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Internal medicine III > 12. Vascular disorders of the kidney > Flashcards

12. Vascular disorders of the kidney Flashcards

1
Q

What are the disease affecting the renal arteries?

A
  • Renal artery stenosis
  • Cholesterol embolism
  • Vasculitis
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2
Q

What is renal artery stenosis and its causes?

A

It is a renovascular disease defined as stenosis of the renal artery or one of its branches

Causes:

  • Atherosclerosis (65%, usually elderly with comorbidities)
  • Fibromuscular dysplasia (35%, < 55-years-old)
  • Rare cases:
    • Takayasu arteritis
    • Antiphospholipid syndrome
    • Post-renal transplant
    • Thromboembolism
    • External compression
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3
Q

What are the hemodynamic consequences of renal artery stenosis?

A
  • Decreased RBF ==> decreased GFR
    • This activates compensatory mechanisms of RAAS ==> systemic hypertension
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4
Q

What are the signs of renal artery stenosis?

A
  • Rapid onset hypertension, resistant to treatment
  • Worsening of renal function after ACEI/ARB in bilateral renal artery stenosis
  • Pulmonary edema of unknown etiology
  • Abdominal and/or carotid or femoral bruits with weak leg pulse
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5
Q

What are the diagnostic tests of renal artery stenosis?

A
  • US: renal size asymmetry (affected side is smaller), disturbances in renal blood flow on doppler US
  • Dynamic renal isotope scan
  • CT/MR angiography (more sensitive)
  • Renal angiography
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6
Q

What is the treatment of renal artery stenosis?

A
  • Objective is to decrease the BP, prevent further loss of renal function and prevent cardiovascular events
    • Comprehensive antihypertensive therapy
      • ACEI, Calcium channel blockers, diuretics, statins, aspirin
    • Transluminal angioplasty and stent placement
    • Revascularization surgery (rare)
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7
Q

From where do cholesterol embolisms arise from?

A
  • May be released from atheromatous plaques (often aorta) –> lodges in microcirculation (renal vessels, peripheral circulation, gut) ==> ischemia!
    • An inflammatory response may lead to fever, myalgia and eosinophil accumulation
  • It is usually the result of endovascular procedures (arterial catheterization)
    • Either immediatly of 1-3 days after the procedure
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8
Q

What are the are the signs of cholesterol embolism?

A

Multisystemic disease:

  • Fever
  • Uncontrolled BP
  • Livedo reticularis
  • Oliguria and AKI
  • Gangrene
  • GI bleeds, ischemia and necrosis

The condition is often progressive and fatal, however, some patients regain renal function after dialysis.

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9
Q

What are vasculitides?

A
  • They are inflammatory disorders of the blood vessel walls ==> destruction (aneurysm/rupture) or stenosis
  • It may be a primary conditions or secondary due to autoimmune disorders, infections or malignant diseases
  • Affects vessels of any organ and the presentation depends on the organ affected
  • Different types prefferentially affect different organs, causing different patterns of symptoms
    • But, the presentation may often only be of overwhelming fatigue with high ESR/CRP.
  • Suspected in any patient presenting with multisystemic disease, which can’t be explained by an infectious process.
    • If presentation doesn’t fit clinically or serologically into a specific category –> Consider malignancy-associated vasculitis
  • A severe vasculitis flare-up is a medical emergency!!!
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10
Q

What is the affected organ presentation of vasculitis?

A
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11
Q

What is the classification of vasculitis?

A
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12
Q

How do we diagnose different vasculitides and how do we manage them?

A
  • Lab tests can help in the diagnosis:
    • ESR/CRP
    • ANCA
    • Creatinine
    • Urinalysis (proteinuria, hematuria, casts)
    • Biopsy (required!)

Management:

  • Steroids (large vessel vasculitis)
  • Combination of steroids + IV cyclophosphamide (medium/small-vessel vasculitis)
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13
Q

What is Giant cell (temporal) arteritis?

A
  • Granulomatous inflammation of the aorta and major branches (often superficial temporal artery) in patients > 50-years-old
  • Typically 2-year course and then complete remission

Symptoms:

  • Headache
  • Temporal artery and scalp tenderness
  • Jaw claudication
  • Sudden blindess (one eye typically)
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14
Q

What is Takayasu’s arteritis?

A
  • Affects more femaly < 50-years-old
  • Similar to Giant cell arteritis
    • Affect aorta and major branches
  • Pulseless disease
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15
Q

What is polyarteritis nodosa (PAN)?

A
  • It is a necrotizing vasculitis causing aneurysms and thrombosis in medium-sized arteries ==> infarction of an organ with severe systemic symptoms

​Symptoms:

  • Constitutional symptoms
  • It affects all organs except the lung
    • Skin rask and punched-out ulcers
    • Renal
    • Cardiac
    • GIT
    • GUT
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16
Q

What is kawazaki arteritis?

A
  • It is a childhood variant of PAN affecting mostly asian populations
  • It affects the coronary arteries and causes lymph node enlargements
17
Q

What is Henoch-Schönlein purpura?

