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Antinuclear Antibody-Negative Lupus Nephritis with Full House Nephropathy: A Case Report and Review of the Literature.

Abstract

Lupus nephritis (LN) is a serious and common complication of systemic lupus erythematosus (SLE) that predisposes to significant morbidity and mortality. Studies show that prompt diagnosis and treatment improves patient survival. We present a case of a 49-year-old female with an atypical presentation of LN who initially presented with new-onset hypertension, edema, arthritis, serositis and recently diagnosed leukocytoclastic vasculitis who later developed acute kidney injury, hematuria and nephrotic syndrome. Laboratory testing showed mixed cryoglobulinemia and elevated perinuclear anti-neutrophil cytoplasmic (p-ANCA) and myeloperoxidase (MPO) antibodies. SLE-related serologies were negative. Kidney biopsy showed diffuse proliferative global glomerulonephritis with a full-house nephropathy pattern on immunofluorescence suggestive of LN. Due to high clinical suspicion and renal biopsy findings, she was treated for LN with prompt renal response to immunosuppression. Cryoglobulins, p-ANCA and MPO titers normalized and the negative SLE serologies remained negative. Literature review on antinuclear antibody (ANA)-negative and seronegative LN revealed the following patient presentations: (1) renal-limited or renal and extra-renal manifestations of SLE with negative serologies and (2) renal and extra-renal manifestations of SLE with negative serologies at presentation who develop positive serologies later in follow-up. Both groups represent a unique and challenging cohort of patients who may require longer follow-up and further testing to rule out other glomerular diseases that may mimic LN on renal biopsy. The absence of SLE-related serologies should be weighed against a high pre-test probability of ANA-negative or seronegative LN. If highly suspected, the patient should be treated promptly with close monitoring.

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  • Authors+Show Affiliations

    ,

    Department of Internal Medicine, Mayo Clinic, Phoenix, Arizona, USA.

    , ,

    Source

    MeSH

    Acute Kidney Injury
    Antibodies, Antinuclear
    Biopsy
    Cell Proliferation
    Cohort Studies
    Cryoglobulinemia
    Female
    Hematuria
    Humans
    Immunosuppressive Agents
    Kidney
    Lupus Erythematosus, Systemic
    Lupus Nephritis
    Microscopy, Fluorescence
    Middle Aged
    Nephrotic Syndrome
    Peroxidase
    Probability

    Pub Type(s)

    Case Reports
    Journal Article

    Language

    eng

    PubMed ID

    26812129

    Citation

    Simmons, Sierra C., et al. "Antinuclear Antibody-Negative Lupus Nephritis With Full House Nephropathy: a Case Report and Review of the Literature." American Journal of Nephrology, vol. 42, no. 6, 2015, pp. 451-9.
    Simmons SC, Smith ML, Chang-Miller A, et al. Antinuclear Antibody-Negative Lupus Nephritis with Full House Nephropathy: A Case Report and Review of the Literature. Am J Nephrol. 2015;42(6):451-9.
    Simmons, S. C., Smith, M. L., Chang-Miller, A., & Keddis, M. T. (2015). Antinuclear Antibody-Negative Lupus Nephritis with Full House Nephropathy: A Case Report and Review of the Literature. American Journal of Nephrology, 42(6), pp. 451-9. doi:10.1159/000443747.
    Simmons SC, et al. Antinuclear Antibody-Negative Lupus Nephritis With Full House Nephropathy: a Case Report and Review of the Literature. Am J Nephrol. 2015;42(6):451-9. PubMed PMID: 26812129.
    * Article titles in AMA citation format should be in sentence-case
    TY - JOUR T1 - Antinuclear Antibody-Negative Lupus Nephritis with Full House Nephropathy: A Case Report and Review of the Literature. AU - Simmons,Sierra C, AU - Smith,Maxwell L, AU - Chang-Miller,April, AU - Keddis,Mira T, Y1 - 2016/01/27/ PY - 2016/1/27/entrez PY - 2016/1/27/pubmed PY - 2016/10/27/medline SP - 451 EP - 9 JF - American journal of nephrology JO - Am. J. Nephrol. VL - 42 IS - 6 N2 - Lupus nephritis (LN) is a serious and common complication of systemic lupus erythematosus (SLE) that predisposes to significant morbidity and mortality. Studies show that prompt diagnosis and treatment improves patient survival. We present a case of a 49-year-old female with an atypical presentation of LN who initially presented with new-onset hypertension, edema, arthritis, serositis and recently diagnosed leukocytoclastic vasculitis who later developed acute kidney injury, hematuria and nephrotic syndrome. Laboratory testing showed mixed cryoglobulinemia and elevated perinuclear anti-neutrophil cytoplasmic (p-ANCA) and myeloperoxidase (MPO) antibodies. SLE-related serologies were negative. Kidney biopsy showed diffuse proliferative global glomerulonephritis with a full-house nephropathy pattern on immunofluorescence suggestive of LN. Due to high clinical suspicion and renal biopsy findings, she was treated for LN with prompt renal response to immunosuppression. Cryoglobulins, p-ANCA and MPO titers normalized and the negative SLE serologies remained negative. Literature review on antinuclear antibody (ANA)-negative and seronegative LN revealed the following patient presentations: (1) renal-limited or renal and extra-renal manifestations of SLE with negative serologies and (2) renal and extra-renal manifestations of SLE with negative serologies at presentation who develop positive serologies later in follow-up. Both groups represent a unique and challenging cohort of patients who may require longer follow-up and further testing to rule out other glomerular diseases that may mimic LN on renal biopsy. The absence of SLE-related serologies should be weighed against a high pre-test probability of ANA-negative or seronegative LN. If highly suspected, the patient should be treated promptly with close monitoring. SN - 1421-9670 UR - https://www.unboundmedicine.com/medline/citation/26812129/Antinuclear_Antibody_Negative_Lupus_Nephritis_with_Full_House_Nephropathy:_A_Case_Report_and_Review_of_the_Literature_ L2 - https://www.karger.com?DOI=10.1159/000443747 DB - PRIME DP - Unbound Medicine ER -