Vascular pathology part 1 (vasculitis) Flashcards
(168 cards)
1
Q
3 layers of the arterial wall include:
A
Endothelial intimal (internal elastic lamina)
Smooth muscle media (external elastic lamina)
Connective tissue adventitia (CT, nerve fibers, & vasa vasorum feeds it!)
2
Q
Granulomatous/large vessel vasculitis classically involves branches of which blood vessels? Which age group/gender is this most common in?
A
Carotid artery & most common in older females (>50)
3
Q
How does LVV/Granulomatous vasculitis present as?
A
1) Headache (temporal artery)
2) Visual disturbances (Opthalmic artery)
3) Jaw claudication (impaired movement)
4) Flu-like symptoms & joint pain
5) ESR is elevated
4
Q
1) Headache (temporal artery)
2) Visual disturbances (Opthalmic artery)
3) Jaw claudication (impaired movement)
4) Flu-like symptoms & joint pain
5) ESR is elevated
A
the classic presentation of LVV/Granulomatous vasculitis
5
Q
What does a biopsy of LVV/Granulomatous vasculitis show?
A
1) Inflamed vessel walls with giant cells & intimal fibrosis
2) Segmental lesions
6
Q
What is the Rx of LVV/Granulomatous vasculitis? Without Rx what is the patient at risk of?
A
Corticosteroids & without Rx patient has a high risk of blindness
7
Q
Blood vessel walls are made up of 3 basic components (not layers)
A
Endothelial cells
Smooth muscle cells
ECM (elastin, collagen, & glycosaminoglycans)
8
Q
________ are principal points of physiologic resistance to blood flow.
A
Arterioles
9
Q
Arteries of 3 types:
Large/elastic arteries
Give an example & what is the media rich in?
A
Aorta & its large branches
Media is rich in elastic fibers
10
Q
Arteries of 3 types:
Medium/muscular arteries. Give an example & what is the media rich in?
A
Coronary & renal arteries
Media is rich in smooth muscle cells
11
Q
Arteries of 3 types:
Small arteries/Arterioles. Where are they located?
A
Within the substance of tissues & organs
12
Q
Inflammation:
Describe a Type II hypersensitivity
A
Complement mediated
13
Q
Inflammation:
Describe a Type III hypersensitivity
A
Immune complex mediated
It’s responsible for most cases of vasculitis
14
Q
Inflammation:
Describe a Type IV hypersensitivity
A
Cell mediated
15
Q
Describe the pathophysiology of Vasculitis induced by type III hypersensitivity?
A
- Immune complex is deposited in BV, this activated the complement & releases C5a
- C5a recruits neutrophils which damage the endothelium causing thrombosis (narrowed lumen) leading to ischemia
16
Q
Henoch-Schönlein purpura is an example of which type of vasculitis
A
Immune complex (III) mediated vasculitis
17
Q
Describe Vasculitis induced by type IV hypersensitivity?
A
A delayed hypersensitivity reaction that can be involved in some types of vasculitis that have granulomas
18
Q
Temporal arteritis is an example of which type of vasculitis induced by hypersensitivity reactions
A
Type IV hypersensitivity induced vasculitis
19
Q
Rickettsia, Meningococcus, & fungus can all cause vasculitis due to
A
Direct invasion of a pathogen
20
Q
What are two key lab findings used when looking for vasculitis?
A
ANCA (Antineutrophil cytoplasmic antibodies)
ESR (Erythrocyte sedimentation rate)
21
Q
How do ANCA’s cause vessel damage & how are they detected?
A
Seen in some small vessel vasculitis
ANCAs activate neutrophils & cause a release of enzymes of free radicals that cause vessel damage
Detected with immunofluorescence
22
Q
What are the 2 types of ANCAs:
Describe Cytoplasmic (c-ANCAs)
What are the vasculitides associated with it?
A
Cytoplasmic (c-ANCAs) that have Ab’s against proteinase 3 in cytoplasmic granules which show cytoplasmic staining pattern
Wegners
Chrug-strauss
Microscopic polyangitis
23
Q
What are the 2 types of ANCAs:
Wegener’s granulomatosis is an example of
A
Cytoplasmic (c-ANCAs)
24
Q
What are the 2 types of ANCAs:
Describe Perinuclear (p-ANCAs)
A
Perinuclear (p-ANCAs) has Abs against myeloperoxidase, it shows perinuclear staining
25
What are the 2 types of ANCAs:
Churg-Strauss syndrome & Polyarteritis nodosa is an example of
Perinuclear (p-ANCAs) diseases
26
Giant cell arteritis (granulomatous) & Takayasu's arteritis are examples of
Large vessel vasculitis
27
Polyarteritis nodosa, Kawasaki's disease, & Thromboangitis obliterans are examples of
Medium vessel vasculitis
28
Hypersensitivity vasculitis (Henoch Schönlein purpura), Churg-Strauss syndrome, & Wegener granulomatosis are all examples of
Small vessel vasculitis
29
Describe the clinical representations of Large vessel vasculitis
It presents with loss of pulse
(vasculitis -> narrowed lumen -> less blood flow)
OR
Stroke (due to carotid artery involvement)
30
Describe the clinical representations of Medium vessel vasculitis
Presents with infarction or aneurysm (due to involvement of med arteries i.e renal, coronary, popliteal infarct damage can also lead to an aneurysm)
31
Describe the clinical representations of Small vessel vasculitis
Presents with palpable purpura
32
What are general warning signs of vasculitis
Fever, Weight loss, malaise, & myalgias
33
What's the disease?
