Vasculitis Flashcards
1
Q
HSP classification
A
Palpable purport with lower limb predominance plus at least one of the following:
- Diffuse abdominal pain
- Biopsy showing typical leukocytoclastic vasculitis OR proliferative glomerulonephritis with predominant IgA deposition
- Arthritis or arthralgia
- Renal involvement (any hematuria and/or proteinuria)
2
Q
Indications for renal biopsy in HSP
A
- Impaired GFR
- Severe or persistent proteinuria
- Renal biopsy should be considered in cases of
- Acute kidney injury with worsening renal function as part of a rapidly progressing glomerulonephritis
- Nephrotic Syndrome (heavy proteinuria, hypoalbuminemia, and edema)
- Nephritis Syndrome (impaired GFR, HTN, hematuria/proteinuria)
3
Q
Principle lesion found in HSP nephritis
A
Endocapillary proliferation with an increase in endothelial and mesangial cells
- IgA, fibrin, C3 and properdim in most involved glomeruli
- Peripheral capillary loops involved in more severe cases
4
Q
Poor prognostic factors in HSP
A
- Mixed nephrotic nephritis syndrome within the first 6 months
- Decreased factor XIII activity
- HTN
- Renal failure at onset
- Increased number of glomeruli with crescents
- TI disease or macrophage infiltration
5
Q
When do GI and renal manifestations typically occur in HSP
A
GI occur usually within a week
Renal usually develops within 6-8 weeks of rash but should monitor for 6 months
6
Q
Lab findings in HSP
A
NORMAL platelet count Moderate leukocytosis Normochromic anemia May have positive stool guiac ANA/ RF negative UA abnormalities Hypoalbuminema with proteinuria Complements usually normal Ancas negative
7
Q
Most common season for HSP
A
Winter
8
Q
Classification criteria for hypersensitivity vasculitis/serum sickness
A
Need 3/6
- Age at onset >16
- Medication at disease onset
- Palpable purpura not related to thrombocytopenia
- Maculopapular rash
- Characteristic biopsy findings
9
Q
Pathology of hypersensitivity
vasculitis
A
- Cellular infiltrate contains a large number of neutrophils and eosinophils
- Granulocytes present in perivascular or extravascular location
10
Q
Labs associated with Hypersensitivity vasculitis
A
- leukocytosis
- Eosinophilia
- ESR is NORMAL
11
Q
Classification criteria for PAN
A
1. Evidence of necrotizing vasculitis in medium or small arteries or angiographic abnormality showing aneursym, stenosis, or occlusion of a medium or small sized artery (histo or angio mandatory) plus at least 1 A. Skin involvement - Livedo - Skin nodules - Infarcts
B. Myalgia or muscle tenderness C. HTN - Systolic/diastolic blood pressure >95th percentile for height D. Peripheral neuropathy - Sensory - Motor mononeuritis multiplex E. Renal involvement - Proteinuria - Hematuria - Impaired renal function
12
Q
Viral/bacterial trigger for PAN
A
Hepatitis B + Strep
13
Q
Genetic mutation that may predispose to PAN
A
MEFV
14
Q
Typical labs for PAN
A
Leukocytosis
Thrombosis
ESR/CRP
UA –> protein, hematuria
ANA and ANCA typically negative
15
Q
Characteristic histopath of PAN
A
Fibrinoid necrosis of the walls of medium or small arteries with a marked inflammatory response within or surrounding the vessel wall
- Lesions tend to be focal and segmental
16
Q
What increases risk of relapse in PAN?
