Vasculitis Flashcards
What are the three principal layers of arteries and veins?
Intima, media, and adventitia
What are elastic arteries?
The aorta and all immediate branches that contain elastin in the tunica media and allow expainson/recoil with ventricular systole and diastole
What are muscular arteries?
Arteries that contain predominantly smooth muscle inthe tunica media and are subject to autonomic control
What are arterioles?
Resistance vessels of the body that have the highest smooth muscle/diameter ratio
What is the difference between a primary and secondary vasculitis?
Primary = comprised of diseases with proven or presumed autoimmune etiology
Secondary = disease secondary to infection, drugs, connective tissue disease, or malignancy
What are two examples of large vessel vasculitis?
Giant cell arteritis, takayasu arteritis
What are two examples of medium vessel vasculitis?
Polyarteritis nodosa and kawasaki disease
What are three examples of ANCA-associated vasculitis?
Granulomatosis with polyangiitis, microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis
What are four examples of immune complex small vessel vasculitis?
Anti-glomerular basement membrane disease (Goodpasture’s disease), cryoglobulinemic vasculitis, IgA vasculitis (Henoch-Schonlein purpura), and hypocomplementemic urticarial vasculitis
What are two examples of variable vessel vasculitis?
Behcet’s disease and Cogan’s syndrome
What are five examples of single organ vasculitis?
cutaneous leukocytoclastic angiitis, cutaneous arteritis, primary central nervous system vasculitis, isolated aortitis, and renal limited vasculitis
What are four examples of secondary systemic vasculitis?
Infectious vasculitis, drug-induced, connective tissue disease associated, and malignancy associated
What are the most susceptible demographics for giant-cell arteritis?
Above age 50, women more than men
What is giant-cell arteritis?
The most common form of arteritis, affects large cranial arteries including temporal artery
What is the clinical presentation of giant-cell arteritis?
Headache, jaw claudication, scalp tenderness, vision loss, and limb claudication
Can also present with hip and shoulder stiffness when associated with polymyalgia rheumatica
What are the pathological findings of giant-cell arteritis?
Granulomatous inflammation
What are the treatments for giant-cell arteritis?
First line = glucocorticoids
Steroid-sparing agents = Tocilizumab (targets IL-6 pathway)
What demographis are most susceptible to Takayasu arteritis?
Young women (under 40), people of Asian descent
What vessels are primarily affected by Takayasu arteritis?
Ascending aorta, its immediate branches, and the subclavian arteries
What is the pathology of Takayasu arteritis?
Granulomatous lesions with intimal fibrosis leading to luminal obstruction
What is the presentation of Takayasu arteritis?
Diminished peripheral pulses, constitutional symptoms, neurologic symptoms, and claudication
What is the treatment for Takayasu arteritis?
Glucocorticoids
What demographics are most susceptible to polyarteritis nodosa?
Middle-aged individuals
What is the pathogenesis of polyarteritis nodosa?
It affects small- to medium-sized arteries and is characterized by acute segmental transmural necrotizing inflammation of muscular arteries
What are the causes of polyarteritis nodosa?
Idiopathic (most cases) or secondary to hep B, hep C, or hairy cell leukemia
What is the clinical presentation of polyarteritis nodosa?
Constitutional symptoms (fever, weight loss), mononeuritis multiplex (peripheral neuropathy), skin nodules, hematuria, new-onset hypertension, abdominal pain, occiasionally muscle involvement, and orchitis (testicle inflammation)
What is the treatment for polyarteritis nodosa?
initially glucocorticoids, progress to cyclophosphamide if severe case
What demographics are most susceptible to Kawasaki disease?
Mostly children under age 4
What is the pathogenesis of Kawasaki disease?
Arteritis affecting large- to medium-sized vessels with genetic, infectious, and environmental factors playing a role
What is the clinical presentation of Kawasaki disease?
Fever, conjunctivitis, strawberry tongue, lymphadenitis, and desquamative skin rash
What is the consequence of untreated Kawasaki disease?
