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Rheumatology > Vasculitides > Flashcards

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

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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
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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!
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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
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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
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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
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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%
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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
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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
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10
Q

Musculoskeletal manifestations of PAN?

A
  • muscle involvement is common
  • myalgias
  • muscular weakness
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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
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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)
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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
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14
Q

Prognosis of PAN?

A
  • if untx: 5 yr survival is only about 13%

- if tx: 5 yr survival is about 80%

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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
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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
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17
Q

Etiology of kawasaki’s?

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

Rheumatology (8 decks)

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