Temporal arteritis look for:
- Prominent temporal artery
- Biopsy shows multinucleated giant cells (granulomatous vasculitis)
34
Old female patient
Presentation:
Headache in the temporal region
Pain in the jaw while chewing
Muscle aches and pains
Develops problems with vision.
Exam & labs:
Nodular and palpable temporal artery.
Elevated ESR
Biopsy:
Granulomatous inflammation & giant cells
What is the diagnosis?
Giant cell (temporal) arteritis
35
Describe the following aspects of Giant cell (temporal) arteritis
- Presentation (key points)
- Exam/labs
- Biopsy
Usually an older female patient
Presentation:
Headache in the temporal region
Pain in the jaw while chewing
Muscle aches and pains
Develops problems with vision.
Exam & labs:
Nodular and palpable temporal artery.
Elevated ESR
Biopsy:
Granulomatous inflammation & giant cells
36
Describe the pathology of Giant cell arteritis
The vessels tend to be cord-like & show nodular thickening
37
Describe the microscopy of Giant cell arteritis
- Focal granulomatous inflammation of the temporal artery
- Fragmented internal elastic lamina
- Giant cell
38
Describe the findings in the histo slide & the likely diagnosis
The Elastic tissue stain shows a section of a temporal artery with:
- Focal destruction of the internal elastic membrane & Intimal thickening (characteristic of long-standing/healed arteritis)
Diagnosis: Temporal (giant cell) arteritis
39
- Temporal/unilateral headaches (near the TA)
- Painful/Palpably enlarged temporal artery
- Generalized muscle ache/stiffness
- Temporary/permanent blindness (Opthalmic artery)
- Jaw Claudication
Key indicators of which condition?
Giant cell temporal arteritis
40
Describe the lab findings of Giant cell (temporal) arteritis
- Elevated ESR
41
How often is a temporal artery biopsy positive?
Approximately 60% of cases
42
This X-ray is indicative of to numerous areas of constriction in the subclavian arteries, patient likely has absent or very weak pulses in the upper extremities. What is the likely diagnosis?
Takayasu's arteritis
43
Middle aged Asian woman
Presentation:
- Visual disturbances
- Decreased BP in upper extremity
- Absent/very weak pulses in upper extremities
Exams/labs:
- Narrowed aortic arch vessels on angiography
Biopsy:
- Granulomatous inflammation with giant cells
Diagnosis?
Takayasu's arteritis (aka pulseless disease)
44
Describe the following aspects of Takayasu's arteritis
- Presentation (key points)
- Exam/labs
- Biopsy
Usually middle aged Asian women
Presentation:
- Visual disturbances
- Decreased BP in upper extremity
- Absent/very weak pulses in upper extremities
Exams/labs:
- Narrowed aortic arch vessels on angiography
Biopsy:
- Granulomatous inflammation with giant cells
45
What are the key differences between Giant cell (temporal) arteritis & Takayasu's arteritis?
GCTA:
- Older women (over 50)
Takayasu's Arteritis:
- Middle aged Asian women (under 50)
46
What arteries does Takayasu's arteritis typically involve?
Usually the aorta & aortic arch vessels (carotids, subclavian, pulmonary, renal, & coronary)
47
Granulomatous inflammation/vasculitis is due to what type of hypersensitivity reaction?
Type IV hypersensitivity reaction
48
What are the findings in the following histological view? What's the diagnosis?
- Destruction of the arterial media via mononuclear inflammation with giant cells
Diagnosis: Takayasu's arteritis
49
Describe the pathophysiology of Takayasu's arteritis
Thickening of the vessels (the aorta & its branches) with narrowing (stenosis) of the lumen causing less blood flow
50
Describe the key clinical findings of Takayasu's arteritis
- Dizziness
- Syncope (fainting)
- Absent upper extremity pulse
- Blood pressure discrepancy (low upper extremity BP)
- Visual disturbance
51
What is the crucial test to find Takayasu's
Angiography
52
- Dizziness
- Syncope (fainting)
- Absent upper extremity pulse
- Blood pressure discrepancy (low upper extremity BP)
- Visual disturbance
Describes which condition?
