Flashcards in Pathology Deck (76):
Congenital heart diseases
1.R to L shunts-early cyanosis-blue babies
2.L to R shunts-late cyanosis with Eisenmengers phenomenon-blue kids
R to L shunts
Compatibility with life unless
Early cyanosis—“blue babies.”
Often diagnosed prenatally or become evident immediately after birth.
Usually require urgent surgical correction and/or maintenance of a PDA.
What are the R to L shunt disorders
The 5 Ts:
1. Truncus arteriosus (1 vessel)
2. Transposition (2 switched vessels)
3. Tricuspid atresia (3 = Tri)
4. Tetralogy of Fallot (4 = Tetra)
5. TAPVR (5 letters in the name)
Persistsnt Truncus arteriosus
Also have what
Truncus arteriosus fails to divide into pulmonary trunk and aorta due to lack of aorticopulmonary septum formation
most patients have accompanying VSD.
Aorta leaves RV (anterior) and pulmonary trunk leaves LV (posterior)
Due to failure of the aorticopulmonary septum to spiral.
If no sx die within weeks
Absence of tricuspid valve and hypoplastic RV
requires both ASD and VSD for viability.
Factor determining the prognosis
Shape of the heart,due to
Most common cause of early childhood cyanosis.
Caused by anterosuperior displacement of the infundibular septum
Pulmonary infundibular stenosis (most important determinant for prognosis)
Right ventricular hypertrophy (RVH)— boot-shaped heart on CXR
A Overriding aorta
early cyanotic “tet spells"
Squatting: SVR, right-to-left shunt, improves
Treatment: early surgical correction.
Pulmonary veins drain into right heart circulation
SVC, coronary sinus
associated with ASD and sometimes PDA to allow for right-to-left shunting to maintain CO.
L to R shunt diseases
Symptoms at birth
Symptoms appear when
Larger ones can causes
Most common congenital cardiac defect.
Asymptomatic at birth.
May manifest weeks later or remain asymptomatic throughout life.
Most self resolve
larger lesions may lead to LV overload and HF.
Two types/common/associated abnormalities
Difference from patent f.ovale
Defect in interatrial septum
loud S1/wide, fixed split S2.
Ostium secundum defects most common and usually occur as isolated findings
ostium primum defects rarer yet usually occur with other cardiac anomalies.
Symptoms range from none to HF.
Distinct from patent foramen ovale in that septa are missing tissue rather than unfused.
Function in fetal period
Neonatal period function
Later on what happens
In fetal period, shunt is right to left (normal).
In neonatal period, lung resistance shunt becomes left to right
progressive RVH and/or LVH and HF-Eisenmengers
Lower extremity cyanosis
Clinical signs 3
Age of onset
Uncorrected left-to-right shunt (VSD, ASD, PDA)
pulmonary blood flow pathologic remodeling of vasculature pulmonary arterial hypertension. RVH occurs to compensate -shunt becomes right to
Causes late cyanosis, clubbing and polycythemia.
Age of onset varies.
Associated conditions 2
Symtopms and signs
Later on CXR changes,due to
Aortic narrowing near insertion of ductus arteriosus (“juxtaductal”).
bicuspid aortic valve
Hypertension in upper extremities and weak, delayed pulse in lower extremities (brachial-femoral delay).
With age, collateral arteries erode ribs (notched appearance on CXR).
