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Flashcards in Vasculitides Deck (18)
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1

Polyarteritis Nodosa:
-what is this?
-cause
-pathogenesis

What: necrotizing arteritis of medium-sized vessels. MC involves the skin, peripheral nerves, mesenteric vessels, heart, and brain but can affect any organ

Cause: idiopathic but may be related to hep B and C as well as hairy cell leukemia

Pathogenesis:
-immune complex formation, thickening of inflamed vessel wall leading to luminal narrowing.
- reduced blood flow and thrombosis resulting in ischemia to the involved organ.
-inflammation can also cause weakening of vessel wall (aneurysm)
*does not involve veins

2

Polyarteritis nodosa:
-sx
-signs
-skin manifestations

Sx: fatigue, wt loss*, weakness, fever*, arthralgias, malaise*

Signs:
-skin lesions, htn, renal insufficiency, neurologic dysfunction, abd pain of multisystem involvement.

Skin manifestation:
-tender erythematous nodules
-purpura*
-livedo reticularis*
-ulcers (infarction, gangrene)*
-bullous or vesicular eruption
*may be focal or diffuse
*more common in the lower extremities

3

Renal manifestations of polyarteritis nodosa

UA results of polyerteritis nodosa affecting the kidneys.

Renal insufficiency and HTN

Perirenal hematoma from rupture of renal arteries

Luminal narrowing leads to glomerular ischemia

UA: minimal protein, moderate hematuria, NO RBC CASTS!

4

Neurologic manifestations of polyangitis nodosa

motor and sensory deficits of the involved nerves

generally asymmetric neuropathy at onset.

5

GI manifestations of polyarteritis nodosa

Abd pain with mesenteric arteritis
--post prandial pain, wt loss, bowel infarction with perforation

Nausea

Vomiting

Melena

Diarrhea

GI bleeding

6

Cardiovascular manifestations of Polyarteritis nodosa?

Coronary artery dz

Heart failure; from vasculitis of the coronary arteries resulting in ischemic cardiomyopathy or from uncontrolled HTN caused by renal dz.

7

Musculoskeletal manifestations of polyarteritis nodosa?

Myalgias

Muscular weakness

8

Other manifestations of polyarteritis nodosa

**ANY ORGAN CAN BE AFFECTED!

Orchitis, breast and uterine pain, ischemic retinopathy, retinal detachment

9

Polyarteritis Nodosa:
-dx
-tx
-prognosis

Dx;
-PE & Hx
-confirm with Bx of angiography if possible
-Labs:
--CMP, CPK, HBV, HCV, UA, ESR, CRP, ANCA (ANCA should be negative)

Tx:
-high dose glucocorticoids (Predisone)
-Cyclophosphamide (azathiprine or methotrexate)

Prognosis:
-if untreated 5 year survival is 13%, if treated its about 80%

10

Kawasaki Disease
-aka
-course
-MC age and gender
-MC race
-etiology

AKA
-Mucocutaneous lymph node syndrome

Course
-typically self limiting lasting an average of 12 days without therapy

MC in boys, ages 3-5

MC in Asians or pacific islanders

Etiology
-UNKNOWN
-possibly genetics or immunologic

11

Kawasaki Disease
-Patho
-Dx criteria
-Dx criteria for incomplete kawasaki dz

Patho
-vasculitis caused by the infiltration of vessel walls with mononuclear cells (monocytes, lymphocytes, and dentritic cells) and later IgA secreting plasma cells. This can result in destruction of the tunica media and aneurysm formation

Dx Criteria
-Fever lasting at least five days with at least four of the five following criteria:
--bilateral bulbar conjunctival injection
--oral mucous membrane changes (injected or fissured lips, injected pharynx, strawberry tongue)
--peripheral extremity changes, including erythema of palms or soles, edema of hand or feet, and periungual desquamation
--polymorphus rash
--cervical lymphadenopathy

Incomplete dx:
-if only 2 of the criteria above are met.

