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ICM Exam 2- Rheumatology > Vasculitis > Flashcards

Vasculitis Flashcards

1
Q

Vasculitis classification based on __ and ___

A

blood vessel size and presence of ANCA

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2
Q

Takayasu presenting patient

Symptoms

A

(young asian female)

Weak/absent UE pulse, Elevated ESR

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3
Q

Takayasu tx

A

High dose CS

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4
Q

Takayasu test/imaging

A

aortigram to visualize occlusion or narrowing of Aorta or branches

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5
Q

Giant cell arteritis presenting patient

A

older female (>50)

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6
Q

Giant cell arteritis aka

A

Temporal GCA

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7
Q

Chief Sx for TGCA

A
  • Headache
  • Jaw claudication
  • Vision loss

(each based on arteritis of the supplying vessel)

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8
Q

TGCA associated with elevated

A

ESR

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9
Q

TGCA vessel appearance

A

Segmental lesions

need LONG segment in biopsy

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10
Q

___ does not rule out TGCA

A

negative biopsy

(due to segmental nature of the lesions)

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11
Q

TGCA can be associated with ______

A

polymyalgia rheumatica

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12
Q

Which vasculitis’s are granulomatous and which are necrotizing

A

Temporal GCA + Takayasu = granuloma

Polyarteritis nodosa + Kawasaki= Necrotizing

Wegners = necrotizing granulomas

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13
Q

Polymyalgia rheumatica Symptoms and sensitivity

A

Sudden onset pain/stiffness in shoulders and hips

Sensitive to prednisone

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14
Q

Gold standard Dx for TGCA

A

temporal artery biopsy

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15
Q

Polyarteritis nodosa involves ___ organs, spares ____

A

multiple organs, spares LUNGS

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16
Q

Polyarteritis nodosa classically presents in ___ as…

A

young adults as:

  • HTN
  • Abdominal pain with melena
  • Neuro disturbance
  • Skin lesions
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17
Q

Skin symptoms of PAN

A

Leucoclastic vasculitis

Livedo reticularis

nodules

Bullous/vesicular eruption

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18
Q

Leucoclastic vasulitis seen as …

A

palpable purpura

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19
Q

Most common involved organ in PAN

A

Kidney

Elevated BUN/Cr/BP

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20
Q

6 Labs for PAN

A
  1. Acute phase
  2. Complement
  3. HBsAg (positive in 10-50%)
  4. Negative ANCA
  5. Angiography
  6. Biopsy of sural nerve, testicle, skin nodules
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21
Q

Treatment for PAN

A
  • CS
  • Cyclophosphamide
  • Plasmapheresis

(also IFN and lamivudine for HBV)

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22
Q

Kawasaki disease classically affects

A

asian children under 4

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23
Q

4 Associated Sx with KD

A
  • Fever
  • Conjunctivitis
  • Lymphadenopathy
  • Indurated edema on hand/foot dorsum, diffuse erythema of the palms and soles
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24
Q

KD treatment

A
  • IVIG within 10 days!
  • Aspirin
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25
Q

Characteristic involvement in KD

A

Coronary artery involvement

(leads to thrombosis or aneurysm)

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26
Q

Buerger disease is..

A

Necrotizing vasulitis involving digits

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27
Q

Small vessel vasculitis’s

A
  • Wegners Granulomatosis (with polyangiitis)
  • Microscopic polyangiitis
  • Churg-Strauss Syndrome
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28
Q

Wegners granulomatosis with polyangiitiss aka…

A

“WeCners”

  1. C (drawn on stick figure) = Nasopharynx, Lung, Kidney
  2. C-ANCA
  3. Cyclphosphamide (and CS)
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29
Q

Abnormal CXR findings

A

nodules

fixed infiltrates

cavities

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30
Q

UA findings for wegners

A

Microscopic hematuria

31
Q

Labs for Wegners

A

ANCA (positive…associated with Proteinase 3 (PR3)

Biopsy granulomatous inflammation

(c-ANCA negative)

32
Q

Churg-Strauss a.k.a

A

Eosinophilic granulomatosis with polyangiitis

33
Q

___ present in 90% of churg strauss

A

Asthma and Lung disease

**cardinal feature!

34
Q

Churg strauss momst often have __ or ____

A

Myeloperoxidase (MPO)

or

p-ANCA

(only 40-60% have ANCA)

35
Q

When you see “eosinophilic pneumonia”, think ….

A

Churg-strauss

36
Q

5 Other manifestations of Churg strauss

A

Upper airwar and ear

Skin nodules

Cardio (HF, pericarditis)

Thromboembolism

Mononeuritis multiplex

37
Q

Treatment for Churg strauss

A

CS

Immunosuppressives

38
Q

Goodpastures a.k.a

A

Anti-glomerular Basement Membrane Disease

39
Q

Major manifestations of goodpastures

A

Rapid GN

Pulmonary (hemorrhage)

40
Q

Diagnosis of goodpastures

A

Demo of anti GBM antibodies (ELISA)

Kidney biopsy

ANCA (40% positive)

41
Q

Cryoglobulinemic Vasculitis pathology

A

precipitation of blood proteins below 37d

42
Q

____ precipitate from serum and plasma

___ precipitates from plasma only

A

CryoGlobulin from Serum+Plasma

Cryofibrinogen from Plasma

43
Q

Types of cryoglobulins

A

1: Monoclonal (waldenstroms macroglobulinemia or MM)
2: Mixture of polyclonal and monoclonal (viral)
3: Polyclonal (connective tissue disease)

44
Q

Cryoglobulinemic Vasculitis Manifestations

A

Hyperviscosity = Neuro Sx

Cutaneous purpura + Raynauds

GN

Small Airway disease

45
Q

Cryoglobulinemic Vasculitis labs

A

Handling is important!

