Chapter 9 - blood vessles Flashcards
Giant cell (temporal) arteritis treatment:
- croticosteroid
- anti-TNF therapies
Clinical features of Giant cell (temporal) arteritis:
- rare before 50yr
- fever, fatigue, weight loss, malaise, muscle aches
- polymalgia rheumatica (flu-like+joint pain)
- facial pain/headache, intense -> superficial temporal artery (painful to palpation)
- vision loss (sudden and permanent).
- Double or blurred vision.
What is the most common form of vasculitis?
Giant cell (temporal) arteritis
Distribution of Giant cell (temporal) arteritis:
- small and medium sized arteries
- cranial arteries (temporal(important), facial, and opthalmic)
- Aortic arch - giant cell aortitis (uncommon)
Giant cell (temporal) arteritis LAB:
elevated ESR (erythrocyte sedimentation rate)
Giant cell (temporal) arteritis morphology:
- patchy along the length of the affected vessel
- nodular intimal thickening (and occasional thromboses) that reduce the lumen diameter and cause distal ischemia
- granulomatous inflammation
- fragmentation of the internal elastic lamina
Pulseless disease=
Takayasu arteritis
Takayasu arteritis epidemiology:
- most common in Asia
- affects young and middle-aged women (ages 15-45)
Takayasu arteritis (pulseless disease) distribution:
- medium-sized to large arteries
- aortic arch and major branches
Takayasu arteritis (pulseless disease) characteristics:
- ocular disturbance
- weakening of the pulses of the upper extremeties
Takayasu arteritis (pulseless disease) pathology:
- transmural scarring
- irregular fibrous thickening of the aorta (especially the aortic arch and great vessels)
- narrowing of the orifices of the major arterial branches
Takayasu arteritis (pulseless disease) clinical features:
- reduced blood pressure and pulse strength in the upper extremities
- fatigue, wight loss, and fever
- neurologic deficit/abnormalities
- visual field defects, retinal hemorrhages, and total blindness
Takayasu arteritis (pulseless disease) treatment:
steroids
What can Takayasu arteritis (pulseless disease) lead to:
- pulmonary artery -> pulmonary hypertention
- renal arteries -> systemic hypertention
Takayasu arteritis (pulseless disease) morphology:
- affect the aortic arch and arch vessels
- dialtion and aortic valve insufficiency
- pulmonary artery. renal and coronary arteries may be affected
- takeoffs of great vessels are narrowed
Polyarteritis nodosa clinical:
- malaise, fever, weight loss
- hypertention due to renal artery involvement
- abdominal pain and bloody stools caused by vascular gastrointestinal lesions
- diffuse muscular aches and pains
- peripheral neuritis
- predominantly affecting motor nerves
What is polyarteritis nodosa?
Necrotizing immune complex inflammation of small or medium-sized muscular arteries, typically involving renal or visceral vessels.
What is the treatment of polyarteritis nodosa?
Corticosteroids, azathioprine, and/or cyclophosphamide
What is an association of polyarteritis nodosa?
Hepatitis B infection (30% of patients)
What are the signs of polyarteritis nodosa?
- Cotton wool spots
- Microaneurysms
- Pericarditis
- Myocarditis
- Palpable purpura
- Elevated ESR
- P-ANCA positive serum
PAN =
polyarteritis nodosa
Polyarteritis nodosa epidemiology:
- young adults
- male>female
Polyarteritis nodosa distribution:
- systemic vasculitis- any organ except for lungs
- kidney, heart, GI tract, muscle etc.
