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Literature DB >> 30642866

Haemophagocytic lymphohistiocytosis with collapsing lupus podocytopathy as an unusual manifestation of systemic lupus erythematosus with APOL1 double-risk alleles.

Bhavin Chokshi¹, Vivette D'Agati², Lilian Bizzocchi³, Beverly Johnson³, Barbara Mendez³, Belinda Jim¹.

Abstract

Haemophagocytic lymphohistiocytosis (HLH) is a potentially life-threatening syndrome caused by excessive immune activation. Secondary HLH has been described in autoimmune diseases. We detail the case of a 28-year-old African American woman who developed HLH in the setting of systemic lupus erythematosus with collapsing lupus podocytopathy superimposed on mesangial proliferative lupus nephritis class II. Genotyping for APOL1 risk alleles revealed the presence of double (G1/G2) risk alleles. Our patient achieved a complete renal recovery and resolution of HLH within 1 month of treatment with steroids and mycophenolate mofetil, highlighting the importance of prompt, aggressive therapy. © BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.

Entities: Chemical Disease Gene Species

Keywords: acute renal failure; haematology (INCL blood transfusion); proteinurea; renal system; systemic lupus erythematosus

Mesh：

Substances：

Year: 2019 PMID： 30642866 PMCID： PMC6340519 DOI： 10.1136/bcr-2018-227860

Source DB: PubMed Journal: BMJ Case Rep ISSN： 1757-790X

20 in total

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Journal: Pediatr Blood Cancer Date: 2007-02 Impact factor: 3.167

3. Collapsing focal segmental glomerulosclerosis: Current concepts.

Authors: Muhammed Mubarak
Journal: World J Nephrol Date: 2012-04-06

4. Parietal epithelial cells participate in the formation of sclerotic lesions in focal segmental glomerulosclerosis.

Authors: Bart Smeets; Christoph Kuppe; Eva-Maria Sicking; Astrid Fuss; Peggy Jirak; Toin H van Kuppevelt; Karlhans Endlich; Jack F M Wetzels; Hermann-Josef Gröne; Jürgen Floege; Marcus J Moeller
Journal: J Am Soc Nephrol Date: 2011-06-30 Impact factor: 10.121

5. Development and validation of the HScore, a score for the diagnosis of reactive hemophagocytic syndrome.

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6. Clinical Features and Histology of Apolipoprotein L1-Associated Nephropathy in the FSGS Clinical Trial.

Authors: Jeffrey B Kopp; Cheryl A Winkler; Xiongce Zhao; Milena K Radeva; Jennifer J Gassman; Vivette D D'Agati; Cynthia C Nast; Changli Wei; Jochen Reiser; Lisa M Guay-Woodford; Martin R Pollak; Friedhelm Hildebrandt; Marva Moxey-Mims; Debbie S Gipson; Howard Trachtman; Aaron L Friedman; Frederick J Kaskel
Journal: J Am Soc Nephrol Date: 2015-01-08 Impact factor: 10.121

7. Renal Survival in Patients with Collapsing Compared with Not Otherwise Specified FSGS.

Authors: Louis-Philippe Laurin; Adil M Gasim; Vimal K Derebail; JulieAnne G McGregor; Jason M Kidd; Susan L Hogan; Caroline J Poulton; Randal K Detwiler; J Charles Jennette; Ronald J Falk; Patrick H Nachman
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Haemophagocytic lymphohistiocytosis with collapsing lupus podocytopathy as an unusual manifestation of systemic lupus erythematosus with APOL1 double-risk alleles.

1. Association of trypanolytic ApoL1 variants with kidney disease in African Americans.

2. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis.

3. Collapsing focal segmental glomerulosclerosis: Current concepts.

4. Parietal epithelial cells participate in the formation of sclerotic lesions in focal segmental glomerulosclerosis.

5. Development and validation of the HScore, a score for the diagnosis of reactive hemophagocytic syndrome.

6. Clinical Features and Histology of Apolipoprotein L1-Associated Nephropathy in the FSGS Clinical Trial.

7. Renal Survival in Patients with Collapsing Compared with Not Otherwise Specified FSGS.

8. Recruitment of podocytes from glomerular parietal epithelial cells.

Review 9. Haemophagocytic syndrome in rheumatic patients. A systematic review.

10. Innate immunity pathways regulate the nephropathy gene Apolipoprotein L1.

Review 1. APOL1 Nephropathy: From Genetics to Clinical Applications.

2. Lupus podocytopathy superimposed on diabetic glomerulosclerosis: A case report.

3. Histiocytic Glomerulopathy Associated With Hemophagocytic Lymphohistiocytosis.