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Practice Topics Exam 8 > Rheum 5 - Vasculitis > Flashcards

Rheum 5 - Vasculitis Flashcards

1
Q

Approaches to classification of vasculitis

A

Clinical syndromes
Vessel size - large arteries, medium and small mm arteries, small vessels (arterioles and venules)
Lab markers
Pathology - immune complex deposition, granulomatous inflammation

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2
Q

Clinical features with vasculitis

A 
Multisystem disease
Unexplained constitutional s/s
Skin lesions (palpable purpura)
Ischemic vascular changes 
Glomerulonephritis
Mononeuritis multiplex
Myalgia, arthralgia/arthritis
Abdominal or testicular pain
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3
Q

Polyarteritis Nodosa (PAN)

A

Necrotizing vasculitis of small and medium arteries
Lesions are segmental
Favor vessel branch points
Different stages of development - simultaneously
Aneurysms

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4
Q

Epidemiology - PAN

A

Uncommon!
Avg age onset = 40 to 60
M:F is 2:1

Higher incidence in pop with HBV

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5
Q

PAN classification

A

Polyarteritis Nodosa

  • Idiopathic
  • Associated with cirus (primarily hep B)
  • Associated with malignancy (mainly hematologic)
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6
Q

ACR criteria for classification of PAN

A 
Need at least 3 of the 10
1 Weight loss more than 4 Kg
2 Livedo reticularis 
3 Testicular pain or tenderness
4 Myalgias, weak, leg tenderness
5 Mono or polyneuropathy
6 Diastolic BP more than 90
7 Elevated BUN/creatinine
8 Hep B
9 Arteriographic abnormaiity
10 Biopsy of small or medium artery containing PMN
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7
Q

PAN cutaneous

A 
25-60% of patiens
Palpable purpura
Infarctions
Ulcerations
Livedo reticularis 
Subcutaneous nodules
Ischemic distal digits
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8
Q

PAN neurologic

A

Up to 70%
LE more than UE
Mononeuritis multiplex (esp affecting superficial peroneal, sural, radial, cubital, median)
Symmetric polyneuropathy (sensory and motor) - stocking glove dysesthesia
CNS - HA, seizure, CN palsy, CVA

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9
Q

PAN - GI

A

Abdominal pain = 25-70%
Intestinal angina
Bowel infarction
Gall bladder or appendix

One of the most sever manifestations - primary causes of death within first year (after infection and cardiac)

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10
Q

PAN - renal

A 
If classic PAN = glomerulus is usually spared 
Vascular neuropathy (35%
HTN - 25-30%
If glomerulonephritis = microscopic polyangitis (MPA)
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11
Q

Labs for PAN

A 
Elevated ESR 95%
Elevated WBC 75%
Anemia of chronic disease 67%
Depressed C3 70%
Pos RF 40% (not pos ANA)
Elevated C reactive protein
HebBSAg
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12
Q

PAN diagnosis

A

Tissue diagnosis - sural nerve, testes, skeletal muscle, renal
Visceral angiography

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13
Q

PAN tx

A

Corticosterois - prednisone
Cyclophosphamide (second choice azathioprine)
Control hyeprtension

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14
Q

IgA Vasculitis (Henoch Scholein Purpura - clinical manifestations

A

Non thrombocytopenic purpura
Arthralgias/arthritis
GI pain - colicky abdominal pain; GI hemorrhage risk, Renal abnormalities

80-90% will have all four of these

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15
Q

IgA Vasculitis (Henoch Scholein Purpura - classic tetrad in 80% of cases

A

1 palpable purpura
2 arthritis
3 abdominal pain
4 renal disease

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16
Q

IgA Vasculitis (Henoch Scholein Purpura - clinical features - demographics

A 
Incidence school age = 13/100,000
Develop at any age - 90% 4-7 yo
2/3 follow resp infection 
Immune complex deposition - IgA 1
F:M is 1:1
Uniphasic 80%, polyphasic 10-20%

Majority are treated quickly and resolve - those that linger tend to have renal involvement
Adults more likley to have severe disease with renal involvement

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17
Q

IgA Vasculitis (Henoch Scholein Purpura - - Skin

A

Palpable purpura (100% prereq for diagnosis)
Usually LE - buttock
Presenting sign - 50%

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18
Q

IgA Vasculitis (Henoch Scholein Purpura - - joint involvement

A

Arthralgias/arthritis (60-85%)
Knees more than ankles more than wrists more than elbows
Transient - self limiting; without permanent joint damage

