Rapidly progressive lupus glomerulonephritis and concomitant microangiopathy in an adolescent

Blake, JS; Butani, L
September 2002
Lupus;2002, Vol. 11 Issue 8, p533
Academic Journal
We describe our experience managing a 16-year-old girl with systemic lupus erythematosus (SLE) who presented concomitantly with rapidly progressive glomerulonephritis (RPGN) and a thrombotic microangiopathic hemolytic anemia (TMAHA). Her renal biopsy showed evidence of diffuse proliferative glomerulonephritis without glomerular microthrombi. The patient was treated with a combination of intravenous corticosteroids and cyclophosphamide, as well as plasmapheresis, with an excellent response resulting in complete disease remission. The purpose of our report is to make health professionals more aware of TMAHA as a complication of SLE, since the occurrence of TMAHA may confuse the clinical picture, and since its treatment with plasmapheresis is life saving, if performed early.


Related Articles

  • Induction therapies for class IV lupus nephritis with non-inflammatory necrotizing vasculopathy: mycophenolate mofetil or intravenous cyclophosphamide. Wang, J.; Hu, W.; Xie, H.; Zhang, H.; Chen, H.; Zeng, C.; Liu, Z.; Li, L. // Lupus;2007, Vol. 16 Issue 9, p707 

    The presence of renal noninflammatory necrotizing vasculopathy (NNV) is often associated with a severe form of lupus nephritis (LN), which is unresponsive to standard therapy. We conducted a 6-month randomized, prospective, open-label trial comparing mycophenolate mofetil (MMF) (1.5�2.0...

  • Clinical characteristics and prognosis of diffuse proliferative lupus nephritis with thrombotic microangiopathy. Hu, W. X.; Liu, Z. Z.; Chen, H. P.; Zhang, H. T.; Li, L. S.; Liu, Z. H. // Lupus;12/01/2010, Vol. 19 Issue 14, p1591 

    We retrospectively analyzed the clinicopathologic characteristics and prognosis of 33 patients with diffuse proliferative lupus nephritis (class IV LN) complicated with thrombotic microangiopathy (TMA). Eighty-one percent of patients had renal dysfunction (mean Scr 3.1 � 2.0 mg/dl), among...

  • Circulating thrombomodulin and vascular cell adhesion molecule-1 and renal vascular lesion in patients with lupus nephritis. Yao, G. H.; Liu, Z. H.; Zhang, X.; Zheng, C. X.; Chen, H. P.; Zeng, C. H.; Li, L. S. // Lupus;Aug2008, Vol. 17 Issue 8, p720 

    Currently, the detection of renal vascular lesions (VLS) in lupus nephritis (LN) mainly depends on biopsy examination, and lack surrogate biomarkers for clinical dynamic evaluation. The aim of the present study is to explore the correlation between circulatory endothelial damage biomarkers and...

  • Rituximab induced pulmonary fibrosis in a patient with lupus nephritis. Rathi, M; Ramachandran, R; Gundlapalli, S; Agarwal, R; Das, A; Kumar, V; Kohli, HS; Jha, V; Sakhuja, V // Lupus;Sep2012, Vol. 21 Issue 10, p1131 

    We describe a 26-year-old woman who was diagnosed eleven years ago with systemic lupus erythematosus and who had suffered multiple relapses. She presented with class IV lupus nephritis with thrombotic microangiopathy, for which she received three doses of rituximab along with plasmapheresis,...

  • Inclusion of renal vascular lesions in the 2003 ISN/RPS system for classifying lupus nephritis improves renal outcome predictions. Wu, Li-Hua; Yu, Feng; Tan, Ying; Qu, Zhen; Chen, Meng-Hua; Wang, Su-Xia; Liu, Gang; Zhao, Ming-Hui // Kidney International;Apr2013, Vol. 83 Issue 4, p715 

    The 2003 International Society of Nephrology/Renal Pathology Society (ISN/RPS) pathological classification system of lupus nephritis specified the importance of vascular damage and indicated this should be included in the diagnostic summary. Few pathological studies of lupus nephritis, however,...

  • Lupus nephritis combined with renal injury due to thrombotic thrombocytopaenic purpura-haemolytic uraemic syndrome. Feng Yu; Ying Tan; Ming-Hui Zhao // Nephrology Dialysis Transplantation;Jan2010, Vol. 25 Issue 1, p145 

    Background. Thrombotic thrombocytopaenic purpura–haemolytic uraemic syndrome (TTP–HUS) in SLE was reported mainly in isolated case reports. The aim of this study is to investigate the clinical and pathological features, outcome and possible pathogenesis of TTP–HUS in...

  • Antiphospholipid syndrome nephropathy (APSN) in patients with lupus nephritis: a retrospective clinical and renal pathology study. Erre, Gian; Bosincu, Luisanna; Faedda, Rossana; Fenu, Patrizia; Masala, Antonio; Sanna, Marcella; Taras, Loredana; Longu, Maria; Piras, Marco; Soro, Giovanni; Satta, Andrea; Passiu, Giuseppe // Rheumatology International;Apr2014, Vol. 34 Issue 4, p535 

    Data about clinical-laboratory features and outcome of antiphospholipid syndrome nephropathy (APSN) in the course of lupus nephritis (LN) are scarce. To determine prevalence, clinical correlations and outcome of APSN in patients with LN, retrospective analysis of renal specimens and review of...

  • Thrombotic thrombocytopenic purpura associated with rapidly progressive lupus nephritis: report of two cases. Kapoulas, S.; Liakos, S.; Karkavelas, G.; Ageloudi, M.; Grekas, D.; Giannoulis, E. // Clinical Nephrology;2005, Vol. 63 Issue 4, p297 

    There are a few reported cases in the literature of thrombotic thrombocytopenic purpura (TTP), associated with systemic lupus erythematosus (SLE). We describe two cases of TTP which have been presented during rapidly progressive lupus nephritis, with grandmal seizures, thrombocytopenia and micro...

  • Case 37-2010. Binder, William D.; Traum, Avram Z.; Makar, Robert S.; Colvin, Robert B. // New England Journal of Medicine;12/9/2010, Vol. 363 Issue 24, p2352 

    The article describes the case of a 16-year-old girl with confusion, anemia and thrombocytopenia admitted to Massachusetts General Hospital. The patient had lupus nephritis and reported abdominal pain on examination. Her medical history includes obesity, dysmenorrhea and a right ovarian simple...


Read the Article


Sign out of this library

Other Topics