Takayasu's arteritis
53
Young male IV drug abuser with a history of HBV
Presentation:
- Hypertension
- Abdominal pain
- Melena (black/tarry stools)
- Muscle aches/pains
- Skin nodulations
Exams/labs:
- HBsAg +ve
- pANCA +ve
Biopsy:
- Segmental transmural inflammation with fibrinoid necrosis (Blood vessels)
Diagnosis:?
Polyarteritis nodosa (PAN)
54
Describe the following aspects of PAN arteritis
- Presentation (key points)
- Exam/labs
- Biopsy
Young male IV drug abuser with a history of HBV
Presentation:
- Hypertension
- Abdominal pain
- Melena (black/tarry stools)
- Muscle aches/pains
- Skin nodulations
Exams/labs:
- HBsAg +ve
- pANCA +ve
Biopsy:
- Segmental transmural inflammation with fibrinoid necrosis (Blood vessels)
55
Describe the pathophysiology of Polyarteritis nodosa
It involves multiple medium sized vessels & it's associated with segmental transmural inflammation of the vessel walls. This weakens the wall focally which causes dilation (aneurysm) that are responsible for the nodules on the skin
56
A systemic disease that affects medium & small sized muscular arteries (i.e kidney, heart, liver, GIT, & skin NOT the lungs) with a strong association to HBV antigenemia hypersensitivity to drugs (ex IV amphetamines)
Polyarteritis nodosa (PAN)
57
Polyarteritis nodosa is mediated by which type of Hypersensitivity reaction?
Type III hypersensitivity (Immune complex deposition via HBsAg/anti-HBsAg)
58
The histological slide shows what? What is the likely diagnosis?
- Fibrinoid necrosis & disruption of the vessel wall
- Neutrophil infiltrates
Likely diagnosis: Polyarteritis
59
The histological slide shows what? What is the likely diagnosis?
- Segmental fibrinoid necrosis & thrombotic occlusion of the lumen of a small artery
- Transmural inflammation (all layers)
Diagnosis: Polyarteritis nodosa
60
Describe the consequences of thrombosis & Weakened focal BV wall in Polyarteritis nodosa
Thrombosis = Infarction
Weakened vessel wall = Aneurysms (Kidney, heart, & Gi)
61
What are the key findings in the target organs (Kidneys, GI, skin, & Coronary arteries) of Polyarteritis nodosa?
Ischemic damage:
Kidneys = Vasculitis/infarction causes hypertension, hematuria, & albuminuria
GI tract = Bowel infarction causes abdominal pain & melena
Skin = Ischemic ulcers & nodules
Coronary arteries = Aneurysms & MI
62
What is the most common cause of death in Polyarteritis nodosa?
Renal failure due to MC COD
63
Describe the common lab findings in Polyarteritis nodosa
- HbsAg +ve in 30% of cases
- Hematuria with RBC casts
64
What is the Rx of Polyarteritis nodosa? What is a major complication if it's left untreated?
Rx: Corticosteroids & Cyclophosphamide
Untreated = Almost fatal
65
Describe the key pathological findings of Churg-Strauss syndrome
A variant of PAN it's a systematic vasculitis that happens in people with asthma (small & medium BVs)
- Inflammation of the vessel wall (lots of eosinophils
- Fibrinoid necrosis
- Thrombosis & infarction
66
A variant of PAN it's a systematic vasculitis that happens in people with asthma (small & medium BVs)
- Inflammation of the vessel wall (lots of eosinophils
- Fibrinoid necrosis
- Thrombosis & infarction
Describes which condition?
Churg-Stauss syndrome
67
What's the Rx for Churg-Strauss syndrome?
Corticosteroids
68
What are the key clinical features of Churg-Strauss syndrome?
- History of atopy
- Bronchial asthma
- Allergic rhinitis
- Peripheral blood eosinophilia
69
What are the usual lab findings for Churg-Strauss syndrome?
- Peripheral eosinophilia
- High serum IgE
- pANCA
70
4-yr old Japanese child
Presentation:
- Fever
- Red eyes/mouth
- Rash (trunk/extremities)
- Peeling skin
- Cervical lymphadenopathy
- Edema
Exam/Labs:
- ECG changes consistent with myocardial ischemia
Diagnosis?
Kawasaki disease (mucocutaneous lymph node syndrome)
71
Describe the following aspects of Kawasaki Disease
- Presentation (key points)
- Exam/labs
- Biopsy
4-yr old Japanese child
Presentation:
- Fever
- Red eyes/mouth
- Rash (trunk/extremities)
- Peeling skin
- Cervical lymphadenopathy
- Edema
Exam/Labs:
- ECG changes consistent with myocardial ischemia
72
Describe the key etiological features and pathophysiology of Kawasaki disease
A childhood vasculitis (under 5yrs) more common in Japanese & Hawaiian children
Transmural inflammation of the vessels via neutrophils & fibrinoid necrosis can lead to coronary thrombosis or aneurysm
73
What's the condition?
