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Renal Week 2 > The Kidney in Systemic Disease I > Flashcards

The Kidney in Systemic Disease I Flashcards

1
Q

What are some vascular injury syndromes?

A
  • ANCA-associated glomerulonephritis
  • Thrombotic microangiopathy
  • Lupus nephritis
  • Scleroderma
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2
Q

Describe how renal disease presents in medium vessel diseases like PAN?

A

You see renal infarcts due to distal glomerular ischemia that causes a decrease in GFR but is not associated with glomerular inflammation with RBC casts (usually ANCA negative)

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3
Q

Describe how renal disease presents in SMALL vessel diseases like Wegener’s and microscopic polyangiitis?

A

focal necrotizing lesions with crescent formation that have active urinary sediment and rapid progression of kidney failure (usually ANCA positive)

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4
Q

What vessels does PAN affect?

A

systemic segmental, necrotizing vasculiitis of medium-sized muscular arteries, not veins

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5
Q

When does PAN usually present?

A

most commonly in middle-aged or older adults

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6
Q

What causes PAN?

A

Usually idiopathic, although may be associated with Hep B, Hep C, and hairy cell leukemia

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7
Q

What is pauci-immune glomerulonephritis?

A

Refers to negative immunofluorescence studies usually in the setting of crescentic glomerulonephritis

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8
Q

What is pauci-immune glomerulonephritis typically associated with?

A

ANCAs with extrarenal findings (arthritis, athralgias, myalgias, fatigue)

ANCA titers may not always parallel disease activity

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9
Q

Which has diffuse cytoplasmic reactivity, C-ANCA (PR3) or P-ANCA (MPO)?

A

C-ANCA

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10
Q

What does a positive C-ANCA (PR3) suggest?

A

Positivity strongly suggests Granulomatosis with polyangiitis (AKA Wegener’s granulomatosis)

can be P-ANCA positive or C-ANCA neg however

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11
Q

Which has perinuclear cytoplasmic reactivity, C-ANCA (PR3) or P-ANCA (MPO)?

A

P-ANCA

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12
Q

Nonmyeloperoxidase P-ANCA has been detected in what diseases?

A
  • sclerosing cholangitis, -ulcerative colitis, and
  • Crohn’s disease
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13
Q

What are the two sinopulmonary renal syndrome?

A

Goodpastures and Wegener’s

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14
Q

What is the difference in the presentation of Wegener’s and Goodpastures?

A

At presentation, patients with Goodpasture’s disease will likely have significant renal dysfunction while it is much more mild in Wegener’s

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15
Q

How does Wegener’s present?

A
  • cough, dyspnea, hemopytsis
  • pulmonary infiltrates on x-ray
  • fever, weight loss, skin lesions
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16
Q

How is Wegener’s treated?

A

lots of immunosuppression- 3 doses of cyclophosphamide based regimens, steroids, and plasmapheresis depending on severity of disease

mortality is 80-90% if left untreated

17
Q

Does Wegener’s relapse often?

A

Yes, about 25-50% will relapse within 3-5 yrs

18
Q

HUS is a thrombotic microangiopathic disease associated with what clinical triad?

A
  • hemolytic anemia (schistocytes)
  • renal dysfunction
  • thrombocytopenia
19
Q

TTP is also a thrombotic microangiopathic disease associated with what clinical pentad?

A
  • Fever
  • Hemolytic anemia
  • Thrombocytopenia
  • Renal dysfunction
  • Neurologic dysfunction (eg. seizures)

need to treat early with plasmapheresis

20
Q

What does thrombotic microangiopathy cause?

A

loss of thromboresistance by endothelial cells leading to platelet activation and deposition of platelets and fibrin thrombi in the lumen of affected vessels giving the vessels an ‘onionskin’ appearance (not-specific- can see in malignant HTN)

21
Q

What things is HUS associated with?

A
  • verotoxin producing E. Coli H7:O157
  • chemo (cyclosporine, gemcitabine, bleomycin /cisplatinum)
  • radiation
22
Q

What causes TTP?

A

Increased levels of von Willebrand factor multimers due to ADAMTS 13 mutation may directly enhance platelet aggregation

TTP also occurs in the setting of autoimmune diseases (antiphospholipid syndrome) associated with autoantibodies to inhibitors of platelet aggregation

23
Q

How can you clinically differentiate between TMA vs DIC?

A

Prothrombin time and partial thromboplastin times are normal in TMA but prolonged in DIC

24
Q

Case. A 27-year-old AA woman with a 10-year history of systemic lupus erythematosus (SLE) but no previous history of renal disease presents with new onset of hematuria and proteinuria.

Previous manifestations of SLE included pericarditis, alopecia, a malar rash, and arthralgias.

Medications include prednisone 5 mg/day and hydroxychloroquine 200 mg/day.

Physical examination reveals a BP of 122/76, an erythematous facial rash, and no edema.

Laboratory results revealed serum creatinine 0.9 mg/dl, spot urine protein to creatinine ratio 2.9, serum albumin 2.8 g/dl, white blood cell count 2400/L, hemoglobin 10.5 g/dl, platelet count 127k/L, anti-nuclear antibody (ANA) titer 1:1280, anti-double stranded DNA antibody 500 IU/ml, low C3 and C4 complement levels.

Urine sediment showed dysmorphic RBCs, no significant white blood cells, and no cellular casts.

The kidneys measure 12.1 and 11.5 cm by ultrasound.

Renal biopsy was performed.

A

SLE

25
Q

What class of lupus has the worst prognosis?

A

Class IV

26
Q

How is SLE treated?

A
  • Aspirin
  • Glucocorticoids

-Immunosuppressive agents
IV Cyclophosphamide (Cytoxan)
Methotrexate
Azathioprine

-Mycophenolate mofetil (Cellcept, Myfortic) for class V
Inhibition of toll-like receptor

-Hydroxychloroquine (Plaquenil)
Hormone manipulation (dehydroepiandrosterone)
Modulation of cell signaling (tacrolimus, sirolimus)

Only aspirin, glucocorticoids, and hydroxychloroquine are approved by the FDA for the treatment of lupus.

27
Q

What is scleroderma?

A

AKA Systemic sclerosis
Involves the connective tissue and the microvasculature with fibrosis and vascular occlusion

Mild renal involvement is frequent, manifesting as mild renal dysfunction, proteinuria, and hypertension

28
Q

What patient population is scleroderma common in?

A

shows a female preponderance, and is more frequent in African-Americans

29
Q

What is the severity of scleroderma based on?

A

Patients are grouped into limited cutaneous (lc) or diffuse cutaneous (dc) SSc subsets based on the pattern of skin involvement

30
Q

Diffuse vs limited

A

Diffuse shows less Raynaud association, more constitutional early symptoms, and more severe skin involvement

31
Q

A patient with scleroderma with kidney involvement would probably be of what type?

A

diffuse cutaneous scleroderma

32
Q

What is scleroderma renal crisis?

A

new onset of accelerated arterial hypertension and/or rapidly progressive oliguric renal failure

33
Q

How is scleroderma renal crisis treated?

A

Treated with ACE inhibitors

34
Q

What are the risk factors for scleroderma renal crisis?

A
  • early diffuse systemic sclerosis
  • rapidly progressive skin disease
  • anti-RNA polymerase antibodies
  • corticosteroid therapy
35
Q

Where does scleroderma happen?

A

small arteries

Renal Week 2 (13 decks)

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