LECTURE 7 (Vasculitis) Flashcards
What is Vasculitis?
Inflammation of blood vessels with a hallmark of leukocytes in blood vessel walls
SYMPTOMS:
- fever
- myalgia
- arthralgias (joint stiffness)
- fatigue
- organ/disease specific symptoms (due to vessel lumen narrowing/occluding from inflammation)
How is Vasculitis classified?
It is classified bases on the type of vessel involved
- LARGE
[Temporal Arteritis, Takayasu’s Arteritis] - MEDIUM
[Polyarteritis Nodosa, Kawasaki Disease, Buerger’s Disease] - SMALL
[Churg-Strauss, Wegener’s granulomatosis, Microscopic polyangiitis, Henoch-Schonlein purpura]
What is Palpable Purpura?
A common finding in vasculitis patients
- “Purpura” are red-purple skin lesions due to extravagation of blood into the skin
- Do not blanch when pressed
What is the difference between Non-palpable purpura and Palpable purpura?
NON-PALPABLE PURPURA
- usually non-inflammatory
- called “Petechiae” when small and “Ecchymoses” when large
- petechiae caused by thrombocytopenia -> non-inflammatory loss of blood in small vessels of skin
PALPABLE PURPURA
- occurs in vasculitis
- raised lesions due to small vessel inflammation
- sometimes called “Leukocytoclastic Vasculitis” -> destruction of cytoplasm of WBC -> infiltration of white cells + debris in vasculature
What is the treatment for Vasculitis?
Steroids or Cyclophosphamide
Explanation: Cyclophosphamide is an immunosuppressant
Describe Large vessel vasculitis
- “Temporal arteritis” and “Takayasu’s arteritis”
- Both involve granulomatous inflammation + narrowing of large arteries
What is Temporal Arteritis?
Also called “Giant cell arteritis” involves narrowing of the temporal artery system
SYMPTOMS:
- headache
- jaw claudication (pain on chewing)
- if not treated -> BLINDNESS due to occlusion of Ophthalmic artery
LAB FINDINGS:
- High ESR (erythrocyte sedimentation rate)
DIAGNOSIS:
- biopsy of temporal artery + finding granulomas
TREATMENT:
- treat with high dose steroids IMMEDIATELY
[if you suspect TA, treat with high dose steroids immediately to prevent blindness - don’t wait for biopsy!]
CLASSIC DEMOGRAPHIC:
- Elderly female with headache
- Pain on chewing
- High ESR
What is Takayasu’s Arteritis?
Granulomatous thickening of aortic arch and branches and is common in young, asian women (average age is 35 years old)
SYMPTOMS:
- weak pulses in one arm
[due to narrowing of subclavian that supplies arms]
- “pulseless disease” since narrowing of proximal great vessels
- BP difference between arms + legs
- Bruits over arteries
LAB FINDINGS:
- high ESR
TREATMENT:
- Steroids
What is Kawasaki disease?
An autoimmune attack of medium vessels that usually occurs in asian children
SYMPTOMS:
- diffuse, red rash on skin
- red rash usually on the palms and soles -> later desquamates and the skin falls off
- “strawberry tongue” + changes in lips/oral mucosa
COMPLICATIONS:
- coronary aneurysms -> can rupture leading to myocardial infarction + death
TREATMENT:
- IV immunoglobulin
- Aspirin
MEDIUM VESSEL VASCULITIS
How can you differentiate Scarlet fever from Kawasaki disease?
Scarlet fever will be preceded with a sore throat
What is Reye’s syndrome?
Involves encephalopathy, liver failure and fatty infiltration and can occur when children are given Aspirin
SYMPTOMS:
- vomiting
- confusion
- seizures
- coma
CAUSE:
- often follows viral illnesses (e.g influenza, varicella)
- caused by diffuse mitochondrial insult
Associated with aspirin use in children (children should NOT be given it) - ONLY exception is Kawasaki
MEDIUM VESSEL VASCULITIS
What is Buerger’s disease?
Also called “Thromboangiitis Obliterans” which occurs due to poor blood flow to hands and feet and usually occurs in male smokers
SYMPTOMS:
- poor blood flow to hands + feet -> gangrene
- autoamputation of digits
- superficial nodular phlebitis (tender nodules develop over course of a vein)
- Raynaud’s phenomenon (poor blow to hands + can develop ulcers)
- segmental thrombosing vasculitis (blood clots that cut off blood flow to hands + feet)
TREATMENT:
- STOP SMOKING!