Kawasaki Disease
74
What's the condition?
Kawasaki disease
75
Describe the clinical findings of Kawasaki disease
High fever
Erythematous rash (trunk/extremities)
Desquamation/peeling skin
Mucosal inflammation (red eyes/mouth)
Erythema/Edema of hands/feet
Cervical lymphadenopathy
Acute MI (Children)
76
Describe the common lab findings of Kawasaki disease
Neutrophilic leukocytosis
Thrombocytosis **
High ESR
Abnormal ECG (hints at acute MI)
77
What is the Rx of Kawasaki disease?
- Aspirin can be used to reduce the long term sequelae of KD
78
What Rx do you want to avoid giving patients with Kawasaki disease? What are the complications of giving this particular Rx?
avoid giving corticosteroids! Unlike most other vasculitis-related conditions it increases the risk for coronary aneurysms/ruptures
79
Young male from Israel who smokes
Presentation:
- Pain in the foot (severe + present at rest)
Exam/Labs:
- Ulcers/necrosis (fingers/toes)
- missing fingers (potentially)
Biopsy:
- Acute inflammation of BV wall with obliteration of the lumen due to a thrombus
Diagnosis?
Thromboangitis Obliterans (Buerger's disease)
80
Describe the following aspects of Thromboangitis Obliterans (Buerger's disease)
- Presentation (key points)
- Exam/labs
- Biopsy
Young male (from Israel NOT always) who smokes
Presentation:
- Pain in the foot (severe + present at rest)
Exam/Labs:
- Ulcers/necrosis (fingers/toes)
- missing fingers (potentially)
Biopsy:
- Acute inflammation of BV wall with obliteration of the lumen due to a thrombus
81
Peripheral vascular disease common in smokers
Thromboangitis Obliterans aka Buerger's disease
82
Describe the features of the earliest stage of Thromboangitis Obliterans (Buerger's disease)
- Acute inflammation (small/medium arteries) in the extremities
- Thrombus formation (due to the inflammation)
- Involvement of the entire neurovascular compartment
83
What does the histo slide show? What's the condition?
An occluded lumen (due to a thrombus) & leukocyte infiltration of the BV wall
Diagnosis: Thromboangitis Obliterans (Buerger's disease)
84
What are the early manifestations of Thromboangitis Obliterans (Buerger's disease)
Intermittent Claudication of hands/feet & cramping in muscles after exercise (relieved by rest)
85
Age range for Thromboangitis Obliterans (Buerger's disease)
25-40yr old men that are heavy smokers, usually from Israel, Japan, or India
86
What are the late manifestations of Thromboangitis Obliterans (Buerger's disease)
Painful ulceration of digits & eventual gangrene that need amputation
87
How do you diagnose & treat Thromboangitis Obliterans (Buerger's disease)
Diagnose via a biopsy
Treat the early states by smoking cessation
88
What is the key differential between Hypersensitivity (leukocytoclastic) vasculitis a Small vessel vasculitis & Polyarteritis nodosa a medium vessel vasculitis
Leukocytoclastic:
- lesions tend to be the same age
- necrotizing glomerulonephritis & involvement of pulmonary capillaries is common
- Purpura are palpable
89
Describe the features of Hypersensitivity (leukocytoclastic) vasculitis
A type III hypersensitivity (immune-complex) mediated vasculitis that's characterized by
- acute inflammation of small BV
- Palpable Purpura (it doesn't usually affect other organs)
90
Which triggers can precipitate Hypersensitivity (Leukocytosis) vasculitis
Exogenous antigens triggered by:
- Drugs (aspirin, penicillin, & thiazide diuretics)
- Infectious organisms (Strep/staph, TB, & viruses)
- Foods
Chronic diseases:
- SLE
- RA
91
Describe the pathological features of Hypersensitivity (Leukocytosis) vasculitis
Acute inflammation (small BV)
Leukocytoclastic (neutrophils undergoing karyorrhexis)
Erythrocyte extravasation
92
What findings are in the skin biopsy slide? What's the likely diagnosis?
Fragmentation of neutrophils in & around the BV walls, the likely diagnosis is Hypersensitivity (Leukocytosis) vasculitis
93
What is the skin biopsy & image showing? What condition is this?
The image shows:
Hypersensitivity (leukocytoclastic) vasculitis
- Palpable purpuric tender papules
The biopsy shows:
- A BV surrounded by pink fibrin & neutrophils most of which are fragmented (leukocytoclastic)
- Extravasated RBCs
94
How does Hypersensitivity (Leukocytosis) vasculitis typically present?