- small and medium size arteries
Polyarteritis nodosa clinical features:
- low-grade fever, malaise, wight loss
- hematouria, renal failure, hypertention
- abdominal pain, diarrhea, and GI bleeding
- myalgia and arthralgia
Polyarteritis nodosa pathology three stages:
- acute lesion: fibrinoid necrosis and neutrophils
- healing lesion: fibroblast proliferation
- healed lesion: nodular fibrosis and loss of internal elastic lamina
Polyarteritis nodosa sequela:
- thrombosis and infarction
- aneurysm (kidneys, heart, and GI tract)
Polyarteritis nodosa LAB:
- antibodies against myeloperoxidase
- P-ANCA
Polyarteritis nodosa treatment:
Corticosteroids and Cyclophosphamide
Kawasaki disease epidemiology:
- Commonly affects infants and young children (age <4)
- Japan, Hawaii, and U.S. mainland
Kawasaki disease clinical features
- Acute febrile illness
- Conjunctivitis; erythema, blistering and erosions of the oral mucosa
- Generalized maculopapular skin rash (desquamative rash)
- edema of the hands and feet
- erythema of the palms and soles
- Lymphadenopathy, cervical lymph node enlargment
Kawasaki disease distribution:
- Large,medium-sized, and small arteries
- Coronary artery commonly affected (70%)
Kawasaki disease pathology:
- Segmental necrotizing vasculitis
- Weakened vascular wall may undergo aneurysm formation
Kawasaki disease def. #1:
is a febrile lymphadenopathy with rash with an associated segmental necrotizing vasculitis with a predilection for the coronary arteries
Kawasaki disease def. #2:
acute, febrile, usually self-limited illness of infancy and childhood (80% of the patients are younger than 4 yrs old) associated with an arteritis of mainly large to medium-sized vessels
(Kawasaki disease) What can coronary arteritis cause?
aneurysm that rupture or thrombose, resulting in myocardial infarction.
Kawasaki disease =
mucocutaneous lymph node syndrome
How are the cardiovascular sequelae that develops because of the Kawasaki disease?
they range from asymptomatic coronary arteritis, to coronary artery ectasia, to large coronary artery aneurysm with rupture or thrombosis, myocardial infarction, and sudden death.
Microscopic polyangiitis =
hypersensitivity vasculitis or leukocytoclastic vasculitis
Microscopic polyangiitis distribution:
- (necrotizing vasculitis)
- capillaries, small arterioles and venules
What is involved in Microscopic polyangiitis?
- skin
- mucous membranes
- lungs
- brain
- heart
- GI tract
- kidneys
- muscle
What is common in Microscopic polyangiitis?
- necrotizing glomerulonephritis (90%)
- pulmonary capillaritis
Microscopic polyangiitis is associated with?
MPO-ANCA
Microscopic polyangiitis is characterized by….
segmental fibrinoid necrosis of the media with focal transmural necrotizing lesions; granulomatous inflammation is absent
are macroscopic infarctions common in Microscopic polyangiitis?
nope
lesions that are “pauci-immune” ->
Microscopic polyangiitis
pauci-immune =
show little or no antibody
Microscopic polyangiitis features:
- hemoptysis
- hematuria
- proteinuria
- abdominal pain or bleeding
- muscle pain or weakness
- palpable cutaneous purpura
C-ANCA
Wegerner’s granulomatosis
What is the treatment of wegener’s granulomatosis?
cyclophosphamide, corticosteroids, and/or methotrexate
What are the symptoms of Wegeners granulomatosis?
- Perforation of nasal septum
- Chronic sinusitis
- Otitis media
- Mastoiditis
- Cough
- Dyspnea
- Hemoptysis
What are the findings of Wegener’s granulomatosis?
- C-ANCA positive
- CXR reveals large nodular lesions
- Hematuria and red cell casts
Wegener’s granulomatosis epidemiology:
- Rare; males> females
- Peak incidence: ages 40-60
Wegener’s granulomatosis distribution:
- Necrotizing vasculitis with granulomas
- Classically involves the nose,sinuses,lungs,and kidneys
- Small size arteries and veins
- small to medium sized vessels (capillaries, venules, arterioles, and arteries)
Wegener’s granulomatosis clinical features:
- Bilateral pnuemonitis with nodular and cavitary pulmonary infiltrates
- Chronic sinusitis
- Nasopharyngeal ulcerations
- Renal disease
- Focal necrotizing glomerulonephritis
- Crescentic glomerulonephritis
Wegener’s granulomatosis LAB:
- Cytoplasmicantineutrophil cytoplasmic autoantibodies (C-ANCA)
- Autoantibody againstproteinase3
- Correlates with disease activity
Wegener’s granulomatosis treatment:
immunosuppressive drugs (cyclophosphamide)
Wegener’s granulomatosis is charachterized by:
- granulomas of the lung and/or upper respiratory tract (ear, nose, sinuses, throat)
- vasculitis of small to medium sized vessels most prominently in lungs and upper respiratory tract
- glomerulonephritis
The widespread form of Wegener’s granulomatosis:
can affect, eyes, skin, and other organs, notably the heart
