otitis media, persistent rhinorrhea, oral or nasal ulcers, purulent/bloody nasal discharge, polychondritis • Saddle nose deformitymore typically in WG

  44. Pulmonary disease • Airways and/or Pulmonary parenchyma: hoarseness, cough, dyspnea, stridor, wheezing, hemoptysis or pleuritic pain; tracheal or subglottic stenosis, pulmonary consolidation • Nodules and patchy or diffuse opacities • Pulmonary fibrosis and pulmonary hypertension • Tumor-like masses, extrathoracic • Breast • Kidney

  45. Renal disease • Acute kidney injury with hematuria, red cell and other casts, and proteinuria • Classifications and outcome: • Focal: > 50% of glomeruli are normal • Crescentic: cellular crescents • Sclerotic: > 50% of glomeruli are globally sclerotic • Mixed: normal; crescentic; globally sclerotic

  46. Renal biopsy • Segmental necrotizing glomerulonephritis • GPA: Granulomatous changes • Almost all Pauci-immune crescentic glomerulonephritis are ANCA positive

  47. ANCA-negative pauci-immune GN • As part of spectrum of WG and MPAsimilar renal biopsy findings and prognosis • Significnatly younger • Significantly higher rate of proteinuria • Significantly lower rate of renal survival and of extrarenal manifestations (pulmonary)

More Related