A
  • Small-vessel vasculitis with dominant IgA immune complex deposits, which affect:
    • Skin
    • GIT
    • Glomeruli (nephritic syndrome or isolated hematuria)
    • Joint
18
Q

What is Wegener’s granulomatosis?

A
  • ≥ 40 years old
  • Necrotizing, granulomatous inflammation of the upper and lower respiratory organs
  • Necrotizing thrombogenic vasculitis of isolated glomeruli: glomerulonephritis
  • Necrotizing pneumonitis, chronic sinusitis, nasopharyngeal ulcerations, renal damage
  • Serum C-ANCA ­ (cytoplasmic anti-neutrophil cytoplasmic antibodies)
19
Q

What is cryoglobulinemia?

A
  • MPGN (nephritic syndrome) may be see in type II cryoglobulinemia
  • The blood contains large amounts of cryoglobulins – proteins (mostly immunoglobulins themselves) that become insoluble at reduced temperatures.
20
Q

What are the ANCA positive GN-producing vasculitides?

A
21
Q

Which are the diseases affecting renal capillaries?

A
  • Hypertensive nephrosclerosis
  • Thrombotic microangiopathies (TMA)
    • Hemolytic uremic syndrome (HUS)
    • Thrombotic thrombocytopenic purpura (TTP)
22
Q

What is hypertensive nephrosclerosis?

A
  • Long-standing benign hypertension leads to slow and progressive loss of renal function
  • Histology demonstrates:
    • Arteriolar thickening and hyalinization
    • Glomerulosclerosis
    • Interstitial inflammation and fibrosis.
  • Other organs are affected:
    • LVH
    • Retinal changes
    • Microalbuminuria
23
Q

What are thrombotic microangiopathies?

A
  • A group of acute syndrome characterized by:
    • Microangiopathic hemolytic anemia (MAHA)
    • Thrombocytopenia
    • Possible, renal (AKI) and CNS abnormalities
  • They share common pathophysiologies:
    • Inciting event triggers thrombosis, platelet consumption and fibrin strand deposition, mainly in renal vasculature
      • Leads to mechanical destruction of passing RBCs

Types:

  • Hemolytic uremic syndrome (HUS)
  • Thrombotic thrombocytopenic purpura (TTP)
  • Transplant nephropathy
  • Radiation nephropathy
  • Scleroderma renal crisis
  • Malignant HTN
  • Antiphospholipid syndrome
  • HELLP syndrome
24
Q

What is hemolytic uremic syndrome (HUS)?

A
  • In 90% of cases, E. Coli strain O157 is the incinting event as it produces shiga toxins that attack endothelial cells
    • Atypical cases can be caused by complement component deficiencies
  • It usually affects young children in outbreaks after eating undercooked contaminated meat

Symptoms:

  • Abdominal pain
  • Bloody diarrhea
  • AKI (hematuria/proteinuria)

Diagnosis:

  • Blood film shows
    • Fragmented RBCs (Schistocytes)
    • Decreased platelet count and Hb concentration

Management:

  • Dialysis may be required in case of AKI
  • Plasmapheresis is used if persistent disease

Prognosis:

  • Worse in non-E. Coli cases
  • Mortality is 3-5%, good prognosis if caught early
25
Q

What is thrombotic thrombocytopenic purpura (TTP)?

A
  • Genetic or acquired deficiency of ADAMTS13, which normally cleaves multimers of VWF
    • Idiopathic (40%)
    • Autoimmunity (e.g. SLE)
    • Cancer
    • Pregnancy
    • Drug-associated (e.g. quinine)
    • Bloody diarrhea prodrome
    • Hematopoietic stem cell transplant
  • The uncleaved VWF multimers can cause platelet aggregation and fibrin deposition in small vessels ==> microthrombi

Clinical features:

  • MAHA (with jaundice)
  • Low platelet
  • Sometimes, AKI, fluctuating CNS signs (seizures, hemiparesis, consciousness, vision)
  • Fever

Treatment:

  • It is a hemolytic emergency and urgent plasma exchange may be life-saving
  • Steroids are used in non-respondes
  • New therapies: eculizumab (target terminal complement pathway C5), rituximab
26
Q

What is the disease affecting renal veins?

A

Renal vein thrombosis (RVT)

27
Q

What is renal vein thrombosis (RVT)?

A
  • It is usually an asymptomatic condition and happens in the setting of nephrotic syndrome or malignancy
    • It may worsen proteinuria and renal function

Etiology:

  • Acute RVT may result from:
    • Trauma
    • Dehydration
    • Hypercoagulable state

Symptoms:

  • Flank pain
  • Hematuria
  • Increased serum LDH
  • Proteinuria
  • Renal failure (if bilateral)

Diagnosis:

  • US, MRI, CT, Venography

Treatment:

  • LMWH + anticoagulation therapy for 6-12 months
  • Catheter thrombectomy if acute RVT
  • Prophylactic anticoagulation is recommended for high risk patients (low albumin, heavy proteinuria)

Internal medicine III (26 decks)

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