Congenital cardiac defect s associations
Alcohol exposure in utero (fetal alcohol syndrome)
Infant of diabetic mother
Prenatal lithium exposure Turner syndrome Williams syndrome
Alcohol exposure in utero (fetal alcohol syndrome)-ASD/VSD/PDA/TOF
Down syndrome-AV canal defects/ASD/VSD
Infant of diabetic mother-TPGV
Marfan syndrome-MVP/AR/thoracic aorta aneurysms and dissections
Prenatal lithium exposure -Ebstein anomaly(TV defect)
Turner syndrome -COA/bicuspid aortic v
Williams syndrome-supravalvular AS
Types and %
Causes for both
contrast X-ray in fibromyscular dysplasia
What's HT urgency
What's HT emergency/eg
Explain high renin HT
Defined as persistent systolic BP ≥ 140 mmHg and/or diastolic BP ≥ 90 mmHg
age, obesity, diabetes, physical inactivity, excess salt intake, excess alcohol intake, family history
black > white > Asian
90% -1° (essential) and related to CO or TPR
-renal/renovascular disease-renal artery stenosis (e.g., fibromuscular dysplasia -usually found in younger women-string of beads appearance)
Hypertensive urgency—severe (≥ 180/≥ 120 mmHg) hypertension without acute end-organ damage.
Hypertensive emergency—severe hypertension with evidence of acute end-organ damage
(e.g., encephalopathy, stroke, retinal hemorrhages and exudates, papilledema, MI, HF, aortic dissection, kidney injury, microangiopathic hemolytic anemia, eclampsia)
1.CVS-CAD, LVH, HF, atrial fibrillation
aortic dissection, aortic aneurysm
3.Renal-chronic kidney disease (hypertensive nephropathy)
B. Renal artery stenosis is a common cause (renovascular hypertension).
Stenosis decreases blood flow to glomerulus.
Juxtaglomerular apparatus (JGA) responds by secreting renin, which converts angiotensinogen to angiotensin 1.
Angiotensin I is converted to angiotensin 11 (ATII) by angiotensin converting enzyme (ACE).
ATII raises blood pressure by (I) contracting arteriolar smooth muscle, increasing total peripheral resistance and (2) promoting adrenal release of aldosterone, which increases resorption of sodium in the distal convoluted tubule (expanding plasma volume).
Leads to HTN with increased plasma renin and unilateral atrophy (due to low blood flow) of the affected kidney; neither feature is seen in primary hypertension
Important causes of stenosis include atherosclerosis (elderly males) and fibromuscular dysplasia (young females).
HLD signs 3
lipid deposited in histiocytes
Seen as plaque sin eye lids
Lipid deposited on tendons
Deposit in cornea
Old age-senile arcus
Premature if high cholesterol
Affects which vessels
Thickening of vessel walls-hardening and loss of elasticity
Medial calcific sclerosis-
Medium size arteries
Elastic and medium and large muscular arteries
Affect which vessels
Hyper plastic Appearance on histo,due to
Classically seen when
Affects small arteries and arterioles.
1.hyaline (thickening of vessel walls in essential hypertension or diabetes mellitus
classically produces glomerular scarring (arteriolonephrosclerosis)that slowly progresses to
chronic renal failure
2.hyperplastic (“onion skinning” in severe hypertension with proliferation of smooth muscle cells).
fibrinoid necrosis of the vessel wall with hemorrhage
classically causes acute renal failure with a characteristic 'flea-bitten' appearance
Medial calcific sclerosis
Blood flow affected
Affects medium-sized arteries.
Calcification of elastic lamina of arteries vascular stiffening without obstruction.
“Pipestem” appearance on x-ray
Does not obstruct blood flow
intima not involved
Vessels affecting frequency
elastic arteries and large- and medium-sized muscular arteries
a form of arteriosclerosis caused by buildup of cholesterol plaques.
Modifiable: smoking, hypertension, hyperlipidemia, diabetes.
Nonmodifiable: age, sex ( in men and postmenopausal women), family history.
Inflammation important in pathogenesis:
endothelial cell dysfunction
macrophage and LDL accumulation
foam cell formation
smooth muscle cell migration (involves PDGF and FGF), proliferation
extracellular matrix deposition
peripheral vascular disease
Abdominal aorta > coronary artery > popliteal artery > carotid artery B .
Angina, claudication, but can be asymptomatic.
Symptom of complications
Tertiary syphillis causes what ,special name
Abdominal aortic aneurysm site
High risk of rupture when
Abdominal aneurysms occure in who
Localized pathologic dilatation of the aorta.