A
Gi involvement
17
Q
Symptoms of cutaneous PAN
A
Fever, nodular, painful non purpuric lesions with or without livedo racemosa, occurring predominantly in the lower extremities and with NO systemic involvement except for myalgias, arthralgia, and nonerosive arthritis
18
Q
Skin biopsy of cPAN
A
necrotizing, non granulomatous small and medium sized vasculitis
19
Q
Bacterial cause for cPAN
A
strep infection
20
Q
Diagnostic Criteria for Kawasaki
A
Fever for more than 5 days plus 4/5 following signs CRASH Conjunctivitis Rash Adenopathy Strawberry tongue Hand and feet swelling
21
Q
Acute febrile phase of KD
A
- Lasts 10-14 days
- Onset of fever is abrupt
- Irritability, vomiting decreased PO, diarrhea, abd pain, cough, rhinnorhea, weakness, arthralgia, arthritis, perineal desquamation in days leading up to presentation
- Over next 3-4 days, cervical LAD, conjunctivitis, changes in oral mucosa, pleomorphic rash, erythema and edema in hands and feet
- No particular order
- If untreated, clinical signs subside after about 12 days
- Myocarditis, pericarditis, abd pain, ascites and hydrops of gallbladder may occur during this phase
22
Q
Subacute phase of KD
A
- 2-4 weeks
- May be entirely asymptomatic if given IVIG
- Desquamation of skin (digit) may be only clinically apparent residual feature
- May develop arthritis
- CAA most commonly first develop during this phase
- Irritability resolves
- Phase ends with return to normal of platelet count and ESR
23
Q
Convalescent phase
A
- Most children asymptomatic
- Beau lines (horizontal ridging of nails) may appear
- Vessels undergo healing, remodeling and scarring
24
Q
IVIG resistance in KD
A
- Higher CRP, LDH, bilirubin
- Hyponatremia
- ELEvated LFTs
- high percentage of bands
- platelets less than 300000
- SHort duration between fever and diagnosis
- <12 months at onset or older than typical
25
Incomplete KD
Seen more frequently in younger infants and older children
- Fever greater than or equal to 5 and 2 or 3 clinical criteria
- Check CRP and ESR >3/40
- Albumin <3
- Anemia for age
- Elevated ALT
- Platelets after 7 days >450k
- WBC >15
- Urine WBC >10
26
Pathology of KD coronaries
Systemic necrotizing vasculitis with fibrinoid necrosis
- Begins with neutrophilic vasculitis (saccular)
- Chronic vasculitis (lymphocytes, eosinophils, macrophages, IgA secretign plasma cells)-- Fusiform anerurysms
- Smooth muscle cells may be converted to myofibroblasts
27
Markers for severe disease
significant neutropenia
| thrombocytopenia
28
Infantile polyarteritis nodosa
onset before age 2, most commonly affects coronaries
| - frequently presents with heart failure, may also have renal, GI, CNS manifestations
29
What is considered an abnormal coronary?
if age less than 5, >3, if 5 and up, >4
Aneursym if the diameter is 1.5x the size of the adajcent vessel
30
Signs of MAS in KD
Thrombocytopenia, anemia and modest elevation of the ESR
31
Small Aneurysm
Z score <= 2.5 to <5 (or less than 4mm)
32
Medium Aneurysm
Z score 5 to <10 (4-8mm)
33
Large Aneurysm
Z >= 10 (>8mm)
34
Antibody associated with MPA
Anti-MPO
35
Three phases of EGPA
1. Very prolonged prodromal period over many years with asthma and other allergic manifestations (chronic rhinitis and nasal polyposis)
2. Eosinophilic phase with eosinophilia and pulmonary infiltrates
3. Vasculitis
36
ACR criteria for GPA
- Requires 2/4
- Nasal or oral inflammation
- Abnormal CXR
- ABnormal UA
- Granulomatous inflammation
Eular includes stenosis and ANCA
37
Limited granulomatosis
No kidney disease
| Disease may just be limited to upper resp tract
38
First sign of pulmonary hemorrhage
increased DLCO
39
What differentiates MPA versus GPA?
- Upper airway disease (saddle nose deformity, nasal septal perforation, subglottic stenosis, dacryocytitis, proptosis, characteristic lung nodules, granulomas)
40
Common antibody associated with GPA
cANCA (PR3)
41
What biologic should NOT be used in GPA and why
- Etanercept
| - Risk of cancer
42
GPA kidney disease
focal segmental necrotizing vasculitis
43
EGPA ACR criteria
4/7
- Asthma
- Eosinophil
- History of allergy
- Mononeuropathy/polyneuropathy
- Pulmonary infiltrates
- Paranasal sinus
- Extravascular eosinophils
44
Risk factors for worse prognosis of EGPA
- Renal insufficiency
- Proteinuria greater than 1g/day
- cardiomyopathy
- CNS involvement
- GI involvement
45
Diagnostic criteria for hypocomplementemic urticaria
Major criteria
- recurrent urticaria
- low complement levels
Minor Criteria (need at least two)
- Venulitis on skin biopsy
- Arthritis
- Ocular inflammation
- Abd pain
- positive c1q antibodies
- glomerulonephritis
Positive ANA, dsDNA are exclusion criteria
46
Criteria for Behcets
- Recurrent oral aphthosis (at least 3/year)
- Genital ulceration typically with a scar
- Skin features (Acneiform lesions, necrotic follicultis, erythema nodosum)
- Ocular involvement (anterior and or posterior uveitis, retinal vasculitis )
- Neurological signs (with the exception of headache)
- Vascular involvement (venous thrombosis, arterial thrombosis, arterial anersuym)
47
Potential triggers for Behcets
- Parvo
- HSV1
- Strep
48
Pathology of Behcets
Misfolding of HLA b51
ERAP1
IL-23/IL17
49
What is Pathergy?