20% risk of coronary artery ectasia (dilation) and aneurysms that are prone to thrombosis or rupture (leading to MI or death)
What is the treatment for Kawasaki disease?
Intravenous immunoglobulin and aspirin
What demographics are most susceptible to granulomatosis with polyangiitis?
Middle-aged adults
What is the pathology of granulomatosis with polyangiitis?
Necrotizing granulomas in small- and medium-sized vessels
What is the clinical presentation of granulomatosis with polyangiitis?
Oral ulcers, bloody nasal discharge/crusting, chronic sinusitis, subglottic stenosis,recurrent otitis media, pulmonary infiltrates, pulmonary nodules, alveolar hemmorhage, pauci-immune glomerulonephritis, constitutional symptoms
can also affect any organ
What antibody is associated with granulomatosis with polyangiitis?
c-ANCA
What is the treatment for granulomatosis with polyangiitis?
High dose glucocorticoids with cyclophosphamide or rituximab (targets B cells)
What demographics are most susceptible to microscopic polyangiitis?
Middle-aged adults
What is the pathogenesis of microscopic polyangiitis?
A necrotizing vasculitis that affects small-caliber vessels (capillaries, arterioles, and venules) thatdoes not have granulomas
What is the clinical presentation of microscopic polyangiitis?
Constitutional symptoms, renal involvement (hematuria, rise in creatinine, RBC casts), rapidly progressive pauci-immune glomerulonephritis, pulmonary manifestations (diffuse alveolar hemorrhage presenting as dyspnea, cough, hemoptysis, etc), PNS involvement
What antibody is associated with microscopic polyangiitis?
p-ANCA
What is the treatment for microscopic polyangiitis?
Glucocorticoids, cyclophosphamide, and rituximab (targets B cells)
Can you have polyangiitis without ANCA positivity?
Yes - it is most common to have ANCA positivity corresponding to the condition, but it is not always present
What happens in the prodromal phase of eosinophilic granulomatosis with polyangiitis?
Asthma, allergic rhinitis, and nasal polyps
What happens in the eosinophilic phase of eosinophilic granulomatosis with polyangiitis?
Peripheral eosinophilia and eosinophilic tissue infiltration
What happens in the vasculitic phase of eosinophilic granulomatosis with polyangiitis?
There are manifestations that can include cutaneous lesions, cardiac involvement, heart failure, pericarditis, neurologic manifestations, renal involvement, eosinophilic gastroenteritis
What antibody is associated with eosinophilic granulomatosis with polyangiitis?
p-ANC, though less associatedthan in other polyangiitis conditions
What are the treatments for eosinophilic granulomatosis with polyangiitis?
Glucocorticoids, cyclophosphamide, and mepolizumab (targets IL-5)
What demographics are most affected by anti-GBM disease?
Older children and adults
What is anti-GBM disease?
An immune complex that affects small vessels and is usually idiopathic but can also occur after pulmonary infection
What is the clinical presentationof anti-GBM disease?
Acute renal failure (proteinuria, hematuria, casts), frequent pulmonary involvement (alveolar hemorrhage)
What antibodies are associated with anti-GBM disease?
anti-GBM antibodies, sometimes ANCAs if there is a concomitant ANCA associated vasculitis
What is the treatment for anti-GBM disease?
Plasmapheresis, glucocorticoids, and cyclophosphamide
What is cryoglobulinemic vasculitis?
An immune complex small-vessel vasculitis where immunoglobulins and complement are found in the vessel wall
What is the most common cause of cryoglobulinemic vasculitis?
Chronic hepatitis C infection
What is the clinical presentation of cryoglobulinemic vasculitis?
Cutaneous purpura, arthralgia/arthritis, neuropathy, renal involvement
What are the renal biopsy findings of cryoglobulinemic vasculitis?
Intraluminal thrombi, diffuse IgM on capillary loops, and subendothelial deposits on EM
What is the treatment for cryoglobulinemic vasculitis?