May cause abdominal and/or back pain
which is a sign of leaking, dissection, or imminent rupture.
Associated with atherosclerosis
Risk factors include history of tobacco use, age, male sex, family history.
May present as palpable pulsatile abdominal mass .
Associated with cystic medial degeneration.
Risk factors include hypertension, bicuspid aortic valve, connective tissue disease (e.g., Marfan syndrome)
Also historically associated with 3° syphilis (obliterative endarteritis of the vasa vasorum).
Below renal vessels above bifurcation
Flank pain/hypotension/pulsatile mass
Involve which parts
If ascending aorta and descending both involves which type
Longitudinal intimal tear forming a false lumen
bicuspid aortic valve
inherited connective tissue disorders (e.g., Marfan syndrome).
Can present with tearing chest pain, of sudden onset, radiating to the back +/− markedly unequal BP in arms.
CXR shows mediastinal widening.
Can result in
Stanford type A (proximal):
involves Ascending aorta.
May extend to aortic arch
or descending aorta.
Treatment is surgery.
Stanford type B (distal):
involves descending aorta and/or aortic arch.
No ascending aorta involvement.
Treat medically with β-blockers, then vasodilators.
Ischemic artery disease types
Coronary steal syndrome
Chest pain due to ischemic myocardium
2° to coronary artery narrowing or spasm
no myocyte necrosis
What's stable angina
Acute ECG changes
Stable—usually 2° to atherosclerosis
exertional chest pain in classic distribution
ST depression on ECG
resolving with rest or nitroglycerin.
Prinze metal angina
Chest painoccurs 2° to coronary artery spasm
Occurs at rest
transient ST elevation on ECG
Known triggers include tobacco, cocaine, and triptans, but trigger is often unknown.
Treat with Ca2+ channel blockers, nitrates, and smoking cessation (if applicable).
Difference to NSTEMI
Increase in frequency or intensity of chest pain or any chest pain at rest.
thrombosis with incomplete coronary artery occlusion
+/− ST depression and/or
T-wave inversion on ECG
no cardiac biomarker elevation (unlike NSTEMI)
Coronary steal syndrome
Basis of what
Distal to coronary stenosis, vessels are maximally dilated at baseline.
Administration of vasodilators (e.g., dipyridamole, regadenoson) dilates normal vessels and shunts blood toward well-perfused areas flow and ischemia in poststenotic region.
Principle behind pharmacologic stress tests.
Due to what
ECG in both
Most often acute thrombosis due to rupture of coronary artery atherosclerotic plaque.
Acute onset typical chest pain
Lasting more than1/2hr
Not responding to rest or GTN
transmural, ECG may show ST elevations (STEMI)
subendocardial, ECG may show ST depressions (NSTEMI)
Cardiac biomarkers are diagnostic.
Sudden cardiac death
Usually due to
Death from cardiac causes within 1 hour of onset of symptoms
most commonly due to a lethal arrhythmia (e.g., ventricular fibrillation).
Associated with CAD (up to 70% of cases), cardiomyopathy (hypertrophic, dilated),
hereditary ion channelopathies (e.g., long QT syndrome, Brugada syndrome).
Progressive onset of HF over many years due to chronic ischemic myocardial damage
Arteries affected frequency 3
2wks -several months
Pale with Tetrazonium stain
Early coagulative necrosis-
release of necrotic cell contents into blood
Reperfusion injury may cause contraction bands (due to free radical damage and hyper contraction from high Ca influx)
Extensive coagulative necrosis.
Tissue surrounding infarct shows acute inflammation with neutrophils.
Complications -post MI fibrinous pericarditis
Pale yellow soft middle
Softest on day 10(high risk of rupture)
Macrophages cause phagocytosis
Granulation tissue formation in the border
Free wall rupture -tamponade
papillary muscle rupture -mitral regurgitation
interventricular septal rupture - VSD
LV pseudoaneurysm (risk of rupture).
