Systemic disease with renal involvement Flashcards
Mechanism of tissue injury in SLE
Type II hypersensitivity –> auto-antibodies
Type III hypersensitivity –> immune complex deposition
Antiphospholipid antibodies
Hematologic manifestations of SLE
Hemolytic anemia
Lymphopenia
Thrombocytopenia
Reason for hematologic manifestations of SLE
Auto-antibodies specific for RBCs, WBCs, and platelets –> opsonize cells and promote phagocytosis and lysis
Deposit description in class I lupus nephritis
Mesangial
Deposit description in class II lupus nephritis
Mesangial proliferative
Deposit description in class III lupus nephritis
Focal proliferative
Deposit description in class IV lupus nephritis
Diffuse proliferative
Deposit description in class V lupus nephritis
Membranous
Deposit description in class VI lupus nephritis
Advance sclerosing nephritis
Renal disease indicated by wire loop lesions on microscopy
Lupus nephritis, class II-VI
Immunofluorescence findings in lupus nephritis
IgG deposits in mesangium and capillary wall
Most important glomerular lesions in diabetic nephropathy
Capillary basement membrane thickening
Diffuse mesangial sclerosis
Nodular glomerulosclerosis
Nodules of PAS positive matrix in the periphery of the glomerulus
Kimmelstiel-Wilson nodules
Pathogenesis involves progressive glomerular hyperfiltration due to nonenzymatic glycosylation of proteins deposited on the glomerular basement membranes. Leads to thickening and deranged cytokines
Diabetic nephropathy
Severe renal hyaline arteriolosclerosis affecting both afferent and efferent arterioles, and tubular BM thickening on PAS stain.
Diabetic nephropathy
Type of amyloid fibril protein derived from a unique non-Ig protein made by the liver
Amyloid associated (AA)
Type of amyloid protein associated with chronic inflammatory conditions
Amyloid associated (AA)
Disease characterized by circulating autoantibodies against NCl domain of the alpha-3 chain of collagen type IV affecting BM of glomeruli and lung alveoli
Goodpasture’s syndrome
Systemic disorders that may cause secondary amyloidosis
Goodpasture syndrome
Microscopic polyangiitis
Wegener granulomatosis
Purpura on extensor surfaces of arms and legs, and on buttocks. Necrotizing vasculitis, abdominal manifestations, non-migratory arthralgia, and renal abnormalities.
Henoch-Schonlein purpura
Pathogenesis renal damage in Henoch-Schonlein purpura
IgA deposits in mesangium, sometimes with IgG and C3 deposits
ANCA type associated with granulomatosis with polyangiitis
PR3-ANCAs
Upper respiratory signs of granulomatosis with polyangiitis
Nasal septum perforation
Chronic sinusitis
Otitis media
Mucosal ulcerations of the nasopharynx
Lower respiratory signs of granulomatosis with polyangiitis
Persistent pneumonitis with bilateral nodular and cavitary infiltrates
Hemoptysis, cough, and dyspnea
ANCA type associated with eosinophilic granulomatosis with polyangiitis
MPO-ANCAs
Classic associations with eosinophilic granulomatosis with polyangiitis
Asthma
Allergic rhinitis
Lung infiltrates
Peripheral hypereosinophilia
Extravascular necrotizing granulomata
ANCA type associated with microscopic polyangiitis
MPO-ANCAs
ANCA associated vasculitis syndrome associated with leukocytoclastic vasculitis
Microscopic polyangiitis
Renal involvement in ANCA associated vasculitis syndrome
Rapidly progressive pauci immune type of crescentic glomerulonephritis
Immunofluorescence findings in renal damage associated with ANCA associated vasculitis syndrome
Minimal to no deposits
Electron microscopy findings in renal damage associated with ANCA associated vasculitis syndrome
Ruptures in the glomerular basement membrane
Conditions that increase incidence and severity of benign nephrosclerosis
HTN and diabetes
Renal vascular disorders
Benign nephrosclerosis
Malignant nephrosclerosis
Renal A stenosis
Thrombotic microangiopathies
Others
Sclerosis of renal arterioles and small arteries leading resulting in focal ischemia leading to scars. The gross kidney shows fine granularity of cortical surfaces due to underlying subcortical scars.
