05.15 - Kidney, Systemic 1 (Nichols) - PP + Handout Flashcards by Ryan Lee

What does factor H regulate

Complement

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Demographic of SLE

Black Female of child-bearing age

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Small vessel vasculitis causes

Focal necrotizing lesions with crescents, active urinary sediment, Rapid progression of kidney failure

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AI necrotizing granulomatous vasculitis of respiratory tract and cause of crescentic GN associated with C-ANCA

Wegener’s

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Small vessel vasculitis involving glomeruli usually causes

Pauci-immune Crescentic GN

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Causes of Thrombotic Microangiopathy includes ___ in children and ___ in adults

Hemolytic Uremic Syndrome, TTP

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Sudden onset of irritability, lethargy, weakness, pallor, and oliguria 5-10 days following gastroenteritis

HUS

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Most common form of Lupus Nephritis

Diffuse Proliferative (Class IV) - Severe disease

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Thrombotic Microangiopathy of Glomeruli in small children due to Shiga toxin from E Coli infection

Hemolytic Uremic Syndrome

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Ab’s in Microscopic Polyangiitis

P-ANCA (anti-mpo)

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What are Classes I, II, and III of Lupus Nephritis

Minimal Mesangial (rare), Mesangial Proliferative (15), Focal Proliferative (25)

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Effect of Microscopic Polyangiitis on kidneys

Glomerulonephritis

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First and second most common Classes/Patterns of GN

Diffuse Proliferative (50), then Focal Proliferative (25)

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Typical patient with TTP

Older adult - Subacute onset of malaise, faitigue, petechiae, pallor, confusion, nausea, abdominal pain, weakness

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Urine feature present in 100% of patients with SLE

Proteinuria

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Manifestation of renal involvement of SS

Mild renal dysfunction, Proteinuria, HTN; or Scleroderma Renal Crisis

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Which arteries show onion skinning in SS of kidneys

Interlobar arteries

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Histological findings in kidney in scleroderma

Onion skinning, Intimal and Medial proliferation, Fibrinoud Necrosis

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Which Class of Lupus Nephritis has significantly worse renal survivial rate

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Immediate consequence of PMN activation by ANCAs

Increased contact and adhesion with endothelial cells and vascular structures

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Most forms of vasculitis involve on

Arteries

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How do you differentiate between TMA and DIC

PT and PTT are normal in TMA, but prolonged in DIC

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There are granular deposits of ___ in ___ locations in most cases of SLE

Ig and Complement, Subepithelial, Mesangial, and Subendothelial locations

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Typical onset of HUS

Sudden irritability, lethargy, weakness, pallor, oliguria; 5-10 days following gastroenteritis

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Vascular changes in Scleroderma Renal Crisis associated with poorer outcome

Mucoid Intimal thickening and thrombosis

Patients with Wegener's develop necrotizing granulomatous inflammation in their \_\_\_\_, in addition to \_\_\_\_

Nose, Paranasal sinuses, and Lungs, in addition to Crescentic GN

What are Classes IV, V, and VI of Lupus Nephritis

Diffuse proliferative (50), Membranous (10), Advanced Sclerosing (?)

HUS + Fever and neurological dysfunction (seizures)

TTP

ANCA-associated GN is usually part of

syndrome with extra-renal signs and symptoms

For SLE, anti-dsDNA and anti-Sm Ab's are less ___ than Anti-nuclear, but much more

Less sensitive, but more specific

T/F: Pauci-immune GN may be ANCA-negative and can occur w/out extra-renal disease

TRUE

Prevalance of anti-nuclear auto-ab's in normal individuals

15%

90% of patients with ANCA-associated Crescentic GN have ___ before they develop symptoms of GN

Flu-like symptoms

PR3-ANCA =

Wegener's

Factor H mutation

Uncontrolled activation of complement with intravascular thrombosis --\> HUS

AI small vessel vasculitis cause of pauci-immune crescentic GN associated with P-ANCA

Microscopic Poly

Only approved tx of Lupus by FDA

Aspirin, Glucocorticoids, Hydroxychloroquine

Full House Immunofluorescence

In SLE, staining of deposits with antisera to all 3 Ig's, C3, and Cr

When both P-ANCA and C-ANCA are present, call it

MPO-ANCA

Finding MHA and Thrombocytopenia with no other explananation besides TTP should prompt

Plasmapharesis

Clincially, patients with Thrombotic Microangiopathies have what 3 things

Microangiopathic Hemolytic Anemia, Thrombocytopenia, Often renal failure

Tx of HUS

Transfusions, Dialysis, Supportive measures

Mortality for HUS

4% if treated, poor if not

Which class of Lupus Nephritis is severe? Which is nephrotic?

