Systemic disease with renal involvement Flashcards

1
Q

Mechanism of tissue injury in SLE

A

Type II hypersensitivity –> auto-antibodies
Type III hypersensitivity –> immune complex deposition
Antiphospholipid antibodies

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2
Q

Hematologic manifestations of SLE

A

Hemolytic anemia
Lymphopenia
Thrombocytopenia

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3
Q

Reason for hematologic manifestations of SLE

A

Auto-antibodies specific for RBCs, WBCs, and platelets –> opsonize cells and promote phagocytosis and lysis

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4
Q

Deposit description in class I lupus nephritis

A

Mesangial

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5
Q

Deposit description in class II lupus nephritis

A

Mesangial proliferative

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6
Q

Deposit description in class III lupus nephritis

A

Focal proliferative

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7
Q

Deposit description in class IV lupus nephritis

A

Diffuse proliferative

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8
Q

Deposit description in class V lupus nephritis

A

Membranous

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9
Q

Deposit description in class VI lupus nephritis

A

Advance sclerosing nephritis

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10
Q

Renal disease indicated by wire loop lesions on microscopy

A

Lupus nephritis, class II-VI

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11
Q

Immunofluorescence findings in lupus nephritis

A

IgG deposits in mesangium and capillary wall

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12
Q

Most important glomerular lesions in diabetic nephropathy

A

Capillary basement membrane thickening
Diffuse mesangial sclerosis
Nodular glomerulosclerosis

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13
Q

Nodules of PAS positive matrix in the periphery of the glomerulus

A

Kimmelstiel-Wilson nodules

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14
Q

Pathogenesis involves progressive glomerular hyperfiltration due to nonenzymatic glycosylation of proteins deposited on the glomerular basement membranes. Leads to thickening and deranged cytokines

A

Diabetic nephropathy

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15
Q

Severe renal hyaline arteriolosclerosis affecting both afferent and efferent arterioles, and tubular BM thickening on PAS stain.

A

Diabetic nephropathy

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16
Q

Type of amyloid fibril protein derived from a unique non-Ig protein made by the liver

A

Amyloid associated (AA)

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17
Q

Type of amyloid protein associated with chronic inflammatory conditions

A

Amyloid associated (AA)

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18
Q

Disease characterized by circulating autoantibodies against NCl domain of the alpha-3 chain of collagen type IV affecting BM of glomeruli and lung alveoli

A

Goodpasture’s syndrome

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19
Q

Systemic disorders that may cause secondary amyloidosis

A

Goodpasture syndrome
Microscopic polyangiitis
Wegener granulomatosis

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20
Q

Purpura on extensor surfaces of arms and legs, and on buttocks. Necrotizing vasculitis, abdominal manifestations, non-migratory arthralgia, and renal abnormalities.

A

Henoch-Schonlein purpura

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21
Q

Pathogenesis renal damage in Henoch-Schonlein purpura

A

IgA deposits in mesangium, sometimes with IgG and C3 deposits

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22
Q

ANCA type associated with granulomatosis with polyangiitis

A

PR3-ANCAs

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23
Q

Upper respiratory signs of granulomatosis with polyangiitis

A

Nasal septum perforation
Chronic sinusitis
Otitis media
Mucosal ulcerations of the nasopharynx

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24
Q

Lower respiratory signs of granulomatosis with polyangiitis

A

Persistent pneumonitis with bilateral nodular and cavitary infiltrates
Hemoptysis, cough, and dyspnea

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25
Q

ANCA type associated with eosinophilic granulomatosis with polyangiitis

A

MPO-ANCAs

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26
Q

Classic associations with eosinophilic granulomatosis with polyangiitis

A

Asthma
Allergic rhinitis
Lung infiltrates
Peripheral hypereosinophilia
Extravascular necrotizing granulomata

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27
Q

ANCA type associated with microscopic polyangiitis

A

MPO-ANCAs

28
Q

ANCA associated vasculitis syndrome associated with leukocytoclastic vasculitis

A

Microscopic polyangiitis

29
Q

Renal involvement in ANCA associated vasculitis syndrome

A

Rapidly progressive pauci immune type of crescentic glomerulonephritis

30
Q

Immunofluorescence findings in renal damage associated with ANCA associated vasculitis syndrome

A

Minimal to no deposits

31
Q

Electron microscopy findings in renal damage associated with ANCA associated vasculitis syndrome

A

Ruptures in the glomerular basement membrane

32
Q

Conditions that increase incidence and severity of benign nephrosclerosis

A

HTN and diabetes

33
Q

Renal vascular disorders

A

Benign nephrosclerosis
Malignant nephrosclerosis
Renal A stenosis
Thrombotic microangiopathies
Others

34
Q

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.