- Usually as palpable/non-blancing purpura on the skin of the lower extremities
- Hemoptysis (coughing blood)
- Abdominal pain
- Hematuria
- Arthralgia
95
How do you diagnose & treat Hypersensitivity (leukocytoclastic) vasculitis
Diagnose with a skin biopsy
Rx: Remove the trigger
96
14yr old child with a history of URT
Presentation:
- Polyarthritis***
- Colicky abdominal pain
- Hematuria with RBC casts
- Palpable purpura (lower limbs & butt)***
Exams/Labs:
- Neutrophilic leukocytosis
- IgA-C3 Immune complex deposition in skin & kidneys (Type III hypersensitivity)
Diagnosis:?
Henoch Schönlein purpura
97
Describe the following aspects of Henoch Schönlein purpura
- Presentation (key points)
- Exam/labs
14yr old child with a history of URT
Presentation:
- Polyarthritis***
- Colicky abdominal pain
- Hematuria with RBC casts
- Palpable purpura (lower limbs & butt)***
Exams/Labs:
- Neutrophilic leukocytosis
- IgA-C3 Immune complex deposition in skin & kidneys (Type III hypersensitivity)
98
Descried the etiopathogenesis of Henoch Schönlein purpura
A variant of hypersensitivity vasculitis in CHILDREN. It usually happens after an URT infection & is caused by deposition of IgA-C3 immune complexes in the BV wall
Involves small BV, skin, GI, Kidney, Musculoskeletal system
99
Henoch Schonlein purpura (HSP): Patient has multiple palpable purpuric lesions in the lower limbs and buttock area.
100
What is the Rx for Henoch Schönlein purpura?
Steroids
101
What is Wegener Granulomatosis characterized by?
- Necrotizing granulomas & vasculitis in the URT/LRT (Granuloma formation with giant cells)
- Granulomatous vasculitis of URT/LRT & kidneys (small vessels)
- High association with cANCA
102
What types of hypersensitivity reactions contribute to Wegener Granulomatosis
Type II, III, & IV hypersensitivity reactions
103
What do the following slide & image show & what's the condition?
Slide:
- Vasculitis
- Giant cells
Image:
- Fatal Wegener granulomatosis with large nodular lesions
104
Describe the following
A saddle nose deformity because the septum is destroyed by granulomatous inflammation
Note congenital syphilis & lepromatous leprosy can have the same deformity
105
Describe the classical triad of Wegener Granulomatosis
1. Granulomas in URT/LRT
2. Saddle nose
3. Granulomatous inflammation of kidneys (causes Renal disease i.e. crescentic glomerulonephritis)
106
middle aged men with the peak being between 40-50yrs old
Presentation:
- URT = Chronic sinusitis, ulcers of nasopharyngeal mucosa, & saddle nose
- LRT = Rec. pneumonia with nodular lesions
- Kidney = Cresentric glomerulonephritis
Exams/Labs:
- cANCA (90% cases)
- Bilateral nodular infiltrates/cavitary lesions (CT)
Diagnosis:?
Wegener Granulomatosis
107
What's the diagnostic tool & treatment for Wegener Granulomatosis
Biopsy
&
Cyclophosphamide & steroids
108
What are the dangers of treating Wegener Granulomatosis with cyclophosphamide? what's the death rate without Rx?
Hemorrhagic cystitis & transitional cell carcinoma
Death rate without Rx = 80% within a year
109
What are the culprits for fungal vasculitis?
MAC:
Mucor
Aspergillus
Candida
110
What are the culprits for Rocky Mountain spotted fever?
Caused by Rickettsia rickettsiae (transmitted via the hard dermacentor andersoni tick)
Look for: fever, rash, & hist tick bite!
- Petechial lesions spreading from extremities to trunk
111
What are the culprits for Disseminated meningococcemia?
Small vessel vasculitis causing petechial hemorrhages
112
What are the culprits for viral vasculitis?
HBV & HCV = immune complex mediated
113
What are the culprits for infective endocarditis?
Immune complex vasculitis thought to be responsible for:
- Roth's spots in retina
- Janeway's lesions on hands (painless)
- Osler's nodes on hands (painful)
- Glomerulonephritis
114
Child presents with palpable purpura on the butt & legs, GI pain/bleeding, & hematuria. They previously had a respiratory tract infection. What is the MOA of the disease?
Vasculitis due to IgA immune complex deposition (Henoch-Schönlein purpura)
115
Patient with a history of asthma, presents with elevated serum p-ANCA levels & eosinophilia surrounding multiple organs especially the heart & lungs. What is the likely diagnosis?
Churg-Strauss Syndrome
116
A middle aged-male with presents with elevated serum p-ANCA, hemoptysis with bilateral nodular lung infiltrates, & hematuria. Upon examination there's necrotizing vasculitis involving multiple organs especially the kidneys & lungs. What is the condition & how would you treat it?
Microscopic Polyangiitis Rx with corticosteroids & cyclophosphamide
117
A middle aged man presents with sinusitis/nasopharyngeal ulceration, hemoptysis with bilateral nodular lung infiltrates, & hematuria. Labs show elevated serum c-ANCA levels. A biopsy would reveal what? What is the condition & the treatment?
