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