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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

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Nephrology conference

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  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)

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