Multisystem Disease Associated With Hematuria Flashcards
Multisystem Disease Associated With Hematuria etiology ……?
systemic infections, systemic lupus erythematosus, Henoch-Schönlein purpura nephritis, Goodpasture disease, hemolytic-uremic syndrome, nephrotoxicity, and renal cortical necrosis.
HIV-associated nephropathy features …..?
HIV-associated nephropathy, direct viral infection of nephrons occurs because renal cells express a variety of lymphocyte chemokine receptors that are essential for and facilitate viral invasion.
The renal expression of HIV infection is quite variable and includes an immune complex injury and a direct cytopathic effect.
The classic histopathologic lesion of HIV-associated nephropathy is focal segmental glomerulosclerosis.
Systemic lupus erythematosus (SLE) is a chronic autoimmune disease characterized by ….?
fever, weight loss, dermatitis, hematologic abnormalities, arthritis, and involvement of the heart, lungs, central nervous system, and kidneys
— — is the most important cause of morbidity and mortality in SLE.
Lupus nephritis
Lupus nephritis is a result of ….?
The deposition of circulating immune complexes, as well as the direct binding of autoantibodies to glomerular components with resultant complement stimulation.
WHO class I nephritis …..
Minimal mesangial lupus nephritis
No histologic abnormalities are detected on light microscopy but mesangial immune deposits are present on immunof luorescence or electron microscopy
WHO class II nephritis ……
Mesangial proliferative nephritis
Light microscopy shows both mesangial hypercellularity and an increased matrix, along with mesangial deposits containing immunoglobulin and complement
WHO class III nephritis ……?
Focal proliferative LN < 50% glomeruli involved
A. Active
A/C. Active and chronic
C. Chronic Lesions
characterized by both mesangial and endocapillary lesions.
WHO class IV nephritis ……..
Diffuse proliferative LN (>50% glomeruli involved); all may be with segmental or global involvement (S or G)
A. Active
A/C. Active and chronic
C. Chronic
WHO class V nephritis
Membranous lupus nephritis
WHO class VI ……?
Advanced sclerosing LN
Characteristics of class 4 lesion in LN
Other signs of active disease include capillary walls that are thickened secondary to subendothelial deposits (creating the characteristic wire-loop lesion), necrosis, and crescent formation
Most children with SLE are adolescent male or females …..?
females (female-to-male ratio of 5 : 1), and present with extrarenal manifestations
Lupus nephritis affects——% of pediatric patients with SLE………?
80%
And although it commonly presents within the first year of diagnosis, may occur at any time during the course of the disease
What is clinical remission in LN ….?
Clinical remission, defined as normalization of renal function and proteinuria, and
A serologic remission, defined as normalization of anti-DNA antibody, C3, and C4 levels.
For patients with more severe forms of nephritis (WHO classes III and IV) treatment choices ?..
More aggressive immunosuppressive regimens induction phase and a maintenance phase
The most commonly employed induction therapy in
LN 3 & 4 …..?
6 consecutive monthly intravenous infusions of cyclophosphamide at a dose of 500-1,000 mg/m2
Pulse intravenous methylprednisolone (1,000 mg/m2) is also used in addition to oral corticosteroids
Induction therapy in LN ?
mycophenolate mofetil was as efficacious as, or even superior to, cyclophosphamide, and is increasingly considered for use in children at a dosage of 600 mg/m2 per dose twice daily
Major side effects of mycophenolate mofetil …..?
diarrhea, leucopenia, and teratogenicity
Treatment for children with proliferative lupus nephritis refractory to steroids and other immunosuppressants …?
Rituximab, a chimeric monoclonal antibody specific for human CD20
Role of plasma paresis in SLE-LN ….?
Plasmapheresis is ineffective in lupus nephritis
unless there is accompanying thrombotic thrombocytopenic purpura or antineutrophilic cytoplasmic antibodyassociated disease
New therapies include in LN ?
Belimumab, a fully humanized monoclonal antibody against a type II transmembrane protein that functions in the normal survival and differentiation of B cells