Blood Vessel diseases lecture Flashcards
Henoch-Schonlein Purpura
- Vasculitis associated with systemic disorders
- Necrotizing vasculitis of small dermal vessels
- Most common vasculitis in children
- Large palpable purpura patches on extensor surfaces of arms, legs, buttocks
- GI/Abdominal pain and bleeding and vomiting
- Arthralgia(non-migratory)
- Due to IgA immune complex deposition and follows an upper respiratory tract infection
- Disease is self-limited
Wegener’s Granulomatous
- Affects small to medium-sized vessels
- Patient presentation: middle aged(5th decade) males
- c-ANCA (cytoplasmic anti neutrophil cytoplasmic antibodies)–> react with proteinase 3 an enzyme in neutrophil granulocyte
- Necrotizing granulomas of upper/lower respiratory tract including ear, sinuses, oral cavity/Necrotizing crescentic glomerulonephritis
- Heavy lung involvement: persistent pneumonitis with bilateral cavitary and nodular infiltrates
Symptoms: Cough, Hemoptysis, Chronic Sinusitis, Otitis media, Saddle nose, Perforation of the nasal septum, glomerulonephritis, hematuri
Describe the histology presentation of Wegners and the treatment
Treatment is cyclophosphamide and steroids; relapses are common
Churg-Strauss syndrome (allergic granulomatous angitis)
- a small and medium vessel autoimmune vasculitis
- respiratory process with eosinophil infiltration and vasculitis
- Granulomatous vasculitis
- Sinusitis/Skin lesions/Peripheral Neuropathy/Associated with Asthma/Eosinophilia
Organs Affected: GI, Heart, Kidney
p-ANCA (perinuclear anca)
-Patient Presentation: 1st Stage–> Involves the sinuses and the onset or worsening of pre-existing allergies. 2nd Stage–> Acute Asthma. 3rd Stage–> Involves various organ systems including vessels of the lungs, GI tract, and peripheral nerves. Also the heart and kdneys with severe nerve pain or peripheral neuropathy.
Leukocytoclastic vasculitis
- White blood cells breaking down
- Presentation looks similar to rashes. Biopsy of the rash will show apoptotic nuclei and debris from PMNs around small vessels
- Nuclear dust
Polyarteritis Nodosa (PAN)
- Medium vessel vasculitis
- Segmental necrotizing inflammation of vessels of the kidney, heart, and liver.
- The LUNGS ARE SPARED(pulmonary artery)
-Necrotizing acute inflammation(immune complex mediated transmural vasculitis), fibrinoid necrosis/thrombotic occlusion of the lumen, thickening of the vessels. Not as granulomatous as other vasculitis syndromes
- Can get aneurysms/ruptures, infarction, ulceration
- Patient presentation: young adult complains of fever, weight loss, ischemia of involved organs with 1)Hypertention(from renal artery involvement) 2) abdominal pain with melena(mesenteric artery involvement), 3) Neurologic disturbances 4) Skin Lesions 5) Myalgia
- Lesions of varying stages are present. Transmural inflammation with fibrinoid necrosis–> eventually heals with fibrosis–> producing a string of perals appearance on imaging
- Treatment is corticosteroids and cyclophosphamide; fatal if not treated
- Hepatitis B seropositivity
Kawasaki disease
Medium sized vessel vasculitis(necrotizing)
- mucocutaneous lymph node disease(lymphadenopathy)
- involves coronary arteries–>coronary aneurysms and death
Patient Presentation: Asian Children under 4 years old, systemic symptoms, fever, eye(conjunctivitis) and mouth mucous membrane redness/erosion(strawberry red tongue), edema, red palms, soles, skin rash, cervical adenopathy
-erythema and edema of the hands and feet
Treatment: IVIG and aspirin
Self limited
Buerger Disease
- medium sized vessels
- Thromboangiitis obliterans
- Big in smokers and cigarette users
- acute and chronic inflammation of arm and leg vessels
- can spread from the arteries to veins, nerves
- Ulcers and gangrene of extremities due to the vasculitis
- autoamputation of fingers and toes
- Raynaud phenomenon is often present
- segmental inflammation/superficial nodular phlebitis
Giant cell (temporal) arteritis
-Older Adults > 50, female , Most common vasculitis syndrome
Branches affected–>Carotid, temporal, ophthalmic arteries
- Patient presentation: severe unilateral headache and radiating facial pain, Jaw claudication, Blindness, 50% risk of ocural symptoms due to involvement of opthalmic artery, 50% present with flu like syndrome with joint stiffness(polymyalgia rheumatic)
- if genetic then look for incidence in increased in patients with HLA-DR4(rhematoid artheritis)
- temporal artery biopsy and look for–> Granulomatous inflammation with giant cells and disruption of the elastic tissue in elastic stain
- Increased ESR, normal serum creatine kinase, steroids
- T-cell mediated immune response–>CD4 + T cells are involved in the destructive granulomatous inflammation of the vessel wall
- fragmentation of internal elastic lamina (segmental lesions)
- intimal thickening causing stenosis with or without thrombosis
Treatment with corticosteroids
Takayasu’s Arteritis
- Typically in young females <50 years old, Asian
- granulomatous inflammation of large arteries branching from aortic arch
- luminal occlusion
Complications include: blindness, neurological impairment, undetectable upper extremity pulses
- fever, myalgia, night sweats, arthritis
- pulseless disease/ blood pressure different in two arms
- ESR elevated
What are the causes of vasculitis syndromes
1) Direct injury to the vessels by infectious organisms
2) Immune-related inflammation caused by antigen-antibody complexes such as
- immune complex formation with damage
- Anti-neutrophil cytoplasmic autoantibodies (ANCAs) directed against enzymes in neutrophil granules
- Perinuclear ANCAs against myeloperoxidase (P-ANCAs)
- Cytoplasmic ANCAs against proteinase (c-ANCAs)
- Antiendothelial cell antibodies
which vasculitis syndromes present with c-ANCAs?
wegner granulomatosis
What are varicose veins and how are they formed?
- Varicose veins are abnormally dilated, elongated, tortuous veins which can be painful.
- It happens due to prolonged increased intraluminal pressure from when venous valves give out and backflow of blood occurs, causing veins to dilate.
Varicose viens happen at 3 important clinical sites what are they
- Superficial veins of legs
- Anorectal venous plexus (hemorrhoids)
- Esophagus
- Vulva varicosities are also common in women who have had many children
Varicose veins of the legs
Upper and lower superficial veins
Most common location
increase with age, obesity, female pregnancy
venous dilation and valve incompetence lead to
- Stasis and congestion/edema in the leg. Thrombosis can occur and cause stasis dermatitis and varicose ulcers (overlying skin often looks abnormal)
- risk of infection and impaired wound healing