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19
Q

IgA Vasculitis (Henoch Scholein Purpura - - GI involvement

A

Colicky abdominal pan, nausea, vomiting - peds 70%, adults 50%
GI bleeding occult (50%)
Major hemorrhage (less than 5%)
Intussusception - ileoileal (mean age 6 yo)
Abdominal pain secondary to submucosal and intramural extravasation of fluid into intestinal wall

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20
Q

IgA Vasculitis (Henoch Scholein Purpura - - renal involvement

A

Incidence 10-50%
Children usually more than 9 yo
Inc incidence of GMN with bloody stools
Less than 1% progress to ESRD

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21
Q

IgA Vasculitis (Henoch Scholein Purpura - Lab

A

Elevated ESR 1/3
Platelet count normal
U/A microscopic hematuria, proteinuria, RBC cast
Elevated IgA 50%
Skin biopsy positive IF for IgA in vessel walls

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22
Q

IgA Vasculitis (Henoch Scholein Purpura - - treatment

A 
Supportive APAP (tyelenol) for analgesia 
Corticosteroids - abomdinal pain, nephritis, edema, ? benefit
Severe HSP nephritis - high dose corticosteroids, cyclophosphamide
23
Q

ANCA - associated vasculitis

A

1 Granulomatosis with polyantitis (Wegeners granulomatosis)
2 Eosinophilic granulomatosis with polyangitis (churg strauss syndrome)
3 Microscopic polyangitis

24
Q

ANCA is what

A

Antineutrophil cytoplasmic antibodies

25
Q

ANCA - Granulomatosis with polyangitis - Wegener’s - GPA is what

A 
Multi organ systemic disease
Granulomas necrotizing vasculitis - predilection upper and lwoer resp tract and kidney
Prevelance 3/100,000
M:F is 1:1
Age - 40 to 60
Mainly cauc
Association with PR3 90-97% specific 
"saddle nose deformity"
26
Q

ANCA - Granulomatosis with polyangitis - Wegener’s - GPA - Lungs

A

Trachea - subglottic stenosis
Pulmonary - fleeting pulmonary infiltrates, ndoules, fulminant alveolar hemorrhage
Asymptomatic, cough, dyspnea, pleurisy, hemoptysis

27
Q

ANCA - Granulomatosis with polyangitis - Wegener’s - GPA - renal

A 
RPGN
Hematuria
RBC cast
Proteinuria
Rising serum creatinine 
Can be rapidly progressive
28
Q

ANCA - Granulomatosis with polyangitis - Wegener’s - GPA - Lab

A 
Elevated ESR (often over 100)
Leukocytosis
Thrombocytosis
Anemia
Mildly positive RF - 60%
Abnormal urinary sediment
cANCA PR3
29
Q

ANCA - Granulomatosis with polyangitis - Wegener’s - GPA - Treatment

A

Prednisone
Cyclophosphamide
Rituxan

Limited disease - prednisone and methotrexate

30
Q

ANCA - Granulomatosis with polyangitis - Wegener’s - GPA - prognosis

A

Prednisone and CTS - 95% remission
Remission relapse is 50%
Mortality 8 yrs - 13%

31
Q

ANCA - microscopic polyangitis (MPA) - Typical manifestations

A

peak age 30-50
Frequent manifestations:
Pulmonary capillaries/diffuse alveolar hemorrhage
Necrotizing glomerulonephritis (almost always)

So mainly lung and kidney involvement

32
Q

ANCA - microscopic polyangitis (MPA) - Other manifestations

A

Fever (50-70%)
Arthralgias (30-65%)
Pupura (40%)
Peripheral or CNS (25-30%)

33
Q

ANCA - microscopic polyangitis (MPA) - Histopathology of MPA

A

Focal, segmental necrotizing GN
Pauci immune (no immune deposits)
Lung capillaritis
Skin leukocytoclastic vasculitis

34
Q

ANCA - microscopic polyangitis (MPA) - diagnosis

A

Clinical features
Biopsy results
Lab data - P ANCA against MPO in 80% (PANCA is bound around MPO)

35
Q

ANCA - Eosinophilic granulomatosis with polyangiitis (churg strauss syndrome)

A

Granulomatous inflammation
Small and medium vessels
Peripheral eosinophilia
Primarily with a prior history of allergic manifestations - Rhinitis (often with nasal plyps), Adult onset asthma

36
Q

ANCA - Eosinophilic granulomatosis with polyangiitis (churg strauss syndrome) - clinical pahses