Cutaneous pustular reaction occurring 24 to 48 hours after a needle puncture of the dermis
50
Indicators of poor prognosis in Behcets
young age at onset
| male
51
Hallmark of Sarcoidosis
Non-caseating epitheliod giant cell granulomas in a variety of tissues and organ systems
52
Classic triad of Blau Syndrome (with NOD2 mutation)
- Poly arthritis
- Uveitis
- Dermatitis
53
Infantile onset Sarcoid without NOD2
- Infantile onset panniculitis with uveitis and systemic granulomatosis
- Panniculitis, severe systemic involvement, persistent fever, hepatosplenomegaly, granulomatous inflammation of joints, eyes, internal organs, CNS
- Progressive
54
Pediatric adult onset sarcoidosis
- Systemic features (pulm and LAD) rather than articular disease
- Malaise, fever, weight loss, lung involvement, hilar LAD, hepatosplenomegaly, cutaneous features, renal sarcoidosis, myositis etc
55
Neurosarcoidosis presenttion of sarcoid
Seizures ( cranial nerve palsy, most common complication in adults )
56
Lofgren's syndrome
Acute onset arthritis (mainly of the ankles), e nodosum, and hilar adenopathy ( rare in peds
57
Mikulicz syndrome
parotid and lacrimal gland enlargement (rare in peds)
58
Uveitis of sarcoid
tightly packed corneal accumulations of lymphocytes with firm edges located in the peripheral cornea
may be confluent and form a snowbanking
- More involvement of posterior portion
59
Koeppe nodules
Present on Iris pupil margin
60
Other gene name for NOD2
CARD15
61
Cause of hypercalcemia and hypercalcuria
Overproduction of 25 hydroxyvitamin D1 alpha hydroxylase which convers 25 OHD to 1-25 by sarcoid macrophages
62
Symptoms of large vessel disease
- Seizures
- Headache
- Focal deficits (hemiparesis, gross and fine motor deficits, gait abnl)
- Cranial neuropathies
- Movement d/o
63
Angiographic appearance of non progressive large vessel disease
Focal segmental stenosis often with alternating areas of narrowing and dilatation with banded or striated appearance
64
Progressive large medium vessel disease
- Uncommon
- Less acute
- Longer standing
- Non focal
- Cognitive, seizures, headaches
65
Small vessel disease
```
Presentation gradual
Headache
Behavioral changes
Atypical presentation of psych disease
Cognitive decline
seizures
```
66
Pathology of small vessel vasculitis
Non granulomatous, lymphocytic infiltration of small vessels; macrophages, PMNs and occasionally eosinophils
- Microglial activation
67
Small vessel vasculitis
Involves vessels throughout the brain and meninges that are not the primary branches of the circle of Willis
68
Large vessel
Targets major arteries, such as the anterior, middle, and posterior
69
Diagnostic criteria of Takayasu
Angiographic abnormalities of the aorta or its main branches demonstrated by angiography, CT or MRI
plus at least 1
- Decreased peripheral pulses and/or claudication of extremities
- Blood pressure difference between limbs >10
- Bruits over the aorta and or its major branches
- Systolic/diastolic HTN
- ER >20 or CRP above normal
70
Sweet syndrome
- Spiking fevers
- Crops of tender, raised, pseudovascular, erythematous plaques or nodules
- Arthritis
- Multifocal osteomyelitis
71
Supradiaphragmatic disease of TA
- Headaches
- Strokes
- Dizziness
- Syncope
- Seizures
72
Infradiaphragmatic disease of TA
- Mid aortic syndrome
- HTN
- Abdominal pain
- Abdominal bruits
73
What vessels are affected in Behcets
Arteries veins. Variable vessel vasculitis
74
Treatment for ocular behcets as opposed to ulcers
AZA for ocular ( thalidomide, dapsone, colchicine for the rest)
75
Mucha habarmann syndrome
Rare idiopathic dermatosis with red scaly papules
Papular, look like chicken pox
Can see fever and joint pain
Tx is steroids and MTX
76
Most common ANCA associated with EGPA
- MPO (<50 percent)
77
How many kids fail first dose of IVIG?
- 10-20 percent
78
How many kids progress to develop CAA?
- 5 percent
79
What pathway is activated in hSP?
- Alternative complement pathway
80
Cryoglobulinemic vasculitis
- 80-90 percent associated with Hep C
Labs
- Low C4, positive RF, mixed II and III cyro, elevated LFTs