Benign nephroscelrosis
Kidney biopsy shows hyaline arteriolosclerosis and fibroelastic hyperplasia. There is focal tubular atrophy, patchy ischemia, interstitial fibrosis, and variety of glomerular changes, including sclerosis.
Benign nephrosclerosis
3 groups at increased risk of renal failure in benign nephroscerosis
African descent
More severe BP elevations
Diabetes
Associated with the malignant or accelerated phase of HTN, often superimposed on pre-existing essential benign HTN.
Malignant nephrosclerosis
Frequent cause of death from uremia in pts with scleroderma. Seen more in younger people, males, and those of African descent.
Malignant nephrosclerosis
Blood work finding in malignant nephrosclerosis
Markedly increased plasma renin
Renal vascular disorder initially characterized by proteinuria and/or hematuria, which is then followed by renal failure.
Malignant nephrosclerosis
Injury to renal arteriolar walls leads to increased permeability and endothelial injury. Leads to fibrinoid necrosis of arterioles and small arteries, and hyperplastic arteriolosclerosis. Triggers RAAS, leading to a self-perpetuating cycle.
Malignant nephrosclerosis
Gross appearance of kidney in malignant nephrosclerosis
Petechial hemorrhages on the cortical surface due to rupture of arterioles and capillaries –> flea-bitten kidney
Microscopic findings in malignant nephrosclerosis
Fibrinoid necrosis
Hyperplastic arteriolitis
Description of hyperplastic arteriolitis
Smooth muscle and collagen concentric in vessels –> onion-skinning
Two types of renal A stenosis
Occlusion by atheromatous plaque
Fibromuscular dysplasia
Drug that can be used in renal A stenosis causing HTN due to increased renin
ACE inhibitor
Two types of thrombotic microangiopathies
TTP
HUS
Characteristics of TTP and HUS
Microangiopathic hemolytic anemia
Thrombocytopenia
Renal failure
Thombi in capillaries and arterioles
Peripheral smear finding in microangiopathic hemolytic anemia, associated with HUS and TTP
Schistocytes
Lab values in TTP and HUS
Normal CT, PT, and PTT
Normal or slightly elevated fibrin split products
Thrombotic microangiopathy type that is predominantly CNS involvement, with renal involvement only in 50%
TTP
Thrombotic microangiopathy type associated with consumption of food contaminated with bacteria producing shiga-like toxin
Typical HUS
Thombotic microangiopathy type associated with inherited mutations of complement-regulatory proteins
Atypical HUS
Acquired causes of endothelial injury that can cause atypical HUS
Antiphospholipid antibodies
Complications of pregnancy or OCP
Vascular renal diseases
Chemotherapeutic and immunosuppressive drugs
Chemotherapeutic and immunosuppressive drugs that can cause atypical HUS
Mitomycin
Cyclosporin
Cisplatin
Gemcitabine
Radiation
Vascular renal diseases that may cause atypical HUS
Scleroderma
HTN
Deficiency often associated with TTP
ADAMTS13
Plasma metalloprotease that regulates the function of vWF
ADAMTS13
Primary cause of HUS
Endothelial injury
Inciting event in TTP
Platelet activation and aggregation
Gross morphology features in TTP and HUS
Patchy cortical necrosis
Subcapsular petechiae
Microscopic features of TTP and HUS
Fibrin thrombi in glomerular capillaries
Mesagniolysis
Fibrinoid necrosis of interlobular arteries and arterioles with splitting (tram-track) appearance of GBM
Mesangiolysis - seen in HUS and TTP
Types of other vascular disorders
Atherosclerotic ischemic renal disease
Atheroembolic renal disease
Diffuse cortical necrosis
Sickle cell nephropathy
Renal infarcts
General signs and symptoms of renal vascular disorders
Hematuria
Patchy papillary necrosis
Proteinuria
Risk factor for diffuse cortical necrosis
Obstetric emergency
Septic shock
Extensive surgery