Lupus nephritis is a severe organ manifestation of systemic lupus erythematosus (SLE) that can affect up to 70% of the SLE population. In childhood SLE, nephritis is present in up to 90% of the cases. This disease is one of the most serious complications of SLE, with a high morbidity and mortality. Clinical manifestations of active lupus nephritis include proteinuria, active urinary sediments, and progressive renal dysfunction. It is initiated by the deposition of immunoglobulins and formation of immune complexes in the glomerular and tubulo-interstitial compartments of the kidney. There is emerging evidence that the interaction between anti-dsDNA antibodies and kidney cells, notably mesangial cells, proximal renal tubular epithelial cells, and glomerular endothelial cells plays a significant role in disease pathogenesis.
Category
Immune system disease; Urinary system disease
Brite
Human diseases [BR:br08402]
Immune system diseases
Allergies and autoimmune diseases
H01500 Lupus nephritis
Urinary system diseases
Kidney diseases
H01500 Lupus nephritis
Human diseases in ICD-11 classification [BR:br08403]
04 Diseases of the immune system
Nonorgan specific systemic autoimmune disorders
4A40 Lupus erythematosus
H01500 Lupus nephritis
Drug
Voclosporin [DR:D09033]
Comment
See also H00080 Systemic lupus erythematosus.
Lupus nephritis is staged according to the classification revised by the International Society of Nephrology (ISN) and the Renal Pathology Society (RPS) in 2003.
Other DBs
ICD-11:
4A40.0Y
ICD-10:
M32.1 N08.5
MeSH:
D008181
OMIM:
607965 607966 607967
Reference
PMID:14747370
Authors
Weening JJ, D'Agati VD, Schwartz MM, Seshan SV, Alpers CE, Appel GB, Balow JE, Bruijn JA, Cook T, Ferrario F, Fogo AB, Ginzler EM, Hebert L, Hill G, Hill P, Jennette JC, Kong NC, Lesavre P, Lockshin M, Looi LM, Makino H, Moura LA, Nagata M
Title
The classification of glomerulonephritis in systemic lupus erythematosus revisited.
Journal
J Am Soc Nephrol 15:241-50 (2004) DOI:10.1097/01.ASN.0000108969.21691.5D
Reference
PMID:22761629
Authors
Yung S, Chan TM
Title
Autoantibodies and resident renal cells in the pathogenesis of lupus nephritis: getting to know the unknown.
Journal
Clin Dev Immunol 2012:139365 (2012) DOI:10.1155/2012/139365
Reference
PMID:12215904
Authors
Quintero-Del-Rio AI, Kelly JA, Kilpatrick J, James JA, Harley JB
Title
The genetics of systemic lupus erythematosus stratified by renal disease: linkage at 10q22.3 (SLEN1), 2q34-35 (SLEN2), and 11p15.6 (SLEN3).