A

1 Prodromal phase - allergic manifestations - fora decade or more
2 Peripheral blood and tissue eosinophilia
3 Life threatening systemic vasculitis

37
Q

ANCA - Eosinophilic granulomatosis with polyangiitis (churg strauss syndrome) - clinical features

A

Paranasal sinus - sinusitis, rhinitis, plyps
Lungs - patchy, shifting infiltrates, nodules, effusion
NS - mononeuritis multiplex, polyneuropathy
Skin - nodules, purpura, infarction
Joints
GI - pain, bloody, diarrhea, masses
CHF
Misc - renal is RARE

38
Q

ANCA - Eosinophilic granulomatosis with polyangiitis (churg strauss syndrome) - diagnosis

A

Clinical and pathologic features
ANCA in 60-70% - primarily vs. MPO with PANCA pattern
Diagnosis made based on clinical hx and often bipsy confirmation

39
Q

Large vessel vasculitis

A

Giant cell arteritis

Takayasu Arteritis

40
Q

Large vessel vasculitis - Giant cell arteritis (GCA) - Temporal arteritis

A

Temporal region - forehead
If can;t feel pulses is more concerning
Might have tenderness to palaption too

41
Q

Large vessel vasculitis - Giant cell arteritis (GCA) - epidemiology

A

Avg age 70
3:1 F more than M
Caucasion almost exclusively

42
Q

Large vessel vasculitis - Giant cell arteritis (GCA) - GCA PMR Syndrome

A

Cranial arteriitis - HA, scalp tenderness, optic neuropathy, jaw claudication, PMR

Large vessel invovlement - limb claudication, unequal pulses, aortic aneur, aoritc insuff, PMR

System inflamm disease - fever, chills, anorexia, weight loss, night sweats, weak, depressed

Isolated PMR - pain in shld and pelvic girdle mm, stiffness

43
Q

Relationship of polymyalgia (PMR) to giant cell arteritis

A

40-60% of pts with GCA have PMR symptoms - in about 1/2 of those, PMR is their first manifestation of GCA

10-15% of pts with PMR have GCA

PMR symptoms can occur before, with , or after GCA symptoms in those with GCA

44
Q

Polymyalgia rheumatica epidemiology

A

Onset after 60
Norther europeans
2:1 F more than M

45
Q

Polymyalgia rheumatica - clincial and lab findings

A 
Pelvic and shoulder girdlle aching
Morning stiffness
Rapid response to low dose of corticoseteroids
Anemia
Elevated ESR and C reactive protein
46
Q

GCA symptoms

A 
HA
Fever
PMR
Weight loss
Anorexia
Jaw claudications
Malaise
Depression
Fatigue
Acute visual loss
Diplopia
47
Q

GCA vessel distribution

A

Cranial

  • superficial tempral
  • vertebral
  • ophthalmic
  • post ciliary
  • less ffrequnty in external and internal carotid

Less common but inc recognized in aorta and branches, and cornary (rare)

48
Q

GCA - lab

A 
Elevated ESR - either more than 50 or 100
Elevated C reactive protein
Anemia of chronic disease
Abnormal liver function test
Elevated IL 6
SPEP inc alpha 2 globulin
49
Q

GCA - temporal artery biopsy

A

Should do

Obtain ASAP - though do not delay starting steroids prior to biopsy if suspicion is high

50
Q

GCA - predictors for a positive temporal artery bipsy

A

Positive predictors = jaw claduication, diplopia, TA beading, TA prominence, TA tenderness

Neg = Absence of TA abnormality, normal ESR

51
Q

GCA - histopathology

A

Usually extracranial arteries
Panarteries - invovles intima, destroys internal elastic membrane
Granulomatous arteritis; inc multinucleated giant cells
Skip lesions

52
Q

ACR classification criteria for GCA

A

NEED 3/5

1 Age over 50 at disease onset
2 new HA
3 Termpoal artery abnormality
4 Elevated ESR over 50
5 Abnormal biopsy
53
Q

GCA tx

A 
Mandatory - prednisone for 4 wks
Slow taper, thereafter
Lower maitenance doeses of prednisone 
Total tx duration is 6 to 24 months
Relpase is common
54
Q

Follow up of GCA pts

A

Vascular complications may occur years after onset
Stenosis or aneurysm
Aortic aneur is 17x mroe likley in those with GCA
also 2.4x mroe likely to develop abdominal aneurysm

Practice Topics Exam 8 (12 decks)

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