1 / 57

Marizen L. Lim, Md

Makati Medical Center Department of Medicine Medical Grandrounds July 12, 2007 8:15Am, Ledesma Hall. Marizen L. Lim, Md. Learning Objectives. To present a case of SLE with lupus nephrtis To present updates on the treatment of lupus nephritis. Idenifying Data. ER 43-y/0 female

jena
Download Presentation

Marizen L. Lim, Md

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. Makati Medical Center Department of Medicine Medical Grandrounds July 12, 2007 8:15Am, Ledesma Hall Marizen L. Lim, Md

  2. Learning Objectives • To present a case of SLE with lupus nephrtis • To present updates on the treatment of lupus nephritis

  3. Idenifying Data • ER • 43-y/0 female • Chief Complaint: Persistently elevated BP

  4. History of the Present Illness • 20 years PTA  malar rash  photosensitivity  polyarthralgia  3x spontaneous abortions • 1 month PTA  severe throbbing headache, vomiting, dizziness, dyspnea on exertion • BP: 200/100  consult  u/a: +3 alb, 60 rbc, hyaline & coarse granular casts; crea 0.9

  5. History of the Present Illness • Metoprolol 50mg OD & Felodipine 2.5mg OD • BP remained uncontrolled • Beta blocker  calcium channel blocker  ACE inhibitor  Angiotensin II receptor blocker • BP persistently elevated

  6. History of the Present Illness • 1 week PTA  progressive dyspnea, periorbital, facial, pitting bipedal edema , noted ↓ urine output • Consult  Leukopenia w/ thrombocytopenia; +3 albumin, 12 wbc, fine granular casts; crea 1.2 from 0.9; K 5.7 Nephrology Admitted

  7. Review of Systems • (-) fever • (+) headache • (+) nausea • (+) body malaise • (+) anorexia • (-) easy bruisability • (-) chest pain/palpitation • (-) pleurisy • (-) abdominal pain • (-) dysuria • (-) depression/mania

  8. Past Medical, Social and Personal, Family History • (-) DM • (-) BA • (-) allergies • Non smoker • Non alcoholic beverage drinker • Officer worker • (+) HPN • (-) DM • (-) connective tissue disease

  9. Physical Examination • General Survey: conscious, coherent, oriented, not in cardiorespiratory distress • Vital Signs: BP: 160/100 CR: 68/min, regular RR: 18/min Temp: 36.2°C • HEENT: no alopecia, no head lesions, anicteric sclera, slightly pale palpebral conjunctivae, no abnormal discharges, no oral or nasopharyngeal ulcers, no tonsillopharyngeal wall congestion, no cervical lymphadenopathy, flat neck veins • Skin: no pallor, no jaundice, no rashes, (-) petechiae/hematoma

  10. Physical Examination • Cardiovascular: adynamicprecordium, distinct S1 and S2, normal rate, regular rhythm, no murmur • Respiratory: symmetrical chest expansion, no intercostals/subcostal retractions, clear breath sounds • Abdomen: flabby, normoactive bowel sounds, soft, no tenderness, no organomegaly, no palpable masses • Extremities: (+) grade 3 pitting bipedal edema, no cyanosis, full and equal pulses

  11. Salient Features • 43 y/o female • ↑ BP • History of malar rash, photosensitivity, arthralgia, 3x miscarriages • Decreased urine output • Pitting bipedal edema • Proteinuria, hematuria, casts • Increasing creatinine 0.9  1.2

  12. Differential Diagnoses ARF Prerenal RenalRPGNImmune Complex GN Postrenal Low C3 Normal C3 Post infectious Lupus nephritis GN

  13. Admitting Diagnosis • Acute renal failure, probably rapidly progressive glomerulonephritis, probably immune-mediated • Hypertension, poorly controlled, probably secondary to a renal pathology • Connective tissue disease t/c systemic lupus erythematosus