IV is severe, 5 is nephrotic

What is Onion Skinning? (in context of kidney in SS)

Concentric Sclerosing Intimal thickening of interlobar arteries

Which is ANCA+: PAN or Microscopic Polyangiitis

Microscopic Polyangiitis

A ____ event is likely necessary for endothelial injury with ANCA-associated vasculitis

Synergistic pro-inflammatory event (like exposure to TNF-alpha)

HUS is usually a complication of

intestinal infection of Shiga-toxin producing E Coli

New onset of accelerated arterial HTN and/or rapidly progressive oliguric renal failure

Scleroderma Renal Crisis

3 general locations affected by Wegener's

URT, LRT, Kidney

Cause of TTP

Deficiency of ADAMTS13 - Cleaving protease of vWF

Demographic of SS

Black women in their 50s

Tx of TTP

Plasmapharesis

Medium vessel arteritis (eg classic PAN) causes

renal infarcts and distal glomerular ischemia

Most common and characteristic forms of lupus nephritis involve

the glomeruli

Negative Ab (pauci-immune) is usually in the setting of

Crescentic GN

T/F: Pauci-immune means no Ab's

False, can still have ANCA's

T/F: Patients with Wegener's can also have P-ANCA

True, or be ANCA negative

Which is usually ANCA positive: Medium or Small vessel vasculitis

Small

Tx of SLE

Steroids, Immunosuppressants: Mycophenolate, Cyclo, MTX, Azathioprine

2 main types of Thrombotic Microangiopathies

HUS, TTP

Vasculitis in SLE

Acute necrotizing vasculitis of small arteries and arterioles with fibrinoid deposits

Extra-renal signs and symptoms in ANCA-associated GN

Athralgias, Arthritis, Myalgias, Fatigue

Fundamental pathogenesis of Thrombotic Microangiopathy

Loss of Thromboresistance by endothelial cells

Type of vasculitis caused by Wegener's

Necrotizing

Tx of Wegener's

Cyclophosphamide, Steroids, sometimes plasmapharesis

What are Hyaline thrombi

Misnomer of wire loop deposits that protrude into lumen

Microscopic Polyangiitis vs PAN effects on Kidney

MP causes GN, whereas PAN causes macroscopic ishcemia and infarction (thrombosis, aneurysm)

Tx of Scleroderma Renal Crisis

ACEi

Scleroderma Renal Crisis

New onset of accelerated HTN and/or rapidly progressive oliguric renal failure

Hemolytic Uremic Syndrome is triad of

Microangiopathic Hemolytic Anemia, Thrombocytopenia, AKI

Eculizamab is used for

Block complement activation (HUS subtype)

Systemic Sclerosis is characterized by

Fibrosis of CT and Vascular Occlusive Disease

Wegener's causes rapidly progressive

Crescentic GN

Microangiopathic Hemolytic Anemia, Thrombocytopenia, AKI

Hemolytic Uremic Syndrome is triad of

3 significant causes of endothelial damage

E Coli toxin (auto-Ab's), Chemo, Radiation

Fibrinoid Necrosis and Thrombosis in Scleroderma

Common

What has prolonged PT and PTT: TMA or DIC

DIC - Consumptive coagulopathy

2 features shared between TMA and DIC

Thrombocytopenia and Microangiopathic Hemolytic Anemia

PR3-ANCA is specific for Granulomatosis w Polyangiitis

True, 95%

Primary target in small vessel vasculitis

Endothelial cells

Effect of PAN on kidneys

Not GN - Macroscopic ischemia and infarction

Which arteries are affected by intimal and medial proliferatioin scleroderma renal crisis

Arcuate

Who is usually affected by HUS

Small children under 5 years

4 most common inflammatory manifestations of SLE

Non-erosive Synovitis (90), Skin lesions (85), Nephritis (50), Cerebritis (50)

2 major features of TTP

MHA, Thrombocytopenia

Thrombotic Microangiopathy must be differentiated from

DIC

Wire loop lesions are inidicative of

Active disease

What do ANCA's do to their targets in granulocytes

Activate neutrophils, which then adhere to endothelial cells; Also prevent inactivation of the targeted granulocyte componens (PR3, MPO, etc)

Which part of glomerulus is affected in SLE

All 3 locations

ANCA cause

Endothelial Cell injury in Glomeruli and Blood Vessels

Segmental Transmural Necrotizing Vasculitis

PAN

In about 10% of cases, HUS in children is due to

Inherited mutation that inactivates Factor H

P-ANCA may be positive in up to \_\_\_% of patients with Anti-GBM disease

30%

5 features of TTP

2 major: MHA, Thrombocytopenia; Others: Neurologic dysfunction, renal dysfunction, fever

What causes wire loops in SLE

Confluent circumferential Subendothelial deposits cause the glomerular capillary walls to be thickened