Lupus nephritis diagnostic study of choice

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Omer Kamal, M.D.[2]

Overview

In SLE, nephritis we suspect renal involvment by an abnormal urinalysis and/or increased serum creatinine. Histopathologic findings on renal biopsy confirm the diagnosis.

Diagnostic criteria

In 2012, Systemic Lupus International Collaboration Criteria (SLICC) developed a new criteria for SLE diagnosis. SLICC criteria for the classification of systemic lupus erythematosus was developed based on the old ACR criteria for the classification of systemic lupus erythematosus to address a more sensitive diagnostic criteria and also to cover weaknesses of the previous ACR criteria.[1][2]

Based on SLICC criteria, diagnosis of SLE is defined as:[3]

  • Meeting at least 4 of 17 criteria, including at least 1 of the 11 clinical criteria and one of the six immunologic criteria 

OR

A criterion is considered positive if one or more of the observations listed in the definition for the criterion are present in the patient. A criterion should only be counted once, regardless of the number of observations in the definition that the patient presents with.

Category Criterion Definition
Clinical Acute cutaneous lupus
Chronic cutaneous lupus
Nonscarring alopecia
Oral or nasal ulcers
Joint disease
Serositis
Renal
Neurologic
Hematologic Hemolytic anemia
  • Hemoglobin less than 12 g/dL in females and 13 g/dL in males
Leukopenia or lymphopenia
Thrombocytopenia
Immunologic ANA
  • ANA level above laboratory reference range
Anti-dsDNA
Anit-SM
Antiphospholipid
Low complement
  • Low C3
  • Low C4
  • Low CH50
Direct Coombs' test

Renal Involvement

Urinanalysis:

Hematuria and cellular cast[4]

Protein excretion and protein to creatinine ratio

Increased anti-DNA titers [5]

Low complement (C3 and C4) levels[6]

Renal Biospy:

Histopathology will guide the treatment.[7]

  • Glomerular deposits: "Full house" immunofluorescence pattern containing IgG, immunoglobulin A (IgA), immunoglobulin M (IgM), C3, and C1q.
  • Mesangial, subendothelial, and subepithelial glomerular deposits.
  • Tubular basement membrane, the interstitial, and blood vessels extraglomerular immune-type deposits.[8]
  • Glomerular endothelial cells containing tubuloreticular inclusions composed of ribonucleoprotein and membrane, usually stimulated by alpha-interferon.

References

  1. Tan EM, Cohen AS, Fries JF, Masi AT, McShane DJ, Rothfield NF, Schaller JG, Talal N, Winchester RJ (1982). "The 1982 revised criteria for the classification of systemic lupus erythematosus". Arthritis Rheum. 25 (11): 1271–7. PMID 7138600.
  2. Hochberg MC (1997). "Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus". Arthritis Rheum. 40 (9): 1725. doi:10.1002/1529-0131(199709)40:9<1725::AID-ART29>3.0.CO;2-Y. PMID 9324032.
  3. Petri M, Orbai AM, Alarcón GS, Gordon C, Merrill JT, Fortin PR, Bruce IN, Isenberg D, Wallace DJ, Nived O, Sturfelt G, Ramsey-Goldman R, Bae SC, Hanly JG, Sánchez-Guerrero J, Clarke A, Aranow C, Manzi S, Urowitz M, Gladman D, Kalunian K, Costner M, Werth VP, Zoma A, Bernatsky S, Ruiz-Irastorza G, Khamashta MA, Jacobsen S, Buyon JP, Maddison P, Dooley MA, van Vollenhoven RF, Ginzler E, Stoll T, Peschken C, Jorizzo JL, Callen JP, Lim SS, Fessler BJ, Inanc M, Kamen DL, Rahman A, Steinsson K, Franks AG, Sigler L, Hameed S, Fang H, Pham N, Brey R, Weisman MH, McGwin G, Magder LS (2012). "Derivation and validation of the Systemic Lupus International Collaborating Clinics classification criteria for systemic lupus erythematosus". Arthritis Rheum. 64 (8): 2677–86. doi:10.1002/art.34473. PMC 3409311. PMID 22553077.
  4. Lee LC, Lam KK, Lee CT, Chen JB, Tsai TH, Huang SC (2007). ""Full house" proliferative glomerulonephritis: an unreported presentation of subacute infective endocarditis". J. Nephrol. 20 (6): 745–9. PMID 18046678.
  5. Haas M, Kaul S, Eustace JA (April 2005). "HIV-associated immune complex glomerulonephritis with "lupus-like" features: a clinicopathologic study of 14 cases". Kidney Int. 67 (4): 1381–90. doi:10.1111/j.1523-1755.2005.00215.x. PMID 15780090.
  6. Ramos-Casals M, Font J, García-Carrasco M, Cervera R, Jiménez S, Trejo O, de la Red G, Sánchez-Tapias JM, Ingelmo M (December 2000). "Hepatitis C virus infection mimicking systemic lupus erythematosus: study of hepatitis C virus infection in a series of 134 Spanish patients with systemic lupus erythematosus". Arthritis Rheum. 43 (12): 2801–6. doi:10.1002/1529-0131(200012)43:12<2801::AID-ANR21>3.0.CO;2-V. PMID 11145039.
  7. Smet AD, Kuypers D, Evenepoel P, Maes B, Messiaen T, Van Damme B, Vanrenterghem Y (November 2001). "'Full house' positive immunohistochemical membranoproliferative glomerulonephritis in a patient with portosystemic shunt". Nephrol. Dial. Transplant. 16 (11): 2258–62. PMID 11682680.
  8. Rich SA (January 1995). "De novo synthesis and secretion of a 36-kD protein by cells that form lupus inclusions in response to alpha-interferon". J. Clin. Invest. 95 (1): 219–26. doi:10.1172/JCI117643. PMC 295410. PMID 7814619.