  14. Course in the Wards • On admission • CBC: H 11.6, H 33.6, wbc 5990 (s 65, L 22, M 11, E2) • K: 6.6↑ • Crea: 1.7 ↑ • U/A: (protein +3, rbc 3.8, wbc 23.1, e.c. 20.3, bact 1784, fine granular casts 5-10/lpf) •  Meropenem 500mg IV q 12hrs

  15. Course in the Wards • Elevated ESR: 74 (n.v. 0-20) • ANA and C3 beta complement • ABG: 100/7.36/34.1/18.8/97.5/+5.7/19.9 • Hyperkalemia regimen: (Furosemide 40mg IV q8; Kalimate QID; Salbutamol neb q6; NaHCO3 drip) • Methylprednisolone (Solumedrol) 1gm in D5W 100cc slow IV push x 3 days • Crea: 1.7  1.8 • K: 5.1  6.6

  16. Course in the Wards • 2nd Hospital day: • Hyponatremia: 134 • Crea: 1.3  1.7  1.8 • BUN: 49↑ • 24-hour urine protein: 864.5mg/24hrs ↑ • 24-hour urine crea: 551.85mg/24° estimated crea clearance: 29.48 ml/min • 24 hour urine vol: 650 cc

  17. Course in the Wards • 3rd Hospital Day: s/p utz-guided kidney biopsy • Result: Lupus Nephritis Class III

  18. RENAL BIOPSY • Specimen consists of a strip of brown tissue measuring 1.1 cm • Block all

  19. Scan X4

  20. HPOX40

  21. HPOX40

  22. HPOX40

  23. HPOX40

  24. HPOX40

  25. PAS HPOX40

  26. Trichrome Scan X4

  27. Trichrome HPO X 40

  28. Trichrome HPO X 40

  29. Final Diagnosis RENAL BIOPSY: Clinically diagnosed Systemic Lupus Erythematosus (SLE) with proteinuria Lupus Nephritis Class III

  30. Course in the Wards • Anemia  Hgb: 9.7 ↓ Hct: 27.4 ↓ • Kidney UTZ: no hematoma • 4th Hospital day: • ANA: positive up to 1:320 serum dilution; speckled pattern • C3 beta complement: 23.80mg% ↓(N.V. 79-152)

  31. Course in the Wards • Rheumatology • Anti-cardiolipin Ab: 11 GPL (N.V. <15) • Full Lupus Panel: • ANA (+) up to 1:320 dilution, speckled pattern anti-DNA: (+) anti-SSA(Ro): (+) anti-Sm: (+) anti-SSB(La): (+) anti-RNP: (-) anti-Jo1: (-)

  32. Course in the Wards • Progression of anemia: Hgb: 9.4  9.7 Hct: 27.9 • EPO (Renogen) 8000 u IV OD • Crea: 2  1.3

  33. Course in the Wards • Prednisone 30mg PO AM, 20mg PO PM (on full stomach) • CaCO3 500mg OD • Urine CS: E.coli sensitive to Ertapenem & Cefuroxime: Meropenem  Cefuroxime 250mg PO BID • Losartan 50mg PO OD

  34. Course in the Wards • 5th Hospital day: • Cyclophosphamide (CYC) IV pulsed therapy CYC 700mg in D5W 500cc x 2 hours • Crea: 1.4  2 • Prednisone

  35. Course in the Wards • 6th Hospital day: • Discharged stable and improved • THM: • EPO (Recormon) 5000U SC, T-Th-Sat • Esomeprazole 40mg OD • Clonidine 75mcg SL TID • Losartan 50mg OD • Cefuroxime 250mg BID x 7 days • Prednisone 5mg/tab, 30mg in AM, 20mg in PM w/ meals • CaCo3 500mg PO OD

  36. Final Diagnoses • Systemic lupus erythematosus, lupus nephritis Class III, ARF 2°, s/p kidney biopsy • Hypertension stage II, secondary to lupus nephritis • Urinary tract infection, on treatment

