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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
Characteristic involvement in KD
Coronary artery involvement | (leads to thrombosis or aneurysm)
26
Buerger disease is..
Necrotizing vasulitis involving digits
27
Small vessel vasculitis's
* Wegners Granulomatosis (with polyangiitis) * Microscopic polyangiitis * Churg-Strauss Syndrome
28
Wegners granulomatosis with polyangiitiss aka...
"WeCners" 1. C (drawn on stick figure) = Nasopharynx, Lung, Kidney 2. C-ANCA 3. Cyclphosphamide (and CS)
29
Abnormal CXR findings
nodules fixed infiltrates cavities
30
UA findings for wegners
Microscopic hematuria
31
Labs for Wegners
**ANCA** (positive...associated with Proteinase 3 (PR3) **Biopsy** granulomatous inflammation (c-ANCA negative)
32
Churg-Strauss a.k.a
Eosinophilic granulomatosis with polyangiitis
33
\_\_\_ present in 90% of churg strauss
Asthma and Lung disease \*\*cardinal feature!
34
Churg strauss momst often have __ or \_\_\_\_
Myeloperoxidase (MPO) or p-ANCA (only 40-60% have ANCA)
35
When you see "eosinophilic pneumonia", think ....
Churg-strauss
36
5 Other manifestations of Churg strauss
Upper airwar and ear Skin nodules Cardio (HF, pericarditis) Thromboembolism Mononeuritis multiplex
37
Treatment for Churg strauss
CS Immunosuppressives
38
Goodpastures a.k.a
Anti-glomerular Basement Membrane Disease
39
Major manifestations of goodpastures
Rapid GN Pulmonary (hemorrhage)
40
Diagnosis of goodpastures
Demo of anti GBM antibodies (ELISA) Kidney biopsy ANCA (40% positive)
41
Cryoglobulinemic Vasculitis pathology
precipitation of blood proteins below 37d
42
\_\_\_\_ precipitate from serum and plasma \_\_\_ precipitates from plasma only
CryoGlobulin from Serum+Plasma Cryofibrinogen from Plasma
43
Types of cryoglobulins
1: Monoclonal (waldenstroms macroglobulinemia or MM) 2: Mixture of polyclonal and monoclonal (viral) 3: Polyclonal (connective tissue disease)
44
Cryoglobulinemic Vasculitis Manifestations
Hyperviscosity = Neuro Sx Cutaneous purpura + Raynauds GN Small Airway disease
45
Cryoglobulinemic Vasculitis labs
Handling is important! Obtained at 37, stored at 4
46
Cryoglobulinemic Vasculitis treatment
Type 1 (monoclonal) = treat underlying Type 2 (mixed) = underlyng AND immunosuppressive (CS, rituximab or cyclophosphamide)
47
HSP primary pathology
Immune vasculitis with IgA deposition
48
HSP primary patient
Kids 3-15
49
Major Sx of HSP
Palpable purpura (without TC or coagulopathy) Athritis Abdominal pain Renal (hematuria WITHOUT proteinuria)
50
defining condition for HSP
"Leucoclastic vasculitis accompanied by IgA immune complexes within affected organs"
51
Henoch Schonlein treatment (4)
* Supportive care * Transfusion (anemia) * Antihypertensives (if renal involvement) * NSAIDs \*\*Corticosteroids = controversial, immunosuppressives are generall not needed
52
Diagnosis for HSP
Kidney or Skin **biopsy** IgA test UA
53
HUV aka
Hypocomplementemic Urticarial Vasculitis
54
Like HSP, HUV also may present as..
leucoclastic vasculitis of small vessels (POSTCAPILLARY VENULES)
55
HUV two defining manifestations
Leucocytoclastic vasculitis + Urticacria
56
Labs for HUV
Elevated Sed rate, CRP Complement deficiency (C1q, C3/4, CH50) POSITIVE ANA
57
Tx for HUV
* Antihistamines (urticaria) * NSAIDs * Corticosteroids * Dapsone * Colchicine MMF, MTX, AZT, CsP for refractory disease
58
Variable vessel vasculitis's
Behcets + Cogan's
59
Behcets classification (Criteria)
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
Clinical manifestations of Behcets are d/t
Vasulitis affecting vessels of ALL sizes
61
Behcets Tx (7)
1. CS 2. Colchicine 3. AZT 4. Cycophosphamide 5. TNFa blockers 6. Thalidomide 7. Rituximab (CD20)
62
Cogans syndrome =
Interstitial keratitis of the eye CN8 dysfunction Vasculitis/Aortitis (small or large vessels)
63
Cogans Tx
Prednisone MTX MMF Cyclophosphamide Cyclosporine Tocilizumab (IL-6 blocker)
64
Single organ vasculitises
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
Good test for primary CNS vasculitis
**Biopsy** from involved area showing **granulomatous** inflammation
66
Tx for CNS vasculitis
High dose CS Cyclophosphamide
67
With CNS vasculiti, beware of
Reversible Cerebral Vasoconstriction Syndrome (RCVS) ...presents with thunderclap headache lik Subarach
68
Workup and Tx for RCVS
Head CT to exclude hemorrhage **Angiography = BEAD LIKE VASCULITIS** Tx = No proven treatment. **Calcium channel blokers** possible, but risk *watershed* infarct
69
Gold standard biopsy for... 1. TGCA 2. ANCA associated vasculitis 3. CNS vasculitis 4. Leucoclastic vasculitis
1. Temporal a 2. Kidney or Lung 3. Brain 4. Skin
70
Thromboangiitis obliterans =
Buerger's
71
Buergers defining characteristics Stereotypical patient?
Highly cellular + inflammatory occlusive thrombus with relative sparing of the blood vessel wall Male, jewish, smoker
72
Buergers associated with \_\_\_\_ involves \_\_\_\_
Smoking Severe digital ischemia \*\*as disease progresses, moves proximally (claudication)
73
Imaging for Beurgers
ANgio Echo to rule out cardiac source fo embolization
74
Treatment for Beurgers
Stop smoking and CCB's

ICM Exam 2- Rheumatology (13 decks)

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