Simultaneous Presentation of ANCA-Associated Pauci-Immune Crescentic Glomerulonephritis and Systemic Lupus Erythematous
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Introduction: The presence of vasculitis may be found in the course of Systemic lupus erythematosus (SLE) but rarely corresponds to an ANCA-associated vasculitis (AAV) furthermore antineutrophil cytoplasmic antibodies (ANCA) positivity can be present in patients with SLE and may be related with a poor prognosis however the clinical expression of an AAV is extremely uncommon. We report a case of a young man fulfilling the criteria for SLE who develop a Rapidly progressive glomerulonephritis (RPGN) and the findings on the kidney biopsy corresponds to a Pauci-immune crescentic glomerulonephritis (PICGN) Case Description: Patient with history of hypertension diagnosed 1 year earlier and previous hospitalization six month ago for an episode of venous thrombosis. Begin oral anticoagulation and was discharged with an estimated glomerular filtration rate (eGFR) of 76ml/min/1.73m2. It was referred to our unit for a decline of 50% in eGFR and glomerular hematuria. In physical exam we found arthritis and peripheral edema. We approach this case by performing levels of complements both were found low. In the setting of RPGN and low complements. We order antinuclear antibodies that resulted positive 1:1,280 with a fine speckled pattern. By this point the patient fulfilled the ACR/EULAR 2019 criteria for SLE. We carry out a kidney biopsy that concluded necrotizing proliferative extracapilar pauciinmune glomerulonephritis, with granulomas without eosinophilic infiltration. Tubulointerstitial nephritis and arteriolar fibrosis were also found. Due to this findings ANCA were perform anti-myeloperoxidase antibody was found positive 1:10 2+ on immunofluorescence and 1.35 in enzyme-linked immunoassay. The patient recieve an induction therapy with methylprednisolone and cyclophosphamide. We achieve no progression in the necessity of renal replacement therapy but the eGFR remains on 35ml/min/1.73m2 after induction therapy Discussion: This case demonstrates that the presence of overlap between SLE and AAV is a rare but feasible entity. Although ANCA may be present in SLE they are typically not related with a clinical expression of the disease. As the seen in this case where the kidney biopsy findings are strongly associated with AAV
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DOI retrouvé dans Crossref DOI retrouvé ; titre concordant.
- Titre Crossref
- Simultaneous Presentation of ANCA-Associated Pauci-Immune Crescentic Glomerulonephritis and Systemic Lupus Erythematous
- Date Crossref
- 01/11/2022
- Éditeur
- Ovid Technologies (Wolters Kluwer Health)
- Type
- journal-article
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