2wks to months
Re canalized vessel
White gray area
Contracted scar complete
Dressler syndrome(AI pericarditis)
true ventricular aneurysm (risk of mural thrombus).
Gold standerd ix in first 6 hours
What indicates evolving or old infarct
Important cardiac enzymes 2
Which is more specific
Trop I rises when
Keeps elevated till
Useful for what
ST elevation (STEMI, transmural infarct)
ST depression (NSTEMI)
hyperacute (peaked) T waves
new left bundle branch block
pathologic Q waves or poor R wave progression (evolving or old transmural infarct).
Trop I- more specific
Cardiac troponin I rises after 4 hours
High for 7–10 days
CK-MB rises after 6–12 hours
Normal in 48 hours
predominantly found in myocardium but can also be released from skeletal muscle.
Useful in diagnosing reinfarction following acute MI because levels return to normal
to normal after 48 hours.
Types of infarcts
Amount of necrosis
Affects entire wall
ST elevation on ECG, Q waves
Due to ischemic necrosis of
Anteroapical (distal LAD) -V3-V4
Anterolateral (LAD or LCX) -V5-V6
Post MI complications
Arrythmia important cause of what
Most common during when
Cardiogenic shock occurs with what
Risk of what
Pseudo aneurysm means
Risks of 3
True v aneurysm means
important cause of death before reaching hospital
common in first few days.
LV failure and pulmonary edema.
Cardiogenic shock (large infarct high risk of mortality).
Ventricular free wall rupture -cardiac tamponade
papillary muscle rupture -severe mitral
interventricular septum rupture -VSD
Ventricular pseudoaneurysm formation (contained free wall rupture)— CO, risk of arrhythmia, embolus from mural thrombus;
greatest risk approximately 3–14 days post-MI (as with rupture).
True ventricular aneurysm—outward bulge during contraction (“dyskinesia”),
associated with fibrosis
arises 2 weeks to several months after MI.
Postinfarction fibrinous pericarditis—friction rub (1–3 days post-MI).
Dressler syndrome—autoimmune phenomenon resulting in fibrinous pericarditis (several weeks
RX for MI
For STEMI which is most important
Two methods of reperfusion
Do how soon
Types of fibrinolytic
SK side effects
Anticoagulation (e.g., heparin)
antiplatelet therapy (e.g., aspirin + clopidogrel),
Symptom control with nitroglycerin and morphine.
STEMI—In addition to above, reperfusion therapy most important (percutaneous coronary intervention preferred over fibrinolysis).
Three types of cardiomyopathies
Dilated -most common
Common etiology 2
Clinical signs 2
Dilatation occures how with fibers
Most common cardiomyopathy (90% of cases)
Often idiopathic or familial.
Other etiologies include
C-Coxsackie B virus myocarditis
C-chronic Cocaine use
Findings: S3, systolic regurgitant murmur
dilated heart on echocardiogram
balloon appearance of heart on CXR.
Treatment: Na+ restriction, ACE inhibitors, β-blockers, diuretics, digoxin, ICD, heart transplant.
Eccentric hypertrophy (sarcomeres added in
Due to want mutation
Complication 2,due to
If high risk
HOCM what, pathophysio ,symptoms
60–70% of cases are familial
commonly a β-myosin heavy-chain mutation
Can be associated with Friedreich ataxia.
Causes syncope during exercise
sudden death in young athletes due to ventricular arrhythmia.
Findings: S4, systolic murmur. May see mitral regurgitation due to impaired mitral valve closure.
cessation of high-intensity athletics
use of β-blocker
non-dihydropyridine Ca2+ channel blockers (e.g., verapamil)
ICD if patient is high risk.
Marked ventricular hypertrophy often septal
Obstructive hypertrophic cardiomyopathy
(subset)—asymmetric septal hypertrophy and systolic anterior motion of mitral valve outflow obstruction
dyspnea, possible syncope.
