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Lupus nephritis and fibrinoid vasculitis of the kidney: a very rare alliance
  1. K Benz1,2,
  2. M Büttner2,
  3. K Rosenthal3,
  4. K Amann2
  1. 1Department of Pediatrics, University of Erlangen-Nürnberg, Erlangen, Germany
  2. 2Department of Pathology, University of Erlangen-Nürnberg, Erlangen, Germany
  3. 3Children's Hospital Nürnberg-Süd, Nuremberg, Germany
  1. Correspondence to Professor Dr Kerstin Amann, Department of Pathology, University of Erlangen-Nürnberg, Krankenhausstr. 12, D- 91054 Erlangen, Germany; kerstin.amann{at}

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Systemic lupus erythematosus (SLE) is a rare systemic autoimmune disease affecting multiple organs and particularly the kidney. Lupus nephritis is the first manifestation of SLE in up to 60% of paediatric and adult patients, being a strong predictor of renal and patient survival.1 2

Case report

A 15-year-old girl presented with recurrent arthritis, arthralgia and episodes of high temperature for 5 months. In the last 2 months, she developed crampy abdominal pain, nausea, episodes of diarrhoea without bleeding and paraesthesia in the hands and feet. Treatment with analgetics and physiotherapy only slightly ameliorated the symptoms. Thereafter she developed haematuria, proteinuria and increased serum creatinine and was admitted to hospital, showing a reduced general condition, conjunctivitis, exanthema at the back of the feet and hands, painful wrist swelling and mild oedema. Laboratory investigations showed anaemia, leucopenia, reduced serum C3 and C4 complement, reduced albumin, increased creatinine (1.21 mg/dl), CRP (6 mg/dl) and d-dimers (4.1 mg/l). Urine analysis revealed gross glomerular proteinuria (9 g/day) and haematuria (400 erythrocytes/μl; 28% dysmorphic). Serologically, antinuclear antibody (ANA,1:5120) and anti-ds DNA antibody titres (>200 U/ml) were elevated, positive cardiolipin antibodies (28 GPL-U/ml) were detected, while anti-neutrophil cytoplasmatic antibodies, anti-GBM, antistreptolysin and infectious serology were negative. Taken together, SLE was suspected, and a kidney biopsy was performed. Twenty-two markedly hypercellular glomeruli with increased mesangial and endothelial cell number (intracapillary proliferations) and cellular …

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  • Funding Supported by German Research Society (DFG, SFB 423, Z2) and the IZKF Erlangen (projects A11, A31 and grant to K.B.).

  • Competing interests None.

  • Patient consent Obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.