Systemic disease with renal involvement

Question 1

Mechanism of tissue injury in SLE

Answer 1

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

Question 2

Hematologic manifestations of SLE

Answer 2

Hemolytic anemia
Lymphopenia
Thrombocytopenia

Question 3

Reason for hematologic manifestations of SLE

Answer 3

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

Question 4

Deposit description in class I lupus nephritis

Answer 4

Mesangial

Question 5

Deposit description in class II lupus nephritis

Answer 5

Mesangial proliferative

Question 6

Deposit description in class III lupus nephritis

Answer 6

Focal proliferative

Question 7

Deposit description in class IV lupus nephritis

Answer 7

Diffuse proliferative

Question 8

Deposit description in class V lupus nephritis

Answer 8

Membranous

Question 9

Deposit description in class VI lupus nephritis

Answer 9

Advance sclerosing nephritis

Question 10

Renal disease indicated by wire loop lesions on microscopy

Answer 10

Lupus nephritis, class II-VI

Question 11

Immunofluorescence findings in lupus nephritis

Answer 11

IgG deposits in mesangium and capillary wall

Question 12

Most important glomerular lesions in diabetic nephropathy

Answer 12

Capillary basement membrane thickening
Diffuse mesangial sclerosis
Nodular glomerulosclerosis

Question 13

Nodules of PAS positive matrix in the periphery of the glomerulus

Answer 13

Kimmelstiel-Wilson nodules

Question 14

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

Answer 14

Diabetic nephropathy

Question 15

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

Answer 15

Diabetic nephropathy

Question 16

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

Answer 16

Amyloid associated (AA)

Question 17

Type of amyloid protein associated with chronic inflammatory conditions

Answer 17

Amyloid associated (AA)

Question 18

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

Answer 18

Goodpasture’s syndrome

Question 19

Systemic disorders that may cause secondary amyloidosis

Answer 19

Goodpasture syndrome
Microscopic polyangiitis
Wegener granulomatosis

Question 20

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

Answer 20

Henoch-Schonlein purpura

Question 21

Pathogenesis renal damage in Henoch-Schonlein purpura

Answer 21

IgA deposits in mesangium, sometimes with IgG and C3 deposits

Question 22

ANCA type associated with granulomatosis with polyangiitis

Answer 22

PR3-ANCAs

Question 23

Upper respiratory signs of granulomatosis with polyangiitis

Answer 23

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

Question 24

Lower respiratory signs of granulomatosis with polyangiitis

Answer 24

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

Question 25

ANCA type associated with eosinophilic granulomatosis with polyangiitis

Answer 25

MPO-ANCAs

Question 26

Classic associations with eosinophilic granulomatosis with polyangiitis

Answer 26

Asthma Allergic rhinitis Lung infiltrates Peripheral hypereosinophilia Extravascular necrotizing granulomata

Question 27

ANCA type associated with microscopic polyangiitis

Answer 27

MPO-ANCAs

Question 28

ANCA associated vasculitis syndrome associated with leukocytoclastic vasculitis

Answer 28

Microscopic polyangiitis

Question 29

Renal involvement in ANCA associated vasculitis syndrome

Answer 29

Rapidly progressive pauci immune type of crescentic glomerulonephritis

Question 30

Immunofluorescence findings in renal damage associated with ANCA associated vasculitis syndrome

Answer 30

Minimal to no deposits

Question 31

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

Answer 31

Ruptures in the glomerular basement membrane

Question 32

Conditions that increase incidence and severity of benign nephrosclerosis

Answer 32

HTN and diabetes

Question 33

Renal vascular disorders

Answer 33

Benign nephrosclerosis Malignant nephrosclerosis Renal A stenosis Thrombotic microangiopathies Others

Question 34

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.

Answer 34

Benign nephroscelrosis

Question 35

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

Answer 35

Benign nephrosclerosis

Question 36

3 groups at increased risk of renal failure in benign nephroscerosis

Answer 36

African descent More severe BP elevations Diabetes

Question 37

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

Answer 37

Malignant nephrosclerosis

Question 38

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

Answer 38

Malignant nephrosclerosis

Question 39

Blood work finding in malignant nephrosclerosis

Answer 39

Markedly increased plasma renin

Question 40

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

Answer 40

Malignant nephrosclerosis

Question 41

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.

Answer 41

Malignant nephrosclerosis

Question 42

Gross appearance of kidney in malignant nephrosclerosis

Answer 42

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

Question 43

Microscopic findings in malignant nephrosclerosis

Answer 43

Fibrinoid necrosis Hyperplastic arteriolitis

Question 44

Description of hyperplastic arteriolitis

Answer 44

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

Question 45

Two types of renal A stenosis

Answer 45

Occlusion by atheromatous plaque Fibromuscular dysplasia

Question 46

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

Answer 46

ACE inhibitor

Question 47

Two types of thrombotic microangiopathies

Answer 47

TTP HUS

Question 48

Characteristics of TTP and HUS

Answer 48

Microangiopathic hemolytic anemia Thrombocytopenia Renal failure Thombi in capillaries and arterioles

Question 49

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

Answer 49

Schistocytes

Question 50

Lab values in TTP and HUS

Answer 50

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

Question 51

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

Answer 51

TTP

Question 52

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

Answer 52

Typical HUS

Question 53

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

Answer 53

Atypical HUS

Question 54

Acquired causes of endothelial injury that can cause atypical HUS

Answer 54

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

Question 55

Chemotherapeutic and immunosuppressive drugs that can cause atypical HUS

Answer 55

Mitomycin Cyclosporin Cisplatin Gemcitabine Radiation

Question 56

Vascular renal diseases that may cause atypical HUS

Answer 56

Scleroderma HTN

Question 57

Deficiency often associated with TTP

Answer 57

ADAMTS13

Question 58

Plasma metalloprotease that regulates the function of vWF

Answer 58

ADAMTS13

Question 59

Primary cause of HUS

Answer 59

Endothelial injury

Question 60

Inciting event in TTP

Answer 60

Platelet activation and aggregation

Question 61

Gross morphology features in TTP and HUS

Answer 61

Patchy cortical necrosis Subcapsular petechiae

Question 62

Microscopic features of TTP and HUS

Answer 62

Fibrin thrombi in glomerular capillaries Mesagniolysis

Question 63

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

Answer 63

Mesangiolysis - seen in HUS and TTP

Question 64

Types of other vascular disorders

Answer 64

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

Question 65

General signs and symptoms of renal vascular disorders

Answer 65

Hematuria Patchy papillary necrosis Proteinuria

Question 66

Risk factor for diffuse cortical necrosis

Answer 66

Obstetric emergency Septic shock Extensive surgery