What's endo myocardial fibroelastosis,occures in who
Loefflers syndrome what's complication
ECG change ,especially in
endocardial fibroelastosis (thick fibroelastic tissue in endocardium of young children)
Löffler syndrome (endomyocardial fibrosis with a prominent eosinophilic infiltrate)
hemochromatosis (dilated cardiomyopathy can also occur).
Diastolic dysfunction ensues.
Can have low- voltage ECG despite thick myocardium (especially amyloid).
What's heart failure
2 main pathologies
RHF-results from or due to
RX-reduce mortality 4
Symtopm relieve only 2
Both by2 ,in who
Bblockers not given when
Symptoms in LHF,RHF
Clinical syndrome of cardiac pump dysfunction
congestion and low perfusion.
reduced EF, high EDV
often 2° to ischemia/MI or dilated cardiomyopathy.
2° to myocardial hypertrophy
preserved EF, normal EDV
RHF most often results from left HF.
Isolated right HF -due to cor pulmonale.
ACE inhibitors or angiotensin II receptor blockers, β-blockers (except in acute decompensated HF),
and spironolactone reduce mortality.
Thiazide or loop diuretics are used mainly for symptomatic relief.
Hydralazine with nitrate therapy improves both symptoms and mortality in select patients.
Shortness of breath when supine: venous return from redistribution of blood (immediate gravity effect) exacerbates pulmonary vascular congestion.
Breathless awakening from sleep: venous return from redistribution of blood, reabsorption of edema, etc.
pulmonary venous pressure pulmonary venous distention and transudation of fluid.
Presence of hemosiderin-laden macrophages (“HF” cells) in lungs.
central venous pressure resistance to portal flow. Rarely, leads to “cardiac cirrhosis.”
High venous pressure.
3.High central venous pressure -fluid transudation
Types of shocks 4
Causes for each
Changes to CO/CVP/SVR
First sign of shock
Last result of shock
Systemic inflammatory response syndrome ≥ 2:
First sign of shock is tachycardia.
Multiple organ dysfunction syndrome (MODS) is the end result of shock.
Most common symptoms
What's necessary for diagnosis
Two main types-onset/cause/virulence/vegetations/valve status/association
Commonly affect which valve
TV affected in who/organisms
Culture neg causes
Non bacterial causes of IE-name/
Other special ix
bacteria FROM JANE
Fever (most common symptom)
Roth spots (round white spots on retina surrounded by hemorrhage)
Osler nodes (tender raised lesions on finger or toe pads)
Janeway lesions (small,painless,erythematous lesions on palm or sole)
septic arterial or pulmonary emboli
splinter hemorrhages on nail bed
Multiple blood cultures necessary for diagnosis.
Acute—S. aureus (high virulence).
Large vegetations on previously normal valves Rapid onset.
Subacute—viridans streptococci (low
Smaller vegetations on congenitally abnormal or diseased valves.
Sequela of dental procedures. Gradual onset.
S. bovis (gallolyticus) is present in colon cancer,
S. epidermidis on prosthetic valves.
Mitral valve is most frequently involved.
Tricuspid valve - associated with IV drug abuse (don’t “tri” drugs).
Associated with S. aureus, Pseudomonas, and Candida.
Culture ⊝—most likely Coxiella burnetii, Bartonella spp., HACEK (Haemophilus, Actinobacillus, Cardiobacterium, Eikenella,
Endocarditis may also be nonbacterial (marantic/thrombotic)
2° to malignancy, hypercoagulable state, or lupus(Libermann sacks)
Transeosophageal Echo to see valves
Affects which valves frequency
MV-early and late lesions
Histological feature-and special cells
pathophysiology-hypersen type /due
Other must have thing for diagnosis
Carditis which type
Endocarditis involve what /vegetation type
Myocarditis Histological feature
Most common cause of death in acute disease
Repeat exposure to bacteria risk
What's chronic RHD
Major pathology to valve-appearance
Change in MV/AV
A consequence of pharyngeal infection with group A β-hemolytic streptococci.
