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.

Long‐term renal outcomes in multi‐ethnic Southeast Asians with lupus nephritis: a retrospective cohort study

“…Among the eight patients seronegative for both ANA and anti‐dsDNA, four had membranous lupus nephropathy. It is possible that these patients had seronegative LN and may develop serological manifestations years after diagnosis of LN . Although our patients with mixed proliferative and membranous LN tended to progress to ESRD, this was not statistically significant, possibly due to the small sample size and low event rate.…”

Evaluation of an automated connective tissue disease screening assay in Korean patients with systemic rheumatic diseases

“…Although there were 3 SLE and 4 MCT patients with negative IIF results, they showed earlier clinical observations with developed positive IIF later in follow-up or negative conversion after initial positivity on presentation because of immunosuppressive treatment according to the review of medical records. Several studies described the variable clinical and serological presentations over time in patients with SLE and MCT [18,19]. …”

Rare Case of Lupus Nephritis With Negative Antinuclear Antibodies, Double-Stranded DNA Antibodies and Positive Anti-Ro/SSA Antibodies

“…Renal involvement is a common complication of SLE affecting almost half of the patients [2]. Although antibodies against DNA play a major role in the pathogenesis and the activity of LN [3][4][5], there are few cases in the literature of LN with no detectable antibodies [6,7].…”