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

Biopsy-proven nephritis compatible with SLE in the presence of antinuclear antibodies (ANA) or anti-double-stranded DNA (dsDNA) antibodies

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	Lupus malar rash Fixed erythema, flat or raised, over the malar eminences, tending to spare the nasolabial folds Bullous lupus Toxic epidermal necrolysis variant of SLE Maculopapular lupus rash Photosensitive lupus rash (in the absence of dermatomyositis); Skin rash as a result of unusual reaction to sunlight, by patient history or physician observation Subacute cutaneous lupus Nonindurated psoriaform and/or annular polycyclic lesions that resolve without scarring, although occasionally with postinflammatory dyspigmentation or telangiectasias
	Chronic cutaneous lupus	Classic discoid rash Localized (above the neck) Generalized (above and below the neck) Hypertrophic (verrucous) lupus Lupus panniculitis (profundus) Mucosal lupus Lupus erythematosus tumidus Chilblains lupus OR Discoid lupus/lichen planus overlap
	Nonscarring alopecia	Diffuse thinning or hair fragility with visible broken hairs (in the absence of other causes, such as alopecia areata, drugs, iron deficiency, and androgenic alopecia)
	Oral or nasal ulcers	Palate, buccal, tongue, or nasal ulcers (in the absence of other causes, such as vasculitis, Behçet's disease, infection, inflammatory bowel disease, reactive arthritis, and acidic foods)
	Joint disease	Synovitis involving two or more joints, characterized by swelling or effusion Tenderness in two or more joints and at least 30 minutes of morning stiffness
	Serositis	Typical pleurisy for more than one day, pleural effusions, or pleural rub Typical pericardial pain (pain with recumbency improved by sitting forward) for more than one day, pericardial effusion, pericardial rub, or pericarditis by electrocardiography in the absence of other causes, such as infection, uremia, and Dressler's syndrome
	Renal	Urine protein-to-creatinine ratio (or 24-hour urine protein) representing 500 mg protein/24 hours Red blood cell casts
	Neurologic	Seizures Psychosis Mononeuritis multiplex (in the absence of other known causes, such as primary vasculitis) Myelitis Peripheral or cranial neuropathy (in the absence of other known causes, such as primary vasculitis, infection, and diabetes mellitus) Acute confusional state (in the absence of other causes, including toxic/metabolic, uremia, drugs)
Hematologic	Hemolytic anemia	Hemoglobin less than 12 g/dL in females and 13 g/dL in males
	Leukopenia or lymphopenia	Leukopenia (<4000/mm3 at least once) (in the absence of other known causes, such as Felty's syndrome, drugs, and portal hypertension) Lymphopenia (<1000/mm3 at least once) (in the absence of other known causes, such as glucocorticoids, drugs, and infection)
	Thrombocytopenia	Thrombocytopenia (<100,000/mm3) at least once in the absence of other known causes, such as drugs, portal hypertension, and thrombotic thrombocytopenic purpura
Immunologic	ANA	ANA level above laboratory reference range
	Anti-dsDNA	Anti-dsDNA antibody level above laboratory reference range (or >twofold the reference range if tested by ELISA)
	Anit-SM	Presence of antibody to Sm nuclear antigen
	Antiphospholipid	Antiphospholipid antibody positivity as determined by any of the following: Positive test result for lupus anticoagulant False-positive test result for rapid plasma reagin Medium- or high-titer anticardiolipin antibody level (IgA, IgG, or IgM) Positive test result for anti-beta 2-glycoprotein I (IgA, IgG, or IgM)
	Low complement	Low C3 Low C4 Low CH50
	Direct Coombs' test	Direct Coombs' test in the absence of hemolytic anemia

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

↑ 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.
↑ 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.
↑ 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.
↑ 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.
↑ 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.
↑ 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.
↑ 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.
↑ 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.

Template:WH Template:WS

[pmid7138600-1] 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.

[pmid9324032-2] 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.

[pmid22553077-3] 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.

[pmid18046678-4] 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.

[pmid15780090-5] 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.

[pmid11145039-6] 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.

[pmid11682680-7] 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.

[pmid7814619-8] 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.

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