12

Kawasaki Dz:
-prodrome sx
-dz sx

Prodrome: precedes sx of dz up to 10 days
-irritabilit or lethargy
-vomiting
-anorexia
-cough or rhinorrhea
-diarrhea, vomiting, or abd pain

Dz sx:
-fever
-conjunctivitis
-mucositis
-edema in extremities
-erythema of palmos or soles
-polymorphous rash
-lymphadenopathy

13

Kawasaki dz:
-describe fever
-describe conjunctivitis
-describe mucositis
-describe extremity changes
-describe polymorphous rash
-describe lymphadenopathy

Fever: at least 5 days, typically 38.5C

Conjunctivitis: begins within days of fever onset, may spare the limbus.
-photophobia and anterior uveitis is common.

Mucositis:
-cracked red lips
-strawberry tongue

Extremity changeS:
-last manifestation to appear
-edema of the dorsum of hands and feet
-erythema of palms and soles
-last phase is sheet like desquamation of hands and feet

Polymorphous Rash:
-occurs in first few days of illness
-macular morbilliform or targetoid skin lesion of the trunk and extremities

Lymphadenopathy:
-absent in 50-75%
-anterior cervical nodes
-may only be able to palpate a single large node

14

Kawasaki Dz:
-what are the cardiovascular complications?
-dx
--labs
--imaging
-tx

Cardio Comps:
-coronary artery aneurysms
-CHF and decreased EF
-MI
-arrhythmias
-peripheral arterial occlusion

Dx:
-labs:
--CBC, CRP, ESR, CMP
---elevated CRP, ESR, leukocytosis, thrombocytosis, anemia, abnormal LFT, hyponatremia

Imaging:
-echo @ dx and repeat at 2wks and at 6-8wks

-CXR to eval for pulm edema

Tx:
-IVIG
--if given within first 10 days of onset of illness this can reduce incidence of coronary aneurysm by 5x**********

AND

-Aspirin
--after afebrile x24hrs then taper..continue for 2mo

Second line:
-methylprednisolone
-TNF inhibitors

15

Wegeners Granulomatosis:
-aka
-what is this?
-affects which vessels?
-what is the triad?
-course
-sx

aka: granulomatosis with polyangitis

What:
-granulomas and patchy necrosis in arteries and veins.

Affects small arteries

Triad:
-necrotizing granulomas of the lower and upper resp tract.
-glomerulonephritis

Course:
-develops over 4-12mo, without treatment survival is less than 1 year

Sx:
-upper resp: nasal congestion, sinusitis, otitis media, mastoiditis, inflamm of gums, stridor
-lower resp: cough, dyspnea, hemoptysis
-fever malaise, and wt loss are common
-arthritis of large joints
-purpura
-dysthesia
-renal insufficiency
-unilateral proptosis (eye)
-red eye
-kidney inflammation

16

Wegeners Granulomatosis:
-PE findings
-labs
-imaging

PE:
-Nose: congestion, ulceration. bleeding, perforation of septum, saddle nose deformity

-Ears: otitis media

-Eyes: proptosis, scleritis, episcleritis, conjunctivitis

-findings suggestive of DVT or PE

Labs:
-mild anemia and leukocytosis
-elevated ESR
-hematuria
-blood cell casts
-C-ANCA is highly specific but may not be present in all pts

Imaging:
-Chest CT:
--infiltrates, nodules, masses, cavities

17

Wegeneres granulomatosis:
-tx

Treatment:
-corticosteroids
-cyclophosphamide
-rituximab
*improvement within days to weeks
*remission will occur btu these pts continue these meds until remission for one full year.

18

Henoch- Schonlein Purpura:
-affects what vessels?
-sx
-course
-tx
-follow up
-MC in who?

affects small vessels

Sx:
-palpable purpura* lower extremities
-abd pain associated with GI bleeding
-arthritis (knees and ankles)
-hematuria or proteinuria d/t glomerulonephritis

Course:
-self limiting, lasts 1-6wks
-may be chronic

Tx:
-systemic steroids

Follow up:
-UA and BP monitoring weekly x2 mo then every 1-2mo for a year.

MC in kids, this is the mc vasculitis in kids.