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Flashcards in Vasculitides Deck (45):

What is Polyarteritis Nodosa?

- necrotizing arteritis of medium sized vessels
- MC involves the skin, peripheral nerves, mesenteric vessels (including renals), heart, brain but can affect any organ


Epidemiology and etiology of Polyarteritis Nodosa?

- MC in middle aged or older adults
- can also occur in kids
- males more than females
- rare (3-4.5/100,000)
- etiology:
idiopathic but may be related to Hep B and C as well as Hairy Cell Leukemia


Pathogenesis of Polyarteritis Nodosa?

- some cases related to immune complex formation
- thickening of the inflamed vessel wall leads to luminal narrowing
- reduced blood flow and thrombosis
- thrombosis results in ischemia to the involved organ
- inflammation can also cause weakening of the vessel wall that leads to aneurysm formation
- doesn't involve the veins!


Signs and sxs of Polyarteritis Nodosa?

- systemic sxs:
fatigue, wt loss, weakness, fever, arathralgias
- signs:
skin lesions, HTN, renal insufficiency, neuro dysfxn, abdominal pain of multisystem involvement


Skin manifestations of PAN?

- tender erythematous nodules
- purpura
- livedo reticularis
- ulcers: infarction, gangrene
- bullous or vesicular eruption
- may be focal or diffuse
- more common on the lower extremities


Renal manifestations of PAN?

- kidneys are most commonly involved organ
- variable degrees of renal insufficiency and HTN
- rupture of renal arteries can result in perirenal hematoma
- luminal narrowing leads to glomerular ischemia but not inflammation or necrosis
- UA may show minimal protein, moderate hematuria, no RBC casts should be seen


Neurologic manifestations of PAN?

- motor and sensory deficits of the involved nerves
one of the MC findings (up to 75%)
- generally asymmetric neuropathy at onset
- CNS involvement up to 10%


GI manifestations of PAN?

- abdominal pain in those w/ mesetneric arteritis:
post prandial pain, wt loss, bowel infarction w/ perforation
- N/V/D
- melena
- GI bleeding


CV manifestations of PAN?

- MI is uncommon: may result from narrowing or occlussion of the coronary arteries
- HF: from vasculitis of the coronary arteries, resulting in ischemic cardiomyopathy or from uncontrolled HTN caused by renal disease


Musculoskeletal manifestations of PAN?

- muscle involvement is common
- myalgias
- muscular weakness


Other manifestations of PAN?

- any organ can be affected
- may manifest as:
breast and uterine pain
eye: ischemic retinopathy, retinal detachment


How do you dx PAN?

- based on physical, Hx, labs
- confirm dx w/ bx or angiography if possible
- basic eval should include: CMP, CPK (muscle enzymes), HBV, HCV, UA, ESR, CRP, ANCA (ANCA should be negative)


Tx of PAN?

- high dose glucocorticoids:
prednisone 1 mg/kg/day w/ max dose of up to 80 mg/day
- cyclophosphamide or other immunosuppresants like azathiprine or methotrexate


Prognosis of PAN?

- if untx: 5 yr survival is only about 13%
- if tx: 5 yr survival is about 80%


What is Kawasaki disease?

- mucocutaneous lymph node syndrome
- one of the MC vasculitides of childhood
- typically self limiting lasting an avg of 12 days w/o therapy


Epidemiology of Kawasaki's?

- MC in ages: 3-5 (80-90% of cases)
- more common in boys
- MC in Asians or Pacific Islanders
- Incidence in Japan 2007-2009 216.9/100,000
- highest incidence outside of Asia in Ontario 26.2/100,000
- increased incidence in summer and winter


Etiology of kawasaki's?

- basically unknown
- genetic predisposition?
- immunologic?
- infectious?
likely viral
new RNA virus


PP of Kawasaki's?

- vasculitis caused by infiltration of vessel walls w/ mononuclear cells (monocytes, lymphocytes, and dendritic cells) and later IgA secreting plasma cells
- can result in destruction of the tunica media and aneurysm formation


Dx criteria for Kawasaki disease?

- reqrs presence of a fever lasting at least 5 days w/o any other explanation combined w/ at least 4/5 following criteria:
- bilateral bulbar conjunctival injection
- oral mucous membrane changes, including injected or fissured lips, injected pharynx, or strawberry tongue
- peripheral extremity changes, including erythema of palms or soles, edema of hands or feet (acute phase), and periungal desquamation (convalescent phase)
- polymorphous rash
- cervical lymphadenopathy (at least one lymph node over 1/5 cm in diameter)


What is incomplete kawasaki disease?

- if only 2 of the criteria are met then it is considered an incomplete form


Classic syndrome of Kawasaki's disease?

often preceded by nonspecific sxs for up to 10 days:
- irritability or lethargy 50%
- vomiting alone 44%
- anorexia 37%
- cough or rhinorrhea 35%
- diarrhea, vomiting or abdominal pain 61%


When should you consider KD in your DDx?

- in all kids w/ fever for 5 days or more
- fever may be intermittent
- minimally responsive to antipyretics and typically remains above 38.5 (101.3)


How common is conjunctivitis in KD?

- present in more than 90% of cases
- bulbar injection begins w/in days of onset of the fever
- may spare the limbus
- frequently occurs w/ photophobia and anterior uveitis


Presentation of mucositis in KD?

- cracked red lips
- strawberry tongue
- may be mild or not occur at all
- if they have other oral lesion such as vesicles, ulcers, tonsillar exudate than likely it isn't KD (probably viral)


Extremity changes in KD?