Late sequelae include rheumatic heart disease, which affects heart valves—mitral > aortic >> tricuspid (high-pressure valves affected most).
Early lesion is mitral valve regurgitation;
late lesion is mitral stenosis.
Aschoff bodies -giant cells
Anitschkow cells -enlarged macrophages with ovoid, wavy, rod-like nucleus
anti- streptolysin O (ASO) titers.
Anti DNAse B titres
Immune mediated (type II hypersensitivity)
not a direct effect of bacteria.
Antibodies to M protein cross-react with self antigens (molecular mimicry).
J♥NES (major criteria):
Joint (migratory polyarthritis) -large joints
♥ (carditis)-pan carditis
Nodules in skin (subcutaneous)
None specific-fever /ESR high
Prior group a infection-ASOT/anti DNAse b titres
Endocarditis involve valves-small vegetations
Aschoff bodies in myocardium
Death due to myocarditis
High risk of chronic disease
CHRONIC RHEUMATIC HEART DISEASE
A. Valve scarring that arises as a consequence of rheumatic fever
B.Results in stenosis with a classic 'fish mouth' appearance
1. Almost always involves the mitral valve;thickening of chordae tendineae and cusps
2. Occasionally involves the aortic valve; fusion of the commissures
3. Other valves are less commonly involved.
C. Complications include infectious endocarditis.
Symptoms-increased by/relieved by/
Commonly presents with sharp pain, aggravated by inspiration,
relieved -sitting up and leaning forward.
Presents with friction rub.
ECG changes include widespread ST-segment elevation and/or PR depression.
Causes include idiopathic (most common-presumed viral),
confirmed infection (e.g., Coxsackievirus), neoplasia,
autoimmune (e.g., SLE, rheumatoid arthritis), uremia
cardiovascular (acute STEMI or Dressler syndrome), radiation therapy.
Results in 2
ECG Changes 2
Compression of heart by fluid (e.g., blood, effusions) in pericardial space
Equilibration of diastolic pressures in all 4 chambers.
Findings: Beck triad (hypotension, distended neck veins, distant heart sounds),
ECG shows low-voltage QRS
electrical alternans (due to “swinging” movement of heart in large effusion).
Pulsus paradoxus— in amplitude of systolic BP by > 10 mmHg during inspiration.
Seen in cardiac tamponade, asthma, obstructive sleep apnea, pericarditis, croup.
Which syphillis affects the aorta?
X Ray feature
3° syphilis disrupts the vasa vasorum of the aorta(end arteritis obliterans) -atrophy of vessel wall -dilatation of aorta and valve ring.
calcification of aortic root and ascending aortic arch.
Leads to “tree bark” appearance of aorta.
Can result in aneurysm of ascending aorta or aortic arch, aortic insufficiency.
What's the commonest tumour in heart
Mets from where,
Other types of cardiac Tumors 2
Rhabdomyomas-who/associated with other diseases/benign or not /site
Most common heart tumor is a metastasis.
Mostly affect the pericardium-effusions
Most common 1° cardiac tumor in adults
90% occur in the atria (mostly left
“ball valve” obstruction in the left atrium
associated with multiple syncopal episodes
early diastolic “tumor plop” sound.
Most frequent 1° cardiac tumor in children associated with tuberous sclerosis
Seen when 4
Increased JVP on inspiration instead of a normal reduction
Inspiration -negative intrathoracic pressure not transmitted to heart -impaired filling of right
ventricle -blood backs up into venae cavae -JVD.
right atrial or ventricular tumors.
Of what tissue
Risk factors 3hepatic angiosarcoma-severity/risk/prognosis/why
blood vessel malignancy
in the head, neck, and breast areas.
Usually in elderly
on sun-exposed areas.
Associated with radiation therapy
chronic postmastectomy lymphedema.
Hepatic angiosarcoma associated with vinyl chloride and arsenic exposures.