Obtained at 37, stored at 4

46
Q

Cryoglobulinemic Vasculitis treatment

A

Type 1 (monoclonal) = treat underlying

Type 2 (mixed) = underlyng AND immunosuppressive (CS, rituximab or cyclophosphamide)

47
Q

HSP primary pathology

A

Immune vasculitis with IgA deposition

48
Q

HSP primary patient

A

Kids 3-15

49
Q

Major Sx of HSP

A

Palpable purpura (without TC or coagulopathy)

Athritis

Abdominal pain

Renal (hematuria WITHOUT proteinuria)

50
Q

defining condition for HSP

A

“Leucoclastic vasculitis accompanied by IgA immune complexes within affected organs”

51
Q

Henoch Schonlein treatment (4)

A
  • Supportive care
  • Transfusion (anemia)
  • Antihypertensives (if renal involvement)
  • NSAIDs

**Corticosteroids = controversial, immunosuppressives are generall not needed

52
Q

Diagnosis for HSP

A

Kidney or Skin biopsy

IgA test

UA

53
Q

HUV aka

A

Hypocomplementemic Urticarial Vasculitis

54
Q

Like HSP, HUV also may present as..

A

leucoclastic vasculitis of small vessels

(POSTCAPILLARY VENULES)

55
Q

HUV two defining manifestations

A

Leucocytoclastic vasculitis + Urticacria

56
Q

Labs for HUV

A

Elevated Sed rate, CRP

Complement deficiency (C1q, C3/4, CH50)

POSITIVE ANA

57
Q

Tx for HUV

A
  • Antihistamines (urticaria)
  • NSAIDs
  • Corticosteroids
  • Dapsone
  • Colchicine

MMF, MTX, AZT, CsP for refractory disease

58
Q

Variable vessel vasculitis’s

A

Behcets + Cogan’s

59
Q

Behcets classification (Criteria)

A

Aphthous ulcer PLUS TWO OF:

  • reurrent genital ulcer
  • eye lesions (ant. uviitis, retinal vasculitis)
  • skin lesions (erythema nodosum-like lesions)
  • Pathergy test (pustulous lesions 2d after skin prick)
60
Q

Clinical manifestations of Behcets are d/t

A

Vasulitis affecting vessels of ALL sizes

61
Q

Behcets Tx (7)

A
  1. CS
  2. Colchicine
  3. AZT
  4. Cycophosphamide
  5. TNFa blockers
  6. Thalidomide
  7. Rituximab (CD20)
62
Q

Cogans syndrome =

A

Interstitial keratitis of the eye

CN8 dysfunction

Vasculitis/Aortitis (small or large vessels)

63
Q

Cogans Tx

A

Prednisone

MTX

MMF

Cyclophosphamide

Cyclosporine

Tocilizumab (IL-6 blocker)

64
Q

Single organ vasculitises

A
  1. Cutaneous Leucocytoclastic Angiitis
    1. (classic appearance of drug associated immune complex)
  2. Primary CNS Vasculitis
    1. Small and medium size
    2. All kinds of CNS sx (headache, stroke, seizure)
    3. Diagnosis = Exclusion!
    4. Acute phase normal,
    5. CSF labs and MRI abnormal
65
Q

Good test for primary CNS vasculitis

A

Biopsy from involved area showing granulomatous inflammation

66
Q

Tx for CNS vasculitis

A

High dose CS

Cyclophosphamide

67
Q

With CNS vasculiti, beware of

A

Reversible Cerebral Vasoconstriction Syndrome (RCVS)

…presents with thunderclap headache lik Subarach

68
Q

Workup and Tx for RCVS

A

Head CT to exclude hemorrhage

Angiography = BEAD LIKE VASCULITIS

Tx = No proven treatment. Calcium channel blokers possible, but risk watershed infarct

69
Q

Gold standard biopsy for…

  1. TGCA
  2. ANCA associated vasculitis
  3. CNS vasculitis
  4. Leucoclastic vasculitis
A
  1. Temporal a
  2. Kidney or Lung
  3. Brain
  4. Skin
70
Q

Thromboangiitis obliterans =

A

Buerger’s

71
Q

Buergers defining characteristics

Stereotypical patient?

A

Highly cellular + inflammatory occlusive thrombus with relative sparing of the blood vessel wall

Male, jewish, smoker

72
Q

Buergers associated with ____

involves ____

A

Smoking

Severe digital ischemia

**as disease progresses, moves proximally (claudication)

73
Q

Imaging for Beurgers

A

ANgio

Echo to rule out cardiac source fo embolization

74
Q

Treatment for Beurgers

A

Stop smoking and CCB’s

ICM Exam 2- Rheumatology (13 decks)

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