Pauci‐Immune Lupus Nephritis: A Case Report

Abstract

A 26-year-old female with systemic lupus erythematosus was admitted because of dyspnea and progressive lower extremity edema. Laboratory testing showed blood urea nitrogen levels of 147 mg/dL, creatinine of 6.7 mg/dL, serum albumin of 1.7 g/dL and the daily protein loss was 12.7 g. Her C3 level was 60.4mg/dL and C4 level was 10.2 mg/dL. The antinuclear antibody titer was 1:320, with a homogeneous pattern, but she was negative for anti-dsDNA. ELISA testing for anti-PR3 antibodies and anti-MPO antibodies were all negative. She was also negative for circulating lupus anticoagulant. Renal biopsy revealed diffuse proliferation of glomerular cells, but immunofluorescent microscopy showed no immune deposits and electron microscopy revealed only scanty electron-dense deposits. She received 1 g/day of methylprednisolone intravenously for 3 days, followed by 60 mg/day of prednisolone. She was discharged with serum creatinine decreased to 4.7 mg/dL, and a great improvement in dyspnea. Diffuse proliferative lupus nephritis that contains little or no subendothelial deposits is rare. The differential diagnosis, possible mechanisms and treatment are discussed.

Pauci-Immune Crescentic Glomerulonephritis in Connective Tissue Disease

“…We found 15 cases in the literature with proliferative and necrotizing crescentic glomerulonephritis in connective tissue diseases other than vasculitis. Most of the patients, except patients reported by Fayaz et al [ 2 ], Akhtar et al [ 3 ], and Li et al [ 4 ], have more than 1+ staining on IF. Only 4 out of 15 patients meet the criteria for pauci-immune crescentic glomerulonephritis.…”

“…Activated T cells recruit and activate macrophages, and these sensitized cells can cause severe glomerular injury independent of humoral immune responses. Couser [ 21 ] and Li et al [ 4 ] suggested delayed-type hypersensitivity as the underlying mechanism of glomerular damage in ANCA-associated pauci-immune GN.…”

“…The criteria for the pauci-immune entity of CrGN have been defined as “low intensity of glomerular immunoglobulin staining by direct immunofluorescence (IF) assay in renal sections in the negative to 1+ range on a scale of 0 to 4+” [ 1 ]. Glomerulonephritis is commonly associated with lupus but the occurrence of pauci-immune CrGN is rare in lupus and connective tissue diseases other than vasculitis [ 2 – 4 ].…”

Clinicopathological Characteristics and Outcomes of Chinese Patients with Scanty Immune Deposits Lupus Nephritis: A Large Cohort Study from a Single Center

“…Accordingly, lupus nephritis was considered to be the classical type of immune complex associated glomerulonephritis. It is rare for lupus nephritis to contain no immune complex deposits, although there have been several cases of scanty immune deposits lupus nephritis reported previously [1–3, 28–30]. The clinicopathological features, outcomes, and possible pathogenesis of scanty immune deposits lupus nephritis should be studied.…”

Unusual association of a positive pANCA pauci-immune extracapillary glomerulonephritis in a lupus patient

“…[2] Diffuse proliferative LN that contains little or no sub-endothelial deposits is rare. [3] Underlying vasculitic lesions induced by ANCA antibodies were recently incriminated in the pathogenesis of these forms. [4]…”