Rituximab (targets B cells) and glucocorticoids
What demographics are most susceptible to IgA vasculitis?
Children aged 3-15
What is the pathology of IgA vasculitis?
Necrotizing lesions with IgA deposit in the capillaries an dpost-capillary venules in small vessels
What often preceeds disease onset of IgA vasculitis?
Upper respiratory infection
What is the clinical presentation of IgA vasculitis?
Skin involvement (palpable purpura on extensor surfaces of arms, legs, and buttocks), arthritis of lower extremities, abdominal pain, nephritis, nephrotic syndrome, or hematuria
What is the treatment for IgA vasculitis?
Supportive care, glucocorticoids if there is renal involvement to prevent progression to CKD
What demographics are most susceptible to Behcets syndrome?
Most common in Turkey and along the silk road (mediterranean to eastern Asia) in people aged 20-40
What vessels are affected by Behcets syndrome?
Vessels of all sizes, both arteries and veins
What is the clinical presentation of Behcets syndrome?
Painful oral ulcers, genital ulcers, cutaneous lesions, uveitis, arthritis, vascular disease, and neurologic involvement
What is the treatment for Behcets syndrome?
Depends on which organ is involved
What disease is associated with hep B?
Polyarteritis nodosa
What disease is associated with mononeuritis multiplex?
Polyarteritis nodosa
What disease is associated with hip and shoulder weakness?
Giant-cell arteritis
due to association with polymyalgia rheumatica
What disease is associated with strawberry tongue?
Kawasaki disease
What disease is associated with PR3-ANCA positivity?
Granulomatosis with polyangiitis (also called c-ANCA)
What disease is associated with MPO-ANCA positivity?
Microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis (to a lesser extent)
What disease is associated with asthma?
Eosinophilic granulomatosis with polyangiitis
What disease involves aspirin as a specific therapy?
Kawasaki disease
What disease is associated with frequent URI?
Granulomatosis with polyangiitis
What disease is associated with nasal polyps?
Eosinophilic granulomatosis with polyangiitis
What disease most commonly affects women of Asian descent?
Takayasu arteritis
What disease causes jaw claudication?
Giant-cell arteritis
What disease causes a desquamative skin rash?
Kawasaki disease
A 9 y.o. boy presents with abdominal pain and dark urine for a week. Exam shows purpuric lesions on his ankles and buttock. Serologic tests are negative for p-ANCA and c-ANCA. Skin biopsy shows necrotizing vasculitis of small dermal vessels. Renal biopsy shows immune complex deposition with IgA rich immune complexes. Which is the most likely diagnosis?
a) Giant cell arteritis
b) Henoch-Schonlein purpura
b) PAN
d) Takayasu
e) Kawasaki’s disease
f) Telaniectasias
g) GPA
b) Henoch-Schonlein purpura
also known as IgA vasculitis
A 70 y.o. white man with chronic cough for past year. Exam shows nasal ulcers and lungs with diffuse crackles.
Creatinine = 4.5
Urinalysis shows 20 RBCs +casts
c-ANCA is positive
CXR has small scattered pulmonary nodules, lung biopsy shows vasculitis of small peripheral arteries/arterioles + granulomatous inflammation adjacent to small arterioles
What is the most likely diagnosis?
a) fibromuscular dysplasia
b) PAN
c) takayasus
d) GPA
d) GPA
A 34 y.o. woman is evaluated for pain and redness of her eye, double vision, and ocular movement impairment for the past 6 days. ESR is high, thyroid function studies/urinalysis/CXR are normal. MRI of orbit reveals superior oblique enlargement and enfiltration with edema. Testing for ANCA is negative. Biopsy of superior oblique muscle shows granulomatous inflammation,tissue necrosis, and capillaritis. What is the most likely diagnosis?
a) GPA
b) sarcoidosis
c) Grave’s thyroiditis
d) lymphoma
a) GPA
it can be GPA even if ANCAs are negative
