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Systemic lupus erythematosus (SLE) is a systemic autoimmune disease associated with anti-nuclear antibodies (ANA), extractable nuclear antigens (ENA), and anti-double stranded DNA antibodies. Glomerulonephritis (GN) with immune complex deposition is found in 50% of cases.1 Twenty per cent of patients with SLE have antineutrophil cytoplasmic antibodies (ANCA), which in certain patients is associated with ANCA-associated vasculitis (AAV).2 The ANCA detected in such cases is reported in the literature as antimyeloperoxidase antibodies (MPO) rather than antiproteinase 3 (PR3) antibodies.3 The prevalence of dual-positive AAV is up to 9% but these cases are not associated with SLE.4 This is the first report of a case of SLE/AAV involving AAV (necrotising pauci-immune GN) with both anti-MPO and anti-PR3 antibodies.
A 70-year-old woman presented with thrombocytopenia (platelets 17×109/L), renal failure (creatinine 260 µmol/L) and deep vein thrombosis. She has a 10-year history of SLE characterised by panniculitis, photosensitivity, polyarthritis, oral ulceration, sicca symptoms, relapsing polychondritis, pulmonary hypertension, interstitial lung disease and pulmonary thromboembolism due to secondary antiphospholipid syndrome. Her laboratory investigations revealed a high titre (>2560) homogeneous ANA, anti-double-stranded-DNA antibodies and persistently elevated anticardiolipin and anti-beta-2 glycoprotein 1 antibodies. Her symptoms were non-responsive to methotrexate and leflunomide. A renal biopsy performed 1 year prior to presentation for a creatinine 110 µmol/L demonstrated hypertensive changes with no features of lupus nephritis.
Following the …
Handling editor Stephen R A Jolles.
Correction notice This article has been corrected since it was published. The title and order of authors have been updated.
Contributors Patient care managed by SLF. Planning, conduct and reporting completed by SLF. Case report reviewed and edited by both authors.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Patient consent for publication Obtained.
Provenance and peer review Not commissioned; externally peer reviewed.