Vasculitides Flashcards
1
Q
What is Polyarteritis Nodosa?
A
- necrotizing arteritis of medium sized vessels
- MC involves the skin, peripheral nerves, mesenteric vessels (including renals), heart, brain but can affect any organ
2
Q
Epidemiology and etiology of Polyarteritis Nodosa?
A
- 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
3
Q
Pathogenesis of Polyarteritis Nodosa?
A
- 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!
4
Q
Signs and sxs of Polyarteritis Nodosa?
A
- systemic sxs:
fatigue, wt loss, weakness, fever, arathralgias - signs:
skin lesions, HTN, renal insufficiency, neuro dysfxn, abdominal pain of multisystem involvement
5
Q
Skin manifestations of PAN?
A
- tender erythematous nodules
- purpura
- livedo reticularis
- ulcers: infarction, gangrene
- bullous or vesicular eruption
- may be focal or diffuse
- more common on the lower extremities
6
Q
Renal manifestations of PAN?
A
- 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
7
Q
Neurologic manifestations of PAN?
A
- 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%
8
Q
GI manifestations of PAN?
A
- abdominal pain in those w/ mesetneric arteritis:
post prandial pain, wt loss, bowel infarction w/ perforation - N/V/D
- melena
- GI bleeding
9
Q
CV manifestations of PAN?
A
- CAD
- 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
10
Q
Musculoskeletal manifestations of PAN?
A
- muscle involvement is common
- myalgias
- muscular weakness
11
Q
Other manifestations of PAN?
A
- any organ can be affected
- may manifest as:
orchitis
breast and uterine pain
eye: ischemic retinopathy, retinal detachment
12
Q
How do you dx PAN?
A
- 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)
13
Q
Tx of PAN?
A
- 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
14
Q
Prognosis of PAN?
A
- if untx: 5 yr survival is only about 13%
- if tx: 5 yr survival is about 80%
15
Q
What is Kawasaki disease?
A
- mucocutaneous lymph node syndrome
- one of the MC vasculitides of childhood
- typically self limiting lasting an avg of 12 days w/o therapy
16
Q
Epidemiology of Kawasaki’s?
A
- 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
17
Q
Etiology of kawasaki’s?
A
- basically unknown
- genetic predisposition?
- immunologic?
- infectious?
likely viral
new RNA virus
18
Q
PP of Kawasaki’s?
A
- 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
19
Q
Dx criteria for Kawasaki disease?
A
- 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)
20
Q
What is incomplete kawasaki disease?
A
- if only 2 of the criteria are met then it is considered an incomplete form
21
Q
Classic syndrome of Kawasaki’s disease?
A
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%
22
Q
When should you consider KD in your DDx?
A
- 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)
23
Q
How common is conjunctivitis in KD?
A
- 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
24
Q
Presentation of mucositis in KD?
A
- 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)
25
Q
Extremity changes in KD?
A
- 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
26
Q
When does polymorphous rash occur in KD? Presentation?
A
- 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
27
Q
Lymphadenopathy characteristics of KD?
A
- 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
28
Q
CV complications of KD?
A
- coronary artery aneurysms
- CHF and decreased EF
- MI
- arrhythmias
- peripheral arterial occlusion
29
Q
DDx for KD?
A
- acute rheumatic fever
- TSS
- scarlet fever
- SSSS
- SJS
- drug rxn
- juvenile RA
- measles
- RMSF
30
Q
Eval for KD?
A
- 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
31
Q
Tx for KD?
A
- 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
32
Q
2nd line therapies for KD?
A
- methylprednisolone
- TNF inhibitors
33
Q
What is Wegener’s Granulomatosis?
A
- AKA granulomatosis w/ polyangitis
- rare disorder (12/1 million)
- ANCA assoc
- w/o tx survival less than 1 yr
34
Q
What does Wegener’s granulomatosis affect?
A
- 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
35
Q
Presentation of Wegener’s Granulomatosis?
A
- 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
36
Q
Physical exam findings of Wegener’s?
A
- 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
37
Q
2 forms of Wegener’s?
A
- isolated forms: affect only one organ
- generalized forms: affects many organs at the same time
38
Q
Labs and imaging for Wegener’s?
A
- labs: mild anemia and leukocytosis elevated ESR hematuria 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: infiltrates nodules masses cavities
39
Q
Mild, early presentation of Wegener’s vs late, destructive presentation?
A
- 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
40
Q
Tx of Wegener’s?
A
- 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
41
Q
What is Henoch-Shconlein Purpura?
A
- MC vasculitis in kids, also may occur in adults
- affects small vessels
42
Q
Typical features of HSP?
A
- palpable purpura:
typically located on lower extremities, purple rash - abdominal pain - assoc w/ GI bleeding
- arthritis: knees and ankles MC
- hematuria or proteinuria: glomerulonephritis
43
Q
Course of HSP?
A
- 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
44
Q
MC affected organs in HSP?
A
- 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
45
Q
Prognosis and F/U for HSP?
A
- 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