  37. Discussion

  38. Lupus Nephritis • 50% of lupus patients will develop clinically relevant nephritis at some time in the course of their illness1 • Varies from isolated abnormalities of the urinary sediment to full-blown nephritic or nephrotic syndrome or chronic renal failure • Formation of immune complexes w/in glomerular capillary wall • Diagnosed by renal biopsy 1http://www.cerebrel.com/lupus/nephritis.php

  39. ISN/RPS(2003)classification of LN

  40. Corticosteroids • Mainstay of tx for any inflammatory life-threatening or organ-threatening manifestations of SLE (proliferative LN) • High dose IV glucocorticoid pulses given slowly over a 3-4 hour period, monthly for 6 months with 0.5 - 1mg of oral prednisone per kg between pulses, to control both renal and extrarenal manifestations

  41. Pulse steroids: How much is enough? Giovanni Franchin, and Betty Diamond Columbia University, Department of Medicine, Division of Rheumatology, 1130 St. Nicholas Ave., Audubon III Room 923, New York, NY 10032, USA Available online 29 August 2005. • High dose pulse intravenous steroids with 1 g of methylprednisolone (MEP) given daily, usually for three days, is an accepted practice to treat severe manifestations of systemic lupus erythematosus (SLE) or systemic vasculitides, despite the lack of definitive data.

  42. Adverse Effects of Steroids • Weight gain • Hirsutism • Acne • Infection • Glucose intolerance • Osteopenia/ osteonecrosis • Glaucoma • PUD

  43. Cytotoxic drugs • Used in severe corticosteroid-resistant disease or in the context of unacceptable steroid side effects • Cyclophosphamide has been shown to reduce progression of scarring in the kidney & reduce risk for end-stage renal failure

  44. Cyclophosphamide • An alkylating agent; Cyclophosphamide given intravenously for prolonged periods is the current gold standard. • National Institution of Health Protocol: Cyclophosphamide 1gm/m2 every month for 6 months (Induction phase) Cyclophosphamide 1gm/m2 every 3 months for 2 years (Maintainance phase)

  45. Adverse effects of Cyclophosphamide • Nausea and vomiting • Alopecia • Ovarian failure or azoospermia • Hemorrhagic cystitis, bladder fibrosis, bladder transitional or squamous CA

  46. Monitoring for patients on CTX • Regular and frequent lab evaluations to screen for: • bone marrow toxicity: CBC • monitor renal function: BUN, crea, electrolytes • avoid major drug-induced bladder complications: urinalysis

  47. Other treatment options • Azathioprine Long-term efficacy of azathioprine treatment for proliferative lupus nephritis H. C. NossentandW. Koldingsnes Department of Rheumatology, University Hospital Tromsø, Norway • Plasmapheresis  The Lupus Nephritis Collaborative Study: A randomized, controlled, multicenter clinical trial (John M. Lachin, Sc.D.)

  48. Mycophenolatemofetil • A new immunosuppressive drug • May be more effective in inducing remission than standard regimen of IV cyclophosphamide • Produces fewer complications than Cyclophosphamide like the loss of child-bearing ability

  49. Updates

  50. Long-Term Study of MycophenolateMofetil as Continuous Induction and Maintenance Treatment for Diffuse Proliferative Lupus Nephritis (Tak-Mao Chan, et al; Feb. 23,2005) • Background: Mycophenolate mofetil (MMF) and the sequential use of cyclophosphamidefollowed by azathioprine (CTX-AZA) demonstrate similar short-termefficacy in the treatment of diffuse proliferative lupus nephritis(DPLN), but MMF is associated with less drug toxicity • Materials and Methods: Thirty-three patients wererandomized to receive MMF, and 31 were randomized to the CTX-AZAtreatment arm, both in combination with prednisolone.

More Related