Biopsy shows = Large necrotizing granulomas with adjacent necrotizing vasculitis
Condition: Wegener Granulomatosis
Rx: Cyclophosphamide & steroids
118
A man who is a heavy smoker comes to the office presenting with blackened finger tips. What is the cause of the necrosis? What is the condition? What is the treatment?
Smoking causes necrotizing vasculitis of the digits leading to ulceration, gangrene, & auto amputation.
Condition: Buerger disease (Thromboangitis Obliterans)
Rx: Smoking cessation
119
A 3yr old Asian child is brought to the ER for fever, conjunctivitis, erythematous rash of the palms & feet, & localized cervical lymphadenopathy.
1. What is the condition?
2. Which artery is involved and what is the child at risk of developing?
3. What's the treatment?
1. Kawasaki disease
2. Coronary artery involvement can lead to thrombosis with MI which can cause aneurysms & ruptures
3. Rx with aspirin & IVIG (the disease is self-limiting)
120
A young woman presents to the clinic with hypertension, abdominal pain, tarry stools (melena), neurological disturbances, & skin lesions.
1. What artery is likely involved to be causing melena?
2. What's the condition?
3. What serum Ab is this condition associated with?
4. What's the treatment?
1. Mesenteric artery = melena
2. Polyarteritis nodosa
3. HBsAg
4. Rx with corticosteroids & cyclophosphamide (if not treated it's fatal within 1 yr)
121
A young man with a history of HBV presents to the clinic for a wellness check & constipation yielding black stools. Upon examination it's determined he has hypertension & imaging shows lesions with a "string of pearls" appearance
1. What's the condition
2. What do the lesions consist of?
1. Early stage Polyarteritis nodosa
2. Lesions consist of transmural inflammation with fibrinoid necrosis
122
A 40-yr old female from China presents to the ER with visual disturbances, weak/absent pulse in the upper extremities. Labs show her ESR are markedly elevated.
1. What is the condition?
2. What artery is classically involved?
3. What's the treatment?
1. Takayasu arteritis
2. It classically involves granulomatous vasculitis in the aortic arch at the branching points
3. Rx with corticosteroids
123
A 70-yr old female presents with a headache, visual disturbances, jaw claudication, & flu-like symptoms.
1. A biopsy would be likely to reveal what findings?
2. What's the condition & arteries involved?
3. What's the treatment?
1. Inflamed vessel wall with giant cells & intimal fibrosis (Segmental lesions)
2. Temporal (giant cell) arteritis involving the Temporal & Opthalmic arteries
3. Rx with corticosteroids (risk of blindness without treatment)
124
Antibodies against neutrophil proteinase 3 indicates, what does it present with & what type of vasculitis?
Nasal mucosal ulcerations and hematuria
125
A 5-year-old boy is brought to the physician by his parents because of a 4-day history of arthralgia, abdominal pain, and lesions on his arms and legs. Ten days ago, he had an upper respiratory tract infection. A photograph of his right leg is shown. Further evaluation is most likely to show which of the following?
IgA vasculitis (Henoch-Schönlein purpura.)
126
A 63-year-old man comes to the physician because of a 2-day history of fever and blood-tinged sputum. He has also had a productive cough for 1 year and has had 3 episodes of sinusitis during this time. Physical examination shows palpable erythematous skin lesions over his hands and feet that do not blanch on pressure. There are ulcerations of the nasopharyngeal mucosa and a perforation of the nasal septum. His serum creatinine is 2.6 mg/dL. Urinalysis shows acanthocytes, 70 RBCs/hpf, 2+ proteinuria, and RBC casts. An x-ray of the chest shows multiple, cavitating, nodular lesions bilaterally. Further evaluation of this patient is most likely to show which of the following findings?
Elevated c-ANCA titers
127
A 54-year-old man comes to the physician because of a cough with blood-tinged sputum for 1 week. He also reports fever and a 5-kg (11 lb) weight loss during the past 2 months. Over the past year, he has had 4 episodes of sinusitis. Physical examination shows palpable nonblanching skin lesions over the hands and feet. Examination of the nasal cavity shows ulceration of the nasopharyngeal mucosa and a depressed nasal bridge. Oral examination shows a painful erythematous gingival enlargement that bleeds easily on contact. Which of the following is the most likely cause of the patient's symptoms?
Neutrophil-mediated damage
128
A 32-year-old woman who recently emigrated to the USA from Japan comes to the physician because of a 3-month history of night sweats, malaise, and joint pain. During this time, she has also had a 6-kg (13-lb) weight loss. Physical examination shows weak brachial and radial pulses. There are tender subcutaneous nodules on both legs. Carotid bruits are heard on auscultation bilaterally. Laboratory studies show an erythrocyte sedimentation rate of 96 mm/h. A CT scan of the chest shows thickening and narrowing of the aortic arch. Microscopic examination of the aortic arch is most likely to show which of the following findings?