A

Benign nephroscelrosis

35
Q

Kidney biopsy shows hyaline arteriolosclerosis and fibroelastic hyperplasia. There is focal tubular atrophy, patchy ischemia, interstitial fibrosis, and variety of glomerular changes, including sclerosis.

A

Benign nephrosclerosis

36
Q

3 groups at increased risk of renal failure in benign nephroscerosis

A

African descent
More severe BP elevations
Diabetes

37
Q

Associated with the malignant or accelerated phase of HTN, often superimposed on pre-existing essential benign HTN.

A

Malignant nephrosclerosis

38
Q

Frequent cause of death from uremia in pts with scleroderma. Seen more in younger people, males, and those of African descent.

A

Malignant nephrosclerosis

39
Q

Blood work finding in malignant nephrosclerosis

A

Markedly increased plasma renin

40
Q

Renal vascular disorder initially characterized by proteinuria and/or hematuria, which is then followed by renal failure.

A

Malignant nephrosclerosis

41
Q

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.

A

Malignant nephrosclerosis

42
Q

Gross appearance of kidney in malignant nephrosclerosis

A

Petechial hemorrhages on the cortical surface due to rupture of arterioles and capillaries –> flea-bitten kidney

43
Q

Microscopic findings in malignant nephrosclerosis

A

Fibrinoid necrosis
Hyperplastic arteriolitis

44
Q

Description of hyperplastic arteriolitis

A

Smooth muscle and collagen concentric in vessels –> onion-skinning

45
Q

Two types of renal A stenosis

A

Occlusion by atheromatous plaque
Fibromuscular dysplasia

46
Q

Drug that can be used in renal A stenosis causing HTN due to increased renin

A

ACE inhibitor

47
Q

Two types of thrombotic microangiopathies

A

TTP
HUS

48
Q

Characteristics of TTP and HUS

A

Microangiopathic hemolytic anemia
Thrombocytopenia
Renal failure
Thombi in capillaries and arterioles

49
Q

Peripheral smear finding in microangiopathic hemolytic anemia, associated with HUS and TTP

A

Schistocytes

50
Q

Lab values in TTP and HUS

A

Normal CT, PT, and PTT
Normal or slightly elevated fibrin split products

51
Q

Thrombotic microangiopathy type that is predominantly CNS involvement, with renal involvement only in 50%

A

TTP

52
Q

Thrombotic microangiopathy type associated with consumption of food contaminated with bacteria producing shiga-like toxin

A

Typical HUS

53
Q

Thombotic microangiopathy type associated with inherited mutations of complement-regulatory proteins

A

Atypical HUS

54
Q

Acquired causes of endothelial injury that can cause atypical HUS

A

Antiphospholipid antibodies
Complications of pregnancy or OCP
Vascular renal diseases
Chemotherapeutic and immunosuppressive drugs

55
Q

Chemotherapeutic and immunosuppressive drugs that can cause atypical HUS

A

Mitomycin
Cyclosporin
Cisplatin
Gemcitabine
Radiation

56
Q

Vascular renal diseases that may cause atypical HUS

A

Scleroderma
HTN

57
Q

Deficiency often associated with TTP

A

ADAMTS13

58
Q

Plasma metalloprotease that regulates the function of vWF

A

ADAMTS13

59
Q

Primary cause of HUS

A

Endothelial injury

60
Q

Inciting event in TTP

A

Platelet activation and aggregation

61
Q

Gross morphology features in TTP and HUS

A

Patchy cortical necrosis
Subcapsular petechiae

62
Q

Microscopic features of TTP and HUS

A

Fibrin thrombi in glomerular capillaries
Mesagniolysis

63
Q

Fibrinoid necrosis of interlobular arteries and arterioles with splitting (tram-track) appearance of GBM

A

Mesangiolysis - seen in HUS and TTP

64
Q

Types of other vascular disorders

A

Atherosclerotic ischemic renal disease
Atheroembolic renal disease
Diffuse cortical necrosis
Sickle cell nephropathy
Renal infarcts

65
Q

General signs and symptoms of renal vascular disorders

A

Hematuria
Patchy papillary necrosis
Proteinuria

66
Q

Risk factor for diffuse cortical necrosis

A

Obstetric emergency
Septic shock
Extensive surgery