# Antineutrophil Cytoplasmic Antibody-Negative Pauci-Immune Crescentic Glomerulonephritis in Systemic Lupus Erythematosus: A Case Report and Literature Review

**Authors:** Mohammed A Miqdad, Zainulabdeen S Al-Saedi, Lina Alatta, Hasan Hulwi, Sheng Kuo

PMC · DOI: 10.7759/cureus.87285 · Cureus · 2025-07-04

## TL;DR

A rare case of ANCA-negative pauci-immune crescentic glomerulonephritis in a systemic lupus erythematosus patient is reported and reviewed.

## Contribution

A novel case of ANCA-negative PICG in SLE is documented and discussed in the context of existing literature.

## Key findings

- An SLE patient developed ANCA-negative PICG requiring renal replacement therapy.
- The patient responded well to treatment despite severe kidney injury.
- Literature review highlights the rarity of PICG in the context of lupus nephritis.

## Abstract

Lupus nephritis (LN) is a common complication of systemic lupus erythematosus (SLE) and often presents as an immune complex-mediated glomerular disease. While pauci-immune crescentic glomerulonephritis (PICG) is commonly associated with antineutrophil cytoplasmic antibodies (ANCAs), its occurrence in the context of LN is rare. We report a case of an SLE patient who developed acute kidney injury due to ANCA-negative PICG. The kidney injury was severe enough to necessitate renal replacement therapy; however, the patient responded well to treatment. We also review the literature and previously reported cases of PICG in patients with SLE.

## Linked entities

- **Diseases:** systemic lupus erythematosus (MONDO:0007915), lupus nephritis (MONDO:0005556)

## Full-text entities

- **Genes:** CD79A (CD79a molecule) [NCBI Gene 973] {aka IGA, IGAlpha, MB-1, MB1}, PRTN3 (proteinase 3) [NCBI Gene 5657] {aka ACPA, AGP7, C-ANCA, CANCA, MBN, MBT}, ALB (albumin) [NCBI Gene 213] {aka FDAHT, HSA, PRO0883, PRO0903, PRO1341}, C1QA (complement C1q A chain) [NCBI Gene 712] {aka C1QD1}, TRIM21 (tripartite motif containing 21) [NCBI Gene 6737] {aka RNF81, RO52, Ro/SSA, SSA, SSA1, TRIM21/Ro52}, SSB (small RNA binding exonuclease protection factor La) [NCBI Gene 6741] {aka LARP3, La, La/SSB, SSB/La}, MPO (myeloperoxidase) [NCBI Gene 4353]
- **Diseases:** malignancies (MESH:D009369), uterine adenocarcinoma (MESH:D000230), SLE (MESH:D008180), infection (MESH:D007239), hypoalbuminemia (MESH:D034141), Klebsiella pneumoniae (MESH:D007710), uremia (MESH:D014511), chills (MESH:D023341), acute kidney injury (MESH:D058186), leukocytosis (MESH:D007964), vomiting (MESH:D014839), acute tubular injury (MESH:D001930), ANCA (MESH:D056648), joint pain (MESH:D018771), renal involvement (MESH:C565423), proteinuria (MESH:D011507), necrosis (MESH:D009336), skin rash (MESH:D005076), marginal zone lymphoma (MESH:D018442), epigastric pain (MESH:D010146), chest pain (MESH:D002637), immune complex (MESH:D007105), Raynaud's phenomenon (MESH:D011928), lung adenocarcinoma (MESH:D000077192), shortness of breath (MESH:D004417), Glomerulonephritis (MESH:D005921), nephrotic (MESH:D009404), nausea (MESH:D009325), glomerular disease (MESH:D007674), oral ulcers (MESH:D019226), bacterial infections (MESH:D001424), alopecia (MESH:D000505), Fabry disease (MESH:D000795), hematuria (MESH:D006417), hemolytic anemia (MESH:D000743), arthritis (MESH:D001168), eosinophilia (MESH:D004802), LN (MESH:D008181), autoimmune diseases (MESH:D001327)
- **Chemicals:** steroid (MESH:D013256), Ceftriaxone (MESH:D002443), nitrites (MESH:D009573), urea nitrogen (MESH:C530477), hydroxychloroquine (MESH:D006886), Rituximab (MESH:D000069283), methylprednisolone (MESH:D008775), creatinine (MESH:D003404), cyclophosphamide (MESH:D003520), Antineutrophil (-), mycophenolate (MESH:D009173), prednisone (MESH:D011241)
- **Species:** Homo sapiens (human, species) [taxon 9606], Streptococcus parasanguinis (species) [taxon 1318], Staphylococcus epidermidis (species) [taxon 1282]

## Full text

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## Figures

3 figures with captions in the complete paper: https://tomesphere.com/paper/PMC12318536/full.md

## References

13 references — full list in the complete paper: https://tomesphere.com/paper/PMC12318536/full.md

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Source: https://tomesphere.com/paper/PMC12318536