Granulomatous inflammation of the media, diagnosis is Takayasu's arteritis
129
A 38-year-old man comes to the physician because of a 3-week history of a painful rash affecting his left foot. For the past 2 years, he has had recurrent episodes of color changes in his fingers when exposed to the cold; his fingers first turn white and then progress to blue and red before spontaneously resolving. He has smoked two packs of cigarettes daily for 20 years. His blood pressure is 115/78 mm Hg. Physical examination shows multiple tender, dark purple nodules on the lateral surface of the left foot with surrounding erythema that follow the course of the lateral marginal vein. There are dry ulcers on the tip of his right index finger and on the distal aspect of his right hallux. Serum lipid studies show no abnormalities. Biopsy of the dorsalis pedis artery will most likely show which of the following findings?
Segmental thrombosing inflammation with sparing of the internal elastic lamina
130
A 45-year-old man with asthma comes to the physician because of a 1-month history of progressively worsening shortness of breath and cough. He also has a history of chronic sinusitis and foot drop. Current medications include an albuterol inhaler and inhaled corticosteroid. Physical examination shows diffuse wheezing over both lung fields and tender subcutaneous nodules on both elbows. Laboratory studies show a leukocyte count of 23,000/mm3 with 26% eosinophils and a serum creatinine of 1.7 mg/dL. Urine microscopy shows red blood cell casts. Which of the following is the most likely diagnosis in this patient?
Eosinophilic granulomatosis with polyangiitis
131
A previously healthy 48-year-old man comes to the physician for a 3-month history of myalgias and recurrent episodes of retrosternal chest pain and dizziness. He has had a 5-kg (11-lb) weight loss during this period. His temperature is 39.1°C (102.3°F), pulse is 90/min, and blood pressure is 160/102 mm Hg. Physical examination shows lacy, purplish discoloration of the skin with multiple erythematous, tender subcutaneous nodules on the lower legs. Some of the nodules have central ulcerations. Serum studies show an erythrocyte sedimentation rate of 76 mm/h and creatinine level of 1.8 mg/dL. Renal MR angiography shows irregular areas of dilation and constriction in the renal arteries bilaterally. Further evaluation of this patient is most likely to show which of the following?
Transmural inflammation with fibrinoid necrosis on arterial biopsy, Diagnosis polyarteritis nodosa
132
transmural inflammation of the arterial wall with leukocytic infiltration and fibrinoid necrosis
Polyarteritis nodosa
133
Wegners granulomatosis (Granulomatosis eith polyangitis)
Features include
1. C-ANCA
2. Effects:
- Lungs
- Nasopharynx
- Kidneys
3. Small arteries
134
1. C-ANCA
2. Effects:
- Lungs
- Nasopharynx
- Kidneys
3. Small arteries
Wegners granulomatosis (Granulomatosis WITH polyangitis)
135
Microscopic polyangitis
features include:
1. P-ANCA
2. Lungs & Kidneys only
3. Small arteries
136
1. P-ANCA
2. Lungs & Kidneys only
3. Small arteries
Microscopic polyangitis
features include:
137
Henoch-Schonlein purpura (IgA vasculitis)
Features includes:
It's due to IgA deposits in the arteries (Small)
Hematuria
Palpable purpura
Arthralgia
Stomach pain**
Recent URI (B19 or S.aureus)**
138
It's due to IgA deposits in the arteries (small)
Hematuria
Palpable purpura
Arthralgia
Stomach pain**
Recent URI (B19 or S.aureus)**
Henoch-Schonlein purpura (IgA vasculitis)
Features includes:
139
Cryoglobulinemic vasculitis (Hep C vasculitis)
Features include
Due to a Hep C infection (small arteries)
Hematuria
Palpable purpura
Arthralgia
Hep C & Cryoglobulin deposits **
Rx the underlying Hep C
140
Chrug-Strauss (Eosinophilic granulomatous with polyangitis)
Features include:
Causes necrotizing eosinophilic granulomatous infiltration of arteries (small)
141
Causes necrotizing eosinophilic granulomatous infiltration of arteries (small)
Chrug-Strauss (Eosinophilic granulomatous with polyangitis)
Features include:
142
Polyarteritis nodosa (PAN)
Features include:
Causes transmural arteritis with fibrinoid necrosis
Involves renal A, coronary A, & Mesenteric A (med)
String of pearls (pattern of necrosis0
143
Causes transmural arteritis with fibrinoid necrosis
Involves renal A, coronary A, & Mesenteric A (med)
String of pearls (pattern of necrosis0
Polyarteritis nodosa (PAN)
Features include:
144
Buerger's (Thromboangitis Obliterans)
Usually men 30-50yrs old, caused by heavy smoking & treated with cessation (med arteries)