- usually last manifestation ot appear
- edema of the dorsum of hands and feet, erythema of the palms and soles
- last phase of the disease: sheet like desquamation of hands and feet


When does polymorphous rash occur in KD? Presentation?

- usually occurs in first few days of illness
- may begin as perineal erythema and desquamation followed by macular, morbilliform or targetoid skin lesion of the trunk and extremities
- if vesicular or bullous lesions than consider another dx


Lymphadenopathy characteristics of KD?

- absent in up to 50-75% of pts
- anterior cervical nodes
- may only be able to palpate a single large node
- if diffuse lymphadenopathy or splenomegaly look for an alternative dx


CV complications of KD?

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


DDx for KD?

- acute rheumatic fever
- scarlet fever
- drug rxn
- juvenile RA
- measles


Eval for KD?

- labs: CBC, CRP, ESR, CMP - elevated CRP, ESR, leukocytosis, thrombocytosis, anemia, abnormal LFTs, hyponatremia
- echo: at dx and repeat at 2 wks and 6-8 wks
- CXR: to eval for pulmonary edema


Tx for KD?

- IV IG 2 g/kg infusion over 8-12 hrs - if given w/in the 1st 10 days of the onset of illness can reduce the incidence of coronary aneurysm by 5x, anti-inflammatory effect, reduces acute phase reactants, cytokines, augments T cell suppressor activity, resolves fever
- + aspirin 80-100 mg/kg/day divided into QID dosing - after afebrile x 48hrs then decrease dose to 3-5 mg/kg/day and continue for about 2 months, antipyretic, anti-inflammatory, and anti platelet effects


2nd line therapies for KD?

- methylprednisolone
- TNF inhibitors


What is Wegener's Granulomatosis?

- AKA granulomatosis w/ polyangitis
- rare disorder (12/1 million)
- ANCA assoc
- w/o tx survival less than 1 yr


What does Wegener's granulomatosis affect?

- small arteries: inflammation restricts blood flow
- triad of upper and lower respiratory tract and glomerulonephritis:
necrotizing granulomas of upper and lower respiratory tract, necrotizing glomerulitis and thromboses of capillary loops
- commonly occurs in 40s-50s
- affects men and women equally


Presentation of Wegener's Granulomatosis?

- develops over 4-12 months
- 90% of pts present w/ upper resp tract sxs: nasal congestion, sinusitis, otitis media, mastoiditis, inflammation of gums, stridor
- pts may present w/ lower respiratory tract sxs: cough, dyspnea, hemoptysis
- fever, malaise and wt loss are common
- arthritis of the large jts
- skin: purpura or other skin lesions
- neuro: dythesia
- kidneys: renal insufficiency
- ocular disease:
unilateral proptosis, red eye

- other possibe sxs:
eye inflammation
jt pain or muscle pain
- rashes or skin sores
- kidney inflammation: renal involvement is usually subclincal until renal insufficiency is advanced


Physical exam findings of Wegener's?

- nose: congestion, ulceration, bleeding, perforation of nasal septum, saddle nose deformity
- ears: otitis media
- eyes: proptosis, scleritis, episcleritsi, conjunctivitis
- findings suggestive of DVT or PE


2 forms of Wegener's?

- isolated forms: affect only one organ
- generalized forms: affects many organs at the same time


Labs and imaging for Wegener's?

- labs:
mild anemia and leukocytosis
elevated ESR
blood cell casts
C-ANCA is highly specific, but not all pts show an elevated C-ANCA
- chest CT is most sensitive to lung changes:


Mild, early presentation of Wegener's vs late, destructive presentation?

- early:
sore eyes, ears, stuffy nose, sore jts, trace of blood in urine, blip in lungs on CXR, granulomas and patchy necrosis in arteries and veins
- late:
destruction of the face, lung cavities and bleeds, perm kidney failure, gangrene


Tx of Wegener's?

- corticosteroids
- cyclophosphamide
- rituximab
- improvement usually occurs w/in days to weeks
- when the disease is in remission, pts will reduce the dosage of these meds, but will continue tx until the disease has been in continuous remission for one yr


What is Henoch-Shconlein Purpura?

- MC vasculitis in kids, also may occur in adults
- affects small vessels


Typical features of HSP?

- palpable purpura:
typically located on lower extremities, purple rash
- abdominal pain - assoc w/ GI bleeding
- arthritis: knees and ankles MC
- hematuria or proteinuria: glomerulonephritis


Course of HSP?

- usually self-limiting
- lasts from 1-6 wks then subsides w/o sequela if renal involvement isn't severe
- chronic courses w/ persistent or intermittent skin disease are more likely in adults than kids
- efficacy of tx isn't well established


MC affected organs in HSP?

- skin: macular wheals, ecchymosis petechiae, palpable purpura - 100% of cases
- jts: transient or migratory arthritis, large jts - 60-84% of cases
- GI tract: more common in kids, N/V, abd pain, ileus, GI bleed, bowel ischemia, perforation, intussusception - up to 75% of cases
- kidneys: more common in adults, hematuria, + protein, nephritic syndrome, renal insufficiency, nephrotic syndrome - 21-54% of cases


Prognosis and F/U for HSP?

- most cases resolve w/in a month
- severe cases may be tx w/ systemic steroids
- recurs in 1/3 of cases, usually w/in 54 months
- main cause of morbidity is renal disease
- f/u:
UA and BP monitoring
weekly x 2 months, then q 1-2 months for a yr