Vasculitides Flashcards
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
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
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!
Neurologic manifestations of polyangitis nodosa
motor and sensory deficits of the involved nerves
generally asymmetric neuropathy at onset.
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
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.
Musculoskeletal manifestations of polyarteritis nodosa?
Myalgias
Muscular weakness
Other manifestations of polyarteritis nodosa
**ANY ORGAN CAN BE AFFECTED!
Orchitis, breast and uterine pain, ischemic retinopathy, retinal detachment
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%
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
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.
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
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
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
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