Distal digit thrombosis (segmental thrombosis)
Autoamputation of digits
145
Usually men 30-50yrs old, caused by heavy smoking & treated with cessation (med arteries)
Distal digit thrombosis (segmental thrombosis)
Autoamputation of digits
Buerger's (Thromboangitis Obliterans)
146
Kawasaki
Features include:
Necrotizing vasculitis involving the coronary artery in (Conjunctivitis) children (Med arteries)
MI in kids
Cervical lymphadenopathy
Strawberry tongue
Arthralgia/edema hand/feet
Conjunctivitis
Desquamating skin
Rx with aspirin & immunoglobulins (side effect retts)
147
Necrotizing vasculitis involving the coronary artery in (Conjunctivitis) children (Med arteries)
MI in kids
Cervical lymphadenopathy
Strawberry tongue
Arthralgia/edema hand/feet
Conjunctivitis
Desquamating skin
Rx with aspirin & immunoglobulins (side effect retts)
Kawasaki
Features include:
148
Takayasu (Pulseless disease)
Features include:
Transmural fibrous thickening of aortic branches, usually Japanese women under 40yrs (large arteries)
Subclavian artery degeneration
Weak/absent upper pulse
low/uneven upper BP
Paresthesia
149
Transmural fibrous thickening of aortic branches, usually Japanese women under 40yrs (large arteries)
Subclavian artery degeneration
Weak/absent upper pulse
low/uneven upper BP
Paresthesia
Takayasu (Pulseless disease)
Features include:
150
Giant cell (Temporal) arteritis
Features include:
Intimal thickening & lamina fragmentation (large arteries)
High ESR
Jaw claudication
Temporal headaches (TA)
Visual disturbances (OA)
Polymyalgia rheumatica
Rx corticosteroids before biopsy!!!
151
Intimal thickening & lamina fragmentation (large arteries)
High ESR
Jaw claudication
Temporal headaches (TA)
Visual disturbances (OA)
Polymyalgia rheumatica
Rx corticosteroids before biopsy!!!
Giant cell (Temporal) arteritis
Features include:
152
Vasculitis with IgA deposition in response to a GI or Respiratory tract infection (mucosal layers)
Henoch Schönlein purpura
Comon in children, presents with non-blanching/ palpable purpura on the butt & legs
153
What would you see histologically in someone with Giant cell (temporal) arteritis?
Granulomatous inflammation with giant cells (type IV hypersensitivity)
154
Granulomatous inflammation with giant cells (type IV hypersensitivity)
Elevated ESR
Jaw claudication
Headache
Giant cell (temporal) arteritis
155
What's the sequelae of giant cell arteritis? What do we administer to avoid it?
Retinopathy (blindness) treat with corticosteroids right way
156
Giant cell granulomatous inflammation within the branches of the aortic arch (esp, subclavian arteries)
Takayasu
157
Negative ANCA
Distal segmental thrombosing vasculitis
Ulcers/necrotic digits
Bueger's (Thromboangitis Obliterans)
Common in smokers
158
Segmental transmural inflammation of the BV wall with fibrinoid necrosis (type III)
Never involves the lungs & is associated with which infection?
Polyarteritis nodosa associated with HBV (HBsAg)
Abdominal pain
Melena
Hypertension
String of pears app
Skin/neuro symptoms
159
Allergic type vasculitis associated with asthma
Necrotizing granulomatous angiitis
P-ANCA +ve
Churg-Strauss (lungs & renal system involvement)
160
Primarily affects the coronary artery in children under 4yrs old
What do you treat it with & what substance secrete by platelet cells does the treatment inhibit
Kawasaki
Rx with Ig's IV aspirin to inhibit thromboxane A2 (COX2/TXA2) from platelets
Acute MI
Conjunctivitis
Strawberry tongue
Desquamation
Erythematous rash hand/feet
Edema
Cervical lymphadenopathy
161
C-ANCA +ve
Necrotizing granulomatous lesions of the nose, sinus, kidney, lungs, (nodular infiltrates & cavitary lesions)
Post UTR/LRT infection
Wegeners granulomatosis
Hematuria (glomerulonephritis)
Gi pain
**Sinusitis
Hemoptysis
Rx Cyclophosphamide (side effects can be hemorrhagic cystitis or transitional cell cancer)
162
P-ANCA +ve
Necrotizing vasculitis in organs like the lungs & kidneys
Micropoly angiitis
163
Wegener's granulomatosis
164
Giant cell granuloma
165
166
167
Key clinical features of Giant cell temporal arteritis:
- Temporal/unilateral headaches (near the TA)
- Painful/Palpably enlarged temporal artery
- Generalized muscle ache/stiffness
- Temporary/permanent blindness (Opthalmic artery)
- Jaw Claudication
168
Describe the findings in the histo slide & the likely diagnosis
The H&E stain shows a section of a temporal artery with:
- Giant cells
- Degenerated internal elastic membrane
Likely diagnosis: Temporal (giant cell) arteritis