Very aggressive and difficult to resect due to delay in diagnosis.
capillary skin papules
found in AIDS patients
Caused by Bartonella henselae infections. Frequently mistaken for Kaposi sarcoma, but has neutrophilic infiltrate.
Frequency with age
Does not regress
Frequency increase with age.
Associated with Turner syndrome.
Arises from modified smooth muscle cells of the thermoregulatory glomus body.
Tissue of origin
Sites -common ,also
most commonly of the skin
but also mouth, GI tract, and respiratory tract. Associated with HHV-8 and HIV.
Frequently mistaken for bacillary angiomatosis, but has lymphocytic infiltrate.
Seen in who 2
Polypoid capillary hemangioma
ulcerate and bleed.
Associated with trauma and pregnancy
Benign capillary hemangioma of infancy Appears in first few weeks of life (1/200 births)
regresses spontaneously by 5–8 years old.
Due to what
Who exposed to
Two types /causes
Reduction in blood flow to the skin due to arteriolar (small vessel) vasospasm
response to cold or stress
color change from white (ischemia) to blue (hypoxia) to red (reperfusion).
Most often in the fingers A and toes.
Raynaud disease when 1° (idiopathic)
Raynaud syndrome when 2° to a disease process such as mixed connective tissue disease, SLE, or CREST (limited form of systemic sclerosis) syndrome.
Treat with Ca2+ channel blockers.
Types of vasculitis according to affecting vessels
Buergers dis(pollyangitis obliterance)
Granulomatosis with polyangitis(wegners)
Eosinophilic Granulomatosis with polyangitis
Affects who commonly
Main symptoms 2
Affect which arteries
RX before what-why
Usually elderly females.
Unilateral headache (temporal artery)
May lead to irreversible blindness due to
ophthalmic artery occlusion.
Associated with polymyalgia rheumatica.
commonly affects branches of carotid artery.
Focal granulomatous inflammation
Temporal artery bx
Treat with high-dose corticosteroids prior to
temporal artery biopsy to prevent blindness.
Symptoms -non specific,specif
Pathology -caused by
Ix-see what RX-2
Hepatitis B seropositivity in 30% of patients.
Fever, weight loss, malaise, headache.
GI: abdominal pain, melena.
Hypertension, neurologic dysfunction,
cutaneous eruptions, renal damage.
Typically involves renal and visceral vessels
not pulmonary arteries.
Immune complex mediated.
Transmural inflammation of the arterial wall
with fibrinoid necrosis.
Innumerable renal microaneurysms and spasms
Treat with corticosteroids, cyclophosphamide.
Granulomatosis with polyangitis
Symptoms affect with systems-they are
Upper respiratory tract:
perforation of nasal septum,
Lower respiratory tract:
hemoptysis, cough, dyspnea.
Renal: hematuria, red cell casts.
Focal necrotizing vasculitis
Necrotizing granulomas in the lung and
c-ANCA (anti-proteinase 3).
CXR: large nodular densities.
Treat with cyclophosphamide, corticosteroids.
Histology absence of
lung, kidneys, and skin
Presentation similar to granulomatosis with polyangiitis but without nasopharyngeal involvement.
MPO-ANCA/p-ANCA H (anti- myeloperoxidase).
Treat with cyclophosphamide, corticosteroids.
Eosinophilia Granulomatosis with polyangitis
Nerve symtop-due to
Asthma, sinusitis, skin nodules or purpura, peripheral neuropathy (e.g., wrist/foot drop).
Can also involve heart, GI, kidneys (pauci- immune glomerulonephritis).
Granulomatous, necrotizing vasculitis with eosinophilia
IgE level high
Associated with what
Most common childhood systemic vasculitis.
Often follows URI.
Skin: palpable purpura on buttocks/legs J Arthralgias
GI: abdominal pain
Vasculitis 2° to IgA immune complex deposition.
Associated with IgA nephropathy (Berger disease).