MEDIUM VESSEL VASCULITIS
What is Polyarteritis Nodosa?
An immune complex mediated disease (Type III hypersensitivity reaction) that causes serious inflammation in blood vessels
CLASSIC DEMOGAPHIC:
- someone who is Hep B+
- presents with nerve defects, skin nodules, purport and renal failure
SYMPTOMS:
- motor/sensory deficits in nerves
- nodules/palpable purpura in skin
- renal failure
COMPLICATIONS:
- many aneurysms and constrictions seen on anteriogram [kidney, liver and mesenteric arteries] + Rosary sign (series of aneurysms)
- if you biopsy - fibrinoid necrosis + inflammation on vessel wall
DIAGNOSIS:
- angiogram to look for aneurysms (usually in mesenteric vessels or vessels that supply kidney + liver)
- tissue biopsy of affected system
TREATMENT:
Corticosteroids + Cyclophosphamide
MEDIUM VESSEL VASCULITIS
What is Henoch-Schonlein Purpura?
The most common childhood systemic vasculitis which causes inflammation of the small blood vessels of the skin, joints, bowels and kidneys
CAUSE:
- often follows an upper respiratory infection
- associated with IgA antibody deposition -> can cause IgA nephropathy in the kidneys
- C3 complement deposition
SYMPTOMS:
- Skin: palpable purpura on buttocks/legs
- GI tract: abdominal pain + melena (dark faeces due to upper GI bleeding)
- Kidney: nephritis
CLASSIC CASE:
- child with recent upper respiratory infection
- palpable purpura
- melena
DIAGNOSIS:
- tissue biopsy
TREATMENT:
usually requires no treatment
COMPLICATIONS:
- renal failure
- severe cases are treated with steroids + cyclophosphamide
SMALL VESSEL VASCULITIS
Which diseases are ANCA mediated diseases?
- Churg-Strauss
- Wegener’s granulomatosis
- Microscopic polyangiitis
Describe the ANCA diseases
ANCA = Anti-neutrophil cytoplasmic antibodies
- all have pulmonary involvement
- all have renal involvement -> cause Crescentic RPGN [crescents form in glomeruli] -> rapid loss of kidney function
- immunofluorescent staining -> “Pauci-immune” -> glomeruli appear black since no presence of immunoglobulins
- all cause nephritic syndrome
- proteinuria + hematuria
What are Anti-neutrophil cytoplasmic antibodies (ANCA)?
Autoantibodies that attack neutrophil proteins
2 PATTERNS THAT DISTINGUISH DISEASES:
- c-ANCA (cytoplasmic)
[usually directed against proteinase 3 inside neutrophil + Wegener’s only + light up cytoplasmic portion of neutrophil in staining]
- p-ANCA (perinuclear)
[usually directed against myeloperoxidase antibodies in neutrophils + immunofluorescent around the nucleus + seen in Churg-Strauss and Microscopic Polyangiitis
What is Churg-Strauss syndrome?
An ANCA disorder marked by blood vessel inflammation
SYMPTOMS:
- asthma developed as an adult
- sinusitis
- neuropathy
- palpable purpura
- granulomatous + necrotising vasculitis
- heart, GI + kidneys symptoms
LAB FINDINGS:
- high level of eosinophils
- p-ANCA positive
- high levels of IgE
TREATMENT:
steroids + cyclophosphamide
SMALL VESSEL VASCULITIS
What is Wegener’s Granulonatosis?
Granulomatosis with polyangiitis and is inflammation of blood vessels in your nose, sinuses, throat, lungs and kidneys
SYMPTOMS:
- upper airway = sinusitis + otitis media
- lower airway = hemoptysis
- renal: hematuria + red cell casts
- purpura
LAB FINDINGS:
- granulomas on biopsy
- c-ANCA biopsy
TREATMENT:
steroids + cyclophosphamide
SMALL VESSEL VASCULITIS
What is Microscopic Polyangiitis?
A rare condition that can cause blood vessel inflammation
SYMPTOMS:
- hemoptysis
- kidney failure
- palpable purpura
TREATMENT:
steroids + cyclophosphamide
SMALL VESSEL VASCULITIS
What differentiates Microscopic Polyangiitis from Wegner’s?
- No upper airway disease (sinusitis)
- p-ANCA not c-ANCA
- No granulomas on biopsy
What differentiates Goodpasture’s syndrome from Wegner’s or Microscopic Polyangiitis?
- Immunofluorescent staining in Goodpasture’s is +ve -> Linear IF staining when staining for IgG + C3
- Absence of ANCA antibodies
