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Morning Report

Morning Report. 8/26/2010. Case. 51 yo F patient sent by her PCP to the hospital for 10 days hx of poor apetite, fatigue, lethargy and AKI. PMH: Scleroderma, HTN, DM, asthma, noncompliance with meds Meds: diovan, metformin, ventolin and prednisone

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Morning Report

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  1. Morning Report 8/26/2010

  2. Case • 51 yo F patient sent by her PCP to the hospital for 10 days hx of poor apetite, fatigue, lethargy and AKI. • PMH: Scleroderma, HTN, DM, asthma, noncompliance with meds • Meds: diovan, metformin, ventolin and prednisone • ROS: as above with occasional chills, rash on face, bil knee pain and swelling • SH: smoking 30 PPY • FH: aunt with some kidney disease • VS: nl except for BP: 158/98 • PE: 3/6 cardiac murmur, bil knee warmness and mild effusions

  3. Renal manifestations of SLE MR 8/25/10

  4. Systemic Lupus Erythematosus • Patients with SLE are subject to a variety of symptoms, complaints, and inflammatory involvement that can affect virtually every organ • The most common pattern is a mixture of constitutional complaints with skin, musculoskeletal, mild hematologic, renal and serologic involvement

  5. Clinical criteria for diagnosis • ARA/ACR revised Criteria for the Classification of SLE • Classical SLE — many criteria • Definite SLE — four or more criteria • Undifferentiated connective tissue disease (UCTD) • 96% sensitivity and specificity

  6. ACR criteria for diagnosis of systemic lupus erythematosus

  7. Autoantibodies • The ANA test is the best screening test for SLE and should be performed whenever SLE is suspected • The ANA is positive in significant titer (usually 1:160 or higher) in virtually all patients with SLE

  8. Autoantibodies 2 autoantibodies are highly specific for SLE: anti-ds DNA ab & anti-Sm ab • Sensitivity – 66 to 95 percent • Specificity – 75 to 100 percent • Predictive value – 89 to 100 percent

  9. Specific antibodies associations: • Ab (RNP): SLE, MCTD, and scleroderma • Ab to Ro (SS-A) and La (SS-B):SLE & Sjögren's • Anti-dsDNA: nephritis & active systemic disease • Anti-RNP (U1 RNP): myositis, Raynaud’s & mild SLE • Anti-Ro/SSA: lymphopenia, photosensitivity, neonatal lupus, C2 deficiency, subacute cutaneous lupus, and Sjögren's • Anti-La/SSB : neonatal lupus & Sjögren's • Anti-ribosomal P protein: neuropsychiatric manifestations

  10. Lupus renal disease • Renal involvement is common in SLE • Abnormal UA (hematuria and/or proteinuria) with or without increase in S.cr observed in up to 75% of SLE patients • The most frequently observed abnormality is proteinuria (80%); while approximately 40% have hematuria and/or pyuria sometime during the course of their illness

  11. Lupus renal disease • Incidence among patients with SLE probably exceeds 90% • Different types of renal disease in SLE: immune complex-mediated glomerular diseases is most common

  12. Lupus renal disease Tubuloreticular structures Normal glomerulus

  13. Lupus renal disease Classification: • 1974 and 1982 WHO • 2004 new classification: 6 classes

  14. Minimal mesangial lupus nephritis (class I) • Identified by immunofluorescence alone or IF and EM, normal LM • Earliest and mildest form • Normal UA and S.Cr • No biopsy needed

  15. Mesangial proliferative lupus nephritis (class II) • Presents clinically as microscopic hematuria and/or proteinuria • Hypertension is uncommon, and the nephrotic syndrome and renal insufficiency are virtually never seen • The renal prognosis is excellent and no specific therapy is indicated unless the patient progresses to more advanced disease

  16. Mesangial proliferative lupus nephritis (class II) Mesangial proliferative GN Normal glomerulus

  17. Focal proliferative lupus nephritis (class III) • 10 to 20% of cases • Hematuria and proteinuria very common • Nephrotic syndrome, hypertension, and an elevated [S.cr] • By definition, less than 50% of glomeruli are affected on light microscopy • Immune deposits in the subendothelial space of the glomerular capillary wall as well as the mesangium on EM!

  18. Focal proliferative lupus nephritis (class III) • Variable prognosis • Better prognosis when less than 25% of the glomeruli are affected on LM • Also classified as active vs chronic (can be segmental or global): • III (A) • III (A/C) • III (C)

  19. Diffuse proliferative lupus nephritis (class IV) • The most common and most severe form of lupus nephritis affecting 40 to 60% of cases • Hematuria and proteinuria always seen • Nephrotic syndrome, hypertension, and renal insufficiency are all frequently seen • Significant hypocomplementemia and elevated anti-ds-DNA levels, especially during active disease • Immunosuppressive therapy is generally required to prevent progression of active diffuse proliferative lupus nephritis to ESRD

  20. Diffuse proliferative lupus nephritis (class IV) • 6 subtypes: • IV-S (A) • IV-G (A) • IV-S (A/C) • IV-G (A/C) • IV-S (C) • IV-G (C)

  21. Diffuse proliferative lupus nephritis (class IV)

  22. Diffuse proliferative lupus nephritis (class IV) Membranoproliferative LN Normal glomerulus

  23. Membranous lupus (class V) • 10 to 20% of patients • Patients typically present with nephrotic syndrome • Microscopic hematuria and hypertension may be seen at presentation, [S.cr] usually normal or slightly elevated • May present with no other clinical or serologic manifestations of SLE • Most patients maintain a normal or near normal plasma creatinine concentration for five years or more and may not require immunosuppressive therapy

  24. Membranous lupus (class V) Membranous LN Normal Glomerulus

  25. Membranous lupus (class V) Membranous LN Normal Glomerulus

  26. Advanced slerosing lupus nephritis (class VI) • > 90% global sclerosing of glomeruli • Consists of healing of previous inflamatory injury and advanced stages of class III, IV or V LN • Slow progressive renal dysfunction • Bland urine sediment

  27. Other Renal manifestations of SLE • In addition to glomerulopathies, there are three other less common forms of lupus renal disease: • interstitial nephritis • vascular disease • renal disease infrequently associated with drug-induced lupus • ?Glomerular podocytopathy?

  28. Tubulointerstitial nephritis • Tubulointerstitial disease (interstitial infiltrate, tubular injury) • Mostly seen with concurrent glomerular disease, occasionaly the only manifestation of LN

  29. Vascular disease • Glomerular and vascular thrombi associated with APL • Fibrin thrombi in the small arteries and glomerular capillaries and sometimes in the larger renal artery branches • Can be primary superimposed upon one of the immune complex forms of lupus nephritis • Rarely, patients with lupus nephritis develop renal vein thrombosis

  30. Drug-induced lupus • Mainly with drugs that are acetylated in the liver, such as hydralazine, procainamide, and less often isoniazid • Renal involvement is uncommon but can occur

  31. Silent Lupus Nephritis • Presence of mesangial, focal or diffuse proliferative GN without evidence of renal disease • Benign renal outcome

  32. Case

  33. Thank you

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