title: 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.
authors.affiliation: Department of Internal Medicine, Mayo Clinic, Phoenix, Arizona, USA.
authors.forename: Sierra C
authors.initials: SC
authors.lastname: Simmons
authors.forename: Maxwell L
authors.initials: ML
authors.lastname: Smith
authors.forename: April
authors.initials: A
authors.lastname: Chang-Miller
authors.forename: Mira T
authors.initials: MT
authors.lastname: Keddis
mesh_terms: D058186:Acute Kidney Injury / Q000150:complications; D000974:Antibodies, Antinuclear / Q000097:blood*; D001706:Biopsy; D049109:Cell Proliferation; D015331:Cohort Studies; D003449:Cryoglobulinemia / Q000097:blood; D005260:Female; D006417:Hematuria / Q000150:complications; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007668:Kidney / Q000473:pathology; D008180:Lupus Erythematosus, Systemic / Q000150:complications; D008181:Lupus Nephritis / Q000097:blood*; D008856:Microscopy, Fluorescence; D008875:Middle Aged; D009404:Nephrotic Syndrome / Q000150:complications; D009195:Peroxidase / Q000097:blood; D011336:Probability
chemical_list: D000974:Antibodies, Antinuclear; D007166:Immunosuppressive Agents; D009195:Peroxidase